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

Abstract

Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome.

Renin-Angiotensin Blockade Reduces Readmission for Acute Chest Syndrome in Sickle Cell Disease

“…Hydroxyurea mainly exerts its effect by increasing fetal hemoglobin. Hydroxyurea is oxidized by heme groups to produce nitric oxide (NO) to form NO metabolites and also has shown to reduce expression of vascular adhesion molecule-1 (VCAM-1) which may contribute to its efficacy even before its effect on fetal hemoglobin [ 31 - 35 ]. However, it does not have a role in the treatment of ongoing vaso-occlusive crises or acute chest syndrome [ 19 ].…”

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.…”

Bone marrow necrosis and fat embolism syndrome in sickle cell disease during COVID‐19 infection treated successfully with sequential red cell and plasma exchange

“…A decision to commence five, one volume plasma exchange (PLEX) sessions with octaplas TM , starting on 9th day of hospital admission was reached. Bone marrow histology (Figure 2A–C) later revealed extensive necrosis and haemorrhage, capillaries occluded with sickle cells and areas of increased macrophage activity consistent with the pathophysiology of FES [3, 4].…”