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.

Plasticity in Classical Hodgkin Composite Lymphomas: A Systematic Review

“…Among the remaining 99 articles, 26 were excluded from the final analysis because they met at least one exclusion criterion: (i) six NLPHL/NHL composite lymphomas without cHL contingent [ 27 , 28 , 29 , 30 , 31 , 32 ]; (ii) two cHL with an Epstein–Barr virus (EBV) post-transplant lymphoproliferative disorder [ 33 ] or EBV mucocutaneous ulcer [ 34 ]; (iii) one with CD30 negative Hodgkin cells [ 35 ]; (iv) four with Hodgkin-like cells within an NHL [ 36 , 37 , 38 , 39 ]; (v) two sequential lymphomas [ 40 , 41 ]; (vi) ten probable transformations of CCL into cHL [ 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 ]; and (vii) one because the final diagnosis could be considered ambiguous [ 52 ].…”