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ALK+ histiocytosis

Thursday 6 November 2008

See also

- histiocytoses
- ALK immunoreactivity

Clinical synopsis

- pallor
- massive hepatosplenomegaly
- anemia
- thrombocytopenia

Microscopical synopsis

- Liver: 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. (18660380)

- Cutaneous infiltrates morphologically resembling juvenile xanthogranuloma.

- Histiocytes 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. (18660380)

Molecular biology

- One case out of 3 with TPM3-ALK fusion gene (18660380)

References

- Chan JK, Lamant L, Algar E, Delsol G, Tsang WY, Lee KC, Tiedemann K, Chow CW. ALK+ histiocytosis: a novel type of systemic histiocytic proliferative disorder of early infancy. Blood. 2008 Oct 1;112(7):2965-8. PMID: 18660380