Effect of hydroxyurea on extramedullary haematopoiesis in thalassaemia intermedia: Case reports and literature review

A. Meo, E. Cassinerio, R. Castelli, D. Bignamini, L. Perego, M. D. Cappellini

Research output: Contribution to journalArticle

Abstract

Extramedullary haematopoiesis (EH) is the production of blood cell precursors outside the bone marrow that occurs in various disorders, such as thalassaemia, sickle cell anaemia, hereditary spherocytosis, polycythaemia vera, myelofibrosis and other haematological diseases. In chronic anaemia, it is a physiological response to increased erythropoietin. In some other conditions, such as myeloid metaplasia, polycythaemia vera or chronic myeloid leukaemia, EH is due to a clonal disorder of haematopoiesis that enables progenitor cells to escape from the marrow and lodge in other organs. EH usually involves the liver, spleen and lymph nodes or it can be paravertebral, intrathoracic, pelvic. It is often asymptomatic but can sometimes lead to symptomatic tumour-like masses. Treatment options are still controversial and limited, including hypertransfusion regimen, surgical treatment, radiotherapy and hydroxyurea (HU). We describe intrathoracic and symptomatic pelvic EH masses in a 48-year-old woman and intrathoracic bilateral masses causing respiratory insufficiency with pleural effusion in a 42-year-old male, both affected by thalassaemia intermedia. Both patients showed a clinical improvement with hydroxyurea therapy and occasional blood transfusions.

Original languageEnglish
Pages (from-to)425-431
Number of pages7
JournalInternational Journal of Laboratory Hematology
Volume30
Issue number5
DOIs
Publication statusPublished - Oct 2008

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Keywords

  • Hydroxyurea
  • Intrathoracic extramedullary haematopoiesis
  • Thalassaemia intermedia
  • Tumour-like masses

ASJC Scopus subject areas

  • Clinical Biochemistry
  • Biochemistry, medical
  • Hematology

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