Nurhasyimah Hisamud-Din1,2, Nadia Mohd Mustafah2,3, Aishah Ahmad Fauzi2, Natiara Mohamad Hashim2,3. 1. Department of Rehabilitation Medicine, Hospital Tuanku Ja'afar, Seremban, Negeri Sembilan, Malaysia. 2. Discipline of Rehabilitation Medicine, Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh, Selangor, Malaysia. 3. Department of Rehabilitation Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Abstract
INTRODUCTION: Extramedullary hematopoiesis (EMH) is the production of blood cell precursors outside the bone marrow that occur in various hematological diseases. In patients with thalassemia intermedia, ineffective erythropoiesis drives compensatory EMH in the liver, pancreas, pleura, spleen, ribs and spine. CASE PRESENTATION: We describe a patient with thalassemia intermedia who presented with acute neurological symptoms caused by paraspinal EMH, which responded well to combination therapy of steroid, hypertransfusion, laminectomy and excision of pseudotumor and hydroxyurea therapy to boost the formation of fetal haemoglobin. DISCUSSION: Prompt recognition of EMH based on clinical presentation and typical radiological findings should be made. Early treatment is recommended to prevent irreversible damage to the spinal cord.
INTRODUCTION: Extramedullary hematopoiesis (EMH) is the production of blood cell precursors outside the bone marrow that occur in various hematological diseases. In patients with thalassemia intermedia, ineffective erythropoiesis drives compensatory EMH in the liver, pancreas, pleura, spleen, ribs and spine. CASE PRESENTATION: We describe a patient with thalassemia intermedia who presented with acute neurological symptoms caused by paraspinal EMH, which responded well to combination therapy of steroid, hypertransfusion, laminectomy and excision of pseudotumor and hydroxyurea therapy to boost the formation of fetal haemoglobin. DISCUSSION: Prompt recognition of EMH based on clinical presentation and typical radiological findings should be made. Early treatment is recommended to prevent irreversible damage to the spinal cord.
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
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