[Kaposiform haemangioendothelioma associated with B-cell acute lymphoblastic leukemia].
Fiche publication
Date publication
mars 2013
Auteurs
Membres identifiés du Cancéropôle Est :
Dr MUNZER Martine
Tous les auteurs :
Fichel F, Eschard C, Zachar D, Munzer M, Bernard P, Grange F
Lien Pubmed
Résumé
BACKGROUND: Herein, we report the first case of kaposiform haemangioendothelioma (KHE) associated with acute B-lymphoblastic leukemia (B-ALL). PATIENTS AND METHODS: A five-month-old infant presented a plaque of angiomatous appearance on the forearm that had increased in volume since birth, as well as pallor and cutaneous haematomas. Kasabach-Merritt syndrome (KMS) was evoked despite hepatomegaly and considerable splenomegaly. Laboratory tests revealed severe anaemia and thrombocytopenia as well as major hyperleukocytosis with 90% blasts. Skin biopsy revealed vast vascular lobules containing cohesive fusiform endothelial cells not expressing Glut1, bound up in a dense infiltrate of B-lymphoblast cells. It was in fact KHE associated with B-ALL confirmed by the myelogram. The child was treated with the INTERFANT 2006 protocol followed by allograft of haematopoietic stem cells, which resulted in complete haematological remission. At the same time, almost total regression of KHE was noted. DISCUSSION: In this infant, KHE had an inflammatory appearance and was associated with thrombocytopenia, evocative of KMS. Analysis of blood and marrow samples resulted in a diagnosis of B-ALL. Histopathological examination of the angioma revealed a typical appearance of KHE associated with dense lymphoblastic proliferation. This appearance could have resulted either from passive contamination by circulating blast cells or from active recruitment of tumor cells at the KHE site. CONCLUSION: HK mimicking KMS may reveal B-ALL.
Référence
Ann Dermatol Venereol. 2013 Mar;140(3):209-14