title: [Kaposiform haemangioendothelioma associated with B-cell acute lymphoblastic leukemia].
abstract: BACKGROUND
  Herein, we report the first case of kaposiform haemangioendothelioma (KHE) associated with acute B-lymphoblastic leukemia (B-ALL).
  
  
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  HK mimicking KMS may reveal B-ALL.
authors: Service de dermatologie, hôpital Robert-Debré, CHU de Reims, avenue du Général-Koenig, 51092 Reims cedex, France. fanny-fichel@hotmail.fr
authors: F
authors: F
authors: Fichel
authors: C
authors: C
authors: Eschard
authors: D
authors: D
authors: Zachar
authors: M
authors: M
authors: Munzer
authors: P
authors: P
authors: Bernard
authors: F
authors: F
authors: Grange
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor; D001706:Biopsy; D003131:Combined Modality Therapy; D036101:Cord Blood Stem Cell Transplantation; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D003951:Diagnostic Errors; D006390:Hemangioendothelioma / Q000150:complications / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology; D006391:Hemangioma / Q000151:congenital / Q000175:diagnosis; D006801:Humans; D007231:Infant, Newborn; D059885:Kasabach-Merritt Syndrome / Q000209:etiology*; D015448:Leukemia, B-Cell / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D009378:Neoplasms, Multiple Primary / Q000473:pathology* / Q000628:therapy; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D011239:Prednisolone / Q000008:administration & dosage; D012074:Remission Induction; D012878:Skin Neoplasms / Q000150:complications / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology; D014184:Transplantation, Homologous
chemical_list: D014408:Biomarkers, Tumor; D003561:Cytarabine; D003520:Cyclophosphamide; D011239:Prednisolone; D015122:Mercaptopurine; D008727:Methotrexate