Composite prolymphocytoid and hodgkin transformation of chronic lymphocytic leukemia.

O'Sullivan MJ, Kaleem Z, Bolger MJ, Swanson PE, Zutter MM
Arch Pathol Lab Med. 2000 124 (6): 907-9

PMID: 10835534 · DOI:10.1043/0003-9985(2000)124<0907:CPAHTO>2.0.CO;2

The indolent course of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is occasionally altered by transformation to a histologically distinct, rapidly progressive, and clinically unresponsive hematologic malignant neoplasm. We report a case of CLL that, after 3 years of slowly progressive disease and treatment with single-agent chemotherapy (fludarabine phosphate), underwent a composite prolymphocytoid and classic Hodgkin lymphoma transformation. The diagnosis of classic Hodgkin lymphoma was based on the presence of Reed-Sternberg cells with typical morphologic structure and immunophenotype (CD15(+), CD30(+), CD45(-), CD20(-)) associated with the characteristic polymorphous inflammatory background consisting of numerous eosinophils, plasma cells, and reactive T lymphocytes. The remainder of the lymph node and the peripheral blood showed increased numbers of prolymphocytes admixed with typical small CLL cells. Recognition of such a transformation is of the utmost importance, since histologically similar Reed-Sternberg-like cells may be seen in Richter transformation. In contrast to prolymphocytoid transformation of CLL, Richter syndrome is rapidly fatal, with a median survival of 4 to 5 months. The patient pursued a clinical course similar to pure prolymphocytoid transformation and died with disease after 30 months following treatment with combination chemotherapy.

MeSH Terms (15)

Aged Antigens, CD Antineoplastic Combined Chemotherapy Protocols Bleomycin Cell Transformation, Neoplastic Dacarbazine Doxorubicin Fatal Outcome Hodgkin Disease Humans Immunophenotyping Leukemia, Lymphocytic, Chronic, B-Cell Male Reed-Sternberg Cells Vinblastine

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