Outcome of Kasabach-Merritt phenomenon: the role of vincristine as monotherapy: report of a case.

Kasabach-Merrit phenomenon (KMP) is a rare disorder of pediatric hematological malignancies which is previously referred to Kasabach-Merrit syndrome (KMS). The disorder is characterized by prominent vascular mass resulting from abnormal proliferation of blood vessels, consumption coagulopathy and thrombocytopenia. The diagnosis is based upon three basic findings as above. The authors describe a 6-month-old girl with a huge ecchymotic mass at left buttock who is found to have thrombocytopenia and consumption coagulopathy. The clinical and imaging studies strongly suggested the diagnosis of KMP. Vincristine was administered after a trial of corticosteroids was failed to show clinical and laboratory improvement. After 2 weeks, the patient showed that the platelet count and fibrinogen level become to be normal without blood transfusion and gradually decreased in tumor size without any surgical procedure in 4 weeks and disappeared in 5 months without any complications. In the present report, the authors present the findings from successfully specific treatment with vincristine alone and supportive treatment for life-threatening hemorrhage with platelet concentrate and cryoprecipitate should be the best management in our situation.

Use of the gel test to follow up chimerism in ABO mismatched bone marrow transplantation patient: a case report.

OBJECTIVE: The authors report here our experience using the gel test to follow up chimerism in a 5 year old girl with beta thalassemia/Hemoglobin E disease (beta thal/HbE), post allogeneic bone marrow transplantation with Hb E trait HLA identical sibling donor. They were ABO blood group major mismatched donor-recipient pairs (donor and recipient blood group are B and O, respectively). MATERIAL AND METHOD: Pre and post transplanted EDTA blood samples from the girl with beta thalassemia/ Hemoglobin E were tested for ABO, Rh and direct antiglobulin test (DAT) using the A-B-AB-D-ctl/ AHG card and the titer of anti-A and anti-B were tested by the conventional tube technique. The sex chromosome study and hemoglobin typing were also examined. RESULTS: In this technique, mixed field agglutination is clearly identified from positive and negative results. The authors detected peripheral recovery, mixed O/B population after transplantation on day +26 with positive DAT. The DAT was negative on day +67 after transplantation and the recipient blood group was completely changed to B on day +123. In addition, Hb typing was changed to Hb E trait with Hb F less than 5 % on day +37. The engraftment of neutrophils, more than 5x10(9)/L, was detected on day+14 and platelet count was more than 20x10(9)/L on day +28. On day +90, the patient was transfusion-independent with the mean Hb level at 11.4 g/dL (10.4-13.1). The sex chromosome and hemoglobin typing were changed to the donor on day +300. CONCLUSION: The gel test is an alternative method which is simple and helpful in detecting mixed red blood cell populations, particularly in the ABO or other blood group mismatched bone marrow transplantation.