[Is alfa-interferon still current in the management of Kasabach-Merritt syndrome?]

Area of Science:

• Pediatric Oncology
• Vascular Biology
• Hematology

Context:

• Kasabach-Merritt syndrome (KMS) is a rare complication of vascular tumors like kaposiform hemangioendothelioma and tufted angioma.
• KMS is characterized by severe thrombocytopenia (low platelet count) and potential coagulopathy.
• This condition requires prompt and effective management to prevent life-threatening complications.

Purpose:

• To report on two infant cases of Kasabach-Merritt syndrome (KMS) with complicated vascular tumors.
• To evaluate the efficacy of high-dose corticosteroids, vincristine, and interferon therapy in managing these complex cases.
• To discuss the role of interferon and novel anti-angiogenic therapies in treatment-resistant KMS.

Summary:

• Two infants presented with vascular tumors and severe thrombocytopenia, indicative of KMS.
• Initial treatment involved corticosteroids and vincristine, followed by interferon as third-line therapy.
• Interferon demonstrated efficacy in one patient within two months, while the second patient responded to a later re-treatment after eight years, with no observed side effects.
• Long-term follow-up showed no adverse events, particularly neurological complications.

Impact:

• These cases highlight the potential utility of interferon in managing refractory Kasabach-Merritt syndrome.
• The findings support further investigation into interferon and other anti-angiogenic agents for complex vascular tumors.
• Successful management of KMS can prevent severe morbidity and mortality in affected infants.