Lupus anticoagulant hypoprothrombinemia syndrome associated with severe thrombocytopenia in a child. Issue 6 (15th November 2016)

• Record Type: Journal Article
• Authors: Foord, Aimee; Baca, Nicole; Buchbinder, David; Mahajerin, Arash
• Is Part Of: Pediatric blood & cancer. Volume 64:Issue 6(2017)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2016-11-15
• Subjects: hypoprothrombinemia -- lupus coagulation inhibitor -- pediatrics -- thrombocytopenia; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26357
• Languages: English
• ISSNs: 1545-5009

Abstract

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.