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CASE REPORT
Year : 2007  |  Volume : 10  |  Issue : 2  |  Page : 115-117
 

Extramedullary haematopoiesis causing spinal cord compression: A rare presentation with excellent outcome


Department of Radiation Oncology, Tata Memorial Hospital, Mumbai - 400012, India

Correspondence Address:
Siddhartha Laskar
Department of Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.33221

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   Abstract 

Extramedullary haematopoiesis (EMH) as a cause of spinal cord compression in patients with thalassaemia major is very rare. Treatment of EMH includes the use of radiotherapy, hypertransfusion, laminectomy and chemotherapy in the form of hydroxyurea We report a presentation of progressive paraparesis due to a sacral mass in a 7 year old child undergoing repeated blood transfusions for thalassaemia major who had a dramatic improvement in symptoms after radiotherapy. A standard recommendation regarding the radiation dose required is not available but a dose of 20Gy delivered over 10 fractions seems to be effective and well tolerated.


Keywords: Extramedullary haematopoiesis, radiotherapy, spinal cord compression


How to cite this article:
Talapatra K, Nemade B, Siddha M, Muckaden MA, Laskar S. Extramedullary haematopoiesis causing spinal cord compression: A rare presentation with excellent outcome. Ann Indian Acad Neurol 2007;10:115-7

How to cite this URL:
Talapatra K, Nemade B, Siddha M, Muckaden MA, Laskar S. Extramedullary haematopoiesis causing spinal cord compression: A rare presentation with excellent outcome. Ann Indian Acad Neurol [serial online] 2007 [cited 2019 Dec 9];10:115-7. Available from: http://www.annalsofian.org/text.asp?2007/10/2/115/33221



   Introduction Top


Extramedullary haematopoiesis (EMH) is associated with many haematological disorders such as thalassaemia, polycythemia rubra vera, myelofibrosis and other haemoglobinopathies and commonly occur in the spleen and liver.[1] EMH as a cause of spinal cord compression in patients with thalassaemia is very rare and was first described by Gatto et al .[2] Treatment of EMH includes the use of radiotherapy, hypertransfusion, laminectomy and chemotherapy in the form of hydroxyurea. Radiotherapy has resulted in excellent responses in patients with spinal cord compression due to EMH.[1],[3] A significant number of patients reported in literature present with progressive paraparesis[4],[5] whereas a few present with sphincter disturbance.[5] We report a child with thalassaemia presenting with bladder and bowel incontinence along with lower extremity weakness as a result of spinal cord compression due to EMH in the sacral region treated with radiotherapy with excellent outcome.


   Case Report Top


A seven years old child diagnosed to have thalassaemia major six years ago, presented with history of bowel and bladder incontinence of two months' duration, weakness of right lower limb of a month's duration and inability to walk. He had been receiving regular blood transfusions for the past six years. On clinical examination he was found to have diminished power in the right, flexors of ankle (2/5) and extensors of hip (grade 2/5) with loss of bladder and bowel control.The plantar reflex on the right lower limb was absent. Patient had perianal sensory loss. Rest of the clinical examination did not reveal any abnormality. His haemoglobin was 10.2 gm%, platelet count was 2.52 lakhs and rest of CBC and biochemistry was normal. MRI of the spine revealed diffuse hypointensity of the axial skeleton with mild expansion and signal changes in S1 vertebra associated with diffuse epidural soft tissue partially encasing the nerve roots within the sacral canal consistent with iron deposits [Figure - 1]. His chest radiograph was normal. CSF studies did not reveal any abnormality. No nerve conduction studies were done on this patients. He was treated with external beam radiation therapy to the area of spinal cord compression delivering a dose of 19.9Gy in 12 fractions over 17 days (@ 1.6Gy per fraction using AP/PA portals prescribed at midplane. The portal size was 7x7 centimetres planned to cover the soft tissue mass adequately. There was significant improvement in the neurological status within a week of starting radiotherapy with reversal of loss of bowel and bladder control. He was able to walk without support at completion of treatment. On his first follow up after three months he had complete recovery of power and a follow up scan showed complete resolution of the soft tissue mass.


   Discussion Top


Paravertebral pseudotumours due to extramedullary haematopoiesis in patients with thalassaemia may be caused as a result of ineffective erythropoiesis.[6] These masses have the risk of development of severe neurological damage due to the proximity to the spinal canal.[6]

The disorders where EMH is commonly seen are thalassaemia, polycythemia rubra vera, myelfibrosis and other haemoglobinopathies.[1] The common sites of EMH are the liver, spleen and lymph nodes. It has also been seen in the adrenal glands, kidneys, breasts, dura mater, adipose tissue and skin.[1],[6]

EMH has been postulated to be due to direct extension from adjacent vertebral bone marrow and development of extramedullary haematopoietic tissue from branches of the intercostal veins. The thoracic spine predisposes to spinal cord compression frequently due to narrow central canal and limited mobility of the thoracic spine.[2]

History and clinical examination clinch the diagnosis of EMH. At times it may be difficult to differentiate EMH from epidural lymphoma. History is important as the patient would generally have a predisposing haematological condition.[7] A significant number of patients reported in literature present with progressive paraparesis[4],[5] whereas a few present with sphincter disturbance.[5] MRI is currently the gold standard for demonstrating spinal EMH which produces superior soft tissue delineation and is highly sensitive.[2] It has minimal gadolinium enhancement, differentiating it from other epidural lesions such as abscesses or metastases.[8] Computed tomography (CT) can be done for patients for whom MRI is unavailable. CT-guided needle biopsy should be avoided as it carries the risk of catastrophic hemorrhage. TC 99 m bone scanning has been reported to diagnose paraspinal EMH, but the diagnosis within the epidural space may be difficult due to proximity to bone marrow.[9]

In our patient the diagnosis of extramedullary haematopoiesis was made in view of the background of thalassaemia major and and an epidural soft tissue mass in the sacral canal consistent with iron deposits on MRI. Biopsy was avoided in view of the possibility of a catastrophic bleed.

Various treatment modalities for EMH include hypertransfusion, radiotherapy and laminectomy.[6] Hydroxyurea which stimulates fetal hemoglobin synthesis has shown promise as a treatment modality.[6] Radiotherapy is very effective as the primary treatment of EMH or as combined modality therapy. The optimal dose of radiation has not yet been established in dose-response studies and there are no standard guidelines or recommendations available regarding the dose fractionation schedule in EMH. However, haematopoietic tissue is known to be very radiosensitive. In a review by Munn et al , the authors reported total radiation doses ranging from 7.5Gy to 35Gy given for EMH resulting in average relapse rates (RR) of 37.5% in patients with at least one year of follow-up. The authors also reported the efficacy of a second course of irradiation for patients with recurrent cord compression after a course of radiation (dose range 14-21Gy).[1] Jackson et al reported a 7 year follow up on a patient without neurological deficit and recommended that low-dose radiotherapy should be considered as a primary treatment modality for the management of this syndrome.[3] Ohta et al reported a 69-year-old woman with a 14-year history of polycythemia vera suffered progressive paraparesis due to epidural involvement of haematopoietic tissue having almost complete clinical recovery with decompressive surgery and radiotherapy.[4] Munn et al have reported improvement in lower limb weakness, ambulation and no further neurologic deficit in 5 years of follow-up in a patient presenting with spinal cord compression secondary to EMH in a patient with beta-thalassaemia intermedia receiving 20Gy over 10 fractions of radiotherapy.[1],[10]

Our patient had a dramatic improvement in neurological symptoms while on treatment with radiotherapy. There was complete recovery of bowel and bladder control and he was ambulatory at the end of the first week of radiotherapy.

He had an excellent quality of life at his follow up at 9 months and the maintained the neurological recovery clinically.

Radiotherapy results in excellent improvement of neurological symptoms associated with EMH thereby improving the patient's quality of life in well selected patients.If the doses are kept within the range 18-20 Gy,chances of growth related problems are minimal. A standard recommendation regarding the radiation dose required is not available but a dose of 20Gy delivered over 10 fractions seems to be effective and well tolerated.

 
   References Top

1.Munn RK, Kramer CA, Arnold SM. Spinal cord compression due to Extramedullary hematopoiesis in beta thalassemia intermedia. Int J Radiat Oncol Biol Phys 1998;42:607-9.   Back to cited text no. 1    
2. Tan TC, Tsao J, Cheung FC. Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia. J Clin Neurosci 2002;9:721-5.  Back to cited text no. 2    
3.Jackson DV Jr, Randall ME, Richards F. Spinal cord compression due to extramedullary hematopoiesis in thalassemia: Long-term follow-up after radiotherapy. Surg Neurol 1988;29:389-92.  Back to cited text no. 3    
4.Ohta Y, Shichinohe H, Nagashima K. Spinal cord compression due to extramedullary hematopoiesis associated with polycythemia vera: Case report. Neurol Med Chir 2002;42:40-3.  Back to cited text no. 4    
5.Rey J, Gagliano R, Christides C, Pillard E, Magnan F, Tourniaire P, et al . Spinal cord compression caused by extramedullary hematopoiesis foci in the course of thalassemia. Presse Med 2001;30:1351-3.  Back to cited text no. 5    
6.Cario H, Wegener M, Debatin KM, Kohne E. Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis. Ann Hematol 2002;81:478-82.  Back to cited text no. 6    
7.Alorainy IA, Al-Asmi AR, del Carpio R. MRI features of epidural extramedullary hematopoiesis. Eur J Radiol 2000;35:8-11.  Back to cited text no. 7    
8.Chourmouzi D, Pistevou-Gompaki K, Plataniotis G, Skaragas G, Papadopoulos L, Drevelegas A. MRI findings of extramedullary haemopoiesis. Eur Radiol 2001;11:1803-6.  Back to cited text no. 8    
9.Saghafi M, Shirdel A, Lari SM. Extramedullary hematopoiesis with spinal cord compression in β -thalassemia intermedia. Eur J Intern Med 2005;16:596-7.  Back to cited text no. 9    
10.da Fonseca SF, Figueiredo MS, Cancado RD, Nakandakare F, Segreto R, Kerbauy J. Spinal cord compression in beta-thalassemia: Follow-up after radiotherapy. Sao Paulo Med J 1998;116:1879-81.  Back to cited text no. 10    


    Figures

  [Figure - 1]


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