scholarly journals Life-Threatening Acute Chest Syndrome in a Patient With Sickle Cell Disease After Switching From Hydroxyurea Therapy to Partial Exchange Transfusions: A Case Report

Cureus ◽  
2021 ◽  
Author(s):  
Ann K Kvam ◽  
Henrik A Torp ◽  
Per O Iversen
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Life Threatening ◽  
Hydroxyurea Therapy

scholarly journals Case Report: Acute Stroke in Young Adult Patient with Moyamoya Syndrome Secondary to Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-13
Author(s):  
Oladipo Cole ◽  
Asia Filatov ◽  
Javed Khanni ◽  
Patricio Espinosa
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Moyamoya Disease ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
African American Female ◽  
Moyamoya Syndrome ◽  
Cell Disease ◽  
Radiographic Findings

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.

A Severe Case of Delayed Haemolytic Transfusion Reaction in a Sickle Cell Disease Patient: Immuno-Haematological Study, Cytokine Transcripts and Lymphocyte Subsets Analysis.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 954-954
Author(s):  
France Noizat-Pirenne ◽  
Helene Ansart-Pirenne ◽  
Anne Plonquet ◽  
Philippe Chadebech ◽  
Anoosha Habibi ◽  
...  
Keyword(s):  
Case Report ◽  
Natural Killer Cells ◽  
Sickle Cell Disease ◽  
Natural Killer ◽  
Sickle Cell ◽  
Lymphocyte Subsets ◽  
Transfusion Reaction ◽  
Cell Disease ◽  
Killer Cells ◽  
Life Threatening

Abstract Background: Delayed haemolytic transfusion reaction (DHTR) may be a life-threatening complication in sickle cell disease (SCD) patients. DHTR is characterized by a marked drop in hemoglobin (Hb) and is frequently accompanied with intensification of the disease symptoms. Pathogenesis of DHTR is not completely understood as autologous RBCs are probably destroyed and allo-antibodies against transfused RBCs in the course of the accident are not always detected. Here we describe a case of serious DHTR and analyse lymphocyte subsets and cytokine transcripts. Case report: A 33-year-old man with SCD was scheduled for hip replacement. He was poly-immunized (anti-RH2, -RH23, -RH30, -FY1, -FY3,-MNS3, -YT2) and had history of 2 DHTR. He received 7 units of crossmatch-compatible blood at day 0 of surgery. On days 5 and 8, sera were still compatible with samples of units received at day 0. Direct antiglobulin test (DAT) and eluate were negative. Hb remained stable at 6 g.dl−1. On day 14, the patient presented pain, fever and signs of hemolysis including a drop in Hb to 3.5 g.dl−1, LDH at 12460 U per L, bilirubin at 111 μmol/L and renal failure. Serological and eluate evaluation revealed the presence of antibodies against all RBCs tested (including units previously transfused at day 0), DAT positive with anti-C3 and anti-IgG. Hb dropped to 2.5 g.dl−1, the patient presenting neurologic symptoms. Because of the life-threatening anemia, he received 4 units that were compatible only with the known antibodies, associated with corticoids and cyclophosphamide. An anti-MNS5 was finally detected explaining partly the positive reactions against all RBCs tested. Then, additional U-negative units were transfused. The patient gradually improved symptomatically and was discharged from the high dependency unit on day 30. Hb level reached 6 g.dl−1. As compared to day 0, vigorous expansion (ten times) of Natural Killer subset (CD56+, CD16+) was observed on day 14 during hemolysis. This expansion was correlated to an increase of IL-10 transcripts whereas IL-2 and IFNγ transcripts were not detected. After treatment (day 32) lymphocyte subsets returned to the day 0 level and IL10 transcripts disappeared. Conclusion : This case report confirms that DHTR can be induced by transfusion of crossmatch-compatible units. In this poly-immunized SCD patient, transfusion has elicited production of auto-antibodies. The observed expansion of Natural Killer cells during the hemolysis suggests that Natural Killer cells could participate, through an ADCC mechanism, to the destruction of both transfused and autologous RBCs. The involvement of IL-10 on Natural Killer FcγRIIIa receptor expression and Natural Killer cytotoxicity will be discussed.

scholarly journals Acute chest syndrome in sickle cell disease/HBE patient, A case report

2021 ◽  
Vol 9 (8) ◽  
Author(s):  
Ibrahim Khamees ◽  
Waail Rozi ◽  
Mohamed A. Yassin
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease

Clinical Characteristics Associated with Survival in Adult Sickle Cell Disease

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3229-3229
Author(s):  
Hany Elmariah ◽  
Melanie E. Garrett ◽  
Kenneth I. Ataga ◽  
Allison E Ashley-Koch ◽  
Marilyn J. Telen
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Survival Data ◽  
Conflicts Of Interest ◽  
Cox Proportional Hazards Model ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Cerebrovascular Events ◽  
Hydroxyurea Therapy

Abstract Abstract 3229 Background: Sickle cell disease (SCD) greatly decreases survival of affected patients, and significant advances will be necessary to decrease the gap in expected survival between SCD patients and non-affected individuals. We examined the relationship of clinical differences among SCD patients to survival, in order to gain greater understanding of major contributors to early mortality. Identification of such factors could guide development of therapeutic options. Methods: Survival data were obtained for 417 adult subjects previously enrolled in a study of clinical outcome modifying genes in SCD from Duke University Medical Center and the University of North Carolina at Chapel Hill. All subjects were ≥18 years at the time of enrollment and were followed for a mean of 7.9 years (range 2.3–10 years). At enrollment, a number of clinical parameters were collected, including hemoglobin (Hb) genotype (SS, SC, Sβ0 thalassemia, or Sβ+thalassemia), baseline laboratory values (Hb, WBC, platelets, reticulocytes, fetal Hb, LDH, MCV, proteinuria), comorbidities (cerebrovascular events, pulmonary hypertension, history of acute chest syndrome, avascular necrosis, priapism, and pain crises - defined as number of hospitalizations in the past 12 months, among others), and medication status (hydroxyurea, narcotics, and others). Levels of soluble adhesion molecules (sICAM, sVCAM, sE-selectin, sP-selectin), NT-proBNP, TNF-α, and interleukins-6, -8, and -10 were measured for a subset of 87 subjects. Regression analysis based on the Cox proportional hazards model was employed to determine the effect of clinical phenotypes on survival time using PROC PHREG in SAS v9.2 (SAS Systems, Cary, NC). All models were adjusted for gender and age at enrollment. Results: Mean age at enrollment was 34 years (range 18 to 84 years). The mean age at death was 45 years (range 24 to 86 years). Subjects with HbSβ0 had the worst prognosis (p=0.0001), followed by subjects with SS, SC, and Sβ+. Lower glomerular filtration rate (GFR, hazard ratio [HR]=1.087 per each ml/min decrease in GFR, p<0.0001), incidence of pain crises (HR=2.038, p=0.005), pulmonary hypertension (HR=2.269, p=0.005), cerebrovascular events (HR=1.875, p=0.008), proteinuria (HR=1.922, p=0.011), seizures (HR=2.138, p=0.012), short-acting narcotics use (HR=1.693, p=0.033) and TIAs (HR=2.407, p=0.043) were significantly associated with decreased survival. Lower baseline Hb was also associated with decreased survival (HR=1.259 per g/dl decrease, p=0.0047), but after controlling for GFR, was no longer significant (p=0.274). Additionally, increased NT-proBNP (HR=1.617, p=0.0004) and sVCAM-1 (HR=2.032, p=0.0003) were associated with decreased survival. Fifty percent of patients were on hydroxyurea therapy, which was not associated with a change in survival (p=0.503). Conclusion: SCD continues to reduce life expectancy for affected individuals, particularly those with Hb Sβ0 and SS. Surprisingly, we found that Sβ0 had a significantly worse survival compared to SS. Cerebrovascular events, pulmonary hypertension, proteinuria, decreased GFR, and more frequent pain crises were also strongly associated with poorer survival. Not only were these comorbidities individually associated with decreased survival, but an additive effect was observed, such that subjects with a greater number of negative endpoints had worse survival (p<0.0001). These traits may provide some utility in predicting prognosis of SCD patients. More importantly, aggressive management of these comorbidities may produce a survival benefit. The association of higher sVCAM-1 levels with decreased survival suggests that targeted therapies to reduce endothelial damage and inflammation may also be beneficial. In contrast to prior studies, hydroxyurea therapy had no influence on survival. This may reflect a failure in some patients to reach the maximum tolerated dose, lack of compliance, or more severe baseline disease in those patients who were treated. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Identification of the Pitfalls in the Attempted Use of Extracorporeal Membrane Oxygenation in an Adult Patient with Sickle Cell Disease and Severe Acute Chest Syndrome

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4854-4854
Author(s):  
Mira T Tanenbaum ◽  
Anna Shvygina ◽  
Vaishnavi Sridhar ◽  
Jennifer E. Vaughn ◽  
Mark Joseph
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Extracorporeal Membrane Oxygenation ◽  
Adult Patient ◽  
Sickle Cell ◽  
Case Reports ◽  
The United States ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Membrane Oxygenation

BACKGROUND: Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Despite being the leading cause of death in adult patients with SCD, current recommendations for treatment of ACS remain largely supportive, consisting of pain management, aggressive fluid resuscitation, respiratory support, and transfusion therapy. Despite these measures, it is not uncommon for patients to require intubation due to progression to acute respiratory distress syndrome (ARDS). Recently, there have been a number of case reports that have successfully utilized extracorporeal membrane oxygenation (ECMO) for the management of ACS in those patients who fail to respond to conventional therapy [Kuo et al., 2013, Sewaralthahab et al, 2018]. However, the use of ECMO in this patient population remains uncommon, and further evaluation of this intervention is needed. This case report details an unsuccessful attempt at the use of ECMO in the case of ARDS secondary to ACS, in an attempt to identify critical pitfalls. CASE REPORT: A 32-year-old African-American female with HbSS disease on hydroxyurea therapy was transferred from an outside hospital following 3 days of respiratory decompensation. Prior to arrival, patient coded once at the outside hospital and once on transfer. Veno-arterial ECMO was initiated with improving oxygen saturation and volume status with continuous renal replacement therapy. To maintain an ECMO-specific goal hemoglobin level of 10 g/dL, 1:1 manual exchange transfusions were performed due to an inability to access equipment for automated RBC exchange. Once stable enough for CT, patient was found to have gray-white inversion suggestive of irreversible severe brain damage. Following another 28 days of supportive care with no neurologic improvement, the family decided to withdraw care, and the patient expired. CONCLUSION: While unsuccessful, this patient's case revealed a need for defining parameters regarding the initiation of ECMO in SCD patients with severe ACS. A review of previously-published literature has shown that the use of ECMO for the management of ARDS in adults is more efficacious than conventional ventilation support [Peek et al., 2009]. In patients with SCD, this improvement in efficacy is not readily reproduced, likely due to unique challenges presented by the pathophysiology of the disease. Notably, patients with SCD face additional risks of venous thromboembolism and strokes while on prolonged bed rest due to a baseline prothrombotic state [Sewaralthahab et al., 2018]. A systematic review of available case reports is needed to develop a protocol for the management of severe ACS that takes SCD-specific risks into account. The present report also makes a case for the training of providers in the early recognition of severe ACS in SCD patients. SCD remains largely undertreated in the United States, likely due to a complex interplay of patient, physician, and institutional factors. Had this patient been transferred immediately to a facility better equipped to provide a higher level of care, her condition could have arguably taken a different course. Despite the aforementioned challenges, ECMO remains a feasible option for the management of severe ACS in patients with SCD, and efforts should be made to standardize current treatment protocols. REFERENCES: Kuo KW, Cornell TT, Shanley TP, Odetola FO, and Annich GM. The use of extracorporeal membrane oxygenation in pediatric patients with sickle cell disease. Perfusion. 2013 September; 28(5): 424-432. Peek GJ, Mugford M, Tiruvoipati R, Wilson A, Allen E, Thalanany M, et al. Efficacy and economic assessment of conventional ventilatory support versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled trial. The Lancet. 2009 October; 374(9698): 1351-1363. Sewaralthahab SS, Menaker J, Law JY. Successful use of veno-venous extracorporeal membrane oxygenation in an adult patient with sickle cell anemia and severe acute chest syndrome. Hemoglobin. 2018 42(1): 65-67. Disclosures No relevant conflicts of interest to declare.

scholarly journals Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature

2019 ◽  
Author(s):  
Nergis Akay ◽  
Deniz Tugcu ◽  
Rumeysa Tuna ◽  
Suheyla Ocak ◽  
Serap Karaman ◽  
...  
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Review Of The Literature ◽  
Cell Disease ◽  
Disease Case
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