Cranial extramedullary hematopoiesis with pancreatic secondary hemochromatosis in a beta thalassemia patient: A case report

Abstract

Thalassemia is one of the most worrying hemolytic disorder with wide range of evolving clinical alterations based on the type, severity of affected hematopoietic mechanisms and repeated transfusions to correct underlying anemia.We present a 13 year old female patient diagnosed with thalassemia major showing features of cranial extra medullary hematopoiesis with severe intracranial infections in the form of cerebellar abscess and otomastoiditis extending in to adjacent sinus venouses, Iron deposition in liver & pancreas and incidental findings of uncomplicated horse shoe kidney.Extra medullary hematopoiesis is a catch up mechanism at the failing medullary hematopoeising to keep in check the hemoglobin levels without causing much physiological imbalance.EMH usually affects visceral structures like liver, spleen lymph nodes and thorax. Less commonly pleura, lungs, GIT, breast, skin, kidneys, adrenals and rarely the cranium and intracranial structures. Here, we present one of the rare presentations of cranial EMH in the background of severe intracranial infection.