Original Article
Volume 3, No.1
April 1999
 Mondber Yedeas (1)
 Kamel Bahri (1,3)
 A. Ben Rejab (1)
 A. Monavvari (3)
 Moncef Khaldi (2)
 (1) Dept of Neurosurgery
 and Histopathology
 Military Hospital of Tunis
 (2) Dept of Neurosurgery
 Neurological Institute of
 Tunis
 (3) Dept of Neurosurgery
 Beaujon Hospital
 Paris v, France
 Correspondence:
 Dr. Kamel Bahri
 Department of Neurosurgery
 Beaujon Hospital
 92110 Clichy
 France
 Fax No:( 33) 14 739 6635  
 
Spinal Cord Compression Secondary to Extramedullary Haematopoiesis in Thalassaemia

   ABSTRACT


Extramedullary haematopoiesis (EMH) in the spinal epidural space is uncommon and the development of spinal cord compression as a consequence of intraspinal EMH is a rare complication in patients with thalassaemia. The rapid onset of neurological deficit prompts immediate neuroradiological investigations and early treatment. The prompt treatment is mandatory in view of the sequel of the cord compression and the promising response to different treatment modalities such as surgical decompression, radiation therapy and repeated blood transfusions. This report is about one patient with thalassaemia who had extradural cord compression at T5-T8 level, treated successfully with decompressive laminectomy.

Keywords: Extramedullary Haematopoiesis, Paraparesis, Spinal Cord Compression and Thalassaemia

INTRODUCTION

Thalassaemia is a genetically determined disorder of blood formation characterised by quantitative defect in the synthesis of one or more polypeptide chains of haemoglobin. (4,5,21)

Neurological consequence in thalassaemia such as Extramedullary Haematopoiesis (EMH) severe enough to cause symptomatic spinal cord compression is even rare. EMH most frequently involves liver, spleen and lymph nodes. However, other locations such as paraspinal tissue, the thymus, hilum of the kidney and retroperitoneum may be involved.(1,9,11)
The reported case is about one patient with thalassaemia who developed spinal cord compression due to mass of EMH tissue in the epidural space of the thoracic spine.

INTRODUCTION

A 37 year-old man who had previously been diagnosed as having thalassaemia intermedia at the age of 5 years was admitted to our Department of Neurosurgery for dorsal pain with weakness of both lower limbs which progressed for 2 months, reduced sensation, inability to walk (PW = 20m) and associated with urinary disturbance. On physical examination, he was pale with facial features characterizing thalassaemia; the spleen is 5 cm below the costal margins.

Neurological examination shows severe spastic paraparesis, hyperactive deep tendon reflexes with sustained clonus in the ankles; a decreased sensation to all modalities from toes up to T5 dermatoma.

A myelogram performed on the same day of admission showed a complete block at T5-T8 level with a configuration of extradural posterior compression. Computed Tomography (CT) of the spine following myelography showed anterior displacement of the spinal cord by an epidural soft tissue mass from T5-T8 area posteriorly (Fig. 1).

 
Because of the worsening of paraparesis following CT-myelography an emergency laminectomy was performed. T5-T8 laminae were removed and lobulated reddish grey and moderately bleeding tissue, located in the epidural space from T5-T8 was easily removed and a good decompression of the dura was completed.

There was no extension of this tissue into the surrounding bones (Fig. 2 and 3). The postoperative course was uneventful and complete recovery of function occurred within a few days.
  Figure 1 — CT myelogram in axial view, at the T6 level shows a large posterior extradural tissue mass, producing compression and anterior displacement of the spinal cord.

  
The histologic examination showed a haematopoietic granulomatous tissue with remarkable erythroid proliferation (Fig. 4).

 
 
 
 
Figure 2
 
Figure 3
 
Frontal and sagittal reconstruction of CT myelography show the level of the compression and the extension of the lesion.


 
 
  Figure 4 — Histologic feature of resected epidural haematopoesis: tissue with numerous darker staining red-cell precursors, and mega-karyocytes.  

DISCUSSION

In Tunisia a, ß thalassaemia and Hbe are common.(4) Extramedullary Haematopoiesis is a compensatory phenomenon that may occur when there is chronic overstimuiation of red cell production or encroachment on bone marrow spaces. (3,7)

Among all etiologies of intrathoracic paraspinal EMH, thalassaemia is the most common. However, spinal cord compression as a complication of paraspinal EMH is rare. (5,15)

EMH in thalassaernia was first described by GATTO et al in 1954. Since then further 50 cases have been reported in English literature. 9,17,29 The incidence is very low as shown by LOGOTHETICS and PRABHAKAR (Less than 1%).1 The majority of reported cases have been seen between the second and third decades of life.(2)

The pathogenesis of this disease is controversial. DAVID and BALASUBRAMANIAM attributed this to the extension of hyperplastic marrow through the thinned out trabeculae at the proximal end of the ribs. ABBASSIOUM believed that the embryonic cell that rest within the epidural space have been transformed into hematopoietic tissue.(11,17,18)

The diagnosis and differential diagnosis in thalassemic patients presenting with spinal cord compression is not very difficult with the modern imaging techniques ie. CT scan, magnetic resonance imaging (MRI). In our case, the diagnosis was based upon clinical presentation and was confirmed by CT-myelography. MRI showed clearly the site and extent of the lesion within the spinal canal, which displaced the spinal cord anteriorly, and has an advantage over CT since sagittal sections demonstrate the exact length of the lesion in the spinal canal. On T1 weighted images, EMH appears as an extramedullary mass of signal intensity slightly higher than that of the adjacent bone marrow of the vertebral bodies (LAU.SK) and Gadolinium should only be given if structural masses can not be detected on non enhanced scans or if meningeal involvement is suspected (SZE. G et al).(3,6,8,13,22)

There is still controversy regarding the optimal management of these patients. Treatment options for spinal cord compression by EMH include surgery, radiation therapy, repeated blood transfusions or a combination of these. (10,20)

Surgical decompression can provide both, a definitive histologic diagnosis and an immediate decompression to prevent permanent damage. (16,17,26) However, the results of surgery are not satisfactory because of the recurrence of the masses and the diffuse nature of the process which does not allow radical removal .(23)

Some authors recommend postoperative radiation. The radiosensitivity may also depend on the affected marrow site. In some cases radiation is given alone as the primary therapy. Advantages of treatment with radiation include the avoidance of a surgical procedure and associated risks (Cardiovascular debility due to anemia and iron overload). (12,14,19,24,25,27)

Regular transfusion treatment has been recommended for patients presenting with severe extramedullary haematopoiesis but its role as a first line treatment has not been fully studied (2,7,28)

In our opinion, surgery is the treatment of choice in the event of acute and severe neurological deficits. Radiotherapy or transfusion therapy may be reserved in cases with mild paraparesis.

CONCLUSION

Extramedullary haematopoiesis is a benign lesion and epidural location is exceptional but could lead to severe neurological complications.

An early diagnosis avoids major surgery for debilitated patients. Radiotherapy and blood transfusions can be regarded as the initial form of management in less severe spinal cord compression by EMH.

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