50 Years Ago in The Journal of Pediatrics: Hemangioma with Thrombocytopenia

Adams, Denise M.

doi:10.1016/j.jpeds.2008.09.045

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Volume 154, Issue 4 , Page 497, April 2009

50 Years Ago in The Journal of Pediatrics:

Hemangioma with Thrombocytopenia

Associate Professor of Pediatrics, Medical Director of the Hemangioma and Vascular Malformations Center, Inpatient Clinical Director, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio

Dargeon HW, Adiao AC, Pack GT. J Pediatr 1959;54:285-95

In 1940, Kasabach and Merritt were the first to report the association of a “capillary hemangioma” with profound thrombocytopenia.¹ This association is re-addressed in Dargeon's article almost 20 years later, in which the cases of 14 patients with “hemangiomas” and thrombocytopenia were reviewed. Critical observations were noted: most lesions occurred in early infancy; splenectomy, although performed in half the patients, was not effective; steroids helped in some cases; and irradiation was successful in half the patients.

It was not until 1982 that a classification system was proposed for vascular anomalies,² and not until 1997 that tumors called Kaposiform hemangioendotheliomas and tufted angiomas were identified as the “hemangiomas” that caused profound thrombocytopenia.³ For many “vascular anomaly specialists,” the patients identified in Dargeon's paper had a spectrum of vascular anomalies (tumors, malformations, congenital hemangiomas) and not the more common infantile hemangioma.

Fifty years later, we have made some progress. Steroids are used for the treatment of hemangiomas and other vascular tumors with varying response, but other therapies such as vincristine and anti-angiogenic agents are beginning to be studied in clinical trials verses retrospective case reports.⁴ The genetic and biologic characteristics of these anomalies are being investigated with vigor. Organizations such as the International Society for the Study of Vascular Anomalies (ISSVA) and patient support groups have helped with the new interest in this expanding field.

Currently, the proper care of patients with vascular anomalies requires the expertise of multiple medical specialists with clinical acumen in surgery, radiology, dermatology, hematology, oncology, pathology, neurology, cardiology, gastroenterology, and basic sciences. This comprehensive multidisciplinary approach offers the best opportunity for proper diagnosis, management, and scientific progress in this field.

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References

Kasabach H, Merritt K. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child. 1940;59:1063–1070
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Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412–422

Sarkar M, Mulliken JB, et al. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg. 1997;100:1377–1386

Adams DM, Wentzel MS. The role of the hematologist/oncologist in the care of patients with vascular anomalies. Pediatr Clin North Am. 2008;55:339–355