Progressive paraparesis due to thoracic extramedullary hematopoiesis in myelofibrosis

Klippel, Nina De; Dehou, Marie‐Françoise; Bourgain, Catherine; Schots, Rik; Keyser, Jacques De; Ebinger, Guy

doi:10.3171/jns.1993.79.1.0125

Cited by 38 publications

(32 citation statements)

References 10 publications

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“…1,6,9,16,17,19,21) The lesions are frequently localized in the mid or lower thoracic regions. 1,4,5,10,21) In the present case, the appearance of the tumors on MR imaging and the known presence of an underlying hematological condition suggested the diagnosis of extramedullary hematopoiesis. The differential diagnosis of an extensive epidural mass includes epidural hematoma, infection (abscess), extramedullary hematopoiesis, and neoplasms such as metastasis.…”

Section: Discussionmentioning

confidence: 87%

Spinal Cord Compression Due to Extramedullary Hematopoiesis Associated With Polycythemia Vera. Case Report.

Ohta¹,

Shichinohe

Nagashima

2002

Neurol. Med. Chir.(Tokyo)

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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.

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Section: Discussionmentioning

confidence: 87%

Spinal Cord Compression Due to Extramedullary Hematopoiesis Associated With Polycythemia Vera. Case Report.

Ohta¹,

Shichinohe

Nagashima

2002

Neurol. Med. Chir.(Tokyo)

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“…The rare complication of cord compression due to extramedullary hematopoiesis has most often been reported in patients with thalassemia. Cord compression caused by extramedullary hematopoiesis has also been reported in cases of myelofibrosis, sickle cell anemia and polycythemia vera (3)(4)(5) . In our case, thoracic cord compression was due to extramedullary hematopoiesis caused by AML that is rarer.…”

Section: Discussionmentioning

confidence: 99%

Spinal Cord Compression Due to Epidural Extramedullary Haematopoiesis in Acute Myeloid Leukemia: Mri Findings

et al. 2008

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A 32 year-old-man with a history of acute myeloid leukemia on remission for one year presented with sudden back pain, weakness and reduced sensation in both legs, and urinary incontinence that had progressed over one weak. MRI of thoracic and lumbar spine was performed on a 1.5 T system using a body coil due to his neurological symptoms. T1-weighted sequence (TR/TE: 617/15 mse) and T2-weighted sequence (TR/TE: 4300/110 mse) on sagittal and axial images with 3 mm slice thickness were used. Gadolinium enhanced T1-weighted axial images were obtained. Magnetic resonance imaging (MRI) demonstrated spinal cord compression by epidural extramedullary hematopoietic tissue. Spinal epidural space is a very rare site for extramedullary hematopoiesis.

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“…In small series of patients palliative doses of RT (15-30 Gy) divided into multiple fractions successfully reversed spinal cord compression [5], while comparatively low doses are adequate in the treatment of EMH-associated pleural (1-2 Gy) [6] or peritoneal (10-12 Gy) [7] effusion. Abdominal pain and discomfort due to hepatomegaly may occur in advanced MMM after splenectomy because EMH relocates from the spleen into the liver.…”

Section: Discussionmentioning

confidence: 99%

Repeated and Preemptive Palliative Radiotherapy of Symptomatic Hepatomegaly in a Patient with Advanced Myelofibrosis

Gmür¹,

Lütolf²

2008

Onkologie

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Background: Patients with advanced myelofibrosis often suffer from symptomatic extramedullary hematopoiesis in spleen and/or liver. In case of drug-refractory disease splenomegaly is treated surgically, whereas hepatomegaly is palliated by radiotherapy (RT). Case Report: A 56-year-old man with advanced and drug-refractory myelofibrosis suffered from extensive hepatomegaly with severe upper abdominal pain, satiety, weight loss, and fatigue 1.5 years after splenectomy. The patient was treated periodically with fractionated RT to the liver in order to obtain symptom control and to prevent severe symptom recurrence. Results: After 2 Gy fractionated RT to a treatment field encompassing nearly the whole liver, symptoms improved and liver size decreased without severe side effects. This treatment regimen was successfully conducted 3 times in trimonthly intervals. Because symptoms recurred periodically, we then continued RT on a preemptive basis in monthly intervals and with single-dose irradiation. The patient responded well to 1 Gy preemptive single-dose RT to the liver, but not to 0.5 Gy single-dose partial liver irradiation. Conclusion: RT is effective in palliation of hepatomegaly in advanced myelofibrosis. Even preemptive RT can benefit selected patients with advanced disease and periodical recurrence of symptoms.

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Progressive paraparesis due to thoracic extramedullary hematopoiesis in myelofibrosis

Abstract: A patient with myelofibrosis who developed a progressive paraparesis caused by spinal cord compression due to thoracic extramedullary hematopoietic tissue is reported. He recovered well after local radiotherapy alone.

Cited by 38 publications

References 10 publications

Spinal Cord Compression Due to Extramedullary Hematopoiesis Associated With Polycythemia Vera. Case Report.

Spinal Cord Compression Due to Extramedullary Hematopoiesis Associated With Polycythemia Vera. Case Report.

Spinal Cord Compression Due to Epidural Extramedullary Haematopoiesis in Acute Myeloid Leukemia: Mri Findings

Repeated and Preemptive Palliative Radiotherapy of Symptomatic Hepatomegaly in a Patient with Advanced Myelofibrosis

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