The role of radiation therapy in the management of spinal cord compression due to extramedullary haematopoiesis in thalassaemia.

Abstract

Extramedullary haematopoiesis associated with thalassaemia leading to spinal cord compression is an extremely rare event in the course of the disease. The efficacy of radiation therapy is advocated in the management of such a complication. Two patients with thalassaemia, who had presented with spinal cord compression, were successfully treated by a modest dose of local radiotherapy. In one of the patients, however, radiotherapy was resorted to after an initial decompressive laminectomy and partial removal of the intraspinal haematopoietic mass proved unsuccessfil. The other patient was managed solely by radiation therapy.Extramedullary haematopoiesis (EMH) is a common manifestation of severe thalassaemia and it may involve various organs of the body such as liver, spleen, and lymph nodes.'-6 The clinical features secondary to EMH are variable and depend on the site of formation of the haematopoietic tissue. The onset of unexpected clinical signs may be the first indication that haematopoiesis is spreading to an atypical area. Spinal cord compression (SCC) as a consequence of EMH in the intraspinal epidural space is an extremely rare complication. We report two cases of thalassaemia that had presented with SCC. A review of the literature is discussed highlighting the role of radiation therapy in the management of such patients.and there was a complete loss of touch and pinprick sensation below the level ofT7.The radiograph of the skull and spine demonstrated a diffuse osteoporosis but no well defined paravertebral mass was evident. Myelogram, however, revealed a complete extradural block at the level of T9. The CSF examination proved noncontributory. His haemoglobin (Hb) was 8-7 g/dl, haematocrit 28% and white blood cell count (WBC) 7.4 x 109/L. The differential count revealed a slight shift to the left (metamyelocyte 2%, myelocyte 2%). Platelets were adequate. The peripheral smear showed anisopoikilcytosis with microcytosis and hypochromia. A few target cells were also noticed. There were three normoblasts/100 WBC. The corrected reticulocyte count was 2%. A haemoglobin electrophoresis showed 21% Hb F and 2-6% Hb A2.At laminectomy a brownish, soft, vascular extradural mass was seen extending from T5-T9. Only a partial excision of the mass could be accomplished. The histopathological examination of the excised mass confirmed it to be haematopoietic tissue. The postoperative course was uneventful. However, in view of the incomplete excision of the mass and a negligible neurological recovery, radiotherapy was started two weeks after surgery. A total dose of 30 Gy was delivered by a telecobalt unit, over a period of three weeks in 15 sessions. There was a marked neurological improvement at the end of radiation treatment and the patient continued to improve. No complication was observed during or after radiotherapy. The patient has been doing well for a period of five years.

Thoracic spinal cord compression due to xtramedullary haemopoiesis in a patient with beta-thalassemia: complete clinical regression with radiation therapy alone

“…Improvement is clinically evident after an average of 3-7 fractions of radiotherapy and near complete recovery is generally observed by the end of treatment. 13 Doses used have ranged from 750-3500 cGy. 8 With these low doses, the only significant toxicity that may occur is a further decrease in blood cell counts which need to be frequently monitored.…”

Spinal Cord Compression due to Extra Medullary Hematopoiesis in a Case Of Thalassemia Minor

“…However it is also known to occur in other organs like pleura, adrenals, breast, thymus, kidneys, gastrointestinal tract, intracranial structures, retroperitoneal tissue, sweat glands, prostate, broad ligaments, peripheral nerves, heart, retroperitoneal fat, Para spinal regions. [1,2,5] . Para spinal location of EMH has been noted in 11-15% cases.…”

“…Surgical treatment Immediate release of SCC Availability of tissue for histopathological diagnosis [4] Difficulty in complete resection due to diffuse nature [2] Increased bleeding due to highly vascular nature of the lesion [4] Sudden clinical deterioration as these tissues are responsible in maintaining the necessary hemoglobin levels [4] Likelihood of recurrence [4,7] Radiation Hematopoetic tissue is highly sensitive to ionizing radiation [2] Anemia may get aggravated in some cases due to decreased hematopoesis in surrounding tissue [1] Edema of spinal cord( could be reduced by simultaneous administration by steroids) [4] Recurrences [4,6] …”

“…HT Therapeutic as well as diagnostic use as EMH is the only condition in which patient will obtain relief due to HT [1,2] Where both surgical management and RT is not possible like in pregnancy [4] Transfusion transmitted infections, Iron overload Alloimmunisation Recurrences [4] HU Myelosuppresion [1] Decrease in globin chain imbalance.…”

“…The 1 st case of spinal cord compression (SCC) due to EMH in a patient with Thalassemia was reported by Gatto in 1954 , while the 1 st Indian case was reported by Prabhakar et al in 1980. [2] Due to rarity of this entity, there is no definite consensus regarding the treatment for this condition which includes several options like surgical resection, Radiotherapy(RT), Hypertransfusion (HT), Hydroxyurea (HU) administration or a combination of the above. [1 ] We present a case of a 43 year old male who presented with low backache, bilateral lower limb weakness and icterus in whom EMH was suspected on intraoperative squash preparation and subsequently proven on histopathology and immunohistochemistry (IHC).…”