Wiskott Aldrich Syndrome - A Case Report

Wiskott Aldrich Syndrome (WAS) is an immunological disorder caused by a genetic abnormality (Brunner, 2017). WAS is a rare genetic disorder of the immune system that primarily affects boys. It is characterized by abnormal immune function and a reduced ability to form blood clots. It is one of the primary immune deficiency disorders (NIAID, 2015) which results in prolonged bleeding and recurrent infections. The platelet abnormality is typically present from birth and can lead to easy bruising or episodes of prolonged bleeding following minor trauma. Early correction with hematopoietic stem cell transplantation or gene therapy is necessary to prevent the risk of Severe Combined Immunodeficiency (SCID), autoimmune complications and Haematological malignancies. This article glimpses a case scenario with WAS disease and the medical and nursing management carried out in a tertiary care hospital in southern India which is one of the pioneer hospitals for Haematopoietic stem cell transplantation in India.