ABSTRACT
Anaplastic large cell lymphoma (ALCL) is a subcategory of the mature T-cell neoplasm characterized by sheets of cluster of differentiation (CD)30-positive pleomorphic large cells mostly present as lymphadenopathy. Here, we describe a case of Small cell variant ALCL with leukemic presentation without lymphadenopathy. A 68-year-old male presented with fatigue and weakness; examination revealed a total leukocyte count of 295,000/uL. The peripheral smear showed cells having cerebriform nuclei comprising 90% of the leukocytes. The flow cytometry showed that the cells were immunopositive for CD3 (weak), CD4, CD7, and negative for the rest of the markers. The cell blocks from the peripheral blood showed cells with immunopositivity for CD30, anaplastic lymphoma kinase (ALK), and Epithelial membrane antigen (EMA). A diagnosis of the small cell variant of ALK-positive ALCL was made. Due to the presence of atypical pleomorphic cells without lymphadenopathy, the case has a diagnostic dilemma with differential diagnosis of Sezary syndrome, T-cell prolymphocytic leukemia, and adult T-cell leukemia/lymphoma. Karyotyping and additional immunohistochemistry help for the confirmation of the diagnosis.
ABSTRACT
Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.
Subject(s)
Aged , Female , Humans , Biopsy , Chromogranin A , Cytoplasm , Desmin , Eosinophils , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Magnetic Resonance Imaging , Melanoma , Myoglobin , Neuroectodermal Tumors , Neuroendocrine Tumors , S100 Proteins , Synaptophysin , Ulcer , VimentinABSTRACT
Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.
Subject(s)
Aged , Female , Humans , Biopsy , Chromogranin A , Cytoplasm , Desmin , Eosinophils , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Magnetic Resonance Imaging , Melanoma , Myoglobin , Neuroectodermal Tumors , Neuroendocrine Tumors , S100 Proteins , Synaptophysin , Ulcer , VimentinABSTRACT
T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy that has an aggressive clinical course and it is a distinct clinico-biological entity from other T-cell disorders. It is now apparent that this disease represents a separate entity from CLL. Clinically, T-PLL presents with hepatosplenomegaly, lymphadenopathy, skin lesion, and marked lymphocytosis exceeding 100x109/L. Because its clinical course is aggressive, the treatment is difficult. We report a case of small cell variant of T-cell with a review of literatures.