Diagnostic features and survival in typical and prolymphocytoid variants of chronic lymphocytic leukemia

Abstract

Diagnostic features were evaluated with regards to survival in 203 cases of typical and prolymphocytoid chronic lymphocytic leukemia. Excluding 27 (13 per cent) patients with second malignancies, survival analyses indicated that the prognostic factors with greatest significance were age at presentation (less than 65 years and 65 years or more: p = 0.0004), proportions of prolymphocytoid cells (less than 10 per cent and 10 per cent or more: p less than 0.0001 age corrected) and surface immunoglobulin (SIg) density (weak and more than weak: p = 0.001 age corrected). In contrast, sex, absolute numbers of prolymphocytoid cells, SIg light chain type and FMC7 expression were not prognostically significant. These observations suggest that the recognition and delineation of prolymphocytoid CLL variants by morphological and immunophenotypic criteria, although important in diagnostic terms, may have less relevance with respect to prognosis and patient management.

Chronic Lymphocytic Leukemia: Diagnosis and Treatment

“…Transformation of CLL to an aggressive form of disease, either PLL (with >55% circulating prolymphocytes) or Richter syndrome (RS) (large cell lymphoma or immunoblastic lymphoma), occurs in a small proportion of patients. [274][275][276][277][278][279][280][281][282] Transformation to acute lymphocytic leukemia, Hodgkin lymphoma, and multiple myeloma has also been described. 282 Richter Syndrome.…”

“…Prolymphocytic Leukemia. Because of the relative rarity of cases, [275][276][277][278][279][280][281] discrepancies in the percentage of circulat-ing prolymphocytes required for a diagnosis of PLL transformation, and lack of serial blood work available for assessment, 277,278,281 data are extremely limited with respect to not only the incidence of PLL transformation but also the median time to transformation, OS, and optimal therapy. [277][278][279][280] In a small series of patients with typical CLL who had serial blood films available for review, most had minor variations or transient increases in the percentage of circulating prolymphocytes, and one third had a steady increase in the proportion of prolymphocytes (but always <55%).…”