TY - JOUR
T1 - B-prolymphocytic leukemia
T2 - Is it time to retire this entity?
AU - El Hussein, Siba
AU - Khoury, Joseph D.
AU - Medeiros, L. Jeffrey
N1 - Publisher Copyright:
© 2021 Elsevier Inc.
PY - 2021/10
Y1 - 2021/10
N2 - B-prolymphocytic leukemia (B-PLL) is included as a distinct entity in the current World Health Organization classification of hematolymphoid neoplasms. However, the diagnosis of B-PLL has presented several challenges since its conception, and over the past decades investigations of B-PLL have revealed substantial biologic and molecular heterogeneity. These data have shown that many B-PLL cases present many similarities with other types of small B-cell lymphomas, and that small B-cell lymphomas can undergo prolymphocytoid transformation. As a result, the frequency of B-PLL has markedly decreased, and currently B-PLL is a very rare entity. Most recent studies focused on B-PLL cases have been conducted on limited cohorts, precluding robust conclusions. In this article, we provide a concise historical review of B-PLL and describe the diagnostic and clinical challenges associated with establishing this diagnosis. We also argue that cases currently classified as B-PLL are unlikely to be a unique biologic entity, but rather represent a state of morphologic transformation characterized by many prolymphocytes that is shared by various types of small B-cell lymphoma.
AB - B-prolymphocytic leukemia (B-PLL) is included as a distinct entity in the current World Health Organization classification of hematolymphoid neoplasms. However, the diagnosis of B-PLL has presented several challenges since its conception, and over the past decades investigations of B-PLL have revealed substantial biologic and molecular heterogeneity. These data have shown that many B-PLL cases present many similarities with other types of small B-cell lymphomas, and that small B-cell lymphomas can undergo prolymphocytoid transformation. As a result, the frequency of B-PLL has markedly decreased, and currently B-PLL is a very rare entity. Most recent studies focused on B-PLL cases have been conducted on limited cohorts, precluding robust conclusions. In this article, we provide a concise historical review of B-PLL and describe the diagnostic and clinical challenges associated with establishing this diagnosis. We also argue that cases currently classified as B-PLL are unlikely to be a unique biologic entity, but rather represent a state of morphologic transformation characterized by many prolymphocytes that is shared by various types of small B-cell lymphoma.
KW - B-PLL
KW - B-prolymphocytic leukemia
KW - Prolymphocytes
KW - Prolymphocytoid
KW - Review
KW - Small B-cell lymphoma
KW - Transformation
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U2 - 10.1016/j.anndiagpath.2021.151790
DO - 10.1016/j.anndiagpath.2021.151790
M3 - Review article
C2 - 34293709
AN - SCOPUS:85110266953
SN - 1092-9134
VL - 54
JO - Annals of Diagnostic Pathology
JF - Annals of Diagnostic Pathology
M1 - 151790
ER -