Literature DB >> 22470615

Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major.

Salil Soman1, David L Rosenfeld, Sudipta Roychowdhury, Richard A Drachtman, Alan Cohler.   

Abstract

We describe a 16 year-old male with β thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH). After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment.

Entities:  

Keywords:  Adolescent extramedullary hematopiesis; cord compression; thalassemia

Year:  2009        PMID: 22470615      PMCID: PMC3303263          DOI: 10.3941/jrcr.v3i1.83

Source DB:  PubMed          Journal:  J Radiol Case Rep        ISSN: 1943-0922


  15 in total

1.  [Compression of the spinal cord due to proliferation of bone marrow in epidural space in a splenectomized person with Cooley's disease].

Authors:  I GATTO; V TERRANA; L BIONDI
Journal:  Haematologica       Date:  1954       Impact factor: 9.941

2.  Diffuse mesenteric extramedullary hematopoiesis with ascites: sonography, CT, and MRI findings.

Authors:  Christopher Holden; Oliver Hennessy; Wai-Kit Lee
Journal:  AJR Am J Roentgenol       Date:  2006-02       Impact factor: 3.959

3.  Successful treatment of spinal cord compression secondary to extramedullary hematopoietic mass by hypertransfusion in a patient with thalassemia major.

Authors:  S M Tai; J S H Chan; S Y Ha; B W Y Young; M S M Chan
Journal:  Pediatr Hematol Oncol       Date:  2006-06       Impact factor: 1.969

4.  Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord.

Authors:  P Niggemann; T Krings; F Hans; A Thron
Journal:  Neuroradiology       Date:  2005-04-06       Impact factor: 2.804

5.  Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

Authors:  Naziha Khen-Dunlop; Robert Girot; Francis Brunelle; Yann Révillon; Claire Nihoul-Fékété; Sabine Sarnacki
Journal:  J Pediatr Surg       Date:  2006-07       Impact factor: 2.545

6.  Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis.

Authors:  H Cario; M Wegener; K-M Debatin; E Kohne
Journal:  Ann Hematol       Date:  2002-08-15       Impact factor: 3.673

Review 7.  Spinal extradural hematopoiesis in adolescents with thalassemia. Report of two cases and a review of the literature.

Authors:  A Amirjamshidi; K Abbassioun; S E Ketabchi
Journal:  Childs Nerv Syst       Date:  1991-08       Impact factor: 1.475

8.  Hydroxyurea in the treatment of major beta-thalassemia and importance of genetic screening.

Authors:  M Alebouyeh; F Moussavi; H Haddad-Deylami; P Vossough
Journal:  Ann Hematol       Date:  2004-01-14       Impact factor: 3.673

Review 9.  Spinal cord compression in beta-thalassemia: case report and review of the literature.

Authors:  S A Salehi; T Koski; S L Ondra
Journal:  Spinal Cord       Date:  2004-02       Impact factor: 2.772

Review 10.  Structural and functional imaging of normal bone marrow and evaluation of its age-related changes.

Authors:  Judy S Blebea; Mohamed Houseni; Drew A Torigian; Chengzhong Fan; Ayse Mavi; Ying Zhuge; Tad Iwanaga; Shipra Mishra; Jay Udupa; Jiyuan Zhuang; Rohit Gopal; Abass Alavi
Journal:  Semin Nucl Med       Date:  2007-05       Impact factor: 4.446

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  3 in total

1.  Incomplete paraplegia caused by extramedullary hematopoiesis in a patient with thalassemia intermedia.

Authors:  Nurhasyimah Hisamud-Din; Nadia Mohd Mustafah; Aishah Ahmad Fauzi; Natiara Mohamad Hashim
Journal:  Spinal Cord Ser Cases       Date:  2017-05-04

Review 2.  Musculoskeletal imaging manifestations of beta-thalassemia.

Authors:  Maryam Hajimoradi; Sara Haseli; Alireza Abadi; Majid Chalian
Journal:  Skeletal Radiol       Date:  2021-02-09       Impact factor: 2.199

3.  Long segment spinal epidural extramedullary hematopoiesis.

Authors:  Kanwaljeet Garg; Pankaj Kumar Singh; Manmohan Singh; P Sarat Chandra; Bhawani Shankar Sharma
Journal:  Surg Neurol Int       Date:  2013-12-26
  3 in total

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