Medical and Pediatric Oncology 28:213–215 (1997) Successful M anagement With Interferon Alpha-2a After Prednisone Therapy Failure in an Infant With a Giant Cavernous Hemangioma Manuel A. Castello, MD, 1 * G. Ragni, MD, 1 A. Antimi, MD, 1 A. Todini, MD, 2 G. Patti, MD, 3 R. Lubrano, MD, 1 A. Clerico, MD, 1 and A. Calisti, MD 3 A giant cavernoushemangioma of the left arm subcutaneous infusions of interferon alpha- with severethrombocytopeniaand consumptive 2a.Coagulopathy rapidly improved and transfu- coagulopathy was observed in a neonate. Initial sionsweredrasticallyreduced.Thehemangioma treatment with prednisone, platelet transfusions, regressed progressively and disappeared after 4 and clotting replacement failed to control the months of treatment. Med. Pediatr. Oncol. 28: bleedings. The child wasthen treated with daily 213–215  1997 Wiley-Liss, Inc. Key words: giant cavernoushemangioma; Kasabach-Merritt syndrome; medical therapy INTRODUCTION and prednisone 7.5 mg daily, but no significant result was obtained. Her general conditions worsened: the consump- Hemangiomas are common in infancy and usually fol- tive coagulopathy, thrombocytopenia (platelets 11,000/ low a benign course. The spontaneous regression rate is mm 3 ) and subsequent anemia (Hb 7.1 g/dl) did not sub- 80–85% [1]. Rarely, they can be giant, produce skeletal side. The giant hemangioma and consequent edema did overgrowth or high cardiac output failure, or be associated not regress, and a large hematoma (maximum diameter with severe thrombocytopenia, microangiopathic hemo- 12 cm) was observed over the left shoulder and latero- lytic anemia, and bleeding. This latter association was cervical region. described for the first time in a 2-year-old child by Kasa- Interferon alpha-2a therapy was then initiated bach-Merritt [2] in 1940. (3,000,000 U/m 2 /day subcutaneously). After the first 10 days of treatment, her general conditions rapidly im- CASE REPO RT proved (prothrombin time, 18 sec; partial thromboplastine time, 31 sec; fibrinogen, 156 mg/dl; Hb, 8.2 g/dl; platelets, A 29-year-old woman (gravida 1, para 1) gave birth 12,000 mm 3 ). The number of transfusions decreased from at term to a baby girl weighing 3.050 kg. The Apgar 28, administered during the first month of life, to eight score was 7 after the first minute and 9 after the fifth. during the second. Postpartum examination revealed a giant hemangioma The hemangioma regressed in size, disappearing from extending from the left anterolateral chest wall to the the forearm and chest wall after the first month of treat- middle third of the forearm (Fig. 1). The limb was main- ment (Fig. 2). Transfusions and clotting factors replace- tained in extension, and the overlying skin was bluish ment were discontinued during the second month of life and intact. She exhibited hematomas and widespread pe- when laboratory data revealed progressively increasing tecchiae over her body. Neither hematuria, nor internal values that returned to normal (prothrombin time, 13.1 hemorrhages, resulted. The ECG showed right ventricular sec; partial thromboplastine time, 28 sec; fibrinogen, 250 overload, and the echocardiogram, a mild ventricle en- mg/dl; Hb, 13.5 g/dl; platelets, 253,000/mm 3 ) during the largement. following month (Fig. 3). After the fourth month of life, Laboratory data revealed a severe consumptive coagu- interferon alpha-2a dosage was decreased to 3,000,000 lopathy (hemoglobin 7.2 g/dl, platelets 10,000 mm 3 , pro- U/m 2 every other day. The hemangioma continued to thrombin time 60 sec (n.v. 13–20 sec), partial thrombo- plastin time 35 sec (n.v. 55 sec), fibrinogen undetectable (n.v. 125–300 mg/dl), unconjugated bilirubin 7.2 mg/dl (n.v. 12 mg/dl), Coombs’ tests were negative). Initial 1 Department of Pediatrics, University “La Sapienza,”; 2 Departments of Angiology and 3 Pediatric Surgery, Ospedale San Camillo, Rome, Italy. management consisted of platelet and red blood cell trans- fusions, clotting factors replacement, and corticosteroids *Correspondence to: Manuel A. Castello, M.D., Universita ` degli Studi di Roma “La Sapienza,” Istituto di Clinica Pediatrica, S.S. Oncologia (prednisone, 7.5 mg daily). During the following 2 weeks, Pediatrica, V. le Regina Elena 324, 00161 Rome, Italy. 12 platelet and eight red blood cell transfusions were Received 20 February 1996; accepted 6 May 1996. administered together with clotting factors replacements,  1997 Wiley-Liss, Inc.