Kasabach-Merritt Syndrome in an Adult: A Comment

To the Editor,

I read with interest the article by Vinod et al. [1] entitled “Kasabach-Merritt Syndrome in an Adult” in a recent issue of the Turkish Journal of Hematology.

Related to this article, I would like to add that Özsoylu [2] reported megadose methylprednisolone (MDMP) treatment for different hematological conditions including hemangiomas, infantile hemangiomatosis, and Kasabach-Merritt syndrome. Özsoylu [2] recommended the daily use of MDMP at 30 mg/kg for 3 days, then 20 mg/kg for 4 days and subsequently 10, 5, 2, and 1 mg/kg with each dose administered for 1 week around 6 AM as a single dose [2,3]. Özsoylu [2] treated patients who were resistant to conventional corticosteroid treatment either orally or intravenously over the course of 10-15 min [2,3]. Conventional corticosteroid treatment and MDMP were comparatively studied for the treatment of hemangiomas and it was observed that MDMP was superior [4].

In our department, eight patients with infantile hemangiomas were treated with interferon-a2a plus oral prednisolone without observing severe complications. In this study, the median age was 4 months (range: 3-12 months); six of them were female and two were male. Three cases were not followed and three patients achieved complete regression while a partial regression was observed in two others. The beneficial results were obtained with interferon-a2a plus oral prednisolone treatment in these cases [5].

On the other hand, vascular endothelial growth factor (VEGF) is recognized as an essential regulator of blood vessel growth. The use of anti-VEGF abolishes this vascular endothelial growth-promoting activity in vitro [6]. Three patients with periocular epithelioid hemangioma were treated with anti-VEGF successfully [7]. Although recent studies have indicated the effectiveness of anti-VEGF treatment in these cases, there is not a significant number of clinical trials reported.

Dear Editor,

We would like to thank Gözdaşoğlu [1] for showing interest in our report of adult Kasabach-Merritt syndrome (KMS) [2]. Being adult hematologists, we should admit that we have limited experience with treatment of KMS, which by and large affects infants and young children. We do not have personal experience with interferon-alpha and have not studied the efficacy of different corticosteroids in different doses and combination of prednisolone with interferon-alfa for treatment of KMS. In this regard, we thank Gözdaşoğlu [1] for sharing her personal experience (effectiveness of prednisolone + interferon-alfa 2a combination therapy) with treatment of infantile hemangiomas and also for highlighting on reports of usefulness of high dose methylprednisolone [3] and anti-VEGF (bevacizumab) therapy for treatment of hemangiomas and KMS. Clinical trials evaluating different therapies would be desirable in the future to clarify on optimal treatment of KMS.

Best Regards,