Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease

Abstract

Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC), which apparently shows a less severe clinical course, may exhibit the same complications of HbSS. These complications are usually manifested late in the course of life, when compared with the HbSS patients. It is noteworthy that HbSC may present a normal hematocrit, and therefore stays unknown until the first complication, that may be disastrous. The authors report a case of an African-Descendant woman, aging 65 years, with no previous diagnosis of anemia who sought medical attention because of a thoracic back pain followed by fever and altered mental status. The clinical picture deteriorated very fast with multiple organ failure and death. The autopsy findings concluded by generalized vaso-occlusive crisis, bone marrow necrosis and bone marrow and fat embolism, mainly to the lungs and kidney. The authors call attention for the knowledge of this severe life threatening complication, mainly in a country with a high Afro-Descendant population.

Sudden death caused by pulmonary fat embolism in a patient with miliary tuberculosis

“…In the present case, the immediate cause of SD was PFE, which most often follows blunt trauma, such as bone fracture, along with many other causes including severe burns, liposuction, acute pancreatitis, alcoholic fatty liver, sickle cell disease, and panniculitis 2 - 6. Bone marrow necrosis (BMN) is another etiology of PFE and is well known to be frequently associated with hematological disorders, such as sickle cell disease, lymphoma, leukemia, and MDS 5 - 9. Occasionally, miliary TB can be responsible for BMN 8 - 11.…”

Bone marrow necrosis and fat embolism syndrome: a dreadful complication of hemoglobin sickle cell disease

“… 6 Although SC hemoglobinopathy has a more protracted course, both SC and SS may present with similar complications. 7 Studies focused on laboratory biomarkers have shown that SCA individuals are more associated with endothelial dysfunction and hemolysis, while the HbSC genotype is more associated with increased blood viscosity and inflammatory disorders. 8 Proliferating retinitis, osteonecrosis, and acute chest syndrome, may have equal or higher incidence in HbSC compared to SCA.…”

The post-mortem diagnosis of vasocclusive crisis in sickle cell disease

“…2 Recently, sickling crisis followed by bone marrow necrosis and fat embolism was shown to be associated with high index of mortality in a patient with double heterozygosis to HbS and HbC. 3 …”