Primary CD30+ anaplastic large cell lymphoma: a distinct clinicopathologic entity (Meeting abstract).

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Primary CD30+ anaplastic large cell lymphoma: a distinct clinicopathologic entity (Meeting abstract).

Fifth International Conference on Malignant Lymphoma, June 9-12, 1993, Lugano, Switzerland, p. 24, 1993.. Unique Identifier : AIDSLINE ICDB/95606902
Kadin ME; Beth Israel Hosp.

Abstract: The morphology of anaplastic large cell lymphoma (ALCL) is associated with a clinical syndrome of peripheral lymphadenopathy (greater than 80%) and frequent extranodal disease (greater than 40%) in children and young adults (median age less than 40 yr). Skin lesions occur in more than 20% of patients; other extranodal sites are bone, soft tissue, GI tract, lung and pleura. Marrow involvement is infrequent (less than 10%). Distinguishing features from Hodgkin's disease (HD) are noncontiguous nodal disease (greater than 50%), infrequent mediastinal mass (less than 20%), and common inguinal lymphadenopathy (greater than 40%). Most patients present with stage III/IV disease. Stage is highly predictive of achieving complete remission, disease-free survival, and overall survival. Localized skin lesions have an excellent prognosis and occasional spontaneous regressions are noted. Distinctive histopathologic features of ALCL are partial lymph node involvement, sinus infiltration, sparing of B-cell regions and tumor cell pleomorphism. Other features are high mitotic rate, necrosis, fibrosis, and plasma cell infiltrate. Morphologic variants resemble anaplastic carcinoma, syncytial variant of nodular sclerosing HD, true histiocytic lymphoma/interdigitating cell sarcoma, and mycosis fungoides. ALCL can be distinguished from morphologically similar disorders by immunophenotype (CD30+, CD45+, EMA+, BNH9+, CD15-, keratin-, lysozyme-). A recurrent cytogenetic translocation, t(2;5)(p23;q35) has been observed among morphologic variants and a CD30+ cell line which includes both small cerebriform and large Reed-Sternberg-like cells. 70% of ALCL are T-cell lineage, 15% B, 5% T/B, and 10% undefined. ALCL appears to be distinct from other peripheral T-cell lymphomas such as HTLV-1+ adult T-cell leukemia, angioimmunoblastic lymphadenopathy, angiocentric T-cell lymphoma and cutaneous T-cell lymphoma, occurring mainly in older patients. These combined clinical, pathologic, immunophenotypic and cytogenetic observations support the concept that ALCL is a distinct clinicopathologic entity.

Keywords: Antigens, CD/ANALYSIS Human Immunophenotyping Lymphoma, Large-Cell, Ki-1/GENETICS/IMMUNOLOGY/*PATHOLOGY Translocation (Genetics) ABSTRACT
950430
M9541151

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