title: A severe bleeding diathesis in a 6-year-old girl secondary to a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis.
abstract: A 6-year-old girl presented with presumed relapse of childhood immune thrombocytopenia. Investigations revealed deranged coagulation parameters, abnormal small bowel thickening, and splenomegaly. A clinically significant bleeding diathesis emerged which was refractory to most hemostatic interventions. Laparatomy revealed a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis. Splenectomy resulted in complete resolution of the coagulopathy. The diagnosis and management of these conditions is inherently complex and without clear guidance. We discuss our perioperative management of the bleeding diathesis. There is a need for long-term follow-up of the underlying pathologies particularly as potentially useful therapeutic agents have emerged.
authors.affiliation: *Department of Haematology, Belfast City Hospital †Royal Belfast Hospital for Sick Children, Belfast, Northern Ireland, UK ‡Our Lady's Children's Hospital, Crumlin, Dublin, Republic of Ireland.
authors.forename: Patrick T
authors.initials: PT
authors.lastname: Elder
authors.forename: Carole
authors.initials: C
authors.lastname: Cairns
authors.forename: Alistair
authors.initials: A
authors.lastname: Dick
authors.forename: William A
authors.initials: WA
authors.lastname: McCallion
authors.forename: Anthony
authors.initials: A
authors.lastname: McCarthy
authors.forename: Owen P
authors.initials: OP
authors.lastname: Smith
authors.forename: Christine
authors.initials: C
authors.lastname: Macartney
mesh_terms: D002648:Child; D005260:Female; D006391:Hemangioma / Q000150:complications* / Q000175:diagnosis; D006474:Hemorrhagic Disorders / Q000209:etiology* / Q000601:surgery; D006801:Humans; D007421:Intestine, Small / Q000473:pathology*; D007813:Laparotomy; D008202:Lymphangioma / Q000150:complications* / Q000175:diagnosis; D011379:Prognosis; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000150:complications* / Q000175:diagnosis; D013921:Thrombocytopenia / Q000209:etiology* / Q000601:surgery