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sickle cell disease and multiorganic failure

Elliott Vichinsky
Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes hemolysis and nitric oxide deficiency. As patients age, most experience neurologic insult. Twenty-five percent of older adults have had a clinical stroke and at least half of the population have had a silent infarct, cortical atrophy, and neurocognitive impairment. Periodic screening with neuroimaging and neurocognitive testing is recommended...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
James E Louie, Caitlin J Anderson, Katayoun Fayaz M Fomani, Alonye Henry, Trevor Killeen, Narla Mohandas, Karina Yazdanbakhsh, John D Belcher, Gregory M Vercellotti, Patricia A Shi
BACKGROUND: Depletion of haptoglobin (Hp) and hemopexin (Hx) with increase in free hemoglobin and heme are important etiologies of vaso-occlusive complications in sickle cell disease (SCD). This study is the first to show an association between clinical improvement in SCD and repletion of Hp and Hx by therapeutic plasma exchange (TPE) using plasma replacement. STUDY DESIGN AND METHODS: Thirteen fresh-frozen plasma (FFP) units derived from consecutive whole blood donations were thawed at 37°C after 10 months of storage; Hp and Hx concentrations immediately postthaw and after 5 days of refrigerated storage were analyzed by enzyme-linked immunosorbent assay (ELISA)...
February 2018: Transfusion
Jonathan M Davis, Nura El-Haj, Nimish N Shah, Garry Schwartz, Margaret Block, James Wall, Mark Tidswell, Ernest DiNino
BACKGROUND: Red blood cell (RBC) transfusion is an important treatment modality during severe sickle cell crisis (SCC). SCC patients who refuse, or cannot accept, RBCs present a unique challenge. Acellular hemoglobin (Hb)-based oxygen carriers (HBOCs) might be an alternative for critically ill patients in SCC with multiorgan failure due to life-threatening anemia. HBOC-201 (HbO2 Therapeutics) has been administered to more than 800 anemic patients in 22 clinical trials, but use of any HBOCs in critically ill sickle cell patients with organ failure is exceedingly rare...
January 2018: Transfusion
Samir K Ballas
Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient...
September 2017: Transfusion
Salimah Valliani, Pradyumna Agasthi, Gloria Westney, Marilyn Foreman
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
Radhika Gangaraju, Vishnu V B Reddy, Marisa B Marques
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...
September 2016: Southern Medical Journal
Pelin Kardaş Karacaoglu, Suheyl Asma, Aslı Korur, Soner Solmaz, Nurhilal Turgut Buyukkurt, Cigdem Gereklioglu, Mutlu Kasar, Demircan Ozbalcı, Selma Unal, Hasan Kaya, Emel Gurkan, Mahmut Yeral, Çagla Sariturk, Can Boga, Hakan Ozdogu
Sickle cell disease (SCD), one of the most common genetic disorders worldwide, is characterized by hemolytic anemia and tissue damage from the rigid red blood cells. Although hydroxyurea and transfusion therapy are administered to treat the accompanying tissue injury, whether either one prolongs the lifespan of patients with SCD is unknown. SCD-related mortality data are available, but there are few studies on mortality-related factors based on evaluations of surviving patients. In addition, ethnic variability in patient registries has complicated detailed analyses...
May 2016: Annals of Hematology
Muhammad Kashif, Hassan Tariq, Mohsin Ijaz, Jose Gomez-Marquez
Histoplasma duboisii, a variant of Histoplasma capsulatum that causes "African histoplasmosis," can be resistant to itraconazole, requiring intravenous amphotericin B treatment. Rarely, these patients do not respond to intravenous antifungal therapy, and in such cases, patients may progress to develop secondary hemophagocytic lymphohistiocytosis (HLH). We present a case of a 34-year-old male patient with sickle cell disease who presented with a 5-month history of an enlarging painless axillary mass, persistent low grade fevers, night sweats, weight loss, and anorexia...
2015: Case Reports in Critical Care
Laurane Cottin, Camille Rouvet, Chadi Homedan, Mathieu Conté, Satar Mortaza, Marie-Christine Rousselet, Anne Corby, Maïlys Le Guyader, Marc Zandecki, Pascal Reynier
We describe the case of a 30-year-old patient, suffering from composite S/β + sickle cell disease. He was hospitalized following a vaso-occlusive attack with acute bone pains. Despite an analgesic treatment and transfusion of three units of red blood cells, a non-regenerative anemia appeared within 24 hours. One day later an acute chest syndrome with atelectasis of the left lung and desaturation and multi-organ failure occurred and necessitated the patient's intubation and required him to be placed in an artificial coma...
September 2014: Annales de Biologie Clinique
Helen Swede, Biree Andemariam, David I Gregorio, Beth A Jones, Dejana Braithwaite, Thomas E Rohan, Richard G Stevens
PURPOSE: Given the relatively high prevalence of sickle cell trait and disease among African Americans and established racial disparities in cancer outcomes, we reviewed the literature regarding adverse events in cancer patients with these hematologic genotypes. Erythrocyte sickling can result from extreme hypoxia and other physiologic stressors, as might occur during cancer therapy. Further, tumoral hypoxia, a poor prognostic and predictive factor, could lead to a cycle of local sickling and increased hypoxia...
March 2015: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Samir K Ballas, Kalpna Gupta, Patricia Adams-Graves
Sickle cell pain includes 3 types: acute recurrent painful crises, chronic pain syndromes, and neuropathic pain. The acute painful crisis is the hallmark of the disease and the most common cause of hospitalization and treatment in the emergency department. It evolves through 4 phases: prodromal, initial, established, and resolving. Each acute painful episode is associated with inflammation that worsens with recurrent episodes, often culminating in serious complications and organ damage, such as acute chest syndrome, multiorgan failure, and sudden death...
November 1, 2012: Blood
Jill Adamski, Courtney A Hanna, Vishnu B Reddy, Silvio H Litovsky, Cristina A Evans, Marisa B Marques
No abstract text is available yet for this article.
June 2012: American Journal of Hematology
Janet I Malowany, Jagdish Butany
Sickle cell disease (SCD) is a hereditary chronic hemolytic anemia with numerous clinical consequences. Intravascular sickling of red blood cells leads to multiorgan dysfunction. Although the pathophysiology of SCD has been well studied, there remains a lack of effective treatment. Refinements in overall care have improved quality of life; however, premature death is still not uncommon. SCD usually presents in childhood and is common in areas where malaria is (or was) common. The association with malaria is apparently of benefit to the individual because these individuals tend to contract a milder form of the disease...
February 2012: Seminars in Diagnostic Pathology
Eldad A Hod, Steven L Spitalnik
Retrospective studies suggest that the transfusion of older, stored red blood cells (RBCs) may be associated with increases in mortality, serious infections, multiorgan failure, thrombosis, and hospital length of stay. Our research is based on the overarching hypothesis that the adverse effects associated with transfusion of older, stored RBCs result from the acute delivery of hemoglobin iron to the monocyte-macrophage system. To test this "iron hypothesis," we are recruiting healthy human volunteers to donate double, leukoreduced, RBC units...
April 2011: Transfusion
Courtney D Fitzhugh, Naudia Lauder, Jude C Jonassaint, Marilyn J Telen, Xiongce Zhao, Elizabeth C Wright, Francis R Gilliam, Laura M De Castro
Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 were enrolled. Forty-three patients (21 males and 22 females) died during the study period. The median age of survival was 39 years for females (95% CI: 34-56), 40 years for males (95% CI: 34-48), and 40 years overall (95% CI: 35-48)...
January 2010: American Journal of Hematology
Venkata Sasidhar Majjiga, Asit K Tripathy, Kusum Viswanathan, Mayank Shukla
No abstract text is available yet for this article.
October 2010: Clinical Pediatrics
Ulrich Hammer, Rudolf Wegener, Horst Nizze, Gunther Wöhlke, Christine Kruse, Bernd Dworniczak, W Nikolaus Kühn-Velten, Gabriele Nöldge-Schomburg, Rainer Hofmockel, Ludwig Jonas
A 20-year old African woman underwent anesthesia for interruption of an unwanted pregnancy. As a consequence of the anesthesia, she went into coma because of an as yet unknown and untested homozygotic state of sickle cell anemia. Her vital functions were maintained for more than 1 year by intensive medicine, but she died finally in multiorgan failure and aspiration pneumonia. Because of the complications under anesthesia and the missing preanesthetic test for hemoglobinopathy, autopsy was conducted in the forensic medicine department and not in the department of pathology...
November 2006: Ultrastructural Pathology
Taiwo Kotila, Kayode Adedapo, Aduragbenro Adedapo, Olayiwola Oluwasola, Eyitayo Fakunle, Biobele Brown
The liver is one of the organs involved in the multiorgan failure that occurs in sickle cell disease, the pathophysiology of liver disease in this condition is complex because of the interrelated multifactorial causes. Liver dysfunction was assessed in both paediatric and adult sickle cell disease patients in the steady state. The transaminases and alkaline phosphatase were analysed by automation while coagulation studies were done manually. The mean (range) of Alanine transaminase (ALT), Aspartate transaminase (AST) and alkaline phosphatase (ALP) were 23...
October 2005: Annals of Hepatology
Matthew M Baichi, Razi M Arifuddin, Parvez S Mantry, Adel Bozorgzadeh, Charlotte Ryan
Very few cases of liver transplantation in patients with sickle cell disease have been reported in peer-reviewed literature. We reviewed the medical records of two patients with sickle cell disease that received liver transplantation at our institution. The first patient was a 27-year-old female who presented with encephalopathy and cholestatic jaundice with a Hemoglobin S (HbS) level of 69.6%. She was diagnosed with acute sickle cell intrahepatic cholestasis. The second patient was a 26-year-old female with sclerosing cholangitis who presented with encephalopathy, bleeding, and cholestatic jaundice...
December 15, 2005: Transplantation
Allen P Burke, Erik Mont, Frank Kolodgie, Renu Virmani
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) shares histologic features with disseminated intravascular coagulation (DIC) but is clinically distinct. TTP may result in myocardial hemorrhages and rapid death. We compared rapidly fatal TTP with heart involvement and DIC cases to determine if the differential diagnosis of TTP and DIC could be aided by immunohistochemical techniques. DESIGN: We examined 11 hearts from seven women and four men dying with TTP (aged 34+/-10 years) and 8 hearts from patients dying with DIC (five women, three men, aged 58+/-16 years)...
May 2005: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
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