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Background: Kasabach-Merritt syndrome represents the association of hemangioma with thrombocytopenia and consumptive coagulopathy. We present a case of Kasabach-Merritt syndrome treatment with orally administered propranolol., Case Presentation: A 4.5-month-old caucasian female infant with congenital giant hemangioma in the posterior region of her neck presented to our Institute for Childhood Diseases where she underwent clinical, laboratory, and radiological investigations. A low blood platelet count indicated the use of corticosteroids and blood components as first-line therapy. The lack of therapeutic response induced the introduction of orally administered propranolol as additive therapy. A 3-week treatment led to a reduction in the size of hemangioma and a rise in platelet count which enabled surgical treatment and definite healing., Conclusion: Orally administered propranolol as monotherapy or in combination with other therapeutic modalities may play a key role in the treatment of Kasabach-Merritt syndrome.

Introduction: Kasabach-Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the gastrointestinal tract, the skin, and the subcutaneous tissue. There is no publication about pulmonary manifestation of angiomatosis in combination with vascular malformation and hemoptysis. This is the first description of a Kasabach-Merritt syndrome-like condition in the lung., Case Presentation: We present the case of a 29-year-old German woman with angiomatosis and associated pulmonary vascular malformation in her lower left lobe with a Kasabach-Merritt syndrome like condition. It was detected after hemoptysis. We also present our case observations and management., Conclusion: In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding.