title: Composite prolymphocytoid and hodgkin transformation of chronic lymphocytic leukemia.
abstract: 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.
authors.affiliation: Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St. Louis, MO 63110, USA.
authors.forename: M J
authors.initials: MJ
authors.lastname: O'Sullivan
authors.forename: Z
authors.initials: Z
authors.lastname: Kaleem
authors.forename: M J
authors.initials: MJ
authors.lastname: Bolger
authors.forename: P E
authors.initials: PE
authors.lastname: Swanson
authors.forename: M M
authors.initials: MM
authors.lastname: Zutter
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001761:Bleomycin / Q000008:administration & dosage; D002471:Cell Transformation, Neoplastic / Q000276:immunology / Q000473:pathology*; D003606:Dacarbazine / Q000627:therapeutic use; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D006689:Hodgkin Disease / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008297:Male; D016539:Reed-Sternberg Cells / Q000276:immunology / Q000473:pathology; D014747:Vinblastine / Q000008:administration & dosage
chemical_list: D015703:Antigens, CD; D001761:Bleomycin; D014747:Vinblastine; D003606:Dacarbazine; D004317:Doxorubicin