BACKGROUND Thrombotic microangiopathy(TMA)is an acute syndrome characterized by microangiopathic hemolytic anemia,thrombocytopenia,and multi-organ dysfunction due to the microcirculation of platelet thrombi.Cancer-ass...BACKGROUND Thrombotic microangiopathy(TMA)is an acute syndrome characterized by microangiopathic hemolytic anemia,thrombocytopenia,and multi-organ dysfunction due to the microcirculation of platelet thrombi.Cancer-associated TMA is a rare and fatal complication,which often occurs during cancer remission.It is frequently misdiagnosed because of limited clinical awareness.CASE SUMMARY A middle-aged female patient presented to our clinic with a 15-days history of back pain,15 months post-gastrectomy.Cancer-associated TMA was confirmed through bone marrow aspiration,biopsy,and imaging.The patient received intermittent transfusions,fluids,nutrition,and microcirculation therapy with partial coagulation improvement.The family refused intensive care unit admission and plasma exchange,preferring palliative care.The patient died of cerebral hemorrhage and herniation due to disease progression.This case indicates that TMA may serve as an early manifestation of various malignancies,particularly gastric cancer.However,it is often misdiagnosed.Its pathogenesis is not well understood and needs to be further investigated.Currently,no standardized treatment have been developed.Plasma exchange is the only intervention available,though other therapies may also be effective.CONCLUSION In this case of gastric signet-ring cell carcinoma complicated by TMA,the patient achieved transient remission with supportive care but died following treatment discontinuation.Further studies are needed to elucidate the pathological mechanisms and therapeutic strategies for cancer-associated TMA.展开更多
Pulmonary tumour thrombotic microangiopathy(PTTM)is a rare but under-recognised cause of rapidly progressive pulmonary hypertension(PH)and cor pulmonale,characterised by diffuse obstruction of small pulmonary arteries...Pulmonary tumour thrombotic microangiopathy(PTTM)is a rare but under-recognised cause of rapidly progressive pulmonary hypertension(PH)and cor pulmonale,characterised by diffuse obstruction of small pulmonary arteries by metastatic tumour cells.These tumour emboli lead to obstructive intimal proliferation and in situ thrombosis within the pulmonary vasculature,further compromising the overall permeability of the pulmonary vascular bed and exacerbating PH.[1]The clinical and imaging manifestations of PTTM often overlap with those of other causes of PH,including chronic thromboembolic PH,pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis,often leading to diagnostic delays.展开更多
Thrombotic microangiopathy(TMA)is an uncommon but serious complication that not only affects native kidneys but also transplanted kidneys.This review is specifically focused on post-transplant TMA(PT-TMA)involving kid...Thrombotic microangiopathy(TMA)is an uncommon but serious complication that not only affects native kidneys but also transplanted kidneys.This review is specifically focused on post-transplant TMA(PT-TMA)involving kidney transplant recipients.Its reported prevalence in the latter population varies from 0.8%to 14%with adverse impacts on both graft and patient survival.It has many causes and associations,and the list of etiologic agents and associations is growing constantly.The pathogenesis is equally varied and a variety of pathogenetic pathways lead to the development of microvascular injury as the final common pathway.PT-TMA is categorized in many ways in order to facilitate its management.Ironically,more than one causes are contributory in PT-TMA and it is often difficult to pinpoint one particular cause in an individual case.Pathologically,the hallmark lesions are endothelial cell injury and intravascular thrombi affecting the microvasculature.Early diagnosis and classification of PT-TMA are imperative for optimal outcomes but are challenging for both clinicians and pathologists.The Banff classification has addressed this issue and has developed minimum diagnostic criteria for pathologic diagnosis of PT-TMA in the first phase.Management of the condition is also challenging and still largely empirical.It varies from simple maneuvers,such as plasmapheresis,drug withdrawal or modification,or dose reduction,to lifelong complement blockade,which is very expensive.A thorough understanding of the condition is imperative for an early diagnosis and quick treatment when the treatment is potentially effective.This review aims to increase the awareness of relevant stakeholders regarding this important,potentially treatable but under-recognized cause of kidney allograft dysfunction.展开更多
BACKGROUND Pulmonary tumor thrombotic microangiopathy(PTTM)is a rare condition in patients with hepatocellular carcinoma(HCC);to date,few cases have been reported.While hepatic dysfunction has been focused on the late...BACKGROUND Pulmonary tumor thrombotic microangiopathy(PTTM)is a rare condition in patients with hepatocellular carcinoma(HCC);to date,few cases have been reported.While hepatic dysfunction has been focused on the later stages of HCC,the management of symptoms in PTTM is important for supportive care of the cases.For the better understanding of PTTM in HCC,the information of our recent case and reported cases have been summarized.CASE SUMMARY A patient with HCC exhibited acute and severe respiratory failure.Radiography and computed tomography of the chest revealed the multiple metastatic tumors and a frosted glass–like shadow with no evidence of infectious pneumonia.We diagnosed his condition as acute respiratory distress syndrome caused by the lung metastases and involvement of the pulmonary vessels by tumor thrombus.Administration of prednisolone to alleviate the diffuse alveolar damages including edematous changes of alveolar wall caused by the tumor cell infiltration and ischemia showed mild improvement in his symptoms and imaging findings.An autopsy showed the typical pattern of PTTM in the lung with multiple metastases.CONCLUSION PTTM is caused by tumor thrombi in the arteries and thickening of the pulmonary arterial endothelium leading to the symptoms of dyspnea in terminal staged patients.Therefore,supportive management of symptoms is necessary in the cases with PTTM and hence we believe that the information presented here is of great significance for the diagnosis and management of symptoms of PTTM with HCC.展开更多
Thrombotic microangiopathy(TMA) is one of the most devastating sequalae of kidney transplantation. A number of published articles have covered either de novo or recurrent TMA in an isolated manner. We have, hereby, in...Thrombotic microangiopathy(TMA) is one of the most devastating sequalae of kidney transplantation. A number of published articles have covered either de novo or recurrent TMA in an isolated manner. We have, hereby, in this article endeavored to address both types of TMA in a comparative mode. We appreciate that de novo TMA is more common and its prognosis is poorer than recurrent TMA; the latter has a genetic background, with mutations that impact disease behavior and, consequently, allograft and patient survival. Post-transplant TMA can occur as a recurrence of the disease involving the native kidney or as de novo disease with no evidence of previous involvement before transplant. While atypical hemolytic uremic syndrome is a rare disease that results from complement dysregulation with alternative pathway overactivity, de novo TMA is a heterogenous set of various etiologies and constitutes the vast majority of post-transplant TMA cases. Management of both diseases varies from simple maneuvers, e.g., plasmapheresis, drug withdrawal or dose modification, to lifelong complement blockade, which is rather costly. Careful donor selection and proper recipient preparation, including complete genetic screening, would be a pragmatic approach. Novel therapies, e.g., purified products of the deficient genes, though promising in theory, are not yet of proven value.展开更多
BACKGROUND Sodium valproate is widely used in the treatment of epilepsy in clinical practice.Most adverse reactions to sodium valproate are mild and reversible,while serious idiosyncratic side effects are becoming app...BACKGROUND Sodium valproate is widely used in the treatment of epilepsy in clinical practice.Most adverse reactions to sodium valproate are mild and reversible,while serious idiosyncratic side effects are becoming apparent,particularly hepatotoxicity.Herein,we report a case of fatal acute liver failure(ALF)with thrombotic microangiopathy(TMA)caused by treatment with sodium valproate in a patient following surgery for meningioma.CASE SUMMARY A 42-year-old man who received antiepileptic treatment with sodium valproate after surgery for meningioma exhibited extreme fatigue,severe jaundice accompanied by oliguria,soy sauce-colored urine,and ecchymosis.His postoperative laboratory values indicated a rapid decreased platelet count and hemoglobin level,severe liver and kidney dysfunction,and disturbance of the coagulation system.He was diagnosed with drug-induced liver failure combined with TMA.After plasma exchange combined with hemoperfusion,pulse therapy with high-dose methylprednisolone,and blood transfusion,his liver function deteriorated,and finally,he died.CONCLUSION ALF with TMA is a rare and fatal adverse reaction of sodium valproate which needs to be highly valued.展开更多
BACKGROUND Interferons(IFNs)are characterized by a wide range of biological effects,which justifies their potential therapeutic use in several pathologies,but also elicit a wide array of adverse effects in almost ever...BACKGROUND Interferons(IFNs)are characterized by a wide range of biological effects,which justifies their potential therapeutic use in several pathologies,but also elicit a wide array of adverse effects in almost every organ system.Among them,renal involvement is probably one of the most complex to identify.CASE SUMMARY We describe four cases of kidney damage caused by different IFN formulations:IFN-β-related thrombotic microangiopathy,IFN-β-induced systemic lupus erythematosus,and two cases of membranous nephropathy secondary to pegylated-IFN-α2B.In each case,we carefully excluded any other possible cause of renal involvement.Once suspected as the casual relationship between drug and kidney damage,IFN treatment was immediately discontinued.In three cases,we observed a complete and persistent remission of clinical and laboratory abnormalities after IFN withdrawal,while the patient who developed thrombotic microangiopathy,despite IFN withdrawal and complement-inhibitor therapy with eculizumab,showed persistent severe renal failure requiring dialysis.CONCLUSION This case series highlights the causal relationship between IFN treatment and different types of renal involvement and enables us to delineate several peculiarities of this association.展开更多
AIM:To investigate thrombotic microangiopathy (TMA)in liver transplantion,because TMA is an infrequent but life-threatening complication in the transplantation field. METHODS:A total of 206 patients who underwent livi...AIM:To investigate thrombotic microangiopathy (TMA)in liver transplantion,because TMA is an infrequent but life-threatening complication in the transplantation field. METHODS:A total of 206 patients who underwent living-donor liver transplantation (LDLT) were evaluated,and the TMA-like disorder (TMALD) occurred in seven recipients. RESULTS:These TMALD recipients showed poor outcomes in comparison with other 199 recipients. Although two TMALD recipients successfully recovered,the other five recipients finally died despite intensive treatments including repeated plasma exchange (PE) and re-transplantation. Histopathological analysis of liver biopsies after LDLT revealed obvious differences according to the outcomes. Qualitative analysis of antibodies against a disintegrin-like domain and metalloproteinase with thrombospondin type 1 motifs (ADAMTS-13) were negative in all patients. The fragmentation of red cells,the microhemorrhagic macules and the platelet counts were early markers for the suspicion of TMALD after LDLT. Although the absolute values of von Willebrand factor (vWF) and ADAMTS-13 did not necessarily reflect TMALD,the vWF/ADAMTS-13 ratio had a clear diagnostic value in all cases. The establishment of adequate treatments for TMALD,such as PE for ADAMTS-13 replenishment or treatments against inhibitory antibodies,must be decided according to each case. CONCLUSION:The optimal induction of adequate therapies based on early recognition of TMALD by the reliable markers may confer a large advantage for TMALD after LDLT.展开更多
BACKGROUND Thrombotic microangiopathy(TMA)is a group of disorders that converge on excessive platelet aggregation in the microvasculature,leading to consumptive thrombocytopenia,microangiopathic hemolysis and ischemic...BACKGROUND Thrombotic microangiopathy(TMA)is a group of disorders that converge on excessive platelet aggregation in the microvasculature,leading to consumptive thrombocytopenia,microangiopathic hemolysis and ischemic end-organ dysfunction.In predisposed patients,TMA can be triggered by many environmental factors.Glucocorticoids(GCs)can compromise the vascular endothelium.However,GC-associated TMA has rarely been reported,which may be due to the lack of awareness of clinicians.Given the high frequency of thrombocytopenia during GC treatment,particular attention should be given to this potentially fatal complication.CASE SUMMARY An elderly Chinese man had a 12-year history of aplastic anemia(AA)and a 3-year history of paroxysmal nocturnal hemoglobinuria(PNH).Three months earlier,methylprednisolone treatment was initiated at 8 mg/d and increased to 20 mg/d to alleviate complement-mediated hemolysis.Following GC treatment,his platelet counts and hemoglobin levels rapidly decreased.After admission to our hospital,the dose of methylprednisolone was increased to 60 mg/d in an attempt to enhance the suppressive effect.However,increasing the GC dose did not alleviate hemolysis,and his cytopenia worsened.Morphological evaluation of the marrow smears revealed increased cellularity with an increased percentage of erythroid progenitors without evident dysplasia.Cluster of differentiation(CD)55 and CD59 expression was significantly decreased on erythrocytes and granulocytes.In the following days,platelet transfusion was required due to severe thrombocytopenia.Observation of platelet transfusion refractoriness indicated that the exacerbated cytopenia may have been caused by the development of TMA due to GC treatment because the transfused platelet concentrates had no defects in glycosylphosphatidylinositol-anchored proteins.We examined blood smears and found a small number of schistocytes,dacryocytes,acanthocytes and target cells.Discontinuation of GC treatment resulted in rapidly increased platelet counts and steady increases in hemoglobin levels.The patient’s platelet counts and hemoglobin levels returned to the levels prior to GC treatment 4 weeks after GC discontinuation.CONCLUSION GCs can drive TMA episodes.When thrombocytopenia occurs during GC treatment,TMA should be considered,and GCs should be discontinued.展开更多
Thrombotic microangiopathy(TMA)is potentially life-threatening condition caused by small-vessel microthrombi and is associated with schistocyte formation,low platelets and end-organ damage that may not be reversible.[...Thrombotic microangiopathy(TMA)is potentially life-threatening condition caused by small-vessel microthrombi and is associated with schistocyte formation,low platelets and end-organ damage that may not be reversible.[1,2]As a life-threatening condition,TMA recognition in hospitalized patients after organ transplantation is key to improving survival.Transplant-associated TMA(TATMA)can occur after both solid organ or hematopoietic stem cell transplantation and often mimics other disease processes such as thrombotic throm-bocytopenic purpura(TTP)with similar constellation of symptoms during presentation.We present a rare case of a patient with TATMA after orthotopic heart transplantation.展开更多
Concentration of supemxide anion in plasma and ecythrocytes in 21 patients with non-Insulin-dependent diabetes mellitus (NIDDM)(14 with complicating microangiopathy) and 34 normal adults was assayed by chemiluinesenc...Concentration of supemxide anion in plasma and ecythrocytes in 21 patients with non-Insulin-dependent diabetes mellitus (NIDDM)(14 with complicating microangiopathy) and 34 normal adults was assayed by chemiluinesence. Both plasma and erythrocyte concentration of .0-2 were ekvated in NIDDM with complicating microangiopathy beyond normal range (P<0.01),whereas in NIDDM without microangiopathy,in plasma was only slightly raised and RBC(.0-2) was within normal range. The authors think that RBC (.0-2) may be used as an indicator for the detection of presence of microangiopathy in NIDDM.展开更多
To the Editor:Thrombotic microangiopathy(TMA)is an uncommon hematological involvement in systemic lupus erythematosus(SLE)patients with an estimated incidence of 0.5–1.0%,including but not limited to thrombotic throm...To the Editor:Thrombotic microangiopathy(TMA)is an uncommon hematological involvement in systemic lupus erythematosus(SLE)patients with an estimated incidence of 0.5–1.0%,including but not limited to thrombotic thrombocytopenic purpura(TTP),hemolytic uremic syndrome(HUS),and other secondary conditions.SLE-TMA has a rapid onset and poor prognosis,manifesting as microvascular thrombosis,thrombocytopenia,and microangiopathic hemolytic anemia.[1]Based on the Chinese SLE Treatment and Research Group(CSTAR)registry,we aimed to summarize clinical characteristics and explore prognostic risk factors in SLE-TMA patients through a retrospective multicenter study.展开更多
BACKGROUND The relationship between diabetes mellitus(DM)and asthma is complex and can impact disease trajectories.AIM To explore the bidirectional influences between the two conditions on clinical outcomes and diseas...BACKGROUND The relationship between diabetes mellitus(DM)and asthma is complex and can impact disease trajectories.AIM To explore the bidirectional influences between the two conditions on clinical outcomes and disease control.METHODS We systematically reviewed the literature on the relationship between DM and asthma,focusing on their impacts,mechanisms,and therapeutic implications.Various studies were assessed,which investigated the effect of glycemic control on asthma outcomes,lung function,and exacerbations.The study highlighted the role of specific diabetes medications in managing asthma.RESULTS The results showed that poor glycemic control in diabetes can exacerbate asthma,increase hospitalizations,and reduce lung function.Conversely,severe asthma,especially in obese individuals,can complicate diabetes management and make glycemic control more difficult.The diabetes-associated mechanisms,such as inflammation,microangiopathy,and oxidative stress,can exacerbate asthma and decrease lung function.Some diabetes medications exhibit anti-inflammatory effects that show promise in mitigating asthma exacerbations.CONCLUSION The complex interrelationship between diabetes and asthma suggests bidirectional influences that affect disease course and outcomes.Inflammation and microvascular complications associated with diabetes may worsen asthma outcomes,while asthma severity,especially in obese individuals,complicates diabetes control.However,the current research has limitations,and more diverse longitudinal studies are required to establish causal relationships and identify effective treatment strategies for individuals with both conditions.展开更多
Thrombotic microangiopathy(TMA)is characterized by systemic microvascular thrombosis,target organ injury,anemia and thrombocytopenia.Thrombotic thrombocytopenic purpura,atypical hemolytic uremic syndrome and Shiga tox...Thrombotic microangiopathy(TMA)is characterized by systemic microvascular thrombosis,target organ injury,anemia and thrombocytopenia.Thrombotic thrombocytopenic purpura,atypical hemolytic uremic syndrome and Shiga toxin E-coli-related hemolytic uremic syndrome are the three common forms of TMAs.Traditionally,TMA is encountered during pregnancy/postpartum period,malignant hypertension,systemic infections,malignancies,autoimmune disorders,etc.Recently,the patients presenting with trauma have been reported to suffer from TMA.TMA carries a high morbidity and mortality,and demands a prompt recognition and early intervention to limit the target organ injury.Because trauma surgeons are the first line of defense for patients presenting with trauma,the prompt recognition of TMA for these experts is critically important.Early treatment of post-traumatic TMA can help improve the patient outcomes,if the diagnosis is made early.The treatment of TMA is also different from acute blood loss anemia namely in that plasmapheresis is recommended rather than platelet transfusion.This article familiarizes trauma surgeons with TMA encountered in the context of trauma.Besides,it provides a simplified approach to establishing the diagnosis of TMA.Because trauma patients can require multiple transfusions,the development of disseminated intravascular coagulation must be considered.Therefore,the article also provides different features of disseminated intravascular coagulation and TMA.Finally,the article suggests practical points that can be readily applied to the management of these patients.展开更多
Objective To study the relationship between growth hormone (GH) and microangiopathy in patients with diabetes mellitus in order to elucidate pathogenesis on microangiopathy in diabetics. Methods GH and insulin (INS) ...Objective To study the relationship between growth hormone (GH) and microangiopathy in patients with diabetes mellitus in order to elucidate pathogenesis on microangiopathy in diabetics. Methods GH and insulin (INS) were detected by radioimmunoassay, and blood sugar (BS) was detected by oxydase method. Results 138 NIDDM diabetics were examined. The concentration of serum GH in diabetics without microangiopathy (2.3±1.2 μg/L) was higher than in normal people (1.0±1.2 μg/L) and GH in diabetics with microangiopathy (5.74±1.94 μg/L) was higher than in diabetics without microangiopathy. The differences were significant ( P <0.01). As the history of diabetes went on, the level of GH in serum increased, and the incidence of microangiopathy increased too. The correlation of GH in serum with BS was parallel. The correlation of GH in serum with INS was not apparent. 27 IDDM diabetics were examined, their level of GH in serum (6.8±3.4 μg/L) was higher than that of NIDDM diabetics (4.6±1.8 μg/L). They were all patients with microangiopathy. Conclusion The rise of GH in serum may be an important pathogeny that causes microangiopathy in diabetics.展开更多
INTRODUCTIONTransplantation-associated thrombotic microangiopathy(TA-TMA)is a complication of hematopoietic stem cell transplantation(HSCT)characterized by small vessel endothelial damage leading to thrombosis and fib...INTRODUCTIONTransplantation-associated thrombotic microangiopathy(TA-TMA)is a complication of hematopoietic stem cell transplantation(HSCT)characterized by small vessel endothelial damage leading to thrombosis and fibrin deposition resulting in hemolytic anemia and thrombocytopenia.The severity of TA-TMA varies from mild self-limited disease to a fulminant variant resulting in death.Here,we review two rare cases and review the literature of TA-TMA.展开更多
BACKGROUND In this study,we retrospectively analysed macrophage infiltration and podocyte injury in three patients with diffuse proliferative lupus nephritis(LN)who un-derwent repeated renal biopsy.CASE SUMMARY Clinic...BACKGROUND In this study,we retrospectively analysed macrophage infiltration and podocyte injury in three patients with diffuse proliferative lupus nephritis(LN)who un-derwent repeated renal biopsy.CASE SUMMARY Clinical data of three diffuse proliferative LN patients with different pathological characteristics(case 1 was LN IV-G(A),case 2 was LN IV-G(A)+V,and case 3 was LN IV-G(A)+thrombotic microangiopathy)were reviewed.All patients underwent repeated renal biopsies 6 mo later,and renal biopsy specimens were studied.Macrophage infiltration was assessed by CD68 expression detected by immunohistochemical staining,and an immunofluorescence assay was used to detect podocin expression to assess podocyte damage.After treatment,Case 1 changed to LN III-(A),Case 2 remained as type V LN lesions,and Case 3,which changed to LN IV-S(A),had the worst prognosis.We observed reduced macro-phage infiltration after therapy.However,two of the patients with active lesions after treatment still showed macrophage infiltration in the renal interstitium.Before treatment,the three patients showed discontinuous expression of podocin.Notably,the integrity of podocin was restored after treatment in Case 1.CONCLUSION It may be possible to reverse podocyte damage and decrease the infiltrating ma-crophages in LN patients through effective treatment.展开更多
Microthrombosis may be involved in the pathogenesis of cardiac microangiopathy due to diabetes.Recent studies have shown that fibrinogen-like protein 2 (fgl2) plays a pivotal role in microthrombosis in viral hepatitis...Microthrombosis may be involved in the pathogenesis of cardiac microangiopathy due to diabetes.Recent studies have shown that fibrinogen-like protein 2 (fgl2) plays a pivotal role in microthrombosis in viral hepatitis, acute vascular xenograft rejection and cytokine-induced fetal loss syndrome.The current study was designed to examine the expression of fgl2 in microvascular endothelial cells and investigate the effects of microthrombi due to fgl2 on cardiac function and structure in rats with type 2 diabetes.Following induction of type 2 diabetes, 24 rats were observed dynamically.Fgl2 expression and related cardiac microthrombosis were examined.Local or circulating TNF-α was measured.Coronary flow (CF) per min was calculated as an index of cardiac microcirculation.Cardiac function and morphology were evaluated.It was found that Fgl2 was highly expressed in cardiac microvascular endothelial cells of rats with type 2 diabetes, which was promoted by local or circulating TNF-α.The Fgl2 expression was associated with cardiac hyaline microthrombosis.In parallel with the fgl2 expression, CF per min, cardiac diastolic or systolic function and cardiac morphology were aggravated to some extent.It was concluded that in rats with type 2 diabetes, microthrombosis due to fgl2 contributes to the impairment of cardiac diastolic or systolic function and morphological changes.展开更多
Hemolytic uremic syndrome （HUS） is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in ...Hemolytic uremic syndrome （HUS） is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the comple-ment proteins are clearly involved in all types of thrombotic microangiopathy： typical HUS, atypical HUS and thrombotic thrombocytopenic purpura （TTP）. Fur-thermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic as-pects of this rare disease, examining both “traditional therapy” （including plasma therapy, kidney and kidney-liver transplantation） and “new therapies”. The latter include anti-Shiga-toxin antibodies and anti-C5 mono-clonal antibody “eculizumab”. Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases Ⅰ and Ⅱ. They include anti-C5 antibodies, which are more purifed, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy.展开更多
Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicate...Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicated with malignant hypertension who underwent renal biopsy between January 2002 and December 2006.Results Of 19 patients,3 were men and 16 were women,with a mean age of 24.4±7.7 years old.All had positive antinuclear antibodies and low serum complement was found in 13 patients.All were anemic and 12 of them were thrombocytopenic.Impaired renal function was found in 17 patients with an average serum creatinine of 184.5±88.9 μmol/L.Severe intrarenal arteriolar lesion was found in all patients.Six patients had lupus vasculopathy,11 patients had renal thrombotic microangiopathy lesion,2 had severe arteriosclerosis.All patients received steroids and immunosuppressive drugs,15 received angiotensin-converting enzyme inhibitor(ACEI)/angiotensin receptor blocker(ARB)with resultant well-controlled blood pressure.Thrombocytopenia and hemolytic anemia resolved remarkably.The renal function improved or recovered in 14 of 17 patients,and 3 developed end-stage renal disease on maintenance dialysis.Conclusions Severe intrarenal vascular lesion complicated with renal nephritis parallels clinical manifestation of malignant hypertension.Renal pathology is the key of treatment strategy emphasizing on the significance of renal vascular involvement and type.On the basis of immunosuppressive drugs and steroids to control systemic lupus activity,timely initiation of ACEI/ARB could be of benefit to blood pressure control and long term renal survival.展开更多
基金Supported by the Research Project for Clinical Research on Precision Diagnosis and Innovative Treatment of Bone Marrow Failure,No.2024YFC2510500Jiangsu Provincial Traditional Chinese Medicine Science and Technology Development Plan,No.YB2020102Nantong Municipal Health Commission Research Project,No.QN2023007.
文摘BACKGROUND Thrombotic microangiopathy(TMA)is an acute syndrome characterized by microangiopathic hemolytic anemia,thrombocytopenia,and multi-organ dysfunction due to the microcirculation of platelet thrombi.Cancer-associated TMA is a rare and fatal complication,which often occurs during cancer remission.It is frequently misdiagnosed because of limited clinical awareness.CASE SUMMARY A middle-aged female patient presented to our clinic with a 15-days history of back pain,15 months post-gastrectomy.Cancer-associated TMA was confirmed through bone marrow aspiration,biopsy,and imaging.The patient received intermittent transfusions,fluids,nutrition,and microcirculation therapy with partial coagulation improvement.The family refused intensive care unit admission and plasma exchange,preferring palliative care.The patient died of cerebral hemorrhage and herniation due to disease progression.This case indicates that TMA may serve as an early manifestation of various malignancies,particularly gastric cancer.However,it is often misdiagnosed.Its pathogenesis is not well understood and needs to be further investigated.Currently,no standardized treatment have been developed.Plasma exchange is the only intervention available,though other therapies may also be effective.CONCLUSION In this case of gastric signet-ring cell carcinoma complicated by TMA,the patient achieved transient remission with supportive care but died following treatment discontinuation.Further studies are needed to elucidate the pathological mechanisms and therapeutic strategies for cancer-associated TMA.
文摘Pulmonary tumour thrombotic microangiopathy(PTTM)is a rare but under-recognised cause of rapidly progressive pulmonary hypertension(PH)and cor pulmonale,characterised by diffuse obstruction of small pulmonary arteries by metastatic tumour cells.These tumour emboli lead to obstructive intimal proliferation and in situ thrombosis within the pulmonary vasculature,further compromising the overall permeability of the pulmonary vascular bed and exacerbating PH.[1]The clinical and imaging manifestations of PTTM often overlap with those of other causes of PH,including chronic thromboembolic PH,pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis,often leading to diagnostic delays.
文摘Thrombotic microangiopathy(TMA)is an uncommon but serious complication that not only affects native kidneys but also transplanted kidneys.This review is specifically focused on post-transplant TMA(PT-TMA)involving kidney transplant recipients.Its reported prevalence in the latter population varies from 0.8%to 14%with adverse impacts on both graft and patient survival.It has many causes and associations,and the list of etiologic agents and associations is growing constantly.The pathogenesis is equally varied and a variety of pathogenetic pathways lead to the development of microvascular injury as the final common pathway.PT-TMA is categorized in many ways in order to facilitate its management.Ironically,more than one causes are contributory in PT-TMA and it is often difficult to pinpoint one particular cause in an individual case.Pathologically,the hallmark lesions are endothelial cell injury and intravascular thrombi affecting the microvasculature.Early diagnosis and classification of PT-TMA are imperative for optimal outcomes but are challenging for both clinicians and pathologists.The Banff classification has addressed this issue and has developed minimum diagnostic criteria for pathologic diagnosis of PT-TMA in the first phase.Management of the condition is also challenging and still largely empirical.It varies from simple maneuvers,such as plasmapheresis,drug withdrawal or modification,or dose reduction,to lifelong complement blockade,which is very expensive.A thorough understanding of the condition is imperative for an early diagnosis and quick treatment when the treatment is potentially effective.This review aims to increase the awareness of relevant stakeholders regarding this important,potentially treatable but under-recognized cause of kidney allograft dysfunction.
文摘BACKGROUND Pulmonary tumor thrombotic microangiopathy(PTTM)is a rare condition in patients with hepatocellular carcinoma(HCC);to date,few cases have been reported.While hepatic dysfunction has been focused on the later stages of HCC,the management of symptoms in PTTM is important for supportive care of the cases.For the better understanding of PTTM in HCC,the information of our recent case and reported cases have been summarized.CASE SUMMARY A patient with HCC exhibited acute and severe respiratory failure.Radiography and computed tomography of the chest revealed the multiple metastatic tumors and a frosted glass–like shadow with no evidence of infectious pneumonia.We diagnosed his condition as acute respiratory distress syndrome caused by the lung metastases and involvement of the pulmonary vessels by tumor thrombus.Administration of prednisolone to alleviate the diffuse alveolar damages including edematous changes of alveolar wall caused by the tumor cell infiltration and ischemia showed mild improvement in his symptoms and imaging findings.An autopsy showed the typical pattern of PTTM in the lung with multiple metastases.CONCLUSION PTTM is caused by tumor thrombi in the arteries and thickening of the pulmonary arterial endothelium leading to the symptoms of dyspnea in terminal staged patients.Therefore,supportive management of symptoms is necessary in the cases with PTTM and hence we believe that the information presented here is of great significance for the diagnosis and management of symptoms of PTTM with HCC.
文摘Thrombotic microangiopathy(TMA) is one of the most devastating sequalae of kidney transplantation. A number of published articles have covered either de novo or recurrent TMA in an isolated manner. We have, hereby, in this article endeavored to address both types of TMA in a comparative mode. We appreciate that de novo TMA is more common and its prognosis is poorer than recurrent TMA; the latter has a genetic background, with mutations that impact disease behavior and, consequently, allograft and patient survival. Post-transplant TMA can occur as a recurrence of the disease involving the native kidney or as de novo disease with no evidence of previous involvement before transplant. While atypical hemolytic uremic syndrome is a rare disease that results from complement dysregulation with alternative pathway overactivity, de novo TMA is a heterogenous set of various etiologies and constitutes the vast majority of post-transplant TMA cases. Management of both diseases varies from simple maneuvers, e.g., plasmapheresis, drug withdrawal or dose modification, to lifelong complement blockade, which is rather costly. Careful donor selection and proper recipient preparation, including complete genetic screening, would be a pragmatic approach. Novel therapies, e.g., purified products of the deficient genes, though promising in theory, are not yet of proven value.
文摘BACKGROUND Sodium valproate is widely used in the treatment of epilepsy in clinical practice.Most adverse reactions to sodium valproate are mild and reversible,while serious idiosyncratic side effects are becoming apparent,particularly hepatotoxicity.Herein,we report a case of fatal acute liver failure(ALF)with thrombotic microangiopathy(TMA)caused by treatment with sodium valproate in a patient following surgery for meningioma.CASE SUMMARY A 42-year-old man who received antiepileptic treatment with sodium valproate after surgery for meningioma exhibited extreme fatigue,severe jaundice accompanied by oliguria,soy sauce-colored urine,and ecchymosis.His postoperative laboratory values indicated a rapid decreased platelet count and hemoglobin level,severe liver and kidney dysfunction,and disturbance of the coagulation system.He was diagnosed with drug-induced liver failure combined with TMA.After plasma exchange combined with hemoperfusion,pulse therapy with high-dose methylprednisolone,and blood transfusion,his liver function deteriorated,and finally,he died.CONCLUSION ALF with TMA is a rare and fatal adverse reaction of sodium valproate which needs to be highly valued.
文摘BACKGROUND Interferons(IFNs)are characterized by a wide range of biological effects,which justifies their potential therapeutic use in several pathologies,but also elicit a wide array of adverse effects in almost every organ system.Among them,renal involvement is probably one of the most complex to identify.CASE SUMMARY We describe four cases of kidney damage caused by different IFN formulations:IFN-β-related thrombotic microangiopathy,IFN-β-induced systemic lupus erythematosus,and two cases of membranous nephropathy secondary to pegylated-IFN-α2B.In each case,we carefully excluded any other possible cause of renal involvement.Once suspected as the casual relationship between drug and kidney damage,IFN treatment was immediately discontinued.In three cases,we observed a complete and persistent remission of clinical and laboratory abnormalities after IFN withdrawal,while the patient who developed thrombotic microangiopathy,despite IFN withdrawal and complement-inhibitor therapy with eculizumab,showed persistent severe renal failure requiring dialysis.CONCLUSION This case series highlights the causal relationship between IFN treatment and different types of renal involvement and enables us to delineate several peculiarities of this association.
基金Supported by the Grant from Uehara Memorial Foundation, No. 200940051, Tokyo, 171-0033, Japan
文摘AIM:To investigate thrombotic microangiopathy (TMA)in liver transplantion,because TMA is an infrequent but life-threatening complication in the transplantation field. METHODS:A total of 206 patients who underwent living-donor liver transplantation (LDLT) were evaluated,and the TMA-like disorder (TMALD) occurred in seven recipients. RESULTS:These TMALD recipients showed poor outcomes in comparison with other 199 recipients. Although two TMALD recipients successfully recovered,the other five recipients finally died despite intensive treatments including repeated plasma exchange (PE) and re-transplantation. Histopathological analysis of liver biopsies after LDLT revealed obvious differences according to the outcomes. Qualitative analysis of antibodies against a disintegrin-like domain and metalloproteinase with thrombospondin type 1 motifs (ADAMTS-13) were negative in all patients. The fragmentation of red cells,the microhemorrhagic macules and the platelet counts were early markers for the suspicion of TMALD after LDLT. Although the absolute values of von Willebrand factor (vWF) and ADAMTS-13 did not necessarily reflect TMALD,the vWF/ADAMTS-13 ratio had a clear diagnostic value in all cases. The establishment of adequate treatments for TMALD,such as PE for ADAMTS-13 replenishment or treatments against inhibitory antibodies,must be decided according to each case. CONCLUSION:The optimal induction of adequate therapies based on early recognition of TMALD by the reliable markers may confer a large advantage for TMALD after LDLT.
基金Supported by Specialized Scientific Research Fund Projects of The Medical Group of Qingdao University,No.YLJT20201002.
文摘BACKGROUND Thrombotic microangiopathy(TMA)is a group of disorders that converge on excessive platelet aggregation in the microvasculature,leading to consumptive thrombocytopenia,microangiopathic hemolysis and ischemic end-organ dysfunction.In predisposed patients,TMA can be triggered by many environmental factors.Glucocorticoids(GCs)can compromise the vascular endothelium.However,GC-associated TMA has rarely been reported,which may be due to the lack of awareness of clinicians.Given the high frequency of thrombocytopenia during GC treatment,particular attention should be given to this potentially fatal complication.CASE SUMMARY An elderly Chinese man had a 12-year history of aplastic anemia(AA)and a 3-year history of paroxysmal nocturnal hemoglobinuria(PNH).Three months earlier,methylprednisolone treatment was initiated at 8 mg/d and increased to 20 mg/d to alleviate complement-mediated hemolysis.Following GC treatment,his platelet counts and hemoglobin levels rapidly decreased.After admission to our hospital,the dose of methylprednisolone was increased to 60 mg/d in an attempt to enhance the suppressive effect.However,increasing the GC dose did not alleviate hemolysis,and his cytopenia worsened.Morphological evaluation of the marrow smears revealed increased cellularity with an increased percentage of erythroid progenitors without evident dysplasia.Cluster of differentiation(CD)55 and CD59 expression was significantly decreased on erythrocytes and granulocytes.In the following days,platelet transfusion was required due to severe thrombocytopenia.Observation of platelet transfusion refractoriness indicated that the exacerbated cytopenia may have been caused by the development of TMA due to GC treatment because the transfused platelet concentrates had no defects in glycosylphosphatidylinositol-anchored proteins.We examined blood smears and found a small number of schistocytes,dacryocytes,acanthocytes and target cells.Discontinuation of GC treatment resulted in rapidly increased platelet counts and steady increases in hemoglobin levels.The patient’s platelet counts and hemoglobin levels returned to the levels prior to GC treatment 4 weeks after GC discontinuation.CONCLUSION GCs can drive TMA episodes.When thrombocytopenia occurs during GC treatment,TMA should be considered,and GCs should be discontinued.
文摘Thrombotic microangiopathy(TMA)is potentially life-threatening condition caused by small-vessel microthrombi and is associated with schistocyte formation,low platelets and end-organ damage that may not be reversible.[1,2]As a life-threatening condition,TMA recognition in hospitalized patients after organ transplantation is key to improving survival.Transplant-associated TMA(TATMA)can occur after both solid organ or hematopoietic stem cell transplantation and often mimics other disease processes such as thrombotic throm-bocytopenic purpura(TTP)with similar constellation of symptoms during presentation.We present a rare case of a patient with TATMA after orthotopic heart transplantation.
文摘Concentration of supemxide anion in plasma and ecythrocytes in 21 patients with non-Insulin-dependent diabetes mellitus (NIDDM)(14 with complicating microangiopathy) and 34 normal adults was assayed by chemiluinesence. Both plasma and erythrocyte concentration of .0-2 were ekvated in NIDDM with complicating microangiopathy beyond normal range (P<0.01),whereas in NIDDM without microangiopathy,in plasma was only slightly raised and RBC(.0-2) was within normal range. The authors think that RBC (.0-2) may be used as an indicator for the detection of presence of microangiopathy in NIDDM.
基金supported by the Chinese National Key Technology R&D Program,Ministry of Science and Technology(No.2021YFC2501300)Beijing Municipal Science&Technology Commission(Nos.Z201100005520022,Z201100005520023,Z201100005520025,Z201100005520026,and Z201100005520027)+2 种基金CAMS Innovation Fund for Medical Sciences(CIFMS)(Nos.2021-I2M-1-005,2022-I2M-1-004,and 2023-I2M-2-005)National High-Level Hospital Clinical Research Funding(Nos.2022-PUMCH-B-013,2022-PUMCH-C-002,and 2022-PUMCH-D-009)Peking Union Medical College Student Innovation Training Project(No.2023zg1c06017).
文摘To the Editor:Thrombotic microangiopathy(TMA)is an uncommon hematological involvement in systemic lupus erythematosus(SLE)patients with an estimated incidence of 0.5–1.0%,including but not limited to thrombotic thrombocytopenic purpura(TTP),hemolytic uremic syndrome(HUS),and other secondary conditions.SLE-TMA has a rapid onset and poor prognosis,manifesting as microvascular thrombosis,thrombocytopenia,and microangiopathic hemolytic anemia.[1]Based on the Chinese SLE Treatment and Research Group(CSTAR)registry,we aimed to summarize clinical characteristics and explore prognostic risk factors in SLE-TMA patients through a retrospective multicenter study.
文摘BACKGROUND The relationship between diabetes mellitus(DM)and asthma is complex and can impact disease trajectories.AIM To explore the bidirectional influences between the two conditions on clinical outcomes and disease control.METHODS We systematically reviewed the literature on the relationship between DM and asthma,focusing on their impacts,mechanisms,and therapeutic implications.Various studies were assessed,which investigated the effect of glycemic control on asthma outcomes,lung function,and exacerbations.The study highlighted the role of specific diabetes medications in managing asthma.RESULTS The results showed that poor glycemic control in diabetes can exacerbate asthma,increase hospitalizations,and reduce lung function.Conversely,severe asthma,especially in obese individuals,can complicate diabetes management and make glycemic control more difficult.The diabetes-associated mechanisms,such as inflammation,microangiopathy,and oxidative stress,can exacerbate asthma and decrease lung function.Some diabetes medications exhibit anti-inflammatory effects that show promise in mitigating asthma exacerbations.CONCLUSION The complex interrelationship between diabetes and asthma suggests bidirectional influences that affect disease course and outcomes.Inflammation and microvascular complications associated with diabetes may worsen asthma outcomes,while asthma severity,especially in obese individuals,complicates diabetes control.However,the current research has limitations,and more diverse longitudinal studies are required to establish causal relationships and identify effective treatment strategies for individuals with both conditions.
文摘Thrombotic microangiopathy(TMA)is characterized by systemic microvascular thrombosis,target organ injury,anemia and thrombocytopenia.Thrombotic thrombocytopenic purpura,atypical hemolytic uremic syndrome and Shiga toxin E-coli-related hemolytic uremic syndrome are the three common forms of TMAs.Traditionally,TMA is encountered during pregnancy/postpartum period,malignant hypertension,systemic infections,malignancies,autoimmune disorders,etc.Recently,the patients presenting with trauma have been reported to suffer from TMA.TMA carries a high morbidity and mortality,and demands a prompt recognition and early intervention to limit the target organ injury.Because trauma surgeons are the first line of defense for patients presenting with trauma,the prompt recognition of TMA for these experts is critically important.Early treatment of post-traumatic TMA can help improve the patient outcomes,if the diagnosis is made early.The treatment of TMA is also different from acute blood loss anemia namely in that plasmapheresis is recommended rather than platelet transfusion.This article familiarizes trauma surgeons with TMA encountered in the context of trauma.Besides,it provides a simplified approach to establishing the diagnosis of TMA.Because trauma patients can require multiple transfusions,the development of disseminated intravascular coagulation must be considered.Therefore,the article also provides different features of disseminated intravascular coagulation and TMA.Finally,the article suggests practical points that can be readily applied to the management of these patients.
文摘Objective To study the relationship between growth hormone (GH) and microangiopathy in patients with diabetes mellitus in order to elucidate pathogenesis on microangiopathy in diabetics. Methods GH and insulin (INS) were detected by radioimmunoassay, and blood sugar (BS) was detected by oxydase method. Results 138 NIDDM diabetics were examined. The concentration of serum GH in diabetics without microangiopathy (2.3±1.2 μg/L) was higher than in normal people (1.0±1.2 μg/L) and GH in diabetics with microangiopathy (5.74±1.94 μg/L) was higher than in diabetics without microangiopathy. The differences were significant ( P <0.01). As the history of diabetes went on, the level of GH in serum increased, and the incidence of microangiopathy increased too. The correlation of GH in serum with BS was parallel. The correlation of GH in serum with INS was not apparent. 27 IDDM diabetics were examined, their level of GH in serum (6.8±3.4 μg/L) was higher than that of NIDDM diabetics (4.6±1.8 μg/L). They were all patients with microangiopathy. Conclusion The rise of GH in serum may be an important pathogeny that causes microangiopathy in diabetics.
文摘INTRODUCTIONTransplantation-associated thrombotic microangiopathy(TA-TMA)is a complication of hematopoietic stem cell transplantation(HSCT)characterized by small vessel endothelial damage leading to thrombosis and fibrin deposition resulting in hemolytic anemia and thrombocytopenia.The severity of TA-TMA varies from mild self-limited disease to a fulminant variant resulting in death.Here,we review two rare cases and review the literature of TA-TMA.
基金Supported by National Natural Science Foundation of China,No.81960136the Science and Technology Department of Yunnan Province,No.202101AT070243.
文摘BACKGROUND In this study,we retrospectively analysed macrophage infiltration and podocyte injury in three patients with diffuse proliferative lupus nephritis(LN)who un-derwent repeated renal biopsy.CASE SUMMARY Clinical data of three diffuse proliferative LN patients with different pathological characteristics(case 1 was LN IV-G(A),case 2 was LN IV-G(A)+V,and case 3 was LN IV-G(A)+thrombotic microangiopathy)were reviewed.All patients underwent repeated renal biopsies 6 mo later,and renal biopsy specimens were studied.Macrophage infiltration was assessed by CD68 expression detected by immunohistochemical staining,and an immunofluorescence assay was used to detect podocin expression to assess podocyte damage.After treatment,Case 1 changed to LN III-(A),Case 2 remained as type V LN lesions,and Case 3,which changed to LN IV-S(A),had the worst prognosis.We observed reduced macro-phage infiltration after therapy.However,two of the patients with active lesions after treatment still showed macrophage infiltration in the renal interstitium.Before treatment,the three patients showed discontinuous expression of podocin.Notably,the integrity of podocin was restored after treatment in Case 1.CONCLUSION It may be possible to reverse podocyte damage and decrease the infiltrating ma-crophages in LN patients through effective treatment.
基金supported by CGICC Medical Science Research Supporting Program (No.08010022)National Key Basic Research Program of China (No.2007CB512000,Sub-Project No.2007CB512005)
文摘Microthrombosis may be involved in the pathogenesis of cardiac microangiopathy due to diabetes.Recent studies have shown that fibrinogen-like protein 2 (fgl2) plays a pivotal role in microthrombosis in viral hepatitis, acute vascular xenograft rejection and cytokine-induced fetal loss syndrome.The current study was designed to examine the expression of fgl2 in microvascular endothelial cells and investigate the effects of microthrombi due to fgl2 on cardiac function and structure in rats with type 2 diabetes.Following induction of type 2 diabetes, 24 rats were observed dynamically.Fgl2 expression and related cardiac microthrombosis were examined.Local or circulating TNF-α was measured.Coronary flow (CF) per min was calculated as an index of cardiac microcirculation.Cardiac function and morphology were evaluated.It was found that Fgl2 was highly expressed in cardiac microvascular endothelial cells of rats with type 2 diabetes, which was promoted by local or circulating TNF-α.The Fgl2 expression was associated with cardiac hyaline microthrombosis.In parallel with the fgl2 expression, CF per min, cardiac diastolic or systolic function and cardiac morphology were aggravated to some extent.It was concluded that in rats with type 2 diabetes, microthrombosis due to fgl2 contributes to the impairment of cardiac diastolic or systolic function and morphological changes.
文摘Hemolytic uremic syndrome （HUS） is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the comple-ment proteins are clearly involved in all types of thrombotic microangiopathy： typical HUS, atypical HUS and thrombotic thrombocytopenic purpura （TTP）. Fur-thermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic as-pects of this rare disease, examining both “traditional therapy” （including plasma therapy, kidney and kidney-liver transplantation） and “new therapies”. The latter include anti-Shiga-toxin antibodies and anti-C5 mono-clonal antibody “eculizumab”. Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases Ⅰ and Ⅱ. They include anti-C5 antibodies, which are more purifed, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy.
文摘Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicated with malignant hypertension who underwent renal biopsy between January 2002 and December 2006.Results Of 19 patients,3 were men and 16 were women,with a mean age of 24.4±7.7 years old.All had positive antinuclear antibodies and low serum complement was found in 13 patients.All were anemic and 12 of them were thrombocytopenic.Impaired renal function was found in 17 patients with an average serum creatinine of 184.5±88.9 μmol/L.Severe intrarenal arteriolar lesion was found in all patients.Six patients had lupus vasculopathy,11 patients had renal thrombotic microangiopathy lesion,2 had severe arteriosclerosis.All patients received steroids and immunosuppressive drugs,15 received angiotensin-converting enzyme inhibitor(ACEI)/angiotensin receptor blocker(ARB)with resultant well-controlled blood pressure.Thrombocytopenia and hemolytic anemia resolved remarkably.The renal function improved or recovered in 14 of 17 patients,and 3 developed end-stage renal disease on maintenance dialysis.Conclusions Severe intrarenal vascular lesion complicated with renal nephritis parallels clinical manifestation of malignant hypertension.Renal pathology is the key of treatment strategy emphasizing on the significance of renal vascular involvement and type.On the basis of immunosuppressive drugs and steroids to control systemic lupus activity,timely initiation of ACEI/ARB could be of benefit to blood pressure control and long term renal survival.
