Vincristine: A new treatment option for Kasabach-Merritt Syndrome

Kasabach-Merritt syndrome (KMS) is characterized by a rapidly enlarging hemangioma, thrombocytopenia, microangiopathic hemolytic anemia and consumption coagulopathy as a result of platelet and red blood cell trapping and activation of clotting system within the vasculature of hemangioma. This syndrome is shown to be associated with kaposiform hemangioendotheliomas or tufted hemangioma

Peripheral gangrene in a child as a result of Primary Thrombocytosis

Essential Thrombocytosis (ET) is a clonal disorder of unknown etiology involving a multipotent hematopoietic progenitor cell and is manifested clinically by the overproduction of platelets without a definable cause. Clinically, ET is most often identified incidentally when a platelet count is obtained during the course of routine evaluation. There are very few case reports in the pediatric age group in which peripheral gangrene is caused by primary thrombocytosis or essential thrombocythemia. This is a case report of a 5 year old male child who presented to us as peripheral gangrene of toes which was caused by ET, one of the Myeloproloferrative disorders.KEYWORDS: Essential thrombocythemia; Gangrene, Myeloproliferative disorder; Primary thrombocytosisInternet Journal of Medical Update 2012 January;7(1):70-7

WEISMANN-NETTER-STUHL SYNDROME: A RARE FORM OF SKELETAL DYSPLASIA

A case of Weismann-Netter-Stuhl syndrome was described because of its rare entity in pediatric age group. The clinical presentation was short stature with bowing of lower legs with characteristic radiological findings. It was differentiated from congenital syphilis and rickets by negative laboratory results and the characteristic radiographic features of anterior and medial bowing of the mid to distal third of the tibiae and fibulae and cortical hyperostosis of the concave margins