BACKGROUND Lymphoplasmacytic lymphoma is a rare non-Hodgkin’s lymphoma,occurring mostly in the elderly.It develops slowly and leads to malignant proliferation of lymphoid line cells in the bone marrow,lymph nodes and...BACKGROUND Lymphoplasmacytic lymphoma is a rare non-Hodgkin’s lymphoma,occurring mostly in the elderly.It develops slowly and leads to malignant proliferation of lymphoid line cells in the bone marrow,lymph nodes and spleen.It may also affect nerve roots and meninges;some patients develop sensorimotor polyneuropathy which may precede general symptoms of lymphoma.CASE SUMMARY We present a case of a 36-year-old man diagnosed in 2012 with chronic inflammatory demyelinating polyneuropathy(CIDP),then he was hospitalized in 2019 due to progressive symptoms of heart failure and significant weight loss over the previous four months.Based on clinical and laboratory findings a diagnosis of lymphoplasmacytic lymphoma was suspected and confirmed by bone marrow flow cytometry.There was no improvement in the results of laboratory tests and the patient's condition after immediate implementation of chemotherapy.Patient died on the fifth day of treatment.CONCLUSION While CIDP and malignant disease co-occurrence is rare,it should be suspected and investigated in patients with atypical neuropathy symptoms.展开更多
BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare.Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies.Synchronous diag...BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare.Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies.Synchronous diagnoses of diffuse large B-cell lymphoma(DLBCL),acute myeloid leukemia(AML),and untreated lymphoplasmacytic lymphoma/Waldenström macroglobulinemia(LPL/WM)in the same patient have not been reported.Here we report one such case.CASE SUMMARY An 89-year-old man had a chest wall mass histopathologically diagnosed as DLBCL.The bone marrow and peripheral blood contained two groups of cells.One group of cells fulfilled the criteria of AML,and the other revealed the features of small B lymphocytic proliferative disorder,which we considered LPL/WM.Multiple chromosomal or genetic changes were detected in bone marrow mononuclear cells,including ATM deletion,CCND1 amplification,mutations of MYD88(L265P)and TP53,WT1 overexpression,and fusion gene of BIRC2-ARAP1,as well as complex chromosomal abnormalities.The patient refused chemotherapy because of old age and died of pneumonia 1 mo after the final diagnosis.CONCLUSION The coexistence of DLBCL,AML,and untreated LPL/WM in the same patient is extremely rare,which probably results from multiple steps of genetic abnormalities.Asymptomatic LPL/WM might have occurred first,then myelodysplastic syndromerelated AML developed,and finally aggressive DLBCL arose.Therefore,medical staff should pay attention to this rare phenomenon to avoid misdiagnoses.展开更多
Autoimmune pancreatitis （AIP） is a particular type of pancreatitis of presumed autoimmune etiology. Currently, AIP should be diagnosed based on combination of clinical, serological, morphological, and histopathologi...Autoimmune pancreatitis （AIP） is a particular type of pancreatitis of presumed autoimmune etiology. Currently, AIP should be diagnosed based on combination of clinical, serological, morphological, and histopathological features. When diagnosing AIP, it is most important to differentiate it from pancreatic cancer. Diagnostic criteria for AIP, proposed by the Japan Pancreas Society in 2002 first in the world, were revised in 2006. The criteria are based on the minimum consensus of AIP and aim to avoid misdiagnosing pancreatic cancer as far as possible, but not for screening AIR The criteria consist of the following radiological, serological, and histopathological items： （1） radiological imaging showing narrowing of the main pancreatic duct and enlargement of the pancreas, which are characteristic of the disease; （2） laboratory data showing abnormally elevated levels of serum y-globulin, IgG or IgG4, or the presence of autoantibodies; （3） histopathological examination of the pancreas demonstrating marked fibrosis and prominent infiltration of lymphocytes and plasma cells, which is called lymphoplasmacytic sclerosing pancreatitis （LPSP）. For a diagnosis of AIP, criterion 1 must be present, together with criterion 2 and/ or criterion 3. However, it is necessary to exclude malignant diseases such as pancreatic or biliary cancer.展开更多
This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves ...This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.展开更多
AIM:To investigate the usefulness of endoscopic ultrasound-guided fine needle aspiration(EUS-FNA) in the differentiation of autoimmune pancreatitis(AIP).METHODS:We retrospectively reviewed 47 of 56 AIP patients who un...AIM:To investigate the usefulness of endoscopic ultrasound-guided fine needle aspiration(EUS-FNA) in the differentiation of autoimmune pancreatitis(AIP).METHODS:We retrospectively reviewed 47 of 56 AIP patients who underwent EUS-FNA and met the Asian diagnostic criteria.On 47 EUS-FNA specimens,we evaluated the presence of adequate material and characteristic features of lymphoplasmacytic sclerosing pancreatitis(LPSP) and idiopathic duct-centric pancreatitis(IDCP) mentioned in the International Consensus Diagnostic Criteria and examined if these findings make a contribution to the differential diagnosis of type 1 and type 2 AIP.A disposable 22-gauge needle was used for EUS-FNA.RESULTS:Adequate specimens including pancreatic tissue for differentiating AIP from cancer were obtained from 43 of 47 patients who underwent EUSFNA.EUS-FNA was performed from the pancreatic head in 21 cases,which is known to be technically difficult when performed by core biopsy;there was no significant difference in the results compared with pancreatic body-tail.Nine of 47 patients met level 1 findings of LPSP and 5 patients met level 2 findings of LPSP.No one met level 1 findings of IDCP,but 3 patients met level 2 findings of IDCP.Of 10 seronegative cases,2 cases were diagnosed with "definitive type 1 AIP",and 3 cases were diagnosed with "probable type 2 AIP" when considering both the level 2 histological findings and response to steroids.CONCLUSION:EUS-FNA is useful in the differentiation of type 1 and type 2 AIP,particularly in seronegative cases.展开更多
AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divide...AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divided 67 AIP patients into diffuse type(D type) and focal type(F type).We further divided F type into head type(H type) and body and/or tail type(B/T type) according to the location of enlargement.Finally,we classified the 67 AIP patients into three groups:D type,H type and B/T type.We compared the three types of AIP in terms of clinical,laboratory,radiological,functional and histological findings and clinical course.RESULTS:There were 34 patients with D-type,19 with H-type and 14 with B/T-type AIP.Although obstructive jaundice was frequently detected in D-typepatients(88%) and H-type patients(68%),no B/T-type patients showed jaundice as an initial symptom(P < 0.001).There were no differences in frequency of abdominal pain,but acute pancreatitis was associated more frequently in B/T-type patients(36%) than in D-type patients(3%)(P = 0.017).Serum immunoglobulin G(IgG)4 levels were significantly higher in D-type patients(median 309 mg/dL) than in B/T-type patients(133.5 mg/dL)(P = 0.042).Serum amylase levels in B/T-type patients(median:114 IU/L) were significantly greater than in H-type patients(72 IU/L)(P = 0.049).Lymphoplasmacytic sclerosing pancreatitis(LPSP) was histologically confirmed in 6 D-type,7 H-type and 4 B/T-type patients;idiopathic duct-centric pancreatitis was observed in no patients.Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients.Steroid therapy was effective in all 50 patients(31 D type,13 H type and 6 B/T type).Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients,no patients relapsed in B/T type.During follow-up,radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION:Clinical features of H-type AIP were similar to those of D-type,but B/T-type differed from D and H types.B/T-type may involve diseases other than LPSP.展开更多
Autoimmune pancreatitis(AIP)is defined as pancreatitis caused by irregular narrowing of the pancreatic duct accompanied by pancreatic swelling,fibrosis and lymphocyte infiltration,events that are related to autoimmune...Autoimmune pancreatitis(AIP)is defined as pancreatitis caused by irregular narrowing of the pancreatic duct accompanied by pancreatic swelling,fibrosis and lymphocyte infiltration,events that are related to autoimmune mechanisms.The 2010 International Consensus Diagnostic Criteria for AIP defined pancreatitis as“type 1”when increased levels of serum IgG4 were present and other organs were involved;lymphoplasmacytic sclerosing pancreatitis was the main histological characteristic.Apart from surgery,endoscopic ultrasonographyguided fine needle aspiration(EUS-FNA)is the only method for the histological diagnosis of AIP;however,this method is difficult.The use of larger-diameter FNA needles and trucut biopsy did not improve the diagnostic performance of EUS-FNA,but it has improved gradually.In this review,we look back at past efforts to improve the diagnostic performance of EUS-FNA and reveal the present state of EUS-FNA for the histological diagnosis of AIP type 1.展开更多
Autoimmune pancreatitis has received considerable attention,especially due to the marked effect of corticosteroid therapy on its clinical course.Knowledge,especially regarding type 1 autoimmune pancreatitis,has signif...Autoimmune pancreatitis has received considerable attention,especially due to the marked effect of corticosteroid therapy on its clinical course.Knowledge,especially regarding type 1 autoimmune pancreatitis,has significantly increased over the last decades,and despite significant differences in pathophysiology and outcomes,both type 1 and 2 autoimmune pancreatitis are still considered different types of the same disease.Some have proposed a different nomenclature reflecting these differences.Although the term steroid-responsive pancreatitides may be interpreted as synonymous to type 1 and 2 autoimmune pancreatitis,these are not the only pancreatic conditions that show a response to steroid therapy.Acute pancreatitis caused by vasculitis and connective tissue diseases and acute pancreatitis secondary to checkpoint inhibitors or programmed cell death receptor antibody-mediated blockage cancer therapy may also benefit from steroid treatment.This review presents current concepts on these disorders,aiming to increase awareness,analyze similarities and differences,and propose a new nomenclature that reflects their specific particularities,clustering them under the term"steroid-responsive pancreatitides".展开更多
Waldenstr?m macroglobulinemia (WM) is a rare lymphoid malignancy. Many studies, including clinicopathological, cytogenetic, gene expression profile, and therapy studies have been reported from the US and Europe, altho...Waldenstr?m macroglobulinemia (WM) is a rare lymphoid malignancy. Many studies, including clinicopathological, cytogenetic, gene expression profile, and therapy studies have been reported from the US and Europe, although only a few reports are available from East Asia, including Japan. To further clarify the clinicopathological, radiological, and cytogenetic features of WM in Japan, we performed a retrospective analysis of WM in our institute between March 2007 and January 2012. Clinical data, laboratory data, the results of flow cytometric analysis (FCM), and chromosomal abnormalities were analyzed, and a radiological review was performed. The treatment regimen, response, and survival were also estimated. Six patients were enrolled in this study. The median age was 71 years. All patients were symptomatic, 3 had hyperviscosity syndrome, 1 had bone lesions, and 1 had an extra-medullary mass. FCM data showed that all patients were positive for CD38, while 2 were positive for CD56. Four had chromosomal abnormalities including some abnormalities also reported in myeloma. On radiological review, four showed diffuse invasion of the retro-peritoneum. Five patients received treatment, 4 of which achieved a response. At a median follow-up of 527 days, 4 were alive and 2 died because of disease progression. The present study revealed that WM in Japan might be heterogeneous and have a unique disease manifestation. Invasion sites other than bone marrow were very common, and the results of clinical, FCM, and cytogenetic studies revealed that WM in Japanese cases might have manifestations of both myeloma and B-cell lymphoma.展开更多
文摘BACKGROUND Lymphoplasmacytic lymphoma is a rare non-Hodgkin’s lymphoma,occurring mostly in the elderly.It develops slowly and leads to malignant proliferation of lymphoid line cells in the bone marrow,lymph nodes and spleen.It may also affect nerve roots and meninges;some patients develop sensorimotor polyneuropathy which may precede general symptoms of lymphoma.CASE SUMMARY We present a case of a 36-year-old man diagnosed in 2012 with chronic inflammatory demyelinating polyneuropathy(CIDP),then he was hospitalized in 2019 due to progressive symptoms of heart failure and significant weight loss over the previous four months.Based on clinical and laboratory findings a diagnosis of lymphoplasmacytic lymphoma was suspected and confirmed by bone marrow flow cytometry.There was no improvement in the results of laboratory tests and the patient's condition after immediate implementation of chemotherapy.Patient died on the fifth day of treatment.CONCLUSION While CIDP and malignant disease co-occurrence is rare,it should be suspected and investigated in patients with atypical neuropathy symptoms.
基金Supported by the National Natural Science Foundation of China,No.81700130Nanjing Medical University Science and Technology Development Fund.
文摘BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare.Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies.Synchronous diagnoses of diffuse large B-cell lymphoma(DLBCL),acute myeloid leukemia(AML),and untreated lymphoplasmacytic lymphoma/Waldenström macroglobulinemia(LPL/WM)in the same patient have not been reported.Here we report one such case.CASE SUMMARY An 89-year-old man had a chest wall mass histopathologically diagnosed as DLBCL.The bone marrow and peripheral blood contained two groups of cells.One group of cells fulfilled the criteria of AML,and the other revealed the features of small B lymphocytic proliferative disorder,which we considered LPL/WM.Multiple chromosomal or genetic changes were detected in bone marrow mononuclear cells,including ATM deletion,CCND1 amplification,mutations of MYD88(L265P)and TP53,WT1 overexpression,and fusion gene of BIRC2-ARAP1,as well as complex chromosomal abnormalities.The patient refused chemotherapy because of old age and died of pneumonia 1 mo after the final diagnosis.CONCLUSION The coexistence of DLBCL,AML,and untreated LPL/WM in the same patient is extremely rare,which probably results from multiple steps of genetic abnormalities.Asymptomatic LPL/WM might have occurred first,then myelodysplastic syndromerelated AML developed,and finally aggressive DLBCL arose.Therefore,medical staff should pay attention to this rare phenomenon to avoid misdiagnoses.
基金Research for Intractable Disease of the Pancreas, Ministry of Health, Labor and Welfare of Japan
文摘Autoimmune pancreatitis （AIP） is a particular type of pancreatitis of presumed autoimmune etiology. Currently, AIP should be diagnosed based on combination of clinical, serological, morphological, and histopathological features. When diagnosing AIP, it is most important to differentiate it from pancreatic cancer. Diagnostic criteria for AIP, proposed by the Japan Pancreas Society in 2002 first in the world, were revised in 2006. The criteria are based on the minimum consensus of AIP and aim to avoid misdiagnosing pancreatic cancer as far as possible, but not for screening AIR The criteria consist of the following radiological, serological, and histopathological items： （1） radiological imaging showing narrowing of the main pancreatic duct and enlargement of the pancreas, which are characteristic of the disease; （2） laboratory data showing abnormally elevated levels of serum y-globulin, IgG or IgG4, or the presence of autoantibodies; （3） histopathological examination of the pancreas demonstrating marked fibrosis and prominent infiltration of lymphocytes and plasma cells, which is called lymphoplasmacytic sclerosing pancreatitis （LPSP）. For a diagnosis of AIP, criterion 1 must be present, together with criterion 2 and/ or criterion 3. However, it is necessary to exclude malignant diseases such as pancreatic or biliary cancer.
文摘This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.
基金Supported by The Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health,Labour,and Welfare of Japan
文摘AIM:To investigate the usefulness of endoscopic ultrasound-guided fine needle aspiration(EUS-FNA) in the differentiation of autoimmune pancreatitis(AIP).METHODS:We retrospectively reviewed 47 of 56 AIP patients who underwent EUS-FNA and met the Asian diagnostic criteria.On 47 EUS-FNA specimens,we evaluated the presence of adequate material and characteristic features of lymphoplasmacytic sclerosing pancreatitis(LPSP) and idiopathic duct-centric pancreatitis(IDCP) mentioned in the International Consensus Diagnostic Criteria and examined if these findings make a contribution to the differential diagnosis of type 1 and type 2 AIP.A disposable 22-gauge needle was used for EUS-FNA.RESULTS:Adequate specimens including pancreatic tissue for differentiating AIP from cancer were obtained from 43 of 47 patients who underwent EUSFNA.EUS-FNA was performed from the pancreatic head in 21 cases,which is known to be technically difficult when performed by core biopsy;there was no significant difference in the results compared with pancreatic body-tail.Nine of 47 patients met level 1 findings of LPSP and 5 patients met level 2 findings of LPSP.No one met level 1 findings of IDCP,but 3 patients met level 2 findings of IDCP.Of 10 seronegative cases,2 cases were diagnosed with "definitive type 1 AIP",and 3 cases were diagnosed with "probable type 2 AIP" when considering both the level 2 histological findings and response to steroids.CONCLUSION:EUS-FNA is useful in the differentiation of type 1 and type 2 AIP,particularly in seronegative cases.
基金Supported by The Research Committee of Intractable Pancreatic Diseases (Principal investigatorTooru Shimosegawa) provided by the Ministry of Health, Labour and Welfare of Japan
文摘AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divided 67 AIP patients into diffuse type(D type) and focal type(F type).We further divided F type into head type(H type) and body and/or tail type(B/T type) according to the location of enlargement.Finally,we classified the 67 AIP patients into three groups:D type,H type and B/T type.We compared the three types of AIP in terms of clinical,laboratory,radiological,functional and histological findings and clinical course.RESULTS:There were 34 patients with D-type,19 with H-type and 14 with B/T-type AIP.Although obstructive jaundice was frequently detected in D-typepatients(88%) and H-type patients(68%),no B/T-type patients showed jaundice as an initial symptom(P < 0.001).There were no differences in frequency of abdominal pain,but acute pancreatitis was associated more frequently in B/T-type patients(36%) than in D-type patients(3%)(P = 0.017).Serum immunoglobulin G(IgG)4 levels were significantly higher in D-type patients(median 309 mg/dL) than in B/T-type patients(133.5 mg/dL)(P = 0.042).Serum amylase levels in B/T-type patients(median:114 IU/L) were significantly greater than in H-type patients(72 IU/L)(P = 0.049).Lymphoplasmacytic sclerosing pancreatitis(LPSP) was histologically confirmed in 6 D-type,7 H-type and 4 B/T-type patients;idiopathic duct-centric pancreatitis was observed in no patients.Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients.Steroid therapy was effective in all 50 patients(31 D type,13 H type and 6 B/T type).Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients,no patients relapsed in B/T type.During follow-up,radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION:Clinical features of H-type AIP were similar to those of D-type,but B/T-type differed from D and H types.B/T-type may involve diseases other than LPSP.
文摘Autoimmune pancreatitis(AIP)is defined as pancreatitis caused by irregular narrowing of the pancreatic duct accompanied by pancreatic swelling,fibrosis and lymphocyte infiltration,events that are related to autoimmune mechanisms.The 2010 International Consensus Diagnostic Criteria for AIP defined pancreatitis as“type 1”when increased levels of serum IgG4 were present and other organs were involved;lymphoplasmacytic sclerosing pancreatitis was the main histological characteristic.Apart from surgery,endoscopic ultrasonographyguided fine needle aspiration(EUS-FNA)is the only method for the histological diagnosis of AIP;however,this method is difficult.The use of larger-diameter FNA needles and trucut biopsy did not improve the diagnostic performance of EUS-FNA,but it has improved gradually.In this review,we look back at past efforts to improve the diagnostic performance of EUS-FNA and reveal the present state of EUS-FNA for the histological diagnosis of AIP type 1.
文摘Autoimmune pancreatitis has received considerable attention,especially due to the marked effect of corticosteroid therapy on its clinical course.Knowledge,especially regarding type 1 autoimmune pancreatitis,has significantly increased over the last decades,and despite significant differences in pathophysiology and outcomes,both type 1 and 2 autoimmune pancreatitis are still considered different types of the same disease.Some have proposed a different nomenclature reflecting these differences.Although the term steroid-responsive pancreatitides may be interpreted as synonymous to type 1 and 2 autoimmune pancreatitis,these are not the only pancreatic conditions that show a response to steroid therapy.Acute pancreatitis caused by vasculitis and connective tissue diseases and acute pancreatitis secondary to checkpoint inhibitors or programmed cell death receptor antibody-mediated blockage cancer therapy may also benefit from steroid treatment.This review presents current concepts on these disorders,aiming to increase awareness,analyze similarities and differences,and propose a new nomenclature that reflects their specific particularities,clustering them under the term"steroid-responsive pancreatitides".
文摘Waldenstr?m macroglobulinemia (WM) is a rare lymphoid malignancy. Many studies, including clinicopathological, cytogenetic, gene expression profile, and therapy studies have been reported from the US and Europe, although only a few reports are available from East Asia, including Japan. To further clarify the clinicopathological, radiological, and cytogenetic features of WM in Japan, we performed a retrospective analysis of WM in our institute between March 2007 and January 2012. Clinical data, laboratory data, the results of flow cytometric analysis (FCM), and chromosomal abnormalities were analyzed, and a radiological review was performed. The treatment regimen, response, and survival were also estimated. Six patients were enrolled in this study. The median age was 71 years. All patients were symptomatic, 3 had hyperviscosity syndrome, 1 had bone lesions, and 1 had an extra-medullary mass. FCM data showed that all patients were positive for CD38, while 2 were positive for CD56. Four had chromosomal abnormalities including some abnormalities also reported in myeloma. On radiological review, four showed diffuse invasion of the retro-peritoneum. Five patients received treatment, 4 of which achieved a response. At a median follow-up of 527 days, 4 were alive and 2 died because of disease progression. The present study revealed that WM in Japan might be heterogeneous and have a unique disease manifestation. Invasion sites other than bone marrow were very common, and the results of clinical, FCM, and cytogenetic studies revealed that WM in Japanese cases might have manifestations of both myeloma and B-cell lymphoma.
