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2017 ; 2017
(ä): 7185604
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Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia-A Case
Report and Review of the Literature
#MMPMID28695026
Alsafwani SA
; Al-Saeed A
; Bukhamsin R
Case Rep Hematol
2017[]; 2017
(ä): 7185604
PMID28695026
show ga
Bone marrow necrosis (BMN) is a rare clinical entity that was first described in
an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined
necrotic cells in an amorphous eosinophilic background with preservation of
cortical bone. The pathophysiology of BMN is not well known; however, occlusion
of the bone marrow microcirculation with subsequent hypoxia and cell injury has
been thought to be common underlying features. Malignancy has been identified to
be the primary cause in 90% of the cases whereas SCD was found in only 2%. In
this report we present an unusual case of SCD with late onset of the disease
whose initial presentation was extensive BMN. The patient was not known
previously to have SCD, when suddenly she presented with severe cytopenias and
marked elevation in serum lactate dehydrogenase (LDH). Bone marrow examination
was done to exclude bone marrow infiltration, and BMN with dilated marrow sinuses
full of irreversibly sickled cells were the unexpected findings. Patients with a
mild SCD phenotype are at high risk of BMN. Thus, a high index of suspicion must
be borne in mind, particularly in an area of high SCD prevalence, to recognize
and prevent this catastrophic complication.