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.
[Show abstract][Hide abstract] ABSTRACT: Primary larynx lymphomas, specifically of the mucosa-associated lymphoid tissue, are a rare but documented phenomenon. Transformation of any type of lymphoma that has the presence of Reed-Sternberg cells is unusual in lymph nodes and exceptional in extranodal sites. Herein, we report the first case (to the best of our knowledge in a review of the English literature [MEDLINE 1966-2001]) in which both of these unusual findings are present; that is, an extranodal marginal zone B-cell lymphoma of laryngeal mucosa-associated lymphoid tissue with Hodgkin-like transformation. The patient is a 78-year-old man who presented with intermittent shortness of breath, progressive dysphagia, and intermittent hoarseness. On examination, a large mass of the left supraglottic larynx was identified with a "ball-valve" effect into the laryngeal inlet with inspiration. Examination of the neck showed no palpable masses. Histologic examination of the incisional biopsy showed replacement of the submucosa by sheets of atypical monocytoid B cells (CD20+, CD79a+, lambda+, CD3-) characterized by nuclear atypia, mitotic activity, plasmacytoid differentiation, and restricted for lambda light chains. Dutcher bodies were easily identified. Interspersed throughout the neoplastic lymphoid population were numerous Reed-Sternberg cells and variants immunoreactive for CD30 and CD15 and nonreactive for CD45RB. The patient was treated with 44 cGy to the neck and larynx and was alive and free of disease at last contact, 2.6 years after the original presentation.
[Show abstract][Hide abstract] ABSTRACT: We describe a case of B-prolymphocytic leukaemia (B-PLL) who, following a long-lasting remission with fluradabine, developed a Hodgkin's lymphoma (HL) with bone marrow involvement. A 75-yr-old male was found to have a lymphocytosis [white blood cell (WBC) count = 146 x 10(9) L(-1)], small volume axillary lymphadenopathy, and hepatosplenomegaly. The majority of circulating lymphocytes had a round nucleus and prominent single nucleolus. The patient did not respond to chlorambucil and then received fludarabine, achieving a good response lasting for 5 yr, when he manifested with B symptoms and pancytopenia. A diagnosis of HL in the bone marrow was made based on histology (Reed-Sternberg cells) and immunohistochemistry (CD30+, CD15+). Epstein-Barr virus (EBV) studies were negative. The patient was treated with chemotherapy but died 6 months later from disease progression.
European Journal Of Haematology 10/2002; 69(3):182-4. DOI:10.1034/j.1600-0609.2002.02758.x · 2.07 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Malignant lymphomas have traditionally been classified as either Hodgkin's (HL) or non-Hodgkin's lymphomas (NHL). The tumor cells in NHL have been identified as being derived from T- or B-lymphocytes, but not until recently was it possible to identify the origin of the tumor cells in HL. Differential diagnostic problems may exist with morphological overlaps between HL and NHL but do not reflect an underlying biological relationship. Recent observations indicate that HL and NHL may be more closely related than previously believed. HL and NHL may occur sequentially or simultaneously in one and the same individual and in a few cases a clonal relationship between the lymphomas has been proven. It is thus possible that a biological relationship between the disorders may exist in addition to morphological overlaps, and the purpose with this review is to summarize the different relations between HL and NHL.
Medical Oncology 02/2003; 20(3):211-20. DOI:10.1385/MO:20:3:211 · 2.63 Impact Factor
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