Extramedullary hematopoiesis in a child with hereditary spherocytosis: an uncommon cause of an adrenal mass

Abstract

We report a case of extramedullary hematopoiesis presenting as an adrenal mass in a young male with hereditary spherocytosis. The unilateral adrenal mass was discovered during an abdominal ultrasound performed for jaundice. CT and MR imaging were subsequently performed, followed by an excisional biopsy at the time of splenectomy and cholecystectomy. Although extramedullary hematopoiesis is a rare cause of an adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder such as hereditary spherocytosis. In this regard, the morbidity of an unnecessary procedure may be avoided.

Extramedullary hematopoiesis in the adrenal: Case report and review of literature

“…Patients with adrenal EMH are generally known to have beta-thalassemia major 4,5 or other major haemoglobinopathies, such as hereditary spherocytosis. 6 This is the first report of its occurrence in delta-beta thalassemia. There have been reports of Tc99 nanocolloid bone scintigraphy being used to detect evidence of reticuolendothelial tissue in the adrenal.…”

Adrenal Extramedullary Hematopoiesis: A Case Report

“…In our review of literature we found reports of adrenal gland in patients with Beta-Thalassaemia, Agnogenic Myeloid Metaplasia and hereditary spherocytosis [1][2][3][4][5][6][7]. Surgery or radiotherapy should be performed only when EMH tumors cause symptoms, such as during spinal cord compression.…”

“…Involvement of the central nervous system may be associated with increased intracranial pressure, altered sensorium, motor and sensory impairment, including cord compression. Its identification is invariably incidental, imaging of the lesion is poorly specific and the diagnosis requires the histological examination [4].…”

Extramedullary Hematopoiesis in a Man With β-Thalassemia: An Uncommon Cause of an Adrenal Mass

“…Masses larger than 6 cm should be considered malignant, until proven otherwise (8). When EMH presents as a soft tissue mass, it often mimics a more serious diagnosis, requiring radically different treatment options (2). The EMH could present special features on radiologic investigations that help in diagnosis.…”

“…Most frequently, it occurs in the spleen, liver, lymph nodes and less frequently, in the lung, pleura, thymus and breasts. Rare cases of EMH were seen in adrenals (2,3). The EMH may occur in congenital or acquired hemolytic diseases, ineffective erythropoiesis, loss of stem cell differentiation or non-myeloid neoplastic diseases (4).…”