title: Lupus anticoagulant hypoprothrombinemia syndrome associated with severe thrombocytopenia in a child.
abstract: Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) comprises lupus anticoagulant, acquired hypoprothrombinemia, and often mild thrombocytopenia or normal platelets. It is usually associated with autoimmunity or postviral illness. We describe a case of a 10-year-old boy with oral bleeding and severe thrombocytopenia initially suggestive of immune thrombocytopenia. Secondary to bleeding, evaluation demonstrated prolonged coagulation tests and subsequently revealed the presence of lupus anticoagulant and hypoprothrombinemia, along with marked autoimmunity, suggestive of LAHPS. He was treated with intravenous immunoglobulin and hydroxychloroquine. This case report and discussion highlight the diagnostic and therapeutic challenges associated with LAHPS and coincident severe thrombocytopenia.
authors.affiliation: Hematology-Oncology Hospitalist Service, Division of Pediatrics, Seattle Children's Hospital, Seattle, Washington.
authors.forename: Aimee
authors.initials: A
authors.lastname: Foord
authors.affiliation: Division of Hematology/Oncology, CHOC Children's Hospital, Orange, California.
authors.forename: Nicole
authors.initials: N
authors.lastname: Baca
authors.affiliation: Division of Hematology/Bone Marrow Transplant, CHOC Children's Hospital, Orange, California.
authors.forename: David
authors.initials: D
authors.lastname: Buchbinder
authors.affiliation: Division of Hematology, CHOC Children's Hospital, Orange, California.
authors.forename: Arash
authors.initials: A
authors.lastname: Mahajerin
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications / Q000628:therapy; D002648:Child; D006801:Humans; D007020:Hypoprothrombinemias / Q000097:blood / Q000150:complications / Q000628:therapy; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D008297:Male; D006472:Oral Hemorrhage / Q000097:blood / Q000209:etiology / Q000628:therapy; D012720:Severity of Illness Index; D013577:Syndrome; D013921:Thrombocytopenia / Q000097:blood / Q000209:etiology / Q000628:therapy
chemical_list: D016682:Lupus Coagulation Inhibitor
keywords: hypoprothrombinemia; lupus coagulation inhibitor; pediatrics; thrombocytopenia