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

2018 ◽  
Author(s):  
Radhika Gangaraju ◽  
Jori E. May ◽  
Lance A. Williams ◽  
Vishnu B. Reddy ◽  
Paul MacLennan ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Fat Embolism ◽  
Bone Marrow Necrosis ◽  
Thrombocytopenic Purpura ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome ◽  
Life Threatening ◽  
Life Threatening Complication

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

2017 ◽  
Vol 7 (4) ◽  
pp. 42-50 ◽  
Author(s):  
Eduardo Pelegrineti Targueta ◽  
André Carramenha de Góes Hirano ◽  
Fernando Peixoto Ferraz de Campos ◽  
João Augusto dos Santos Martines ◽  
Silvana Maria Lovisolo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fat Embolism ◽  
Cell Disease ◽  
Bone Marrow Necrosis ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

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

2016 ◽  
Vol 109 (9) ◽  
pp. 549-553 ◽  
Author(s):  
Radhika Gangaraju ◽  
Vishnu V.B. Reddy ◽  
Marisa B. Marques
Keyword(s):  
Bone Marrow ◽  
Fat Embolism ◽  
Bone Marrow Necrosis ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

Bone marrow necrosis and fat embolism syndrome in sickle cell disease: A rapidly deteriorating complication

Transfusion ◽  
2021 ◽  
Author(s):  
Mohammad Barouqa ◽  
James Szymanski ◽  
Randin Nelson ◽  
Sebastian Jofre ◽  
Monika Paroder
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fat Embolism ◽  
Cell Disease ◽  
Bone Marrow Necrosis ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

scholarly journals Differentiating Fat Embolism Syndrome Due to Bone Marrow Necrosis in Patients with Sickle Hemoglobinopathies from Thrombotic Thrombocytopenic Purpura (TTP)

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1090-1090
Author(s):  
Radhika Gangaraju ◽  
Jori May ◽  
Paul MacLennan ◽  
Vishnu V.B. Reddy ◽  
Marisa B. Marques
Keyword(s):  
Bone Marrow ◽  
Back Pain ◽  
Mental Status ◽  
Fat Embolism ◽  
Categorical Variables ◽  
Red Cell ◽  
Bone Marrow Necrosis ◽  
Peripheral Smear ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

Abstract BACKGROUND: Fat Embolism Syndrome (FES) is common in trauma patients and those with long bone fractures. It can also be a rare complication of bone marrow necrosis (BMN) in patients with sickle cell hemoglobinopathies (SC), particularly HbSβ+ and HbSC, due to marrow ischemia resulting from sickling with subsequent hematopoietic and stromal cell death. Release of intramedullary fat emboli causes multiorgan failure with anemia and thrombocytopenia, often mimicking TTP. Here, we discuss the clinical presentation and outcomes of 16 patients with SC and FES, and attempt to distinguish it from TTP. METHODS: We compared SC patients with FES treated at our institution between April, 2010 and January, 2018 with 2 TTP patients matched for age, gender and race. We analyzed their presenting symptoms and labs aiming at identifying characteristics that differentiate the two conditions. A univariate exact conditional logistic regression was used to examine significant differences in likelihood of FES for continuous and categorical variables. A Friedman test was used to test for significant differences in schistocytes on admission. Ordinal values were created based off the value of the dependent variable, i.e., presence of schistocytes (none=0, rare=1, few=2, occasional=3, moderate=4, and many=5/high power field). This test accounts for the 1:2 matching and the null hypothesis is that there is no difference in ranks by patient group. The study was approved by our Institutional Review Board. RESULTS: Sixteen patients with SC (10 with HbSβ+, 4 with HbSC and 2 with HbSS disease) were treated for FES at our institution, mean age 40.6y. At presentation, 11 (69%) complained of severe back pain, 11 (69%) had respiratory compromise, and 9 (56%) had altered mental status. On admission, all patients were anemic, with a mean hemoglobin of 9.2 g/dL. Thrombocytopenia was present in 12 patients (75%) at admission, and the rest developed it during the hospitalization. Six (38%) patients had a leukoerythroblastic picture on admission with >20 nucleated RBC/ 100 leukocytes, and another 7 (total, 81%) developed it later. Of the 10 patients who had ferritin measured, all had an increased level (mean 5792 ng/mL; ref: 10-291 ng/mL). Of the 12 patients who received RBC transfusion (10 exchange and 2 simple transfusion), 9 had full recovery, 2 had neurologic impairment and 1 died. Those with delayed recognition of FES either died (n=3) or suffered permanent neurologic damage (n=1). Table 1 compares the clinical characteristics of the SC patients who developed FES with matched TTP patients. The latter had significantly lower platelet count on admission and at nadir during their hospital-stay compared to FES patients (p<0.0001) (Fig 1). Also, back pain was reported significantly more in patients with FES (p<0.0001). Indices on admission that were not significantly different between the two groups included hemoglobin, lactate dehydrogenase, creatinine, or a leukoerythroblastic pattern on peripheral smear. FES patients were less likely to have moderate/many schistocytes on peripheral smear compared to TTP patients (p=0.035) (Fig 2). DISCUSSION: BMN and embolization of fat and necrotic marrow is a rare but potentially fatal complication in SC patients. It can be the first manifestation of SC disease in previously undiagnosed patients, leading to delayed diagnosis. In several published fatal cases of BMN, the diagnosis was only made at autopsy. The etiology and precipitating factors of BMN/FES remain poorly understood, though association with Parvovirus infection has been reported. In the past 8 years, we have diagnosed 16 patients with FES, who presented with an array of nonspecific symptoms such as fatigue, mental status changes, bone pain, fever, anemia, thrombocytopenia, and leukoerythroblastic picture in the peripheral blood. Since FES and TTP have similar clinical and laboratory features, identifying early signs that differentiate the two is essential for optimal treatment and survival. While both disorders have mortality approaching 90% if untreated, therapeutic plasma exchange is life-saving in TTP, and patients with FES appear to benefit from red cell exchange. Increasing awareness of hematologists, maintaining a high index of suspicion for FES, establishing the correct diagnosis early, and instituting rapid intervention with red cell exchange or simple transfusion is essential to prevent permanent dysfunction or death. Disclosures No relevant conflicts of interest to declare.

Bone marrow necrosis and fat embolism syndrome: a near fatal complication in previously undiagnosed sickle beta + thalassaemia

2021 ◽  
Vol 14 (1) ◽  
pp. e238317
Author(s):  
Nibash Budhathoki ◽  
Sunita Timilsina ◽  
Bebu Ram ◽  
Douglas Marks
Keyword(s):  
Bone Marrow ◽  
Multiorgan Failure ◽  
Rare Condition ◽  
Altered Mental Status ◽  
Fat Embolism ◽  
Bone Marrow Necrosis ◽  
Embolism Syndrome ◽  
Hb S ◽  
High Index Of Suspicion ◽  
Specific Symptoms

Prevalence of haemoglobin sickle-β+ thalassaemia (Hb S/β+thal) is variable with geography ranging from 0.2% to 10% among sickle cell patients. Clinical presentation of Hb S/β+thal patients depends on HbA level, with milder disease often going undiagnosed. However, rarely these patients can present with a fulminant vaso-occlusive crisis (VOC). Given VOC can present with non-specific symptoms, the diagnosis and treatment is often delayed. Here, we present a patient who initially developed altered mental status, pancytopenia and multiorgan failure due a critical VOC resulting in bone marrow necrosis and fat embolism. Subsequent workup confirmed that our patient had Sickle-β+ thalassaemia, which had gone undiagnosed, despite subclinical evidence of haemolysis on routine lab work for years. Following diagnosis and initiation of RBC exchange, he improved significantly and was discharged home. High index of suspicion and bone marrow biopsy is vital for early diagnosis and management of this rare condition.

scholarly journals A rare case of fat embolism syndrome secondary to abdominal liposuction and gluteal fat infiltration

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Salvador Recinos ◽  
Sabrina Barillas ◽  
Alejandra Rodas ◽  
Javier Ardebol
Keyword(s):  
Right Ventricular Dysfunction ◽  
Signs And Symptoms ◽  
Fat Embolism ◽  
Fat Infiltration ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome ◽  
Threatening Condition ◽  
Life Threatening ◽  
Bone Marrow Harvesting ◽  
Dermatologic Manifestations

Abstract Fat embolism syndrome (FES) is a rare, life-threatening condition habitually associated with traumatic events such as fractures and, less commonly, burns, liposuction and bone marrow harvesting and transplant [ 1]. The biochemical theory for this condition suggests that fat droplets embolize and convert into fatty acids, eventually leading to toxic injury and inflammation, which results in increased vascular permeability, edema and hemorrhage [ 2]. FES may have an asymptomatic interval lasting 12–72 hours after the insult; however, in some cases, signs have also been seen intraoperatively. Pulmonary signs and symptoms are customarily the earliest and manifest in 75% of patients. Nevertheless, neurologic and dermatologic manifestations are also characteristic, and most severe cases could perhaps present with disseminated intravascular coagulation, right ventricular dysfunction, shock or death. The following case consists of a 37-year-old patient that presented with fat embolism syndrome during liposuction and gluteal fat infiltration.

EARLY DETECTION OF FAT EMBOLISM SYNDROME IN TRAUMATOLOGY (LITERATURE REVIEW)

2021 ◽  
pp. 23-30
Author(s):  
MAKSIM D. OSIPOV
Keyword(s):  
Early Detection ◽  
Literature Review ◽  
Fat Embolism ◽  
High Energy ◽  
Severe Condition ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome ◽  
Threatening Condition ◽  
Life Threatening ◽  
The Many

Fat embolism syndrome is one of the many complications in traumatology, which carries a serious danger due to the difficult diagnosis at the early stages of its development. In many medical sources, fat embolism syndrome is described as a severe condition characterized by obturation of blood vessels by embolus, which are represented by fat droplets larger than 7-9 microns. Fat embolism is usually caused by trauma, accompanied by crushing of tissues. This is especially common in fractures of the tubular bones as a result of high-energy trauma. As exemplified in the medical literature about 6-7 % of isolated fractures and 37 % of combined injuries lead to the appearance of fat embolism syndrome. Much more often, fat embolism develops in open fractures, and the frequency of its occurrence increases with a combination of open and closed fractures. Due to the fact that fat embolism is a life-threatening condition, early detection of this pathology is necessary. The purpose of this literature review is to study the pathogenesis of fat embolism, as well as the possibilities, problems and methods of early diagnosis of this pathology in trauma practice...

scholarly journals Experimental Parametric Study of the Factors Leading to Elevated Femoral Intramedullary Pressure and Fat Embolus Syndrome in Orthopaedic Procedures

2021 ◽  
Author(s):  
Daniel Dobrjanski
Keyword(s):  
Bone Marrow ◽  
Fat Embolism ◽  
Great Promise ◽  
Petroleum Jelly ◽  
Marrow Fat ◽  
Fat Embolism Syndrome ◽  
Intramedullary Pressure ◽  
Embolism Syndrome ◽  
Intramedullary Canal ◽  
Radial Gap

During orthopaedic procedures such as total knee arthroplasty (TKA), total hip arthroplasty (THA), and intramedullary nailing, it is necessary to hammer implants into the intramedullary canal of long bones. This hammering action can generate a high intramedullary pressure, leading to the release of bone marrow fat globules into the cardiovascular system, and ultimately, the possible development of fat embolism syndrome. In the present study, the effect of parameters such as implant tip geometry, peak impact force, hammer tip material, bone to implant radial gap, and marrow viscosity, on the resulting intramedullary pressure generated when hammering implants into a simulated femur analogue was examined. The bone analogue consisted of a porous plastic cylinder, having similar porosity and pore size to human femoral bone, with bone marrow being represented by a paraffin wax/petroleum jelly mixture. It was found that intramedullary pressure is only slightly lowered by a change in implant tip geometry, and that the use of a steel tipped (as opposed to rubber) hammer resulted in an increase in average pressure in the proximal portion of the bone, but a decrease distally. A lower implant insertion speed, lower hammering force, and a larger bone to implant radial gap were found to significantly reduce the intramedullary pressure. The number of hammer strikes required to insert an implant was found to increase significantly with marrow viscosity, but the average intramedullary pressure was found to decrease with increasing viscosity. Numerical modelling was also found to offer great promise for analysing hammering procedures for orthopaedic research into fat embolism syndrome. Numerical and experimental results were matched with approximately a 20% deviation.

Sign in / Sign up

Export Citation Format

Share Document