Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature.
BACKGROUND
Extramedullary hematopoiesis (EH) is a compensatory phenomenon that results in the production of blood cell precursors outside the marrow in patients with chronic hemolytic anemia and ineffective erythropoiesis. EH usually involves the liver, spleen, and lymph nodes. It can also be found at paravertebral, intrathoracic, or pelvic locations. Intracranial EH is a rare entity and often asymptomatic but can sometimes lead to symptomatic tumor-like masses. Treatment options are controversial and include hypertransfusion, surgical excision, radiotherapy, and hydroxyurea (HU).


METHODS
Successful treatment of an intracranial EH mass with HU and blood transfusions in a beta-thalassemia major patient was discussed along with a review of the published literature on intracranial EH in thalassemia.


RESULTS
In our patient, the extramedullary hematopoietic mass in the interhemispheric fissure showed a marked improvement after 6 months of HU and hypertransfusion therapy. In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes.


CONCLUSIONS
There is no standard treatment approach in patients with symptomatic EH. HU with hypertransfusion regimen is a reasonable first-choice modality in treating intracranial EH masses.
Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey. emreeskazan@hotmail.com
Ahmet Emre
AE
Eskazan
Muhlis Cem
MC
Ar
Zafer
Z
Baslar
D000328:Adult; D001803:Blood Transfusion; D001921:Brain / Q000473:pathology*; D006411:Hematopoiesis, Extramedullary / Q000502:physiology*; D006801:Humans; D008297:Male; D018781:Tension-Type Headache / Q000209:etiology* / Q000473:pathology*; D017086:beta-Thalassemia / Q000150:complications* / Q000473:pathology / Q000628:therapy