Background: T-cell prolymphocytic leukemia (T-PLL), formerly categorized as T-cell chronic lymphocytic leukemia, is a rare and aggressive hematologic malignancy. Although the skin is characteristically involved, it is...Background: T-cell prolymphocytic leukemia (T-PLL), formerly categorized as T-cell chronic lymphocytic leukemia, is a rare and aggressive hematologic malignancy. Although the skin is characteristically involved, it is not a well-recognized entity in the dermatologic literature. Methods: Six cases of cutaneous T-PLL are presented from a clinical, light microscopic, and phenotypic perspective. Results: The patient population comprised 2 women and 4 men, with a mean age of 69.8 years. The disease was associated in all with skin involvement with facial preference; edema, purpura, and lesional symmetry were characteristic. The skin biopsies demonstrated a largely non-epidermotropic angiocentric lymphocytic infiltrate with accompanying hemorrhage. The cells showed irregular-to reniform-shaped nuclei with small nucleoli and eosinophilic rims of cytoplasm. Phenotypic studies revealed three prevailing profiles: CD4 dominant in 4, CD8 dominant in one, and co-expression of CD4 and CD8 in one. CD3 loss was seen in one case. All expressed T-cell leukemia 1 (TCL-1) and CD7; cutaneous lymphocyte antigen expression was discernible in a dot-like perinuclear array. All cases tested excluding one expressed TCL-1 and CD52. In two cases tested, T-cell receptor beta rearrangements were observed. Cytogenetic studies demonstrated a paracentromeric chromosome 14 inversion. Polysomy 8 and MYC amplification was seen in one case, manifesting an aggressive clinical course. Four patients died from their disease within 18 months of diagnosis. Limitations: Cytogenetic MYC amplification, FISH, and TCR beta studies were conducted on each of 2 cases, respectively, due to limitations of tissue block samples and/or peripheral blood. cMYC translocation studies were conducted on 3 of the 6 cases, again due to limitations imposed by the tissue samples on the cases. The last case was recently diagnosed and, therefore, long-term follow-up is not possible. Conclusion: T-PLL is a distinctive post thymic T-cell malignancy with frequent cutaneous tropism. A diagnosis is possible in almost all cases based on characteristic clinical, light microscopic, phenotypic, and cytogenetic features. While a chromosome 14 inversion is highly characteristic, additional inherent cytogenetic differences, such as trisomy 8 with CMYC over-amplification, may account for some case to case variation in clinical course.展开更多
Background: The histological diagnosis of malignant melanoma can be challenging. Immunohistochemical techniques may define a critical role in certain cases, specifically in establishing a primary diagnosis of melanoma...Background: The histological diagnosis of malignant melanoma can be challenging. Immunohistochemical techniques may define a critical role in certain cases, specifically in establishing a primary diagnosis of melanoma. CD34 is a hemopoietic stem cell antigen expressed in bone marrow and endothelial cells, and may also be expressed in vascular and spindle cell tumors; it is generally negative in malignant melanoma. Case report: An 83-year-old white female presented with a 3-4 mm area on her right upper back, which had been present for several years. Histologic sections showed a polypoid distortion by sheets and nodules of transformed amelanotic melanocytes lying in intimate apposition to an attenuated epidermis without a concomitant radial growth phase. Tumor cells were extensively S-100 and CD34 positive and showed focal immunoreactivity with melan-A and HMB-45. Discussions: We present a case of malignant melanoma of nodular subtype, which strongly expressed CD34. The spectrum of abnormal phenotypes in malignant melanoma is reviewed, and a possible explanation for the presence of GD34 is discussed. This case demonstrates the potential of malignant melanoma to express CD34, defining an infrequently recognized aberrant phenotype. Whether or not expression of this marker is associated with a more aggressive clinical course remains to be determined.展开更多
Background:Lichenoid and granulomatous dermatitis defines a distinctive pattern of cutaneous inflammation that may be part of the morphologic spectrum of idiopathic lichenoid reactions such as lichen planus and as wel...Background:Lichenoid and granulomatous dermatitis defines a distinctive pattern of cutaneous inflammation that may be part of the morphologic spectrum of idiopathic lichenoid reactions such as lichen planus and as well may be seen with lichenoid drug reactions,endogenous T-cell dyscrasias and as a feature of certain systemic diseases especially Crohn’s disease and rheumatoid arthritis.Results:We encountered three cases of lichenoid and granulomatous dermatitis in which the basis was one of primary cutaneous Mycobacterium infection.In all three cases acid fast stains revealed pathogenic organisms and as well cultures were positive for Mycobacterium kansasii in one case and Mycobacterium marinum in another.Other features included a prominent perineural and periadnexal lymphocytic infiltrate.Conclusions:The differential diagnosis of lichenoid and granulomatous dermatitis should also encompass primary cutaneous Mycobacterium infection in addition to the other more characteristic entities associated with this distinctive reaction pattern.Infection with Mycobacterium induces a TH1 dominant response which would hence produce an infiltrate.展开更多
Background: A reduction in the expression of the pan T-cell markers CD7 and CD62L supports an endogenous T-cell dyscrasia. Previously, clone availability for CD62L restricted its application to frozen tissue sections....Background: A reduction in the expression of the pan T-cell markers CD7 and CD62L supports an endogenous T-cell dyscrasia. Previously, clone availability for CD62L restricted its application to frozen tissue sections. Materials and methods: A nonavidin/biotin technique to examine CD3, CD62L, and CD7 in paraffin formalin-fixed tissue in non-neoplastic and neoplastic T- cell infiltrates. Results: In the reactive group, CD62L manifested a 15 and 22% reduction in epidermal and dermal staining, respectively; there was a 42 and 31% reduction in epidermal and dermal CD7 staining. In lymphomatoid hypersensitivity, CD62L showed a 24 and 9% reduction in epidermal and dermal staining, respectively; CD7 staining demonstrated reduced staining by 70 and 66% in the epidermis and dermis. In the non-lymphomatous endogenous T-cell dyscrasia and lymphoma categories, an 80% diminution in CD62L and CD7 expression was seen. Conclusions: CD62L can be successfully applied in formalin- fixed tissue and exhibits enhanced specificity compared to CD7 in the evaluation of cutaneous T-cell infiltrates. Both CD62L and CD7 in paraffin-embedded, formalin- fixed tissue are useful diagnostic adjuncts, especially in regard to the discrimination of lymphomatoid hypersensitivity reactions from true endogenous T-cell dyscrasia.展开更多
Introduction:Drugs may be an important cause of atypical lymphocytic infiltration.Often times,these infiltrates are in the context of pseudolymphomata.We report a patient who developed lymphocytoma cutis temporally as...Introduction:Drugs may be an important cause of atypical lymphocytic infiltration.Often times,these infiltrates are in the context of pseudolymphomata.We report a patient who developed lymphocytoma cutis temporally associated with initiation of fluoxetine therapy that later went on to develop cutaneous marginal zone B-cell lymphoma.The response of peripheral blood lymphocytes to fluoxetine and other drugs was examined in an attempt to ascertain the potential role for drugs in the propagation of these infiltrates.Materials and Methods:Routine light microscopic analysis and phenotypic studies were performed on tissue obtained from a skin biopsy.Lymphocyte mitogenic studies were carried out using increasing concentrations of fluoxetine,bupropion,and two anticonvulsants.Results:An initial biopsy was consistent with lymphocytomacutis.The patient stopped fluoxetine associated with lesional regression.The lesions recurred despite being off fluoxetine;a repeat biopsy was compatible with marginal zone lymphoma.Lymphocyte proliferation assays revealed a suppressive effect on T-lymphocyte proliferation at physiologic concentrations.Other tested drugs did not have a similar suppressive effect.Conclusion:Fluoxetine may be associated with pseudolymphomata and marginal zone lymphoma.The inhibitory effects on T-lymphocyte function and more specifically T-suppressor function may lead to excessive antigen-driven B-cell proliferation.展开更多
文摘Background: T-cell prolymphocytic leukemia (T-PLL), formerly categorized as T-cell chronic lymphocytic leukemia, is a rare and aggressive hematologic malignancy. Although the skin is characteristically involved, it is not a well-recognized entity in the dermatologic literature. Methods: Six cases of cutaneous T-PLL are presented from a clinical, light microscopic, and phenotypic perspective. Results: The patient population comprised 2 women and 4 men, with a mean age of 69.8 years. The disease was associated in all with skin involvement with facial preference; edema, purpura, and lesional symmetry were characteristic. The skin biopsies demonstrated a largely non-epidermotropic angiocentric lymphocytic infiltrate with accompanying hemorrhage. The cells showed irregular-to reniform-shaped nuclei with small nucleoli and eosinophilic rims of cytoplasm. Phenotypic studies revealed three prevailing profiles: CD4 dominant in 4, CD8 dominant in one, and co-expression of CD4 and CD8 in one. CD3 loss was seen in one case. All expressed T-cell leukemia 1 (TCL-1) and CD7; cutaneous lymphocyte antigen expression was discernible in a dot-like perinuclear array. All cases tested excluding one expressed TCL-1 and CD52. In two cases tested, T-cell receptor beta rearrangements were observed. Cytogenetic studies demonstrated a paracentromeric chromosome 14 inversion. Polysomy 8 and MYC amplification was seen in one case, manifesting an aggressive clinical course. Four patients died from their disease within 18 months of diagnosis. Limitations: Cytogenetic MYC amplification, FISH, and TCR beta studies were conducted on each of 2 cases, respectively, due to limitations of tissue block samples and/or peripheral blood. cMYC translocation studies were conducted on 3 of the 6 cases, again due to limitations imposed by the tissue samples on the cases. The last case was recently diagnosed and, therefore, long-term follow-up is not possible. Conclusion: T-PLL is a distinctive post thymic T-cell malignancy with frequent cutaneous tropism. A diagnosis is possible in almost all cases based on characteristic clinical, light microscopic, phenotypic, and cytogenetic features. While a chromosome 14 inversion is highly characteristic, additional inherent cytogenetic differences, such as trisomy 8 with CMYC over-amplification, may account for some case to case variation in clinical course.
文摘Background: The histological diagnosis of malignant melanoma can be challenging. Immunohistochemical techniques may define a critical role in certain cases, specifically in establishing a primary diagnosis of melanoma. CD34 is a hemopoietic stem cell antigen expressed in bone marrow and endothelial cells, and may also be expressed in vascular and spindle cell tumors; it is generally negative in malignant melanoma. Case report: An 83-year-old white female presented with a 3-4 mm area on her right upper back, which had been present for several years. Histologic sections showed a polypoid distortion by sheets and nodules of transformed amelanotic melanocytes lying in intimate apposition to an attenuated epidermis without a concomitant radial growth phase. Tumor cells were extensively S-100 and CD34 positive and showed focal immunoreactivity with melan-A and HMB-45. Discussions: We present a case of malignant melanoma of nodular subtype, which strongly expressed CD34. The spectrum of abnormal phenotypes in malignant melanoma is reviewed, and a possible explanation for the presence of GD34 is discussed. This case demonstrates the potential of malignant melanoma to express CD34, defining an infrequently recognized aberrant phenotype. Whether or not expression of this marker is associated with a more aggressive clinical course remains to be determined.
文摘Background:Lichenoid and granulomatous dermatitis defines a distinctive pattern of cutaneous inflammation that may be part of the morphologic spectrum of idiopathic lichenoid reactions such as lichen planus and as well may be seen with lichenoid drug reactions,endogenous T-cell dyscrasias and as a feature of certain systemic diseases especially Crohn’s disease and rheumatoid arthritis.Results:We encountered three cases of lichenoid and granulomatous dermatitis in which the basis was one of primary cutaneous Mycobacterium infection.In all three cases acid fast stains revealed pathogenic organisms and as well cultures were positive for Mycobacterium kansasii in one case and Mycobacterium marinum in another.Other features included a prominent perineural and periadnexal lymphocytic infiltrate.Conclusions:The differential diagnosis of lichenoid and granulomatous dermatitis should also encompass primary cutaneous Mycobacterium infection in addition to the other more characteristic entities associated with this distinctive reaction pattern.Infection with Mycobacterium induces a TH1 dominant response which would hence produce an infiltrate.
文摘Background: A reduction in the expression of the pan T-cell markers CD7 and CD62L supports an endogenous T-cell dyscrasia. Previously, clone availability for CD62L restricted its application to frozen tissue sections. Materials and methods: A nonavidin/biotin technique to examine CD3, CD62L, and CD7 in paraffin formalin-fixed tissue in non-neoplastic and neoplastic T- cell infiltrates. Results: In the reactive group, CD62L manifested a 15 and 22% reduction in epidermal and dermal staining, respectively; there was a 42 and 31% reduction in epidermal and dermal CD7 staining. In lymphomatoid hypersensitivity, CD62L showed a 24 and 9% reduction in epidermal and dermal staining, respectively; CD7 staining demonstrated reduced staining by 70 and 66% in the epidermis and dermis. In the non-lymphomatous endogenous T-cell dyscrasia and lymphoma categories, an 80% diminution in CD62L and CD7 expression was seen. Conclusions: CD62L can be successfully applied in formalin- fixed tissue and exhibits enhanced specificity compared to CD7 in the evaluation of cutaneous T-cell infiltrates. Both CD62L and CD7 in paraffin-embedded, formalin- fixed tissue are useful diagnostic adjuncts, especially in regard to the discrimination of lymphomatoid hypersensitivity reactions from true endogenous T-cell dyscrasia.
文摘Introduction:Drugs may be an important cause of atypical lymphocytic infiltration.Often times,these infiltrates are in the context of pseudolymphomata.We report a patient who developed lymphocytoma cutis temporally associated with initiation of fluoxetine therapy that later went on to develop cutaneous marginal zone B-cell lymphoma.The response of peripheral blood lymphocytes to fluoxetine and other drugs was examined in an attempt to ascertain the potential role for drugs in the propagation of these infiltrates.Materials and Methods:Routine light microscopic analysis and phenotypic studies were performed on tissue obtained from a skin biopsy.Lymphocyte mitogenic studies were carried out using increasing concentrations of fluoxetine,bupropion,and two anticonvulsants.Results:An initial biopsy was consistent with lymphocytomacutis.The patient stopped fluoxetine associated with lesional regression.The lesions recurred despite being off fluoxetine;a repeat biopsy was compatible with marginal zone lymphoma.Lymphocyte proliferation assays revealed a suppressive effect on T-lymphocyte proliferation at physiologic concentrations.Other tested drugs did not have a similar suppressive effect.Conclusion:Fluoxetine may be associated with pseudolymphomata and marginal zone lymphoma.The inhibitory effects on T-lymphocyte function and more specifically T-suppressor function may lead to excessive antigen-driven B-cell proliferation.
