Two cases showing clonal progression with full evolution from aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome to myelodysplastic syndromes and leukemia.

Abstract:

:We report 2 paroxysmal nocturnal hemoglobinuria (PNH) patients who were initially diagnosed with aplastic anemia and sequentially developed PNH, myelodysplastic syndromes (MDS), and leukemia. Flow cytometry and cytogenetic analysis showed the initial appearance and expansion of PNH clones, gradual replacement of PNH clones by MDS clones with monosomy 7, and then expansion of MDS clones or their subclones with additional chromosomal abnormalities. In relation to these developments, expression increased of the Wilms' tumor gene WT1, a marker for leukemic progression. These patients not only shared bone marrow failure but also might have harbored a hematopoietic environment favorable for the emergence of abnormal clones leading to leukemogenesis.

journal_name

Int J Hematol

authors

Ishihara S,Nakakuma H,Kawaguchi T,Nagakura S,Horikawa K,Hidaka M,Asou N,Mitsuya H

subject

Has Abstract

pub_date

2000-08-01 00:00:00

pages

206-9

issue

2

eissn

0925-5710

issn

1865-3774

journal_volume

72

pub_type

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