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Studies are presented on a 20-year-old man with congenital giant hemangioma occupying the whole left side of the body, with alterations in the clotting system characteristic of a consumption-coagulopathy with “defensive” fibrinolysis. There were also changes in red cell morphology, consiste

An infant with hemangioma-thrombocytopenia syndrome who was successfully treated with x-irradiation over the hemangioma is described. Studies showed suggestive platelet sequestration in the tumor (as well as spleen), decreased platelet survival, and red cell changes compatible with cell trauma. Speculations concerning the mechanism of the thrombocytopenia are discussed. It is suggested that careful attention should be given to red cell morphology in cases of giant hemangioma in children, particularly those associated with thrombocytopenia.

Summary A case of giant splenic hemangioma associated with thrombocytopenia and fibrinogen deficiency is described. The case presented clinically with severe bleeding and splenomegaly. Treatment with prednisone and transfusions of fresh blood and fibrinogen, was unsuccessful in controlling the hemorrhage. Following splenectomy, the patient improved considerably, the bleeding tendency disappeared and the blood findings became normal. The relationship between giant heman-giomata, either visceral or cutaneous, and the disturbed mechanisms of blood clotting is discussed.

Summary The fifteenth case of giant hemangioma with associated thrombocytopenic purpura is presented. This patient was successfully treated with radiation, prednisone, and whole blood transfusions. Although the ultimate cure of this disorder resulted from the destruction of the tumor by radiation, the effect of the use of prednisone on this patient was lifesaving in preventing the destruction of platelets during the interim between radiation and the effects of radiation on the hemangioendothelioma. This is the first reported case in which this effect of prednisone has been noted with this syndrome.

An eight year old boy with the Kasabach-Merritt syndrome was studied with respect to consumption coagulopathy, which was demonstrated by simultaneous coagulation studies performed on venous and hemangioma blood. Venous blood samples showed only thrombocytopenia, moderate fibrinogenopenia and factor IX deficiency; whereas hemangioma blood samples revealed prolonged recalcification, one-stage prothrombin, cephalin-Stypven, ® thrombin, calcified thrombin, P and P and partial thromboplastin times. There was a plasma defect in the thromboplastin generation test. Besides thrombocytopenia and a more pronounced fibrinogenopenia, deficiencies of factors V, VIII and IX and an increase in the fibrinolytic activity were found. The factor IX deficiency was considered to be a coincidental finding. The patient also presented signs of microangiopathic hemolytic anemia, thought to be related to the giant hemangioma.

SUMMARY Description of a case of large hemangioma with thrombocytopenia, hemorrhage, and anemia, reacting well to X-ray treatment. Forty-nine cases from the literature are reviewed. These show-great variation in the localisation and histological findings, clinical course and type of treatment. This disease should not be regarded as harmless in view of the possibility of serious hemorrhages and the not negligible chance of underlying malignancy. An acceptable concept, combining regression of the tumor and simultaneous increase in platelet count, is not known. Until now, radiation treatment and surgical removal of the tumor have been the only successful methods of treatment. Where possible, surgical treatment is to be preferred, since high doses of radiation seem to be required and relapses may follow. In some cases spontaneous regression appears to occur. Fibrinogenopenia of not fully understood etiology has been noted in some cases.

A case of giant hemangioma with thrombocytopenia has been presented. Isotopic studies with Cr-51 tagged platelets indicated that the basic mechanism of thrombocytopenia in this patient involved platelet sequestration in the tumor. The platelet response in the peripheral blood following irradiation of the hemangioma gives additional evidence that the thrombocytopenia is related to pathological changes within the hemangioma.

The importance of recognizing disseminated intravascular coagulation as the cause of severe bleeding diathesis in a patient with giant hemangioma is illustrated. A sixteen year old boy with multiple giant cavernous hemangiomas had massive local bleeding after each of the three times surgical excision of the hemangiomas was carried out. Detailed laboratory studies performed on the third occasion provided evidence of disseminated intravascular coagulation as shown by the decreased platelet counts and plasma levels of fibrinogen and factors V and VIII, the prolongation of prothrombin time, partial thromboplastin time, thrombin time, and arvin time, the presence of fibrinogen degradation products, and the increased fibrinolytic activity. Cessation of bleeding accompanied by a return of the laboratory values to within normal limits occurred after correction of the disseminated intravascular coagulation with heparin and acetylsalicylic acid. The use of epsilon aminocaproic acid is contraindicated in this situation.

thrombocytopenia in the newborn infant treated by total excision, J. PEDIAT. 59" 238, 1961. 4. James, D. H., and Tuttle, A. H.: Congenital hemangioma with thrombocytopenia, J. PEDIAT. 59: 234, 1961. 5. Katz, H. P., and Askin, J.: Multiple hemangiomata with thrombocytopenia, Amer. J. Dis. Child. 115: 351, 1967. 6. Raphan, H.: Multiple hemangiomas of the skin, liver and intestinal tract, Helv. Paediat. Acta h 56, 1966. 7. Sutherland, D. A., and Clark, H.: Hemangioma associated with thrombocytopenia, Amer. J. Med. 33: t50, 1962. 8. Weissman, J., and Tagnon, H. J.: Syndrome of hemangioma and thromboeytopenic purpura in infants, Arch. Intern. Med. 92: 523, 1953. 9. Wilson, C. H., and Haggard, M. E.: Giant vascular tumors and thrombocytopenia, Arch. Derm. 81: 432, 1960. 10. Kasabach, H. H., and Merritt, K. K.: Capillary hemangioma with extensive purpura: Report of a case, Amer. J. Dis. Child. 59: 1063, 1940. 11. Thatcher, L. G., Clatanoff, D. V., and Stiehm, E. R.: Splenic hemangioma with thrombocytopenia and afibrinogenemia, J. PEDIAT. 73: 345, 1968. 12. Zervos, N., Vlachos, J., Karpathios~ T., and Mantas, J.: Giant hemangioma of the spleen with thrombocytopenia and fibrinogen deficiency, Acta Paediat. Scand. 172: 206, 1967. (Suppl.) 13. Berendes, H.: The structure and scope of the collaborative project on cerebral palsy, mental retardation and other neurological and sensory disorders of infancy and childhood, in Chipman, S. S., Lilienfeld, A. M., Greenberg, B. G., and Donnelly, J. F., editors: Research methodology and needs in perinatal studies, Springfield, Illinois, 1966, Charles C Thomas, Publisher, pp. 118-138. 14. Froehlich, L. A., Fujikura, T., and Fisher, P.: Chorioangiomas and their clinical implications. In press. 15. Siddall, R. S.: Chorioangiofibroma (Chorioangioma), Amer. J. Obstet. Gynec. 8: 430, 1924. I6. Benson, P. F., and Joseph, M. C.: Cardiomegaly in a newborn due to placental chorioangioma, Brit. Med. J. h 102, 1961. 17. Wolman, I. J.: Thrombocytopenic purpura of the newborn, in Laboratory applications in clinical pediatrics, New York, 1957, McGraw-Hill Book Company, Inc., pp. 283284. 18. Rodriguez, S. A., Leikin, S. L., and Hiller, M. C.: Neonatal thrombocytopenia associated with ante-partum administration of thiazide drugs, New Eng. J. Med. 270: 881, 1964.

To the Editor:— The report of Drs. Richardson and Smith ( 203 :518, 1968) of successful heparin sodium therapy for a pregnant woman with thrombotic thrombocytopenia purpura (TTP) prompts this letter, suggesting the feasibility of heparin therapy for a different thrombocytopenic syndrome. Thrombocytopenia is an uncommon, but serious, sometimes lethal complication of giant hemangiomas of infancy. Since hemangiomas are malformations that as a rule involute spontaneously, no treatment is indicated. However, when thrombocytopenia or other deficits of coagulation factors occur, active treatment is required, consisting of local radiation, which usually results in rapid clinical improvement and eventual restoration of blood coagulation factors. Irradiation of infants is always cause for concern, however, so that alternative treatments ought to be weighed. Heparin therapy is promising for two reasons: (1) Heparin has been proven effective for the microangiopathies of which TTP is an example. 1 (2) Giant hemangioma-thrombocytopenia syndrome may also be considered one

The management of the giant and moderate size infantile haemangiomas are challenging problems, especially in health systems with limited resources in developing countries. The aim of presenting this case is to take a lesson from another discipline’s decision in managing haemangioma.The author provide information based on clinical examination and surgical records of the patient with giant hemangioma which was consulted to plastic surgery team. A four month year old boy was consulted by pediatric surgery team with a giant size haemangioma on the right hemithorax.The treatment option for each haemangioma are different based on the case itself. Especially for this case, it seems better to be treated conservatively due to several reason, such as the phase of hemangioma, the location of the mass, the size, the donor morbidity. Early surgical excision of a moderate size infantile haemangioma may be justified especially when there is dificulty of follow-up. This approach will prevent growth deformation, impact on nearby vital organs and psychological problems.

Reversible changes in the gastrointestinal tract of adults due to bleeding and edema have been described clinically (11, 14) and produced experimentally (11). It is not unexpected that examples should be found in the pediatric age group of diseases of diverse etiology with common roentgen manifestations, relating to hemorrhage, ischemia, edema, and necrosis with return to normal. Schonlein-Henoch purpura, giant hemangioma with platelet trapping, classic hemophilia, and postoperative ischemia of a colonic transplant for reconstructing the esophagus in esophageal atresia have all presented with signs of gastrointestinal bleeding and examples of “thumbprinting” and “pseudotumor” defects on barium studies. Schonlein-Henoch purpura (nonthrombocytopenic purpura) In children with Schonlein-Henoch nonthrombocytopenia or anaphylactoid purpura, swollen painful joints, cutaneous purpura, and abdominal pain with associated bleeding from the intestinal tract are characteristic. The abdominal pain, often colicky, may p...

Thrombocytopenia associated with giant hemangioma in the infant produces a disorder featured by hemorrhagic tendency and the occurrence of bleeding into the hemangioma site. Hemorrhagic manifestations in these patients are frequently severe enough to threaten life, and infants have died of this disease. Weissman and Tagnon 1 recently pointed out that enough cases of thrombocytopenia occurring in conjunction with large hemangiomas have been reported to justify considering the simultaneous occurrence of these disorders in the infant as a clinical syndrome. In spite of the established clinical association, the biologic relationship between the thrombopenia and hemangioma remains obscure, and some authors still consider the simultaneous occurrence of these disorders to be coincidental. It is the purpose of this paper to summarize reported experience with cases of thrombocytopenia and hemangioma and to report three additional cases of our own. A review of the available literature indicates that a relationship between the hemangioma

Cases of giant hemangioma in infancy complicated by thrombocytopenia and bleeding have been reported in the literature.1-9The purpuric manifestations are frequently mild and intermittent, although serious intracranial and visceral hemorrhages have been encountered. Recovery follows surgical removal, radiation therapy, or, rarely, spontaneous regression. Mechanical sequestration of platelets in the vascular channels of the hemangioma is believed to be the mechanism of thrombocytopenia which results in bleeding.2 This report describes a case of hemangioma associated with thrombocytopenia in a 7-day-old infant. The patient was transfused with Cr51-tagged platelets, and survival of the donor platelets was studied. Scintillation counting over the hemangioma and spleen to detect sequestration of platelets was performed. Report of a Case A white baby girl, 1 day of age, was admitted to Columbus Children's Hospital on May 31, 1961, with a tentative diagnosis of isoimmunization disease. The baby was born of a gravida

Association of hemangioendothelioma with thrombocytopenic purpura has been described repeatedly. 1,2 The tumor is usually obvious at birth. Radiation therapy has been thought to be of value; surgical removal of the tumor has been performed 3 ; splenectomy is apparently not beneficial. As the tumor shrinks, purpura ceases, and platelet counts return to normal. There are a few reports of spontaneous involution of cavernous hemangioma. 4 The interest in the following case revolves about the spontaneous regression of a huge hemangioma with thrombocytopenic purpura in the first 6 months of life. Case History The patient, a boy, was admitted to Children's Hospital, at the age of 3 days, because of jaundice, hematemesis, and a large tumor on the left leg. He was born of a full-term pregnancy; normal delivery. His birth weight was 3175 gm. (7 lb.); there was no neonatal distress. Jaundice was noticed at 12 hours. The patient vomited

Benign vascular tumors may occur in any tissue in the body. The skin is the structure most commonly affected. From histopathologic studies, according to Stout, 1 hemangiomas of the skin may be divided into two groups: those of the capillary or telangiectatic type, which consist of numerous tubules lined by endothelium and surrounded by cellular intercapillary tissue of varying thickness, and those of the cavernous type, in which blood channels are irregular and more widely dilated. Lymphangiomas are mainly composed of capillaries containing lymph or of widened cystic lymph spaces. Not infrequently both lymph and blood vessel channels may be found in the same tumor, which is then described as a hemangiolymphangioma. The most characteristic feature of the angioma is its red or purple color, of varying intensity, which is due to the large blood content of the tumor. Thrombopenic purpura associated with benign hemangioma is rare. In fact, we