Acta Neurologica Indonesia
Vol. 1 No. 01 (2023): Acta Neurologica Indonesia

Spinal Cord Compression Due to Intradural Extramedullary Hematopoiesis in a Young Adult with Cooley’s Anemia

Taufik Mesiano (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Al Rasyid (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Mohammad Kurniawan (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Rakhmad Hidayat (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
David Pangeran (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Anna Mira Lubis (Department of Internal Medicine Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Mohamad Saekhu (Department of Neurosurgery Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Kevin Gunawan (Department of Neurosurgery Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Stefanie Karina Putri (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Arizari Haj Rahmana (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)
Salim Harris (Department of Neurology Cipto Mangunkusumo National Hospital, Faculty of Medicine Universitas Indonesia Jakarta)



Article Info

Publish Date
31 Dec 2023

Abstract

Cooley's anemia, also known as beta thalassemia major, is an inherited multisystemic disorder characterized by skeletal and non-skeletal complications resulting from hemoglobinopathies. Extramedullary Hematopoiesis (EMH) is a complication of thalassemia major due to insufficient erythropoiesis expansion. The incidence rate of paraspinal EMH in beta-thalassemia is rare but tends to be on the rise. We present a case of spinal cord compression due to intradural EMH in a 21-year-old man with Beta Thalassemia major, who exhibited acute lower motor, sensory, and autonomic disorder, along with severe anemia, and electrolyte imbalance. Patients were treated with corticosteroids, blood transfusions, electrolyte correction and pain medications. Several days later, the patient experienced clinical improvement in reduced pain and motor improvement. The patient was planned to undergo elective surgery and radiotherapy after reaching the stabilized condition. Management options of spinal cord compression due to EMH include corticosteroids, adequate blood transfusion, hydroxyurea, radiotherapy, surgical decompression, exchange transfusion, or a combination of these approaches. The choice of therapy should be based on the patient's clinical condition, diagnostic evaluations, and the size of the mass exerting pressure on the spinal cord. The optimal management of EMH remains uncertain. We need further research to establish effective treatment strategies of spinal cord compression due to EMH in Cooley’s Anemia.

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Journal Info

Abbrev

journal

Publisher

Subject

Medicine & Pharmacology Neuroscience Public Health

Description

Acta Neurologica Indonesia is the official publication of the Department of Neurology, Faculty of Medicine, Universitas Indonesia under Directorate of Administration, Data, and Product Management of Research and Innovation Universitas Indonesia. This journal is published in trimonthly cycle with ...