Spinal cord compression due to extramedullary hematopoiesis in thalassemia: Long-term follow-up after radiotherapy

Jackson, Don V.; Randall, Marcus E.; Richards, Frederick

doi:10.1016/0090-3019(88)90047-x

Cited by 59 publications

(47 citation statements)

References 4 publications

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“…While end-stage, postmitotic cells, such as erythrocytes and granulocytes, are relatively radioresistant, mitotic cells (especially stem cells) arc highly radiosensitive. In the literature, the dose of radiation given to patients who present with spinal cord compression caused by extra medullary hematopoiesis has varied from 8.5 to 30 Gy [3][4][5][7][8][9]. While the range of radiation doses is large, much of the variability can be attributed to the size of the hematopoietic tissue mass and the radiation tolerance of surrounding normal tissues.…”

Section: Discussionmentioning

confidence: 99%

Combined Radiotherapeutic and Surgical Management of a Spinal Cord Compression by Extramedullary Hematopoiesis in a Patient with Hemoglobin E Beta-Thalassemia

et al. 1994

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A 22-year-old male with a history of hemoglobin E β°-thalassemia presented with thoracic spinal cord compression due to extrameduUary hematopoietic tissue in the epidural space. Following decompressive laminectomy, this epidural mass was irradiated to 20.4 Gy in 12 fractions. The patient made a complete recovery from his neurological symptoms following treatment. Follow-up radiographs showed significant reductions in size of the extradural tumor. Results from this case supported by review of the literature suggest that radiation therapy, with or without decompressive surgery, is an effective therapy in treatment of patients with spinal cord compression due to epidural extrameduUary hematopoietic tissue.

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Section: Discussionmentioning

confidence: 99%

Combined Radiotherapeutic and Surgical Management of a Spinal Cord Compression by Extramedullary Hematopoiesis in a Patient with Hemoglobin E Beta-Thalassemia

et al. 1994

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“…Surgery is not always possible because of the diffuse nature of the mass and the likelihood of recurrence. Furthermore, immediate total resection of extramedullary hematopoietic masses can lead to clinical decompensation and deterioration because these masses play a crucial role in maintaining an adequate hemoglobin level (Jackson et al 1988). …”

Section: Extramedullary Hematopoietic Pseudotumorsmentioning

confidence: 99%

-Thalassemia Intermedia: A Clinical Perspective

Musallam

Taher²,

Rachmilewitz

2012

Cold Spring Harbor Perspectives in Medicine

117

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Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with b-thalassemia intermedia has substantially increased over the past decade. Earlier studies observed that patients with b-thalassemia intermedia experience a clinical-complications profile that is different from that in patients with b-thalassemia major. In this article, a variety of clinical morbidities are explored, and their associations with the underlying disease pathophysiology and risk factors are examined. These involve several organs and organ systems including the vasculature, heart, liver, endocrine glands, bone, and the extramedullary hematopoietic system. The effects of some therapeutic interventions on the development of clinical complications are also discussed.

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“…Recurrence rates of about 19% have been reported but these cases are amenable to treatment with further radiation. 8,14 Niggeman et al described a case for whom radiation therapy originally appeared effective, but who then experienced recurrence of symptoms after a short period, making an operative approach necessary. 15 Most reported cases of paraplegia due to EMH from any cause have been treated with surgical decompression with or without radiation therapy.…”

Section: Discussionmentioning

confidence: 99%

Thoracic spinal cord compression due to xtramedullary haemopoiesis in a patient with beta-thalassemia: complete clinical regression with radiation therapy alone

Saha

Chattopadhyay

Azam

et al. 2014

Int J Cancer Ther Oncol

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Case Report AbstractSpinal cord compression due to extramedullary haemopoesis in beta-thalassemia is extremely rare. Controversies are there between the two modalities of treatment surgery vs radiation therapy. We present here a case of beta thalassemia major in a twenty one year old female patient who presented with features of spinal cord compression due to extramedullary haemopoesis. She was then treated with 3000 cGy of radiation therapy targeted to the T5-T8 region, as 200 cGy/fraction daily, 5 fractions/week, over 6 weeks. The patient's haemoglobin was elevated from 6.1g/dl to 10.1g/dl, with her haematocrit rising from 26.3% to 32.8%. Steroid dose was tapered on hospital day number 7. She achieved near full neurological recovery after medical treatment with steroids, blood transfusion and radiation therapy.

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Spinal cord compression due to extramedullary hematopoiesis in thalassemia: Long-term follow-up after radiotherapy

Cited by 59 publications

References 4 publications

Combined Radiotherapeutic and Surgical Management of a Spinal Cord Compression by Extramedullary Hematopoiesis in a Patient with Hemoglobin E Beta-Thalassemia

Combined Radiotherapeutic and Surgical Management of a Spinal Cord Compression by Extramedullary Hematopoiesis in a Patient with Hemoglobin E Beta-Thalassemia

-Thalassemia Intermedia: A Clinical Perspective

Thoracic spinal cord compression due to xtramedullary haemopoiesis in a patient with beta-thalassemia: complete clinical regression with radiation therapy alone

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