1/24. spinal cord compression in beta-thalassemia: follow-up after radiotherapy.CONTEXT: spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including beta-thalassemia. CASE REPORT: We report the case of a patient with intermediate beta-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. DISCUSSION: Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
2/24. MRI features of epidural extramedullary hematopoiesis.A case of beta-thalassemia intermedia with spinal cord compression due to extramedullary hematopoiesis, which was successfully treated by blood transfusion, is presented. Emphasis was made on the MRI appearance of extramedullary hematopoiesis on different pulse sequences. The theories that aimed to explain the involvement of the epidural space by extramedullary hematopoiesis are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = thalassemia (Clic here for more details about this article) |
3/24. spinal cord compression due to extramedullary hematopoiesis associated with polycythemia vera--case report.A 69-year-old woman with a 14-year history of polycythemia vera suffered progressive paraparesis due to epidural involvement of hematopoietic tissue. Magnetic resonance (MR) imaging demonstrated extensive epidural masses. Decompressive surgery and radiotherapy were performed and she made an almost complete clinical recovery. Serial MR imaging showed no regrowth of the other epidural masses. Extramedullary hematopoiesis occurs in patients with various hematologic disorders involving a chronic increase in the production of red blood cells, and is often associated with thalassemia, but is less common with polycythemia vera. The most frequent sites are the spleen, liver, and kidney. Extramedullary hematopoietic tissue occurring within the spinal canal and causing cord compression is very rare. Total surgical excision is not usually feasible because of the diffuse nature of extramedullary hematopoietic tissue and the possibility of recurrence, but acute neurological deterioration does require emergency surgery. Extramedullary hematopoiesis is radiosensitive and displays a rapid response to low dosages, so radiation therapy is recommended for residual tumors. Considering the possibility of central nervous system extramedullary hematopoiesis in patients with polycythemia vera, an early diagnosis is necessary for a favorable prognosis.- - - - - - - - - - ranking = 0.16666666666667keywords = thalassemia (Clic here for more details about this article) |
4/24. Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia.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.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
5/24. Hypertransfusion: a successful method of treatment in thalassemia intermedia patients with spinal cord compression secondary to extramedullary hematopoiesis.SUMMARY OF BACKGROUND DATA: Extramedullary hematopoiesis is a common compensatory phenomenon to chronic hemolytic anemias including thalassemia. Several sites can be involved including the liver, the spleen, the lymph nodes, and other less common locations. spinal cord compression may result in the rare cases wherein the hematopoietic develops intraspinally. Treatment of such conditions still is controversial. OBJECTIVE: This article reviews the literature and reports two cases of thalassemia intermedia involving patients who presented with neurologic symptoms after acute spinal cord compression secondary to extramedullary hematopoiesis. methods: The diagnosis was established by magnetic resonance imaging. Hypertransfusion therapy was used as our first-line treatment method. RESULTS: Complete neurologic recovery was achieved. Improvement in the neurologic status started as soon as the first week of treatment. CONCLUSIONS: Clinical awareness is important for early diagnosis and prevention of irreversible neurologic complications in such cases. magnetic resonance imaging is the radiologic method of choice for diagnosing extramedullary hematopoietic masses and for delineating the extent of spinal cord involvement. Hypertransfusion seems to be a promising treatment method that should be recommended as a first-line approach or as an adjuvant therapy to other methods.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
6/24. spinal cord compression in beta-thalassemia: case report and review of the literature.STUDY DESIGN: A case report of thoracic spinal cord compression in a 34-year-old male with beta-thalassemia is reported. OBJECTIVES: In patients with thalassemia, neurologic complaints should lead to a high index of suspicion for spinal cord compression from marrow expansion, ectopic bone formation and resultant stenosis. Initial presentation, diagnosis, radiographic findings, surgical treatment and follow-up are reviewed. SETTING: This case is reported from chicago, illinois. METHOD: A chart review is performed for the purposes of this case report. RESULTS: Patient underwent decompressive laminectomy with good surgical outcome. CONCLUSION: Rapid diagnosis and treatment of such a condition is essential to optimize the chances of recovery.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
7/24. Asymptomatic spherocytosis presenting with spinal cord compression: case report.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.- - - - - - - - - - ranking = 0.16666666666667keywords = thalassemia (Clic here for more details about this article) |
8/24. spinal cord compression due to epidural extramedullary haematopoiesis in thalassemia.A case of spinal epidural extramedullary haematopoiesis in thalassemia is reported. role of computed tomography in this rare manifestation is emphasized with brief review of literature.- - - - - - - - - - ranking = 0.83333333333333keywords = thalassemia (Clic here for more details about this article) |
9/24. Successful treatment of spinal cord compression secondary to extramedullary hematopoietic mass by hypertransfusion in a patient with thalassemia major.A 15-year-old girl with thalassemia major who suffered from paraparesis with a history of progressive lower limb weakness for 2 years immigrated from mainland china to hong kong. She had not received regular blood transfusion since the age of 6 years after splenectomy. MRI of the spine showed thoracic spinal cord compression secondary to extramedullary hematopoietic mass. She made significant recovery with hypertransfusion therapy alone. MRI of the spine repeated 3 months later showed nearly complete resolution of the extramedullary hematopoietic mass.- - - - - - - - - - ranking = 0.83333333333333keywords = thalassemia (Clic here for more details about this article) |
10/24. Spinal extradural hematopoiesis in adolescents with thalassemia. Report of two cases and a review of the literature.Extramedullary hematopoietic tissue occurring within the extramedullary space and causing cord compression is an unusual occurrence in childhood and adolescence, and only four cases have been reported during the first two decades of life. We had the opportunity to treat a 16-year-old girl and a 14-year-old boy with paraparesis secondary to extradural extramedullary hematopoiesis. Findings in these cases are discussed, and an attempt is made to stress once more the possibility of congenital hematopoietic rests as the origin of such hyperplastic tissue. The various modes of therapy are discussed and related literature is reviewed.- - - - - - - - - - ranking = 0.66666666666667keywords = thalassemia (Clic here for more details about this article) |
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