Blue Rubber Bleb Nevus Syndrome with Disseminated Intravascular Coagulation and Thrombocytopenia: Successful Treatment with High-Dose Intravenous Gammaglobulin

• Published in: The Tohoku Journal of Experimental Medicine Vol. 163; no. 2; pp. 111 - 117
• Main Authors: AIHARA, MORIO, KONUMA, YUZURU, OKAWA, KEIZO, KOMAI, RYUKO, KUDO, IKUO, MORIOKA, RITSUYO, KARIYA, KATSUTOSI, TAKAMI, HIDEKI, SAWADA, YOSHIHIKO, MUNAKATA, AKIHIRO, YOSHIDA, YUTAKA
• Format: Journal Article
• Language: English
• Published: Japan Tohoku University Medical Press 01-02-1991
• Subjects: blue rubber bleb nevus syndrome; disseminated intravascular coagulation; Disseminated Intravascular Coagulation - etiology; Disseminated Intravascular Coagulation - therapy; Female; Femoral Fractures - complications; Hemangioma - complications; Hemangioma - congenital; Humans; Immunization, Passive; Injections, Intravenous; Middle Aged; Nevus - complications; Nevus - congenital; Skin Neoplasms - complications; Skin Neoplasms - congenital; Syndrome; Thrombocytopenia - etiology; Thrombocytopenia - therapy; thrombocytopenia and high-dose gammaglobulin
• ISSN: 0040-8727; 1349-3329
• DOI: 10.1620/tjem.163.111

AIHARA, M., KONUMA, Y., OKAWA, K., KOMAI, R., KUDO, I., MORIOKA, R., KARIYA, K., TAKAMI, H., SADWADA, Y., MUNAKATA, A. and YOSHIDA, Y. Blue Rubber Bleb Nevus Syndrome with Disseminated Intravascular Coagulation and Thrombocytopenia: Successful Treatment wiith High-Dose Intravenous Gamaglobulin. Tohoku J. Exp. Med., 1991, 163 (2), 111-117 - A 63-year-old woman was diagnosed as having blue rubber bleb nevus syndrome (BRBNS) with disseminated intravascular coagulation (DIC). Hematological data showed typical DIC: PT 13.2sec, activated PTT 55.3sec, fibrinogen 20mg/100ml, FDP-E 928ng/ml, D-dimer 3, 477ng/ml, platelet count 25×103/μl. Although hypofibrinogenemia was successfully controlled by the continuous infusion of heparin, 10, 000 units/day, thrombocytopenia has continued. Based on shortened platelet life span, high level of platelet associated IgG, and increased number of megakaryocyte in the bone marrow, the thrombocytopenia was thought to be due to antiplatelet antibody. Her platelet count returned to normal after intravenous infusion of high-dose gamma globulin (IVIg, Sandoz) at the dose of 400mg/kg for 2-5 days, while corticosteroid, Gabexate mesilate, synthetic thrombin inhibitor MD-805, urinastatin and warfarin had no effect. Thus, DIC or thrombocytopenia may become a serious complication in some patients with BRBNS and IVIg may be useful for correcting thrombocytopenia in the patient.