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. 2010;78 Suppl 1(Suppl 1):S35-41.
doi: 10.1002/cyto.b.20546.

CD5-positive chronic B-cell lymphoproliferative disorders: diagnosis and prognosis of a heterogeneous disease entity

Roxana S Dronca  1 , Dragan Jevremovic, Curtis A Hanson, Kari G Rabe, Tait D Shanafelt, William G Morice, Timothy G Call, Neil E Kay, Charles S Collins, Susan M Schwager, Susan L Slager, Clive S Zent
Affiliations

CD5-positive chronic B-cell lymphoproliferative disorders: diagnosis and prognosis of a heterogeneous disease entity

Roxana S Dronca et al. Cytometry B Clin Cytom. 2010.

Abstract

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.

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Figures

Figure 1
Figure 1
Selection of patients of CD5+ B cell chronic lymphoproliferative disorder (CD5+B-CLPD) study subgroups. This flow diagram summarizes the methods of selection of the patient cohorts reported in this study. CLL: chronic lymphocytic leukemia/small lymphocytic lymphoma, MCL: mantle cell lymphoma, MCR: Mayo Clinic Rochester, FC: flow cytometry, BM: bone marrow, MBL: monoclonal B cell lymphocytosis.
Figure 2
Figure 2
Time to treatment (A) and overall survival (B) for CD5+B-CLPD patients and unclassified (non-MBL) CD5+ B-CLPD patients compared to CLL patients. Statistically significant differences exist between CD5+ B-CLPD patients and CLL patients for age-adjusted time to treatment (p=0.004) and age-adjusted survival (p<0.001), but the differences do not exist between unclassified (non-MBL) CD5+ B-CLPD patients and CLL patients (time to treatment p=0.49; age-adjusted survival p=0.15)
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