MAY, 1969 GIANT HEMANGIOENDOTHELIOMA WITH THROM- BOCYTOPENIA AND HYPOFIBRINOGENEMIA By OSCAR K. WILLIAMS, M.D., FREDERICK W. VAN BUSKIRK, M.D., STANLEY BURNS, M.D., and R. W. PAUL MELLISH, M.D. BURLINGTON, VERMONT S INCE when the first description of capillary hemangioma with extensive purpura was published by Kasabach and Merritt,5 at least 57 additional case histor- ies have been recorded.7 Most of these re- port patients in whom thrombocytopenia was associated with giant hemangiomata. In addition there have been 5 patients in whom giant hemangiomata were associated with hypofibrinogenemia as well as throm- bocytopenia.’ The mechanisms for thrombocytopenia and hypofibrinogenemia have been dis- cussed in several previous publica- tions.2’3’4’6’8’9 Most commonly the bleeding diathesis has been explained by platelet trapping or intravascular clotting subse- quently followed by fibrinolysis. Treatment has consisted of multiple ap- proaches. Attempts have been made to treat the bleeding diathesis by transfusion of fresh whole blood and fibrinogen, and by administration of epsilon-amino-caproic acid, corticosteroids and anticoagulants. Surgical excision and the use of scierosing solutions or roentgen therapy have been used to ablate the hemangiomata. When this entity was first described by Kasabach and Merritt roentgen therapy was the mo- dality employeti and it resulted in a cure of their patient. It is the purpose of the authors to report a case of giant hemangioendothelioma with thrombocytopenia and hypofibrinogenemia treated by roentgen therapy to a successful end result. REPORT OF A CASE On August 31, 1966 a 17 month old male in- fant was admitted via the emergency room to the DeGoesbriand Memorial Hospital with a bruise on the left side of the thorax and with blood in the diapers. Three weeks prior to ad- mission a small bruise had been noted at the base of the left chest anteriorly which dis- appeared spontaneously. On the evening before admission a much larger bruise appeared in the same area. No history of trauma was elicited. Blood clots were noted in the diapers just prior to bringing the child to the hospital. There was no history of epistaxis, hematemesis, melena or hematuria. There was no past history or family history of abnormal bleeding. Physical examination revealed extensive cc- chymotic areas overlying a palpable, slightly raised subcutaneous soft swelling involving the entire left anterior, lateral and posterior chest wall. There was mottling on both knees and a reticular mottling of the feet, greater on the right than the left. Petechiae were noted on the palate and in the posterior pharynx. Liver and spleen were not palpable. The laboratory findings revealed a hemoglo- bin of io. gm. per ioo ml.; hematocrit 33 per cent; white blood cell count 21,300 per cu. mm. with a normal differential; and a platelet count of 12,ooo per cu. mm. Bone marrow by aspir- ation was normal. Roentgenograms of the chest and abdomen revealed a marked dorsal scoliosis of the lower thoracic and upper lumbar verte- brae. On the third hospital day treatment was started for idiopathic thrombocytopenic pur- pura with prednisone. By the twelfth hospital day the platelet count had risen to 346,000 per cu. mm. The patient was discharged to continue corticosteroids in gradually reducing dosages. He was admitted 2 weeks later because of sudden reappearance of the ecchymoses in pre- cisely the same distribution as on the previous admission. The patient had received no steroid therapy for i week prior to the second admis- slon. Examination at this time revealed a large, flat, soft tissue mass, with overlying ecchy- moses, extending over the left side of the thorax anteriorly, laterally and posteriorly. Petechiae * From the Departments of Radiology, Medicine and Surgery, University of Vermont College of Medicine, Burlington, Vermont. 204 Downloaded from www.ajronline.org by 171.243.65.178 on 05/14/23 from IP address 171.243.65.178. Copyright ARRS. For personal use only; all rights reserved
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