Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature

Eşkazan, Ahmet Emre; Ar, Muhlis Cem; Başlar, Zafer

doi:10.1111/j.1537-2995.2011.03499.x

Cited by 21 publications

(14 citation statements)

References 21 publications

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“…Several effective treatment options have been described for EMH involving the paraspinal and epidural space, including multiple blood transfusions, 36 radiotherapy, 25,26,28 chemotherapy with agents that diminish hematopoietic drive (such as hydroxyurea) 16 and even laminectomy for spinal cord decompression and resection in selected symptomatic patients who did not respond to initial nonsurgical measures. 38 The ideal management remains controversial, however.…”

Section: Treatment Optionsmentioning

confidence: 99%

Ectopic extramedullary hematopoiesis: evaluation and treatment of a rare and benign paraspinal/epidural tumor

Mattei

Higgins

Flynn

et al. 2013

SPI

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Ectopic extramedullary hematopoiesis (EMH), defined as the formation of blood cells outside the bone marrow, usually occurs in a scenario of chronic anemia when, even after conversion of the bony yellow marrow to red marrow, the body is still unable to meet the demand for red blood cells. Ectopic extramedullary hematopoiesis most commonly occurs in the liver and spleen but may, in fact, occur almost anywhere in the body. Although previous reports have documented EMH presenting as paraspinal masses, such lesions have almost always been associated with a predisposing hematological disorder such as hemolytic anemia, myelofibrosis or myelodysplastic syndromes, thalassemia, polycythemia vera, leukemia, or lymphoma. The authors of this report describe the first reported instance of EMH in a patient presenting with a symptomatic epidural and paraspinal cervical lesion arising from the posterior spinal elements and no known predisposing hematological disease. Initial radiographs revealed a bony lesion arising posteriorly from the C2-3 laminae and spinous processes. Subsequent imaging suggested the diagnosis, which was confirmed by CT-guided biopsy, peripheral blood smears, and bone marrow aspirate. Despite epidural compression and slight displacement of the cervical cord and thecal sac, the patient's symptoms were limited to pain and diminished cervical range of motion. Therefore, surgery was deferred in favor of nonsurgical therapy. Several alternative modalities for the treatment of EMH have been suggested in the literature, including cytotoxic agents and radiotherapy. The authors opted for an approach utilizing directed low-dose radiotherapy of a total of 25 Gy divided in 2.5-Gy fractions. At the 3-month follow-up, the patient continued to be asymptomatic, and MRI demonstrated a significant reduction in the dimensions of the lesion. Extramedullary hematopoiesis with spinal cord compression in the absence of a preexisting hematological disorder has not been described in the context of clinical neurosurgical practice. Recognizing that EMH may present as an epidural or paraspinal lesion is important since chemotherapy and radiotherapy are effective therapeutic options in the majority of patients who suffer few if any symptoms. Extensive evaluation for underlying hematological disorders is necessary before undertaking directed therapy. Inadvertent resection of these highly vascularized masses may risk catastrophic intraoperative hemorrhage with no proven benefit as compared with medical treatment, which usually provides excellent long-term outcomes.

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Section: Treatment Optionsmentioning

confidence: 99%

Ectopic extramedullary hematopoiesis: evaluation and treatment of a rare and benign paraspinal/epidural tumor

Mattei

Higgins

Flynn

et al. 2013

SPI

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“…EMH 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 (7). Paraspinal involvement is the associated with morbidity secondary to spinal cord compression (8).…”

Section: Introductionmentioning

confidence: 99%

Extramedullary hematopoiesis: A report of two cases

Zhang

Liu

et al. 2016

Experimental and Therapeutic Medicine

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Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

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“…To date, cases of intracranial EMH have been described only in the form of extra-axial, epidural, or subdural, mass-like lesions [3]. The majority of the published cases were asymptomatic, presenting with symptoms only in cases of elevated intracranial pressure due to a tumor-like mass [5].…”

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confidence: 99%

Cerebral intraparenchymal extramedullary hematopoiesis in polycythemia vera

2019

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Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature

Abstract: 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.

Cited by 21 publications

References 21 publications

Ectopic extramedullary hematopoiesis: evaluation and treatment of a rare and benign paraspinal/epidural tumor

Ectopic extramedullary hematopoiesis: evaluation and treatment of a rare and benign paraspinal/epidural tumor

Extramedullary hematopoiesis: A report of two cases

Cerebral intraparenchymal extramedullary hematopoiesis in polycythemia vera

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