CD5‐positive chronic B‐cell lymphoproliferative disorders: Diagnosis and prognosis of a heterogeneous disease entity

• Published in: Cytometry. Part B, Clinical cytometry Clinical cytometry, 2010, Vol.78B (S1), p.S35-S41
• Main Authors: Dronca, Roxana S., Jevremovic, Dragan, Hanson, Curtis A., Rabe, Kari G., Shanafelt, Tait D., Morice, William G., Call, Timothy G., Kay, Neil E., Collins, Charles S., Schwager, Susan M., Slager, Susan L., Zent, Clive S.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; B cell; B-Lymphocytes - immunology; B-Lymphocytes - metabolism; B-Lymphocytes - pathology; Biomarkers - metabolism; CD5; CD5 Antigens - metabolism; chronic lymphoproliferative disorders; CLL; Female; Humans; Leukemia, Lymphocytic, Chronic, B-Cell - diagnosis; Leukemia, Lymphocytic, Chronic, B-Cell - immunology; Leukemia, Lymphocytic, Chronic, B-Cell - metabolism; lymphoma; Lymphoma, B-Cell - diagnosis; Lymphoma, B-Cell - immunology; Lymphoma, B-Cell - metabolism; Lymphoma, B-Cell, Marginal Zone - diagnosis; Lymphoma, B-Cell, Marginal Zone - immunology; Lymphoma, B-Cell, Marginal Zone - metabolism; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - immunology; Lymphoma, Large B-Cell, Diffuse - metabolism; Lymphoproliferative Disorders - diagnosis; Lymphoproliferative Disorders - immunology; Male; Middle Aged; Prognosis; SLL; Waldenstrom Macroglobulinemia - diagnosis; Waldenstrom Macroglobulinemia - immunology; Waldenstrom Macroglobulinemia - metabolism; Young Adult
• ISSN: 1552-4949; 1552-4957
• DOI: 10.1002/cyto.b.20546

Background: The pathology and clinical course of patients with CD5+ chronic B‐cell lymphoproliferative disorders, excluding those that present with typical chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL) or mantle cell lymphoma, (i.e. CD5+B‐CLPD) are poorly defined. Methods: We studied patients with CD5+B‐CLPD to (1) more completely define the clinical features and pathology of CD5+B‐CLPD, (2) compare these features to patients presenting with typical CLL, and (3) test the hypothesis that a subset of patients with CD5+B‐CLPD could have a unique B‐cell malignancy. Results: We identified 229 patients with CD5+B‐CLPD. A definitive pathological diagnosis was made in all 61 (27%) CD5+B‐CLPD patients with nonbone marrow (BM) biopsy specimens considered adequate for a comprehensive pathological examination. The most common diagnosis among these 61 patients was CLL (44%) followed by the leukemic phase of marginal zone lymphoma (34%), lymphoplasmacytic lymphoma (11%), diffuse large B cell lymphoma (8%), and high‐grade B cell lymphoma not otherwise specified (2%). In contrast, among 168 patients without a non‐BM tissue biopsy specimen, a specific diagnosis could be made on review of all available data in only 24 (14%) with 144 (86%) remaining “unclassified.” Conclusions: In patients with CD5+B‐CLPD, a definitive diagnosis can be made on an adequate non‐BM tissue biopsy suggesting that this entity does not include a novel disease. We recommend that all patients with CD5+B‐CLPD should have a non‐BM tissue biopsy to make a definitive diagnosis prior to initiation of treatment. © 2010 International Clinical Cytometry Society