AJM onlineDiagnostic dilemmaInside Out: Bone Marrow Necrosis and Fat Embolism Complicating Sickle-β+ Thalassemia
Section snippets
Presentation
Neurologic symptoms initially attributed to infection were ultimately the result of a devastating hematologic condition. The patient, a 57-year-old woman, was rushed to the emergency department for somnolence and slurred speech. She had experienced low back pain during the preceding week. Her medical history was notable for a ventriculoperitoneal shunt placed several years ago after spontaneous intracranial hemorrhage.
Assessment
On examination, the patient was difficult to arouse and was emergently intubated for airway protection. She was febrile to 39.3°C with a heart rate of 144 beats/min. An oxygen saturation was not recorded. Her physical examination results were otherwise unremarkable. The electrocardiogram revealed sinus tachycardia and T-wave inversions in the anterior and lateral leads.
Laboratory investigation revealed a white blood cell count of 11.1 × 103/μL, hemoglobin of 11.2 g/dL, and platelet count of
Diagnosis
With this additional information, the patient was diagnosed with bone marrow necrosis and fat embolism syndrome. Bone marrow necrosis refers to the death of hematopoietic cells and medullary stroma within cortical bone. Approximately 90% of cases of bone marrow necrosis are the result of hematologic malignancies, but it can also occur secondary to drug toxicity, infection, and heterozygous hemoglobin S states.1 The unifying pathophysiology is hypothesized to be a failure of the marrow
Management
The patient underwent emergency red blood cell exchange on day 3. She required 10 units of O negative packed red blood cells to decrease the hemoglobin S concentration by at least 70% at the end of the procedure. The hemoglobin electrophoresis after the exchange revealed hemoglobin S 6.3%, hemoglobin A 90.1%, hemoglobin A2 2.9%, and fetal hemoglobin 0.7%. After the exchange, the patient's platelet count and renal function rapidly recovered (Figure 3). Furthermore, other laboratory parameters
References (6)
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Bone marrow necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non-SS genotypes and a possible association with human parvovirus B19 infection
Blood Rev
(2014) - et al.
Bone marrow necrosis: ten-year retrospective review of bone marrow biopsy specimens
Am J Clin Pathol
(2015) - et al.
Bone marrow embolism in sickle cell disease: a review
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Funding: None.
Conflict of Interest: None.
Authorship: All authors had access to the data and played a role in writing this manuscript.