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Table of Contents    
CASE REPORT
Year : 2012  |  Volume : 60  |  Issue : 3  |  Page : 328-331

Paraplegia treated with blood transfusion


1 Department of Neurology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
2 Department of Radiology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
3 Department of Medicine, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India

Date of Submission24-May-2012
Date of Decision24-May-2012
Date of Acceptance31-May-2012
Date of Web Publication14-Jul-2012

Correspondence Address:
Ajoy Sodani
Department of Neurology, Sri Aurobindo Medical College and PG Institute, Indore-Ujjain Highway, Near MR 10 Junction, Indore 453111, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.98528

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 » Abstract 

Extramedullary hematopoiesis is a compensatory response in patients with thalassemia and other chronic anemia and can result in compressive myelopathy, if untreated. Two young adults with history of thalassemia presented with symptoms of spinal cord compression. Presence of extramedullary hematopoiesis was confirmed by magnetic resonance imaging. Both the patients were treated with blood hypertransfusion and showed improvement clinically and radiologically. Although there are various options in the management of such condition, including decompression surgery and radiation treatment, hypertransfusion can be very effective even in severe compression of the spinal cord. Hypertransfusion should be tried as the first line of management in patients with thalassemia presenting with compressive myelopathy to decrease the bulk of extramedullary hematopoietic tissue.


Keywords: Compressive myelopathy, extramedullary hematopoiesis, hemochromatosis, thalassemia


How to cite this article:
Sodani A, Chawla A, Maheshwari A, Naigaonkar M, Porwal V. Paraplegia treated with blood transfusion. Neurol India 2012;60:328-31

How to cite this URL:
Sodani A, Chawla A, Maheshwari A, Naigaonkar M, Porwal V. Paraplegia treated with blood transfusion. Neurol India [serial online] 2012 [cited 2021 Sep 17];60:328-31. Available from: https://www.neurologyindia.com/text.asp?2012/60/3/328/98528



 » Introduction Top


Extramedullary hematopoiesis (EMH) is commonly seen in patients with thalassemia. [1] When EMH occurs in the tissue in and around spinal canal, it may result in cord compression. This is an uncommon complication of thalassemia, for which various modes of treatment have been used in the past. [2] We report two cases of thalassemia intermedia who presented with paraparesis and were managed with blood hypertransfusion.


 » Case Reports Top


Case 1

A 24-year-old male, a known case of thalassemia intermedia, was receiving regular blood transfusion since the age of 4 years. Three years prior to admission, he stopped taking blood transfusion on his own. He noticed weakness in lower limbs and change in gait after walking for 10-15 min since 6 months. The disability was slowly progressive and forced him to stop and take rest after walking for a short distance. It was not associated with sensory symptoms. Eight days prior to admission, lower limb weakness rapidly progressed to make him bedridden. There were no bladder and bowel symptoms. Examination revealed massive splenomegaly and mild hepatomegaly. He was unable to walk due to severe spasticity and weakness in lower limbs (grade 3/5 at hip, 3/5 at knee, and 4/5 at ankles). Deep tendon reflexes in the lower limbs were exaggerated with ill-sustained right patellar clonus and bilateral ankle clonus. Plantar response was extensor bilaterally. Pain sensation was diminished below D9 and proprioception was reduced up to ankle joint. No bony tenderness was present on spine. Blood picture showed hemoglobin (Hb) of 7.5 gm/dl (HbA1 62.6%, HbA2 3%, HbF 34.4%, mean corpuscular volume (MCV) 77.5 fl, mean corpuscular hemoglobin (MCH) 23.4 pg, mean corpuscular hemoglobin concentration (MCHC) 30.1 gm%), with 4% reticulocytes and no premature cells. Magnetic resonance imaging (MRI) of dorsal spine revealed extramedullary soft tissue masses in dorsal spine, causing severe compression of the spinal cord [Figure 1] and [Figure 2]. The cord was flattened and showed bright T2 signals suggestive of cord edema or ischemia. The posterior end of ribs was widened. MRI also showed diffuse loss of signals from axial skeleton due to retroconversion of marrow. There was marked hepato-splenomegaly with signal loss in liver and spleen, suggesting iron deposition.
Figure 1: Case 1. Sagittal T2W image of dorsal spine shows large extramedullary masses in dorsal spinal canal, causing severe compression of the cord

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Figure 2: Axial T1W image at D8– D9 level shows paraspinal and extramedullary masses compressing the cord. The white arrow shows flattened cord. Note the expanded ribs

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Hypertransfusion of blood was started. Standard doses of desferroxamine as iron chelating agent were also administered to treat associated hemochromatosis. Patient showed dramatic response to hypertransfusion and was ambulatory with minimal support after the third transfusion. He was discharged after 10 days with mild residual spasticity and minimal disability. He was followed at weekly interval and transfused blood to maintain Hb level above 10 gm/dl. Eight weeks after the initial presentation, repeat MRI showed significant decrease in the size of extramedullary hematopoietic tissue as well as reduction in the compression of dorsal cord [Figure 3] and [Figure 4].
Figure 3: Post-treatment sagittal T2W image of dorsal spine shows decrease in the size of extramedullary masses

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Figure 4: Post-treatment axial T1W image at D8– D9 level shows decrease in the size of paraspinal and extramedullary masses. White arrow points at relatively oval appearing cord

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Case 2

A 25-year-old male presented to emergency department with progressive difficulty in walking of 20 days duration with rapid deterioration over the last 8 days. He was a diagnosed case of thalassemia intermedia and on blood transfusion, but was not taking regular transfusion due to financial constraints. He started having numbness near umbilical area 20 days before admission with difficulty in walking in the form of unsteadiness. This gradually progressed, and few days prior to presentation, he became bedridden. No symptoms pertaining to upper limbs, bladder, and bowel were reported. Examination showed pallor, ashy skin hue, mild splenomegaly, and mild hepatomegaly. Neurological examination revealed increased tone in lower limbs and motor power of grade 2/5 in right hip and grade 3/5 in right knee and ankle. Left lower limb had a power of grade 3/5 in all muscle groups. Deep tendon reflexes were exaggerated while abdominal reflexes were absent. Bilateral plantars were extensor. He had decreased sensation to pinprick and proprioception in the lower extremities below D4 level. Investigation showed an Hb of 5.1 gm/dl. MRI of dorsal spine showed soft tissue masses in paravertebral region and epidural space of dorsal spine, causing severe compression of dorsal cord [Figure 5] and [Figure 6]. The compressed cord itself showed T2 bright signals. He was also treated with hypertransfusion and iron chelating agent to maintain Hb level near 10 gm/dl. He reported dramatic improvement after completion of the fourth transfusion. He started walking without any assistance on day 10 of admission. He was discharged after 2 weeks and followed up in neurology outdoor clinic. A follow-up MRI done 4 weeks after the initial admission showed significant reduction in the cord compression and EMH tissue [Figure 7] and [Figure 8].
Figure 5: Case 2. Sagittal T2W image of dorsal spine shows severe compression of dorsal cord secondary to extramedullary masses

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Figure 6: Axial T2W image at D7 level shows bilateral paraspinal masses. The dorsal cord is compressed by extramedullary masses. The compressed cord shows T2 bright signals (white arrow)

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Figure 7: Post-treatment sagittal T2W image reveals significant decrease in the size of extramedullary masses

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Figure 8: Post-treatment axial T2W image at D7 level. It is to be noted that the T2 bright signals in compressed cord (white arrow) have decreased, along with compression

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 » Discussion Top


EMH is a common occurrence in thalassemia, but the associated neurological complications particularly related to spinal cord are rare. The available literature suggests that this complication usually occurs in diagnosed cases of thalassemia and the presentation varies from acute onset with rapid deterioration within 24 h to indolent onset with slow progression over several months. Though back pain is not a very consistent symptom, when present, it could be nonspecific low back pain resistant to symptomatic treatment [3] and could present with leg pains with rapid deterioration. [4] A patient with history of weakness and tiredness brought out by walking, as in our patients, has been described. [5] The patient reported by Singhal et al. [5] had one-level spinal cord compression on myelography. In our patients, MRI showed diffuse involvement of the spinal canal, but was worse in dorsal region.

Review of English literature from 1954 to 2009 revealed that of the total 59 reported cases, [3],[4],[5] only 4 patients were treated with hypertransfusion alone and all of them showed fairly good response as judged by full recovery of the neurological deficit. [6],[7],[8] In all other instances, surgical removal of the offending tissue remained the core treatment with use of radiotherapy and blood transfusion as and when required. Both our patients showed extensive involvement of dorsal spine, so surgery and extensive radiotherapy seemed impractical. Since EMH is a physiological response to severe anemia and resultant generalized hypoxia, [7] removal of such tissue may actually be detrimental, as it will deny the patient of his/her only hope to fight future episode of hypoxemia. Besides, radiotherapy itself could lead to radiation myelitis with near-permanent neurological complications. Furthermore, EMH is a process that may recur at variable sites in future as the underlying disease itself is chronic and not curable. Therefore, radiotherapy or surgery does not stand to reason as a primary choice of treatment.

Sustained clinical remission in our patients was accompanied by significant radiological improvement, which can be taken as an evidence that hypertransfusion addresses the key problem which leads to EMH and resultant cord compression. It is therefore suggested that hypertransfusion therapy should take precedence over other interventions when treating compressive myelopathy in patients of thalassemia due to EMH.

 
 » References Top

1.Al-Habib H, Hadzikaric N. Spinal cord compression due to intraspinal extramedullary hematopoesis in thalassemia intermedia. Neurosciences 2007;12:261-4.  Back to cited text no. 1
[PUBMED]    
2.Malik M, Pillai LS, Gogia N, Puri T, Mahapatra M, Sharma DN, et al. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. Haematologica 2007;92:e28-30.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Moncef B, Hafedh J. Management of spinal cord compression caused by extramedullary hematopoiesis in beta-thalassemia. Intern Med 2008;47:1125-8.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Cross JN, Morgan OS, Gibbs WN, Cheruvanky I. Spinal cord compression in thalassaemia. J Neurol Neurosurg Psychiatry 1977;40:1120-2.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Singhal S, Sharma S, Dixit S, De S, Chander S, Rath GK, et al. The role of radiation therapy in the management of spinal cord compression due to extramedullary haematopoiesis in thalassaemia. J Neurol Neurosurg Psychiatry 1992;55:310-2.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Dewan U, Kumari N, Jaiswal A, Behari S, Jain M. Extramedullary hemopoiesis with undiagnosed, early myelofibrosis causing spastic compressive myelopathy: Case report and review. Indian J Orthop 2010;44:98-103.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.Taher AT, Musallam KM, Cappellini MD. Thalassaemia Intermedia: an Update. Mediterr J Hematol Infect Dis 2009;1:e2009004. Available from: http://www.mjhid.org/article/view/4709 [Last accessed on 2012 May 10].  Back to cited text no. 7
    
8.A, Aoun E, Koussa S, Skoury H, Koussa S, Taher A. Hypertransfusion: A successful method of treatment in thalassemia intermedia patients with spinal cord compression secondary to extramedullary hematopoiesis. Spine 2003;28:E245-9.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]

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