Reversal of Severe Multiorgan Failure Associated With Sickle Cell Crisis Using Plasma Exchange: A Case Series

2019 ◽  
Vol 35 (2) ◽  
pp. 140-148
Author(s):  
Gulrukh Z. Zaidi ◽  
Juliana A. Rosentsveyg ◽  
Katayoun F. Fomani ◽  
James P. Louie ◽  
Seth J. Koenig
Keyword(s):  
Length Of Stay ◽  
Organ Failure ◽  
Plasma Exchange ◽  
Sickle Cell ◽  
Multiorgan Failure ◽  
Tertiary Care ◽  
Laboratory Data ◽  
Case Series ◽  
University Hospital ◽  
Sickle Cell Crisis

Objective: Red blood cell exchange (RBCE) is the standard of care for patients with sickle cell disease (SCD) who present with severe vaso-occlusive crisis (VOC). However, subsets of these critically ill patients have progressive multiorgan failure (MOF) despite RBCE therapy. The purpose of this case series is to describe the use of plasma exchange (PLEX) for the treatment of SCD-related MOF that is refractory to RBCE. Methods: A retrospective case review of patients with severe MOF from sickle cell crisis unresponsive to RBCE who underwent PLEX in a 14-bed adult medical intensive care unit (ICU) at a tertiary care university hospital over a 4-year time period. Key laboratory data including complete blood count, indices of hemolysis, and markers of organ failure were recorded before and after both RBCE and PLEX. Results: Our primary objective is to evaluate the effectiveness of PLEX, in addition to RBCE, on organ dysfunction, laboratory indices, and mortality. Of the 7 patients, 6 survived. Of the patients who survived, all remained hemodynamically stable during PLEX sessions and showed both clinical and laboratory evidences of improvement in hemolysis and organ function. Average time from completion of first PLEX treatment to initial laboratory signs of organ failure reversal for patients who survived was 15.6 hours, the average length of stay in the ICU was 5.6 days, and the average total length of stay in the hospital was 14 days. Conclusions: Plasma exchange, in addition to RBCE, may be a novel synergistic treatment option to decrease risk of mortality in patients with refractory VOC and MOF.

Evaluating Thrombocytopenia and Other Biomarkers in Multi-Organ Failure during Sickle Cell Crisis

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4858-4858
Author(s):  
Krithika Shanmugasundaram ◽  
Ixavier Higgins ◽  
Fuad A El Rassi ◽  
Morgan L. McLemore
Keyword(s):  
Platelet Count ◽  
Organ Failure ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Laboratory Data ◽  
Gram Negative Bacteria ◽  
Gram Negative ◽  
Time To Death ◽  
Multi Organ Failure ◽  
Sickle Cell Crisis

Background: In sickle cell anemia, very few markers of disease have been shown to correlate with activity and severity of crisis. We aimed to identify any patterns amongst biomarkers and co-morbidities that correlate with death from multi-organ failure when admitted for sickle cell crisis. Methods: We identified 20 patients with sickle cell anemia who died of multi-organ failure at Grady Memorial Hospital from 2010-2016. We described baseline characteristics, such as hemoglobin phenotype, age, gender as well as features at presentation such as vital signs, laboratory data, clinical signs and symptoms (I.e. worsening pain), and co-morbidities. We examined bivariate associations between clinical characteristics and the length of survival after multi-organ failure. In a secondary analysis, we investigated clinical and demographic features that most accurately predict infection with gram-negative bacteria. To achieve this, we fit a multivariate logistic regression model and perform leave-one-out cross validation to assess the predictive performance of the fitted model on unseen data. Results: At baseline, the average age of a patient is 43.8 years (sd=14.7 years). A majority of our cohort are women (60%), do not smoke (70%), and were not on hydroxyurea (80%). Time to death from multi-organ failure was 3.05 days (95% C.I.: 0.20, 5.90), and only 43% identified having "worsening pain." Among complications, 23% had a stroke and 25% had an infection with gram-negative bacteria. With regards to laboratory data, average WBC was 13.4 (95% C.I.: 11.04, 15.70), Platelets 155 (95% C.I.: 102.22, 208.38), d-dimer 12,892 (95% C.I.: -2498.47, 33437.87) and ferritin 3108 (95% C.I.: 795.07, 5421.63). When using a univariate analysis, we identified that stroke (p=0.008), age (p=0.022) and platelet count at the time of presentation (p=0.039) correlated with time to death. We also identified a statistically significant decrease in the platelet count from baseline to time of multi-organ failure (-105.6, p<0.001). Body mass index (BMI) and worsening pain jointly predict infection with gram-negative bacteria with small error (0.133). Conclusion: We have described a cohort of patients with sickle cell anemia who died of multi-organ failure and found that some factors may have correlated with time to death. The most poignant factors include age, platelet count, and drop in platelet count from baseline. It is also interesting that a proportion of these patients developed a gram-negative bacterial infection, which is a common cause of mortality in sepsis. Although our cohort is small, these data may help guide future studies with larger cohorts of patients to identify what puts these patients at risk of death from multi-organ failure. Disclosures No relevant conflicts of interest to declare.

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

Transfusion ◽  
2017 ◽  
Vol 58 (2) ◽  
pp. 470-479 ◽  
Author(s):  
James E. Louie ◽  
Caitlin J. Anderson ◽  
Katayoun Fayaz M. Fomani ◽  
Alonye Henry ◽  
Trevor Killeen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Plasma Exchange ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Multiorgan Failure ◽  
Case Series ◽  
Therapeutic Plasma Exchange ◽  
Cell Disease

scholarly journals Successful Use of Plasma Exchange Preceding RBC Exchange in Sickle Cell Disease Hyperhemolytic Crisis with Hepatic Sequestration: A Case Report

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4279-4279
Author(s):  
Samir Atiya ◽  
Rosalyn I Marar ◽  
Aleh Bobr
Keyword(s):  
Case Report ◽  
Liver Failure ◽  
Organ Failure ◽  
Plasma Exchange ◽  
Sickle Cell ◽  
Case Reports ◽  
Multiorgan Failure ◽  
Multi Organ Failure ◽  
Free Heme ◽  
Pain Crisis

Abstract Introduction Hyperhemolytic crisis is an uncommon complication of SCD that may cause multiorgan failure and lead to significant mortality. There are no current national or international guidelines for management of hyperhemolytic crisis and associated complications. There have been limited number of case reports and series that demonstrated utility of plasma exchange in the patients with multiorgan failure resulting from hemolysis complications (Zaidi GZ et al.,2020). We are presenting the case where hyperhemolytic crisis was complicated by hepatic sequestration and acute liver failure, that was dramatically reversed by 2 plasma exchange treatments followed by RBC exchange. Case report We present a case of a 35-year-old African American male with SCD and beta thalassemia trait with frequent hospitalizations for sickle cell pain crisis. He presented with pain typical for his acute pain crises and was admitted for intravenous hydration and pain control. The next morning, lab work showed bicytopenia with a drop in hemoglobin from 10.5 to 5.8 g/dL and platelets (PLT) from 100 to 22 X10E3/uL. Lactate dehydrogenase (LDH) increased from 434 to 2848 U/L, haptoglobin was 36 mg/dL, but disseminated intravascular coagulation (DIC) and Heparin-induced Thrombocytopenia (HIT) antibody panel were negative. The blood urea nitrogen (BUN) creatinine (Cr) ratio was also elevated (30.6) suggesting renal damage as well. He was transferred to the intensive care unit and started on Intravenous Immunoglobulin (IVIG) 0.4 grams/kilogram daily for 5 days and methylprednisolone 500 mg daily for 2 days followed by a prednisone taper. Liver enzymes continued to trend upward with AST of 19,866 U/L and ALT of 3,675 U/L on day 3 of hospitalization. Ultrasound of abdomen demonstrated mild splenomegaly with a spleen measuring 13.3 cm. The clinical presentation and hepatocellular pattern of injury was consistent with hepatic sequestration crisis. Despite receiving 1 unit of platelet 3 units of pRBC, there was little improvement and apheresis service was consulted. Plasma exchange was initiated for 2 procedures on consecutive days followed by RBC exchange with rapid improvement in clinical status and laboratory findings with a reduction of LDH (1304), AST (129), ALT (204), Hgb (8.0), PLT (41), BUN/Cr (20.0). He was discharged on day 7 at baseline status. Discussion Although the mechanism of development of hyperhemolysis in SCD is not fully understood, the hemolysis leads to release of free hemoglobin (Hb) and free heme that activate neutrophils, and vascular endothelial cells via TLR-4. This ultimately leads to inflammatory, coagulative, and cytotoxic damages and decreased nitric oxide (NO) bioavailability which further contributes to SCD complications such as pulmonary and systemic vasculopathy, pain crisis and acute chest syndrome and multi organ failure (Louie JE et al., 2018). This provides a rationale for plasma exchange - removal of free heme from the patient plasma and replenishing exhausted haptoglobin and hemopexin reserves from donor plasma. Hemolytic crisis causing visceral organ damage is relatively rare. There are no current guidelines for management of such patients. In 1996 Betrosian et al. discussed the first case of liver failure in a SCD with vasa-occlusive crisis treated with RBC and plasma transfusions (Betrosian A et al., 1996). Since then, there have been case reports/series of plasma exchange/plasma transfusions in SCD with multi organ failure (Geigel EJ et al., 1997, Louie JE et al., 2018) but reports about use of plasma exchange in SCD patients with hepatic sequestration have not been identified by our literature review. Our case demonstrates that plasma exchange in hyperhemolysis and hepatic sequestration is: Safe Leads to quick and significant improvement in hemolysis laboratory values. Results in quick and durable reversal of hepatic sequestration and associated liver failure. Adds plasma exchange as therapeutic apheresis modality in addition to previously accepted RBC exchange. Provides data about priority of plasma exchange over RBC exchange in this clinical situation. Disclosures No relevant conflicts of interest to declare.

scholarly journals Commonly Used Severity Scores Are Not Good Predictors of Mortality in Sepsis from Severe Leptospirosis: A Series of Ten Patients

2012 ◽  
Vol 2012 ◽  
pp. 1-6
Author(s):  
Dimitrios Velissaris ◽  
Menelaos Karanikolas ◽  
Nikolaos Flaris ◽  
Fotini Fligou ◽  
Markos Marangos ◽  
...  
Keyword(s):  
Small Sample Size ◽  
Multiorgan Failure ◽  
Good Condition ◽  
Case Series ◽  
Scoring Systems ◽  
Small Sample ◽  
University Hospital ◽  
Severity Scores ◽  
Retrospective Data Collection ◽  
Severe Leptospirosis

Introduction. Severe leptospirosis, also known as Weil's disease, can cause multiorgan failure with high mortality. Scoring systems for disease severity have not been validated for leptospirosis, and there is no documented method to predict mortality.Methods. This is a case series on 10 patients admitted to ICU for multiorgan failure from severe leptospirosis. Data were collected retrospectively, with approval from the Institution Ethics Committee.Results. Ten patients with severe leptospirosis were admitted in the Patras University Hospital ICU in a four-year period. Although, based on SOFA scores, predicted mortality was over 80%, seven of 10 patients survived and were discharged from the hospital in good condition. There was no association between SAPS II or SOFA scores and mortality, but survivors had significantly lower APACHE II scores compared to nonsurvivors.Conclusion. Commonly used severity scores do not seem to be useful in predicting mortality in severe leptospirosis. Early ICU admission and resuscitation based on a goal-directed therapy protocol are recommended and may reduce mortality. However, this study is limited by retrospective data collection and small sample size. Data from large prospective studies are needed to validate our findings.

scholarly journals "Prevalence and Outcome of Avascular Necrosis of the Hip (AVN) Among Young Omani Patients with Sickle Cell Disease"

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3577-3577
Author(s):  
Iman Al Fadhali ◽  
Farah Al-Kindy ◽  
Naema Alshibli ◽  
Salam Alkindi ◽  
Murtadha Al-Khabori ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Tertiary Care ◽  
University Hospital ◽  
Average Dose ◽  
Cell Disease ◽  
Advisory Committees ◽  
Female Ratio

Background: Sickle cell disease is a common hemaglopinapthy worldwide and in Oman. Avascular necrosis (AVN) of the hip causes significant morbidity to patients with sickle cell disease and has a profound impact on their quality of life. This study aims to identify the prevalence and outcome of AVN among young Omani patients with sickle cell disease. Methods: The is a cross sectional study done in the main tertiary care and referral facility in Oman, Sultan Qaboos University Hospital. Out of 3000 registered patients 85 patients found to have clinical and radiological proven AVN, between June 2017-January 2019. Data obtained included demographics, the affected joint, uni or bilateral), radiological staging by FICAT (MRI), hydroxyurea use, dose and duration as well as laboratory data at time of diagnosis. Results: The prevalence of AVN among SCD Omani children and young adults is 2.8 %. Their age ranges between 5-25 (Mean 14.6 +3.4). Male to female ratio was 1.6:1. Based on FICAT system score, most of the cases (82%) are AVN grade 3 and above at diagnosis. Thirty-six patients (42%) were diagnosed to have stage IV AVN. Regarding the hydroxyurea use, 43 patients (50.6%) were already started on HU before the development of AVN. Their mean duration of HU use was 5.3 years, with an average dose of 15.7 mg/kg/day. Fifteen patients developed AVN and were started on HU as part of their management. The follow up MRI of 4 of them showed improvement of their AVN stage (from 4 to 2). Fifteen patients (17.6%) underwent joint replacement because of chronic pain and disability and almost half of them (7 patients) were on hydroxyurea. Conclusion: Prevalence of AVN in young patients with SCD in the main tertiary care referral facility in Oman is 2.8% which is less than the internationally reported. Patients are diagnosed at later stages (3 and above) indicating a possibility of underdiagnosis of asymptomatic stage one and 2 patients. Hydroxyurea use has improved the severity of the AVN in few patients. Disclosures Al-Khabori: SOBI: Honoraria; AstraZeneca: Honoraria; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; Roche: Membership on an entity's Board of Directors or advisory committees; Amgen: Membership on an entity's Board of Directors or advisory committees; Servier: Membership on an entity's Board of Directors or advisory committees; Shire (Takeda): Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees; NovoNardisk: Membership on an entity's Board of Directors or advisory committees. Wali:Sultan Qaboos University Hospital: Employment.

Using Internet Cloud Platform To Characterize Neurosurgical Patients in an Emergency Hospital Admission Setting: Retrospective Observational Study (Preprint)

2021 ◽  
Author(s):  
Wei Chen ◽  
Xiangkui Li ◽  
Xiangkui Li
Keyword(s):  
Length Of Stay ◽  
Vascular Disease ◽  
Tertiary Care ◽  
Multivariate Logistic Regression Analysis ◽  
Emergency Admission ◽  
University Hospital ◽  
Cloud Platform ◽  
Non Profit ◽  
Neurosurgical Patients ◽  
Medical Big Data

BACKGROUND Neurosurgical patients are admitted to hospital via emergency department admissions is common; however, studies designed to describe their features are not available. OBJECTIVE This study aims to investigate the characteristics of patients admitted to the neurosurgery department in an emergency manner by using medical big data integration and application platform (internet cloud platform). METHODS We derived data from the internet cloud platform of West China Hospital, Sichuan University. The data of consecutive patients admitted to the department of neurosurgery as emergency admissions in a non-profit tertiary care university hospital was collected. Data on demographic information, clinical characteristics and outcomes were collected and evaluated through the platform. Patients were stratified into five disease groups (vascular disease, trauma, oncology, spine and others) according to their main diagnoses at the time of admission. RESULTS A total of 4,149 cases (median age 52 years, 54.5% male) were identified in this study. Vascular disease was the most common reason for emergency admission (73.5%). Significant differences were found among the five disease groups in sex (P<.001), age (P<.001), surgery (P<.001) and season (P=.009) but not in the length of stay (P=.784). Multivariate logistic regression analysis identified male sex, older age, short length of stay, surgery not performed and disease type (particularly trauma) as independently associated with in-hospital mortality. CONCLUSIONS By using internet cloud platform, we identified significant demographic and clinical differences among neurosurgical patients admitted to the neurosurgery department as an emergency. These findings may assist health care practitioners in shifting tasks and clinical resources toward those patients who may benefit from particular intervention approaches.

Congenital Nasal Pyriform Aperture Stenosis: Diagnosis and Management

2009 ◽  
Vol 46 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Marion Devambez ◽  
Alexis Delattre ◽  
Pierre Fayoux
Keyword(s):  
Surgical Treatment ◽  
Genetic Disorder ◽  
Tertiary Care ◽  
Case Series ◽  
University Hospital ◽  
Treatment Modalities ◽  
Nasal Decongestants ◽  
Sublabial Approach ◽  
Pyriform Aperture

Objectives: To review diagnosis and treatment modalities in congenital nasal pyriform aperture stenosis in a newborn population. Study Design: A 6-year retrospective case series review. Mean follow-up of 21.8 months. Setting: University hospital, tertiary care center. Patients: Twenty-one consecutive patients treated for congenital nasal pyriform aperture stenosis. Diagnosis was suspected on physical examination and confirmed by computed tomography scan. Associated abnormalities were present in 15 patients. Surgical Treatment: Nineteen patients underwent surgical treatment focused on external bony margins of pyriform aperture and the osseous anterior edge of the inferior turbinate, drilling by a sublabial approach and associated with a partial turbinectomy in seven cases. A stent was placed for 7 to 10 days. A conservative treatment based on topical nasal decongestants and gastroesophageal reflux treatment was proposed for two patients. Results: Postoperative follow-up revealed septal ulceration in five cases with septal perforation in one case and development of synechiae in two cases. Follow-up revealed normal nasal breathing and pyriform aperture growth after surgery. Conclusion: Our experience confirmed the link described between congenital nasal pyriform aperture stenosis and holoprosencephaly, but associated extracraniofacial malformations suggest that congenital nasal pyriform aperture stenosis should be integrated in a systemic malformative syndrome or genetic disorder and lead us to propose an exhaustive dysmorphology assessment. Some infants may be treated by conservative management, and severely affected patients may undergo surgery by a sublabial approach.

Endoscopic Lateral Cartilage Graft Tympanoplasty

2017 ◽  
Vol 157 (4) ◽  
pp. 683-689 ◽  
Author(s):  
Cameron C. Wick ◽  
Demetri Arnaoutakis ◽  
Vivian F. Kaul ◽  
Brandon Isaacson
Keyword(s):  
Tympanic Membrane ◽  
Tertiary Care ◽  
Case Series ◽  
University Hospital ◽  
Long Term Results ◽  
Lateral Cartilage ◽  
Cartilage Graft ◽  
Closure Rate ◽  
Tympanic Membrane Perforations ◽  
The Mean

Objective To describe a novel technique for lateral graft tympanoplasty. Study Design Case series with chart review. Setting Tertiary care university hospital. Subjects and Methods Pediatric and adult patients with tympanic membrane perforations deemed unfavorable for a medial graft technique due to the perforation characteristics or myringitis. Results Between 2014 and 2016, 34 ears from 31 patients underwent a transcanal endoscopic lateral cartilage graft tympanoplasty. The mean age was 24.4 years (range, 6-71 years), and 22 patients (65%) were younger than 18 years. All patients had tympanic membrane perforations. Eighteen patients (53%) had total or near-total perforations, leaving a minimal anterior remnant, and 16 patients (47%) had extensive myringitis. A bisected tragal cartilage-perichondrium shield graft was used in 33 patients (97%). The mean (SD) follow-up length was 9.8 (5.7) months. Initial perforation closure rate was 88.2% (30/34). Three of the persistent perforations underwent a revision endoscopic medial graft tympanoplasty with successful closure, leaving a final closure rate of 97.1% (33/34). Five patients (15%) required topical therapy for postoperative myringitis. Mean (SD) pure-tone average and air-bone gap significantly improved by 11.5 (10.7) dB ( P < .001) and 11.4 (10.6) dB ( P < .001), respectively. Twenty-seven patients (79%) closed their air-bone gap within 20 dB. Conclusion Transcanal endoscopic lateral cartilage graft tympanoplasty is feasible, and initial data support favorable outcomes. Further data are necessary for evaluation of long-term results and efficacy comparisons.

scholarly journals INCIDENCE OF HEMATOLOGICAL MALIGNANCIES IN SICKLE CELL PATIENTS FROM AN INDIAN TERTIARY CARE TEACHING HOSPITAL

2018 ◽  
Vol 11 (3) ◽  
pp. 205 ◽  
Author(s):  
Sima Chauhan ◽  
Santosh Kumar Swain ◽  
Mahesh Chandra Sahu
Keyword(s):  
Sickle Cell ◽  
Hematological Malignancies ◽  
Tertiary Care ◽  
Case Series ◽  
Organ Damage ◽  
Population Based ◽  
Variable Degree ◽  
Multiple Systems ◽  
Institutional Ethics ◽  
High Degree

 Objective: Sickle cell disease (SCD) involves multiple systems and is manifested by variable degree of anemia, acute vaso-occlusive episodes, and chronic organ damage. Small case series have hinted at increased incidence of cancer among SCD patients, but no examination of population-based data in Odisha has been reported. Here, we have reported the association of hematological malignancies and SCD in our region.Methods: In this prospective study, we have documented all the demographic and clinical data of sickle cell patients during the past 3 years from June 2013 to May 2016. Simultaneously, the bone marrow (BM) aspiration and biopsy were carried out for all patients. This study was approved from the Institutional Ethics Committee.Results: A total number of 267 sickle cell patients were screened for malignancy by both BM aspiration and biopsy. Among them, 6 cases of malignancies were detected by both BM aspiration and biopsy method.Conclusion: Our study shows a association between SCD and hematological malignancies which could be due to treatment with hydroxyurea or accumulation of multiple genetic abnormalities due to a high degree of proliferative activity of marrow cells.

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