Accelerated phase of Chediak Higashi Syndrome: An Unusual case of pancytopenia
Keywords:
Chediak Higashi, Pancytopenia, accelerated phase, leukocytes, bone marrow.Abstract
Chediak Higashi Syndrome (CHS) is a rare and fatal disease with varied clinical features and laboratory findings. Early diagnosis can be made by screening the blood smear and marrow for giant granules in leukocytes. Accelerated phase has poor prognosis. Hematopoietic stem cell transplantation (HSCT) has good role, if done in early (chronic) stage of disease. Hence, a prompt and accurate diagnosis should be given, so that timely intervention can be done. The present case highlights an accelerated phase of CHS in an infant presenting for the first time with recurrent infections and organomegaly.Â
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Copyright (c) 2016 Manjari Kishore, Sadhna Marwah, Vijay Kumar, Pooja Suteri, A.S. Nigam
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