Acute transformation of chronic large granular lymphocyte leukemia into an aggressive form associated with preferential organ involvement.

Abstract:

:In most patients with large granular lymphocyte (LGL) leukemia, the disease appears to progress slowly if at all, and no therapy is generally required. We present a patient with CD3+ CD8+ CD16+ LGL leukemia, who showed a benign clinical course for more than 7 months without therapy, but subsequently developed aggressive disease. A feature of considerable interest was the transformation into an acute or aggressive form in this patient affected preferentially the liver and spleen, and was not associated with the hematologic features of the circulating leukemic cells, as assessed by morphologic, phenotypic and molecular analyses. The patient was treated with combination chemotherapy; this resulted in clinical remission with persistence of the abnormal clone in the peripheral blood. The mechanism responsible for the preferential organ involvement and the process of progression from the chronic to the acute form in this case are discussed from the viewpoint of a lymphoproliferative disorder.

journal_name

Acta Haematol

journal_title

Acta haematologica

authors

Matsubara A,Matsumoto M,Takada K,Hato T,Hasegawa H,Tamai T,Yasukawa M,Fujita S

doi

10.1159/000204336

subject

Has Abstract

pub_date

1994-01-01 00:00:00

pages

206-10

issue

4

eissn

0001-5792

issn

1421-9662

journal_volume

91

pub_type

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