Literature DB >> 27108419

Cortical bone invasion in non-transfusion-dependent thalassemia: tumefactive extramedullary hematopoiesis reviewed.

Karim Z Masrouha1, Joelle Wazen2, Anthony Haddad3, Fadi Saadeh4, Ali Taher3, Nabil J Khoury5.   

Abstract

OBJECTIVE OF THE STUDY: To assess the prevalence of cortical bone invasion (CBI) with secondary extramedullary hematopoiesis (EMH) in patients with non-transfusion-dependent thalassemia (NTDT), to determine its predilection sites on thoracic and abdominal imaging, to determine whether there is an association between various clinical and hematological parameters, and to evaluate its various findings mainly on magnetic resonance imaging (MRI), in addition to computed tomography (CT) scans.
MATERIALS AND METHODS: This is a retrospective cohort study of 57 patients with NTDT imaged by CT or MRI. Both clinical and laboratory data were gathered. An imaging scoring system was used to describe the appearance of CBI by MRI.
RESULTS: Twenty-seven patients (47.4 %) were found to have CBI and EMH with the most common location being the thoracic spine. Splenectomy and lower hemoglobin level were found to be independent risk factors for its development. Most lesions were homogenous (70 %), had predominant red marrow signal (67 %), and well-defined margins (89 %).
CONCLUSION: CBI and secondary tumefactive EMH are common findings in patients with NTDT, with distinct imaging and clinical characteristics. An increased risk was seen in patients with splenectomy and lower hemoglobin. The imaging scoring system described is helpful in diagnosing and describing this entity, hence precluding unnecessary biopsies.

Entities:  

Keywords:  Cortical bone invasion; Extramedullary hematopoiesis; Non-transfusion-dependent thalassemia; Soft tissue lesion

Mesh:

Substances:

Year:  2016        PMID: 27108419     DOI: 10.1007/s11547-016-0638-1

Source DB:  PubMed          Journal:  Radiol Med        ISSN: 0033-8362            Impact factor:   3.469


  19 in total

1.  Paraparesis induced by extramedullary haematopoiesis.

Authors:  Paolo Savini; Arianna Lanzi; Giorgio Marano; Chiara Carli Moretti; Giovanni Poletti; Giuseppe Musardo; Francesco Giuseppe Foschi; Giuseppe Francesco Stefanini
Journal:  World J Radiol       Date:  2011-03-28

2.  Paravertebral mass in a patient with thalassemia intermedia.

Authors:  R Alam; K Padmanabhan; H Rao
Journal:  Chest       Date:  1997-07       Impact factor: 9.410

Review 3.  Extramedullary hemopoiesis.

Authors:  Eleni Orphanidou-Vlachou; Chrysa Tziakouri-Shiakalli; Christos S Georgiades
Journal:  Semin Ultrasound CT MR       Date:  2013-12-19       Impact factor: 1.875

4.  Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation.

Authors:  J Tsitouridis; S Stamos; E Hassapopoulou; K Tsitouridis; P Nikolopoulos
Journal:  Eur J Radiol       Date:  1999-04       Impact factor: 3.528

Review 5.  The inherited diseases of hemoglobin are an emerging global health burden.

Authors:  David J Weatherall
Journal:  Blood       Date:  2010-03-16       Impact factor: 22.113

6.  MR of thoracic cord compression caused by epidural extramedullary hematopoiesis in myelodysplastic syndrome.

Authors:  D A Dibbern; L A Loevner; A P Lieberman; K E Salhany; A Freese; P J Marcotte
Journal:  AJNR Am J Neuroradiol       Date:  1997-02       Impact factor: 3.825

7.  Presacral extramedullary hematopoiesis: a diagnostic confusion concerning a rare presentation.

Authors:  Abdulla Al-Aabassi; Basil A Murad
Journal:  Med Princ Pract       Date:  2005 Sep-Oct       Impact factor: 1.927

8.  Case report: chronic spinal cord compression from extramedullary haematopoiesis in thalassaemia--MRI findings.

Authors:  L Pantongrag-Brown; N Suwanwela
Journal:  Clin Radiol       Date:  1992-10       Impact factor: 2.350

9.  An interesting presentation of intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia.

Authors:  B Turgut; G E Pamuk; O Vural; M Demir; E Unlü; H Celik; B Cakir; K Kutlu
Journal:  Clin Lab Haematol       Date:  2003-12

10.  Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study.

Authors:  Ali T Taher; Khaled M Musallam; Mehran Karimi; Amal El-Beshlawy; Khawla Belhoul; Shahina Daar; Mohamed-SalahEldin Saned; Abdul-Hamid El-Chafic; Maria R Fasulo; Maria D Cappellini
Journal:  Blood       Date:  2009-12-23       Impact factor: 22.113

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