Acute leukemia with a pure erythroid phenotype: underrecognized morphologic and cytogenetic signatures universally associated with primary refractory disease and a dismal clinical outcome.

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Acute leukemia with a pure erythroid phenotype: underrecognized morphologic and cytogenetic signatures universally associated with primary refractory disease and a dismal clinical outcome.

Histopathology. 2017 Mar 06;:

Authors: Park DC, Ozkaya N, Lovitch SB

Abstract
AIMS: Pure erythroid leukemia (PEL) is a very rare and aggressive subtype of acute myeloid leukemia defined by the World Health Organization (WHO) as a neoplastic proliferation of immature cells committed exclusively to the erythroid lineage comprising >80% of bone marrow cells and not meeting criteria of other well-defined myeloid neoplasms. The aim of this study was to describe the clinicopathologic features of acute leukemias with a pure erythroid phenotype (ALPEP) irrespective of their WHO classification and determine if ALPEP represents a distinct clinicopathologic entity.
METHODS AND RESULTS: We identified 7 cases of ALPEP in which immature cells fulfilled WHO morphologic and immunophenotypic criteria for PEL. All patients except for one were male with a median age of 60 years. Three cases represented de novo PEL, 3 were therapy-related myeloid neoplasms, and 1 was a blast phase of a myeloproliferative neoplasm. Extensive tumor necrosis was present in 5 cases (71%). Five cases with available modal karyotypes all demonstrated a complex karyotype involving the TP53 gene locus with 3 cases (60%) also showing a monosomy 5 or deletion 5q and additional material on chromosome 19q13. All patients died of their disease with a mean overall survival of 189 days and 64.7 days in cases without and with necrosis on the initial biopsy, respectively.
CONCLUSIONS: We describe previously unreported but relatively common findings of extensive tumor necrosis and recurring cytogenetic abnormalities in ALPEP. Our findings strongly suggest that ALPEP represents a distinct clinicopathologic entity regardless of its WHO classification. This article is protected by copyright. All rights reserved.

PMID: 28261852 [PubMed - as supplied by publisher]

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