A case of paraparesis due to extramedullary haemopoiesis in HbE-thalassaemia

Mihindukulasuriya, J. C.; Chanmugam, D.; Machado, Vânia Maria Vasconcelos; Samarasinghe, C. A.

doi:10.1136/pgmj.53.621.393

Cited by 20 publications

(13 citation statements)

References 6 publications

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“…A possible explanation for this phenomenon may consist in the rather long period of time required by the compensatory proliferative mechanism to become symptomatic and cause cord compression [4], Close et al [ 10] T5-T12 radiotherapy Sorsdahl et al [9] intermedia T5-T8 total none Hongladarom et al [7] T7-T9 none Cauthen et al [6] intermedia T4-T8 total none intermedia T4-T8 subtotal radiotherapy Luyendijk et al [18] homozygous T5-T7 subtotal radiotherapy Cross etal. [11] intermedia T4-T9 subtotal radiotherapy Cross et al [8] intermedia T6 subtotal radiotherapy Mihindukulasurya et al [8] T6 total none Prabhakar et al [ 1 ] homozygous T7-TI0 subtotal radiotherapy Issaragrisil et al [16] T4-T11 radiotherapy Abassioun and Amir-Jamshidi [3] T4-T11 subtotal radiotherapy Luitjes et al [ 17] homozygous T4-T8 subtotal radiotherapy David and Balasubramaniam [14] T4-T8 subtotal radiotherapy Ibrahim et al [5] homozygous T4-T8 subtotal none Mann et al [12] heterozygous T3-T8 subtotal transfusional Amirjamshidi et al [4] homozygous T9-TI0 total transfusional intermedia T5-T1I subtotal radiotherapy Present study intermedia T3-T8 total transfusional There is still a controversy regarding the optimal man agement of these patients. Modes of therapy used in the past include surgical excision of the mass, radiotherapy, and overtransfusion, or various combinations of the above.…”

Section: Discussionmentioning

confidence: 99%

Spinal Cord Compression in Homozygous Beta-Thalassemia Intermedia

Cardia¹,

Toscano²,

Rosa³

et al. 1994

Pediatr Neurosurg

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Symptomatic spinal cord compression caused by an epidural mass of extramedullary hematopoietic tissue in patients with β-thalassemia is a rare occurrence, that becomes exceptional in childhood and adolescence. The literature is not uniform about the optimal treatment of these patients and different modes of therapy have been proposed so far, including surgical excision or decompressing laminectomy followed by local irradiation and/or hypertransfusional regimens. We report the successful treatment of such a complication in an adolescent with homozygous β-thalassemia by surgery followed by repeated blood transfusions aimed at maintaining hematocrit at normal levels.

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Section: Discussionmentioning

confidence: 99%

Spinal Cord Compression in Homozygous Beta-Thalassemia Intermedia

Cardia¹,

Toscano²,

Rosa³

et al. 1994

Pediatr Neurosurg

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“…For this reason, it is not possible to compare the long term results in these patients. Six authors pointed out the serious problems associated with stopping bleeding [1,14,18,21,23,29]. A multiple distribution of the hematopoietic foci (in our case, mid-thoracic to sacral) is a surgical obstacle.…”

Section: Discussionmentioning

confidence: 79%

“…Four authors [14,21,23,29] achieved good results with surgery and did not, give radiation treatment. All of the others carried out additional radiation therapy [1,3,6,7,9,18,19,27].…”

Section: Discussionmentioning

confidence: 94%

Spinal space occupying lesions in thalassemia major

Reif

Graf

1989

Neurosurg. Rev.

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A 17 year old patient suffering from thalassemia, who had been dependent on transfusions since the age of three, showed an increasing incomplete sensory and motor transverse cord lesion below T-10. The causes were found to be multiple epidural erythropoietic foci, confirmed by myelography, CT, and biopsy. The lesions were located in the thoracic, lumbar, and sacral regions. Complete remission of the neurological symptoms was achieved by an immediate hypertransfusion regimen and subsequent local radiation therapy of the neuroaxis. Thirty-six cases of thalassemia and spinal space occupying lesions have been reported in the literature. The therapeutic results of these are presented and compared with our findings.

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“…Any of the fetal blood forming centres may thus be activated but the organs most commonly involved are liver, spleen and the lymph nodes. [1][2][3][4][5][6] It occurs rarely in the kidney, breast, heart, adrenals, pleura, retroperitoneal fat, para vertebral gutter and ribs.235 EMH in the spinal epidural space is uncommon and the development of SCC as a consequence of intraspinal EMH is an extremely rare complication in thalassaemic patients.Though this complication has been reported relatively more commonly in thalassaemia compared with various other haematological disorders, its occurrence is still rare.5 7 Logothetis et al8 in a survey of 138 cases of thalassaemia major were unable to find a single case with SCC. Prabhakar et al9 found only one case presenting with SCC in a survey of 120 thalassaemic patients.…”

Section: Discussionmentioning

confidence: 99%

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

Singhal

Sharma

Dixit

et al. 1992

Journal of Neurology, Neurosurgery & Psychiatry

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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.

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A case of paraparesis due to extramedullary haemopoiesis in HbE-thalassaemia

Cited by 20 publications

References 6 publications

Spinal Cord Compression in Homozygous Beta-Thalassemia Intermedia

Spinal Cord Compression in Homozygous Beta-Thalassemia Intermedia

Spinal space occupying lesions in thalassemia major

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

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