Literature DB >> 21705991

Paraplegia in a thalassaemic patient with short stature.

Saveria Campisi1, Antonino Mangiagli, Vincenzo De Sanctis, Michela Giovannini.   

Abstract

Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that occurs in almost all chronic hemolytic anemia, especially in transfusion independent thalassemia intermedia, and can involve many organs or tissues, including the epidural space leading to spinal cord compression syndrome. We present a case of EMH in a 29 year old woman with thalassemia major, regularly transfused since the time of diagnosis (age 21 months), who presented with sudden muscle weakness, difficulty walking and maintaining the upright position. Magnetic Resonance Imaging (MRI) of the thoracic spine showed spinal cord compression secondary to extramedullary hematopoiesis in the spinal canal, leading to early therapy. The neurosurgical treatment (decompressive laminectomy D3-D6) in our patient brought a significant and rapid recovery. The next two MRI of the spine (after 6 and 18 months) were both negative for recurrence.

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Year:  2011        PMID: 21705991

Source DB:  PubMed          Journal:  Pediatr Endocrinol Rev        ISSN: 1565-4753


  1 in total

1.  Extramedullary hematopoiesis presenting as a right adrenal mass in a patient with Beta thalassemia.

Authors:  Hossein Karami; Mehrnoush Kosaryan; Mehrdad Taghipour; Rayka Sharifian; Aili Aliasgharian; Mohsen Motalebi
Journal:  Nephrourol Mon       Date:  2014-09-05
  1 in total

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