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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extramedullary haemopoiesis causing spinal cord compression is a rare manifestation of thalassemia. We describe a 17 year old male with thalassemia intermedia who presented with progressive paraplegia and sphincter disturbance. Magnetic resonance imaging revealed an epidural lesion extending from T5 to T8 compressing the spinal cord. The patient recovered completely after surgical decompression with postoperative radiation therapy. Histological examination of the lesion confirmed the diagnosis of extramedullary haemopoiesis. Clinical awareness of this phenomenon with early treatment is essential for optimizing the neurological outcome.
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PMID:Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia. 1260 97

Extramedullary hematopoiesis is an unusual cause of spinal cord compression. We report the case of a 39-year-old man who presented symptoms of progressive dorsal spinal cord compression. Hemogramm showed erythrocytosis with microcytosis. Hemoglobin electrophoresis revealed heterozygous beta-thalassemia. Diagnosis of extramedullary hematopoiesis was based on magnetic resonance imaging findings. Radiological and therapeutic options in this rare condition are briefly discussed.
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PMID:[A new case report of spinal cord compression secondary to beta-thalassemia]. 1277 4

Extramedullary hematopoiesis (EMH) is a common manifestation in beta-thalassemia, but can also occur in several clinical hematologic disorders or neoplasms. It has been reported in almost all sites of the body. Often asymptomatic, it can be manifested by compression of the adjacent organs. Spinal cord compression as a consequence of EMH in the intraspinal epidural space is an extremely rare complication. We report a case of a 34 year old man who was hospitalised for complete paraplegia and spasticity of both lower limbs; spinal cord compression on D4-D8 by EMH was diagnosed by MRI and confirmed by histologic examination.
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PMID:[Cord compression secondary to extramedullary hematopoiesis in a patient with beta-thalassaemia]. 1577 16

A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained.
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PMID:Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord. 1581 33

Extramedullary hematopoiesis (EMH) is a compensatory mechanism occurring in patients with chronic anemia, which occurs most frequently with thalassemia. The authors report the case of a 57-year-old man, with no history of clinical or hematological disease, presenting with spinal cord compression. Magnetic resonance (MR) imaging demonstrated a homogeneous posterior epidural mass extending from T-3 to T-6. Following decompressive surgery, the patient's symptoms improved. Histological analysis showed features consistent with a diagnosis of EMH. Subsequent workup was remarkable for an asymptomatic spherocytosis without anemia. There was no family history of anemia. An EMH-related presentation of mild spherocytosis has been described in the literature, but its epidural location led to spinal cord compression. The MR imaging features were suggestive of EMH, but in the presence of spinal cord compression and in the absence of a history of chronic anemia, the authors did not believe that nonsurgical management would have been reasonable.
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PMID:Asymptomatic spherocytosis presenting with spinal cord compression: case report. 1587 92

Spinal cord compression due to extramedullary hematopoiesis is a rare manifestation of thalassemia. We present a 28-year-old woman with beta-thalassemia intermedia and progressive paraparesis. She had a thoracic extradural extramedullary mass lesion on MRI. She improved after receiving multiple transfusions. Clinical awareness of this phenomenon with early treatment is essential for a successful outcome.
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PMID:Extramedullary hematopoiesis with spinal cord compression in beta-thalassemia intermedia. 1631 43

We report a 24 years old patient with thalassaemia intermedia and gynecomastia, complicated by paraplegia and urinary/fecal incontinence due to spinal cord compression by an extramedullary erythropoiesis (EE) mass, treated with long-term hydroxyurea (HU). Neurological improvement occurred during the first 6 weeks of HU therapy (1,000 mg/day) and magnetic resonance imaging showed a reduction in EE mass. HU dosage was reduced to 500 mg/day after 5 months, and treatment was discontinued after 25 months. Five months later there was a partial recurrence of neurological symptoms, which responded to radiotherapy. HU may have a role in the symptomatic treatment of spinal cord compression due to EE, particularly when radiotherapy is unavailable.
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PMID:Paraplegia due to spinal cord compression by extramedullary erythropoietic tissue in a thalassaemia intermedia patient with gynecomastia secondary to cirrhosis: successful treatment with hydroxyurea. 1646 20

Extramedullary hematopoiesis affects about 15% of the patients treated for thalassemia intermedia. Usually seen in adulthood, the most common location is the paraspinal region. Diagnosis and treatment of extramedullary hematopoiesis located in the pelvis of a young 15-year-old girl is discussed. The young age of the patient and the uncommon site of the mass first lead to the diagnosis of an ovarian dermoid cyst. Because of the clinical history and the typical feature on computed tomography scan, extramedullary hematopoiesis was concluded. A specific treatment based on blood transfusion and hydroxyurea was first proposed but remained inefficient. Surgical excision was thus successfully performed. Whereas surgery is limited to spinal cord compression in paraspinal extramedullary hematopoiesis, this observation argues for surgical treatment in symptomatic intraabdominal extramedullary hematopoiesis when medical treatment fails.
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PMID:Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis. 1681 41

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.
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PMID:Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. 1740 52

Thalassemia is a chronic hemolytic anemia, endemic around the Mediterranean basin. Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that can involve many organs or tissues, including the epidural space, leading to a spinal cord compression syndrome. In almost all cases, the clinical and MR patterns are those of a lower dorsal spinal cord progressive compression secondary to an epidural expanding process. The management still remains controversial, including mainly blood transfusions, decompressive surgery and radiotherapy. We present a case of EMH in a thalassemic adolescent boy presenting with a T12 level of compression, treated successfully with blood transfusion, surgical decompression and radiotherapy.
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PMID:Management of spinal cord compression caused by extramedullary hematopoiesis in beta-thalassemia. 1855 71


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