Extramedullary hematopoiesis - adrenal

Case contributed by Dr Noriza Zainol Abidin

Presentation

Known haemoglobin H-constant spring (Hb H-CS) thalassaemia with right abdominal discomfort

Patient Data

Age: 16 years
Gender: Female
CT

Contrast enhanced CT abdomen pelvis

There is a well circumscribed heterogeneous large right adrenal mass measuring 6.7 x 6.7 x 9.1 cm (AP x ML x CC).  It has central necrosis. The soft tissue component demonstrates enhancement on the portovenous phase. No calcification or fatty component within. No surrounding fat streakiness.

On coronal images, it causing mass effect onto the right kidney.

Hepatosplenomegaly and cholelithiasis.

No ascites.

Impression:  Right adrenal mass with underlying hematological disorder suggestive of extramedullary hematopoietic tumor.

Ultrasound guided biopsy of the right adrenal mass was performed.

Histopathological examination revealed proliferation of erythroblasts, myeloblasts and megakaryocytes, along with red blood cells and haemosiderin-laden macrophages. Findings consistent with adrenal extramedullary haematopoiesis.

Case Discussion

Extramedullary hematopoiesis (EMH) is a compensatory mechanism in response to an imbalance between bone marrow erythropoiesis and circulatory blood demand. It occurs most often due to hemolytic anemias e.g. thalassemia, hereditary spherocytosis, and sickle cell disease.

Adrenal EMH tumor should be considered in thalassemic patients with an adrenal mass, thus avoiding unnecessary surgical procedures.

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Case information

rID: 51579
Published: 1st Mar 2017
Last edited: 24th Mar 2019
Inclusion in quiz mode: Included

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