ALK+ histiocytosis: a novel type of systemic histiocytic proliferative disorder of early infancy.

Abstract:

:We report 3 cases of a previously uncharacterized form of histiocytosis presenting in early infancy and showing ALK immunoreactivity. The patients presented with pallor, massive hepatosplenomegaly, anemia, and thrombocytopenia. Liver biopsy showed infiltration of the sinusoids by large histiocytes with markedly folded nuclei, fine chromatin, small nucleoli, and voluminous lightly eosinophilic cytoplasm that sometimes was vacuolated or contained phagocytosed blood cells. One patient developed cutaneous infiltrates that morphologically resembled juvenile xanthogranuloma. The histiocytes were immunoreactive for histiocytic markers (CD68, CD163, lysozyme), S100 protein, ALK (membranous and cytoplasmic pattern), and dendritic cell markers (fascin, factor XIIIa), but not CD1a and langerin. One case successfully analyzed by molecular techniques revealed TPM3-ALK fusion. Thus the spectrum of diseases exhibiting ALK translocation should be expanded to include ALK(+) histiocytosis. The disease in the 3 patients (2 having been given chemotherapy) resolved slowly over many months.

journal_name

Blood

journal_title

Blood

authors

Chan JK,Lamant L,Algar E,Delsol G,Tsang WY,Lee KC,Tiedemann K,Chow CW

doi

10.1182/blood-2008-03-147017

subject

Has Abstract

pub_date

2008-10-01 00:00:00

pages

2965-8

issue

7

eissn

0006-4971

issn

1528-0020

pii

blood-2008-03-147017

journal_volume

112

pub_type

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