Extramedullary Hematopoiesis With Spinal Cord Compression in a Child With Thalassemia Intermedia

İleri, Talia; Azk, Fatih; Ertem, Mehmet; Uysal, Zümrüt; Gözdaşoĝlu, Sevgi

doi:10.1097/mph.0b013e3181a71843

Cited by 19 publications

(21 citation statements)

References 14 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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“…High-resolution 3D studies locate erythropoietic islands in the inter-trabecular spaces of the bone marrow [4] and modifications in the vascular structure, in the proportion of hematopoietic lineages and in the remodeling of the trabecular bone are expected to assure replenishment of erythrocytes in response to chronic erythropoietic stress. These hemolytic conditions are also characterized by dysfunctional bone marrow erythropoiesis [5], [6], [7] and extramedular erythropoiesis [8], [9], [10].…”

Section: Introductionmentioning

confidence: 99%

Alterations in Bone and Erythropoiesis in Hemolytic Anemia: Comparative Study in Bled, Phenylhydrazine-Treated and Plasmodium-Infected Mice

et al. 2012

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Sustained erythropoiesis and concurrent bone marrow hyperplasia are proposed to be responsible for low bone mass density (BMD) in chronic hemolytic pathologies. As impaired erythropoiesis is also frequent in these conditions, we hypothesized that free heme may alter marrow and bone physiology in these disorders. Bone status and bone marrow erythropoiesis were studied in mice with hemolytic anemia (HA) induced by phenylhydrazine (PHZ) or Plasmodium infection and in bled mice. All treatments resulted in lower hemoglobin concentrations, enhanced erythropoiesis in the spleen and reticulocytosis. The anemia was severe in mice with acute hemolysis, which also had elevated levels of free heme and ROS. No major changes in cellularity and erythroid cell numbers occurred in the bone marrow of bled mice, which generated higher numbers of erythroid blast forming units (BFU-E) in response to erythropoietin. In contrast, low numbers of bone marrow erythroid precursors and BFU-E and low concentrations of bone remodelling markers were measured in mice with HA, which also had blunted osteoclastogenesis, in opposition to its enhancement in bled mice. The alterations in bone metabolism were accompanied by reduced trabecular bone volume, enhanced trabecular spacing and lower trabecular numbers in mice with HA. Taken together our data suggests that hemolysis exerts distinct effects to bleeding in the marrow and bone and may contribute to osteoporosis through a mechanism independent of the erythropoietic stress.

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“…However, it is understood that >80% of cases do not have signs and symptoms related directly to the disorder, and the lesions are typically discovered incidentally by radiologic techniques (15–17). The development of neurologic symptoms is suggested to depend on the chronicity of the disease, with neurologic symptoms most frequently being reported during the third and fourth decades of life (18), although few reports described presentation as early as the first decade of life (19–21). The male to female ratio reaches 5:1 (18).…”

Section: Discussionmentioning

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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Extramedullary Hematopoiesis With Spinal Cord Compression in a Child With Thalassemia Intermedia

Cited by 19 publications

References 14 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

Alterations in Bone and Erythropoiesis in Hemolytic Anemia: Comparative Study in Bled, Phenylhydrazine-Treated and Plasmodium-Infected Mice

Extramedullary hematopoiesis: A report of two cases

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