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 Blood, 1 October 2008, Vol. 112, No. 7, pp. 2965-2968.
Prepublished online as a Blood First Edition Paper on July 25, 2008; DOI 10.1182/blood-2008-03-147017.

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NEOPLASIA

Brief Report

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

John K. C. Chan1, Laurence Lamant2, Elizabeth Algar3,4, Georges Delsol2, William Y. W. Tsang1, King C. Lee1, Karin Tiedemann4, and Chung W. Chow3,5

1 Department of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong; 2 Inserm, U563, Centre de Physiopathologie de Toulouse Purpan, and Université Toulouse III Paul Sabatier, Toulouse, France; 3 Department of Pediatrics, University of Melbourne, Australia; and 4 Children's Cancer Center and 5 Department of Anatomical Pathology, Royal Children's Hospital, Melbourne, Australia

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.


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