Spinal cord compression in b-thalassemia: follow-up after radiotherapy

Authors

  • Silvana Fahel da Fonseca SPMJ
  • Maria Stella Universidade Federal de São Paulo
  • Rodolfo Delfini Cançado SPMJ
  • Fernando Nakandakare SPMJ
  • Roberto Segreto SPMJ
  • José Kerbauy SPMJ

Keywords:

Extramedullary hematopoiesis, Spinal cord compression, Intermediate b-thalassemia

Abstract

CONTEXT: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including b-thalassemia. CASE REPORT: We report the case of a patient with intermediate b-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. DISCUSSION: Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms.

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Author Biography

Maria Stella, Universidade Federal de São Paulo

Disciplina de Hematologia e Hemoterapia UNIFESP - EPM

References

Gemenis T, Philippou A, Gouliamos A, et al. Atypical location of extramedullary hematopoietic masses in thalassemia. Radiologe 1989;29:295-6.

Konstantopoulos K, Vagiopoulos G, Kantouni R, et al. A case of spinal cord compression by extramedullary haematopoiesis in a thalassaemic patient: a putative role for hydroxyurea? Haematologica 1992;77:352-4.

Singounas EG, Sakas DE, Hadley DM, et al. Paraplegia in a pregnant thalassemic woman due to extramedullary hematopoiesis: successful management with transfusions. Surg Neurol 1991;36:210-5.

Gatto I, Terrana V, Biondi L. Compressione submidollo spinale da proliferazions di midollo osseo nello spazio epidurale in sogetts affeto da malattia di Colley splenectomizzato. Haematologica 1954;38:61-76.

Coraddu M, Floris F, Meleddu V, et al. Compression médullaire et de la queue de cheval chez deux patients atteints de béta-thalassémie intermédiaire. Neurochirurgie 1991;37:58-60.

Kaufmann T, Coleman M, Giardina P, Nisce LZ. The role of radiation therapy in the management of hematopoietic neurologic complications in thalassemia. Acta Haematol. 1991;85:156-9.

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Published

1998-11-11

How to Cite

1.
Fonseca SF da, Maria Stella, Cançado RD, Nakandakare F, Segreto R, Kerbauy J. Spinal cord compression in b-thalassemia: follow-up after radiotherapy. Sao Paulo Med J [Internet]. 1998 Nov. 11 [cited 2025 Oct. 16];116(6):1879-81. Available from: https://periodicosapm.emnuvens.com.br/spmj/article/view/2380

Issue

Section

Case Report