Prolonged fever of unknown origin and hemophagocytosis evolving into acute lymphoblastic leukemia.

Abstract:

:Hemophagocytic syndrome (HPS) is an unusual acute syndrome presenting with fever, hepatosplenomegaly, and cytopenias. The hallmark of HPS is the accumulation of activated macrophages that engulf hematopoietic cells in the reticuloendothelial system. Most cases of HPS in adults are secondary to infection or malignancy, and thus investigation of the underlying disease is necessary. We describe a patient with prolonged fever, HPS, and chromosomal abnormalities in the bone marrow who underwent thorough evaluation for the cause of his symptoms. A final diagnosis of acute lymphoblastic leukemia (ALL) was established in a fourth, repeated bone marrow biopsy performed more than 2 months after the first presenting symptom appeared. This unusual case demonstrates the importance of cytogenetic abnormalities found in cases of HPS and the importance of repeated testing when an underlying disease is suspected.

journal_name

Am J Hematol

authors

Goldschmidt N,Gural A,Kornberg A,Spectre G,Shopen A,Paltiel O

doi

10.1002/ajh.20123

keywords:

subject

Has Abstract

pub_date

2004-08-01 00:00:00

pages

364-7

issue

4

eissn

0361-8609

issn

1096-8652

journal_volume

76

pub_type

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