Fat Embolism Syndrome Secondary to Bone Marrow Necrosis in Patients with Hemoglobinopathies

Abstract

Bone marrow necrosis with subsequent embolization of the fat and necrotic tissues into the systemic circulation causing fat embolism syndrome and multiorgan failure is a rare complication of patients with hemoglobinopathies. The exact etiology of this condition is not known. Because it occurs more often in patients with compound heterozygous conditions than in sickle cell disease, some patients are unaware of their predisposition. The initial symptoms are nonspecific, such as back and/or abdominal pain, fever, and fatigue, which may rapidly progress to respiratory failure and severe neurologic compromise. Common laboratory tests reveal anemia without reticulocytosis, thrombocytopenia, leukoerythroblastic picture with immature white cells and nucleated red blood cells, increased lactate dehydrogenase, high ferritin, and, sometimes increased creatinine. The diagnosis can be delayed because of an apparent lack of awareness about bone marrow necrosis with fat embolism syndrome, its rarity, and its similarities with other conditions such as thrombotic thrombocytopenic purpura. Although a bone marrow biopsy is diagnostic, waiting for it delays definitive treatment, which appears to be essential for the recovery of end-organ damage, such as neurologic and pulmonary damage. In our experience, either multiple units of red blood cell transfusion or, preferably, red cell exchange initiated promptly, is lifesaving.

Three neglected numbers in the CBC: The RDW, MPV, and NRBC count

“…In addition, leukoerythroblastosis appears in a rare complication of sickle cell hemoglobinopathies: bone marrow necrosis with fat embolism syndrome. 20,21 As the marrow ne-croses, fat emboli are released in the systemic circulation causing micro-and macrovascular occlusions and multiorgan failure. The largest case series in the literature reports 58 patients with bone marrow necrosis with fat embolism syndrome.…”

How I investigate bone marrow necrosis

“…Other benign noninfectious causes include anorexia, medication and chemical effect, alcohol abuse, G‐CSF administration, non‐Hgb S hemoglobinopathies, acute graft vs host disease after allogenic bone marrow transplant, and hemophagocytosis . Bone marrow necrosis can sometimes be associated with fat embolization syndrome (FES), a life‐threatening disorder associated with neurologic dysfunction and multiorgan failure; FES is more commonly seen in patients with hemoglobinopathies …”

“…Clinical findings associated with bone marrow necrosis include bone pain and fever, cytopenias, elevated LDH and ferritin, and leukoerythroblastosis . Rarely, such as in fat embolization syndrome (FES), bone marrow necrosis can be associated with thrombotic microangiopathy, neurologic dysfunction, and multiorgan failure …”

Fat embolism syndrome due to bone marrow necrosis in patients with hemoglobinopathies: A life‐threatening complication mimicking thrombotic thrombocytopenic purpura

“…We also attempted to calculate fat embolism risk scores (Gurd and Wilson's criteria, and Schonfeld's criteria) for FES patients, to test their applicability in this context. 2 The Institutional Review Board at our institution approved this study and granted a waiver of informed consent.…”

“…4 While bone marrow biopsy is diagnostic, treatment with transfusion should not wait for biopsy confirmation based on our experience. 2 was scored on a 0-100 scale with 10 points given for each correct answer. Regardless of whether their answers were correct or incorrect, learners were then given access to the full set of thalassemia learning module in which relevant educational information about thalassemia along with key references from the literature.…”