scholarly journals Fat Embolism Syndrome in Sickle Cell Disease

2020 ◽  
Vol 9 (11) ◽  
pp. 3601
Author(s):  
Dimitris A. Tsitsikas ◽  
Jessica Bristowe ◽  
Jibril Abukar
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Complete Recovery ◽  
Neurological Impairment ◽  
Fat Embolism ◽  
Red Cell ◽  
Cell Disease ◽  
Embolism Syndrome ◽  
Severe Neurological Impairment

Fat embolism syndrome is a devastating complication of sickle cell disease resulting from extensive bone marrow necrosis and associated with high mortality rates, while survivors often suffer severe neurological sequelae. Despite that, the syndrome remains under-recognised and under-diagnosed. Paradoxically, it affects exclusively patients with mild forms of sickle cell disease, predominantly HbSC and HbSβ+. A significant number of cases occur in the context of human parvovirus B19 infection. We provide here a brief summary of the existing literature and describe our experience treating 8 patients in our institution. One patient had HbSS, 6 HbSC and 1 HbSβ+. All patients developed type I respiratory failure and neurological involvement either at presentation or within the first 72 h. The most striking laboratory abnormality was a 100-fold increase of the serum ferritin from baseline. Seven patients received emergency red cell exchange and 1 simple transfusion. Two patients (25%) died, 2 patients (25%) suffered severe neurological impairment and 1 (12%) mild neurological impairment on discharge, while 3 (38%) patients made a complete recovery. With long-term follow-up, 1 patient with severe neurological impairment and one patient with mild neurological impairment made dramatic improvements, making the long-term complete recovery or near complete recovery rate 63%. Immediate red cell exchange transfusion can be lifesaving and should be instituted as soon as the syndrome is suspected. However, as the outcomes remain unsatisfactory despite the increasing use of red cell exchange, we suggest additional therapeutic measures such as therapeutic plasma exchange and pre-emptive transfusion for high risk patients.

Coma secondary to cerebral fat embolism syndrome due to sickle cell disease fully recovering following red cell exchange transfusion

2021 ◽  
Vol 14 (11) ◽  
pp. e245051
Author(s):  
Nourah Alajeel ◽  
Kefaya Abdulmalek ◽  
Abdulrahman Al-Fares
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Fat Embolism ◽  
Red Cell ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Epileptiform Discharges ◽  
Embolism Syndrome ◽  
The Brain

A 51-year-old woman known for sickle cell disease presented with 2 weeks of headache and bilateral lower limb pain. During admission, she suffered from multiple generalised tonic-clonic seizures but had an unremarkable CT of the brain. Incidentally, she had worsening baseline renal function. She was admitted to the intensive care unit with an acute confusional state. A bedside electroencephalogram showed triphasic waves and diffuse slow activity suggestive of encephalopathy with no epileptiform discharges. She remained obtunded despite appropriate medical therapy of hydration, antiepileptic and pain control. Lumbar puncture failed to identify an infectious cause. An urgent MRI of the brain was done and revealed features compatible with fat embolism syndrome (FES). Her haemoglobin S was 84.2%. Urgent red cell exchange transfusion was done, and within 3 days she fully regained her orientation and motor function. This represents the first case of such profound obtundation due to FES with a complete response to exchange transfusion.

scholarly journals Cerebral fat embolism syndrome in sickle cell disease without evidence of shunt

2019 ◽  
Vol 14 ◽  
pp. 19-20 ◽  
Author(s):  
Cody L. Nathan ◽  
Whitley W. Aamodt ◽  
Tanuja Yalamarti ◽  
Calli Dogon ◽  
Paul Kinniry
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fat Embolism ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome ◽  
Cerebral Fat Embolism Syndrome

scholarly journals Successful outcome of three patients with sickle-cell disease and fat embolism syndrome treated with intensive exchange transfusion

2016 ◽  
Vol 5 (1) ◽  
pp. 39-43 ◽  
Author(s):  
Paul Greaves ◽  
Vivek Mathew ◽  
Catherine Peters ◽  
Susan Rowe ◽  
Roger J. Amos ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Fat Embolism ◽  
Successful Outcome ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

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

scholarly journals Sickle cell disease, fat embolism syndrome, and “starfield” pattern on MRI

2018 ◽  
Vol 8 (2) ◽  
pp. 162-164 ◽  
Author(s):  
Jennifer H. Kang ◽  
Charles William Hargett ◽  
Theresa Sevilis ◽  
Matthew Luedke
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fat Embolism ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome

Manual Chronic Partial Exchange In Children and Adolescents with Sickle Cell Disease: Impact on Clinical Outcome and Iron Overload

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4821-4821
Author(s):  
Michel Aloni ◽  
Alina Ferster ◽  
Phu-Quoc Le ◽  
Catherine Heijmans ◽  
Sophie Huybrechts ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Outcome ◽  
Iron Overload ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Red Cell ◽  
Cell Disease ◽  
Hb S ◽  
The Mean

Abstract Abstract 4821 Background: Red cell exchange transfusion is frequently used in the management of patients with sickle cell disease either electively or therapeutically to maintain an hemoglobin S (Hb S) level < 30–50%. This target is often difficult to maintain. In order to assess the effects of chronic partial exchange transfusion (CPET) a) on level of Hb and Hb S, b) on iron overload c) the need for chelation, d) on risk of long term adverse events and e) clinical outcome, we analyzed the data of sickle cell disease patients treated by long term CPET in our center. Methods/subjects: In the cohort of 163 SCD patients followed at University Children's Hospital at Brussels (Belgium), 10 benefit from CPET. Main reasons for CPET were neurologic disease (4), frequent ACS (3), previous severe hepatic cholestasis (2), leg ulcer (1) and pulmonary hypertension (1). The median age at start of treatment was 13 years (range 4 –19). These patients (6 males and 4 females) account for 248 exchanges during a median follow-up of 20 months (range 6– 36). These exchanges are until now performed manually and the volume exchanged is calculated taking into account the Hb level and the last HbS percentage. It is usually between 30 and 40 ml/kg BW. Except if severe anemia occurs, the goal of these exchanges is to keep a constant hematocrit level. All patients had a full red cell phenotype performed and received blood matched for ABO, Rhesus, Kell and Duffy antigens systems. The estimation of iron balance (iron intake- iron removed) was calculated yearly. Results: The pre-exchange Hb value was 9.5 g/dl (median; range: 7.7–10.9 g/dl) and the mean post value was 9.4/dl (range: 8.4– 11.1 g/dl). These values are not statistically different (p> 0.05). The majority of patients (9/10) are reached an HbS < 50% when measured 3–5 weeks after PET (just before the next procedure) with a median HbS value of 40% (range: 30–54). At start of CPET program, the median ferritin level was 439 ng/ml (range: 80 – 1704). Five patients had already a ferritin > 500 ng/mL due to numerous previous transfusions. At last evaluation, the median ferritin did not change significantly and was 531 ng/ml (range 84– 3840). The two patients with ferritin higher than 1000 ng/ml start chelation with good result for one. One The mean annual net RBC load were 1.72 ml RBC/kg/yr provided approximately 1.85 mg of iron/kg/yr. Individual data are given in table 1. CPET-treated patients exchanged showed clinical improvement with disappearance of SCD crisis and related complications. The procedure was well tolerated by most patients, and adverse effects were limited to mild hypotension (3/10). No autoimmune hemolysis or allo-immunisation was documented in this cohort. All children remained negative for HIV and hepatitis C virus infections. Conclusion: Manual CPET seems to be safe to prevent middle-term iron overload and the need of elation therapy in most of patients. CPET can therefore be recommended for SCD patients who required decreased in Hb S levels either prophylactically or therapeutically. Manual are safe, effective and easy to use when mechanized exchanges are not possible for technical reasons. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Phospholipase A2 levels in acute chest syndrome of sickle cell disease

Blood ◽  
1996 ◽  
Vol 87 (6) ◽  
pp. 2573-2578 ◽  
Author(s):  
LA Styles ◽  
CG Schalkwijk ◽  
AJ Aarsman ◽  
EP Vichinsky ◽  
BH Lubin ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Phospholipase A2 ◽  
Sickle Cell ◽  
Fat Embolism ◽  
Clinical Severity ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Marrow Fat ◽  
Embolism Syndrome

Acute chest syndrome (ACS) is associated with significant morbidity and is the leading cause of death in patients with sickle cell disease (SCD). Recent reports suggest that bone marrow fat embolism can be detected in many cases of severe ACS. Secretory phospholipase A2 (sPLA2) is an important inflammatory mediator and liberates free fatty acids, which are felt to be responsible for the acute lung injury of the fat embolism syndrome. We measured SPLA2 levels in 35 SCD patients during 20 admissions for ACS, 10 admissions for vaso-occlusive crisis, and during 12 clinic visits when patients were at the steady state. Eleven non-SCD patients with pneumonia were also evaluated. To determine if there was a relationship between sPLA2 and the severity of ACS we correlated SPLA2 levels with the clinical course of the patient. In comparison with normal controls (mean = 3.1 +/- 1.1 ng/mL), the non- SCD patients with pneumonia (mean = 68.6 +/- 82.9 ng/mL) and all three SCD patient groups had an elevation of SPLA2 (steady state mean = 10.0 +/- 8.4 ng/mL; vaso-occlusive crisis mean = 23.7 +/- 40.5 ng/mL; ACS mean = 336 +/- 209 ng/mL). In patients with ACS sPLA2 levels were 100- fold greater than normal control values, 35 times greater than values in SCD patients at baseline, and five times greater than non-SCD patients with pneumonia. The degree of SPLA2 elevation in ACS correlated with three different measures of clinical severity and, in patients followed sequentially, the rise in SPLA2 coincided with the onset of ACS. The dramatic elevation of SPLA2 in patients with ACS but not in patients with vaso-occlusive crisis or non-SCD patients with pneumonia and the correlation between levels of SPLA2 and clinical severity suggest a role for SPLA2 in the diagnosis and, perhaps, in the pathophysiology of patients with ACS.

scholarly journals Fat Embolism Syndrome in a Child with Sickle Cell Disease

2019 ◽  
Vol 214 ◽  
pp. 236
Author(s):  
Arielle Maroni ◽  
Stéphane Dauger ◽  
Maryline Chomton
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fat Embolism ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome
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