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Year : 2013  |  Volume : 4  |  Issue : 3  |  Page : 114-117

Bone marrow necrosis in sickle cell disease: A case report and review of the literature

1 Department of Hematology and Blood Bank, Theodor Bilharz Research Institute, Giza, Egypt; Prince Sultan Military Medical City, Central Military Laboratory and Blood Bank, Riyadh, Kingdom of Saudi Arabia
2 Prince Sultan Military Medical City, Central Military Laboratory and Blood Bank, Riyadh, Kingdom of Saudi Arabia
3 Department of Adult Clinical Hematology and Stem Cell Therapy, Riyadh, Kingdom of Saudi Arabia

Correspondence Address:
Ghaleb Elyamany
Theodor Bilharz Research Institute, Egypt, Consultant Hematopathology, PO Box 7897, Riyadh 11159, Kingdom of Saudi Arabia

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1658-5127.123313

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The clinicopathologic entity of bone marrow necrosis (BMN) was first reported by Wade and Stevenson in 1941. BMN is defined as necrosis of the myeloid tissues and stroma without cortical bone involvement. The pathophysiology of BMN remains unclear; however, the occlusion of microcirculation, accompanied by hypoxemia, causes damage to the cells and plays an important role. Severe BMN is rarely diagnosed in living patients. Herein, we report a rare case of severe BMN in a patient with sickle cell disease (SCD) who presented with an unusually severe vaso-occlusive crisis complicated by acute chest syndrome (ACS), pancytopenia, and disseminated intravascular coagulation. Although the clinical features are highly suggestive of BMN, the diagnosis still required a bone marrow (BM) examination because early transfusion therapy can be lifesaving. The prognosis of severe BMN seems to reflect the poor outcomes of such underlying conditions; however, the prognosis of generalized BMN is not so poor when associated with SCD.

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