Adrenal hematopoiesis in a pediatric patient with spherocytosis: A case report and literature review

Abstract

Extramedullary hematopoiesis (EMH) is extremely rare in the adrenal glands, especially in children. Because this is an underrecognized condition, the vast majority of cases are diagnosed only after adrenalectomy. An 11-year-old female with hereditary spherocytosis presented with dull right hypochondrial pain. Ultrasonography showed chronic calculous cholecystitis, and splenomegaly. A computed tomography revealed a 4 × 1.7 cm mass in the right adrenal gland with no fat content. Laparoscopic cholecystectomy with adrenalectomy was done. Histopathological examination of the adrenal mass revealed hematopoietic precursors demonstrating megakaryocytes, admixed myeloid precursors, and erythroid precursors without adipose tissue. Nearly 30 cases of adrenal EMH have been documented in the literature. Despite being a benign condition and most of the cases diagnosed incidentally, adrenalectomy was done for the majority of patients even though conservative treatment has shown good results in several case reports. Approximately 75% of cases occurred in the right adrenal gland, with bilateral and left side constituting 12.5% each. Patients with hereditary spherocytosis may present with an adrenal mass due to EMH, even in pediatric age groups. In patients with known hematologic diseases, preoperative diagnosis through functional imaging or biopsy may prevent the need for surgical intervention.

Keywords

• Extramedullary hematopoiesis
• Incidentaloma
• Erythropoiesis
• Adrenal gland
• Spherocytosis