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immunology of sickle cell anemia

Marianne E M Yee, Cassandra D Josephson, Anne M Winkler, Jennifer Webb, Naomi L C Luban, Traci Leong, Sean R Stowell, John D Roback, Ross M Fasano
BACKGROUND: Chronic transfusion therapy for sickle cell anemia reduces disease complications by diluting sickle-erythrocytes with hemoglobin A (HbA)-containing erythrocytes and suppressing erythropoiesis. Minor antigen mismatches may result in alloimmunization, but it is unknown if antigen mismatches or recipient characteristics influence HbA clearance posttransfusion. STUDY DESIGN AND METHODS: Children with sickle cell anemia on chronic transfusion therapy were followed prospectively for 12 months...
April 17, 2018: Transfusion
Hui Zhong, Karina Yazdanbakhsh
PURPOSE OF REVIEW: Hemolytic anemias caused by premature destruction of red blood cells occur in many disorders including hemoglobinopathies, autoimmune conditions, during infection or following reaction to drugs or transfusions. Recent studies which will be reviewed here have uncovered several novel mechanisms by which hemolysis can alter immunological functions and increase the risk of severe complications in hemolytic disorders. RECENT FINDINGS: Plasma-free heme can induce the formation of neutrophil extracellular traps (NETs) through reactive oxygen species signaling...
May 2018: Current Opinion in Hematology
Marianne E M Yee, Cassandra D Josephson, Anne M Winkler, Jennifer Webb, Naomi L C Luban, Traci Leong, Sean R Stowell, Ross M Fasano
BACKGROUND: Red blood cell (RBC) alloimmunization occurs at a high frequency in sickle cell anemia (SCA) despite serologic matching for Rh (C/c, E/e) and K antigens. RBC minor antigen genotyping allows for prediction of antigens and RH variants that may lead to alloimmunization. STUDY DESIGN AND METHODS: RBC antigen genotyping was performed on chronically transfused pediatric SCA patients, using PreciseType human erythrocyte antigen (HEA), RHCE, and RHD BeadChip arrays...
November 2017: Transfusion
Giuseppe Leone, Eligio Pizzigallo
Splenectomy, while often necessary in otherwise healthy patients after major trauma, finds its primary indication for patients with underlying malignant or nonmalignant hematologic diseases. Indications of splenectomy for hematologic diseases have been reducing in the last few years, due to improved diagnostic and therapeutic tools. In high-income countries, there is a clear decrease over calendar time in the incidence of all indication splenectomy except nonmalignant hematologic diseases. However, splenectomy, even if with different modalities including laparoscopic splenectomy and partial splenectomy, continue to be a current surgical practice both in nonmalignant hematologic diseases, such as Immune Thrombocytopenic Purpura (ITP), Autoimmune Hemolytic Anemia (AIHA), Congenital Hemolytic Anemia such as Spherocytosis, Sickle Cell Anemia and Thalassemia and Malignant Hematological Disease, such as lymphoma...
2015: Mediterranean Journal of Hematology and Infectious Diseases
Robert S Nickel, Jeanne E Hendrickson, Marianne M Yee, Robert A Bray, Howard M Gebel, Leslie S Kean, David B Miklos, John T Horan
Blood transfusions can induce alloantibodies to antigens on red blood cells (RBCs), white blood cells and platelets, with these alloantibodies affecting transfusion and transplantation. While transfusion-related alloimmunization against RBC antigens and human leucocyte antigens (HLA) have been studied, transfusion-related alloimmunization to minor histocompatibility antigens (mHA), such as H-Y antigens, has not been clinically characterized. We conducted a cross-sectional study of 114 children with sickle cell disease (SCD) and tested for antibodies to 5 H-Y antigens and to HLA class I and class II...
July 2015: British Journal of Haematology
Benoît Vingert, Marie Tamagne, Anoosha Habibi, Sadaf Pakdaman, Julie Ripa, Rahma Elayeb, Frédéric Galacteros, Philippe Bierling, Hélène Ansart-Pirenne, Pablo Bartolucci, France Noizat-Pirenne
Alloimmunization against red blood cells (RBCs) is the main immunological risk associated with transfusion in patients with sickle cell disease (SCD). However, about 50-70% of SCD patients never get immunized despite frequent transfusion. In murine models, CD4(+) T cells play a key role in RBC alloimmunization. We therefore explored and compared the CD4(+) T-cell phenotypes and functions between a group of SCD patients (n = 11) who never became immunized despite a high transfusion regimen and a group of SCD patients (n = 10) who had become immunized (at least against Kidd antigen b) after a low transfusion regimen...
June 2015: European Journal of Immunology
Nisha G Sosale, Tahereh Rouhiparkouhi, Andrew M Bradshaw, Rumiana Dimova, Reinhard Lipowsky, Dennis E Discher
A macrophage engulfs another cell or foreign particle in an adhesive process that often activates myosin-II, unless the macrophage also engages "marker of self" CD47 that inhibits myosin. For many cell types, adhesion-induced activation of myosin-II is maximized by adhesion to a rigid rather than a flexible substrate. Here we demonstrate that rigidity of a phagocytosed cell also hyperactivates myosin-II, which locally overwhelms self-signaling at a phagocytic synapse. Cell stiffness is one among many factors including shape that changes in erythropoiesis, in senescence and in diseases ranging from inherited anemias and malaria to cancer...
January 15, 2015: Blood
Howard M Lederman, Margaret A Connolly, Ram Kalpatthi, Russell E Ware, Winfred C Wang, Lori Luchtman-Jones, Myron Waclawiw, Jonathan C Goldsmith, Andrea Swift, James F Casella
BACKGROUND AND OBJECTIVE: Susceptibility to encapsulated bacteria is well known in sickle cell disease (SCD). Hydroxyurea use is common in adults and children with SCD, but little is known about hydroxyurea's effects on immune function in SCD. Because hydroxyurea inhibits ribonucleotide reductase, causing cell cycle arrest at the G1-S interface, we postulated that hydroxyurea might delay transition from naive to memory T cells, with inhibition of immunologic maturation and vaccine responses...
October 2014: Pediatrics
Matthew M Hsieh, Courtney D Fitzhugh, R Patrick Weitzel, Mary E Link, Wynona A Coles, Xiongce Zhao, Griffin P Rodgers, Jonathan D Powell, John F Tisdale
IMPORTANCE: Myeloablative allogeneic hematopoietic stem cell transplantation (HSCT) is curative for children with severe sickle cell disease, but toxicity may be prohibitive for adults. Nonmyeloablative transplantation has been attempted with degrees of preparative regimen intensity, but graft rejection and graft-vs-host disease remain significant. OBJECTIVE: To determine the efficacy, safety, and outcome on end-organ function with this low-intensity regimen for sickle cell phenotype with or without thalassemia...
July 2, 2014: JAMA: the Journal of the American Medical Association
Hyacinth I Hyacinth, Oluwatoyosi A Adekeye, Christopher S Yilgwan
Sickle cell anemia (SCA) is a genetic disease that affects mostly individuals of African and/or Hispanic descent, with the majority of cases in sub-Saharan Africa. Individuals with this disease show slowed growth, delayed sexual maturity, and poor immunologic function. These complications could partly be explained by the state of undernutrition associated with the disease. Proposed mechanism of undernutrition include protein hypermetabolism, decreased dietary intake possibly from interleukin-6-related appetite suppression, increased cardiac energy demand/expenditure, and increased red cell turnover...
January 1, 2013: Journal of Social, Behavioral and Health Sciences
Erik J Uhlmann, Shalini Shenoy, Lawrence T Goodnough
BACKGROUND: Hyperhemolysis syndrome is a serious transfusion reaction mostly reported in association with sickle cell disease, characterized by destruction of both donor and host red blood cells (RBCs) by an unknown mechanism. CASE REPORT: A 21-year-old man with sickle cell disease and multiple prior transfusions received two phenotype-matched, compatible RBC units during a brief admission for pain crisis. He developed rapid-onset progressive anemia and hemoglobinuria...
February 2014: Transfusion
Khaled Saad, Sherif A Mohamed, Kotb A Metwalley
OBJECTIVES: Recurrent/persistent pneumonia in children continues to be a major challenge for the pediatricians. The aim of our study was to establish the prevalence and underlying causes of recurrent/persistent pneumonia in children in Upper Egypt. SETTINGS: Assiut University Children Hospital, Assiut, Egypt. PATIENTS AND METHODS: Patients, admitted for pneumonia to the hospital during 2 years, were investigated with microbiological, biochemical, immunological and radiological tests in order to establish the prevalence of recurrent/persistent pneumonia and to find out its underlying causes...
2013: Mediterranean Journal of Hematology and Infectious Diseases
David S Terman, Benjamin L Viglianti, Rahima Zennadi, Diane Fels, Richard J Boruta, Hong Yuan, Mathew R Dreher, Gerald Grant, Zahid N Rabbani, Ejung Moon, Lan Lan, Joseph Eble, Yiting Cao, Brian Sorg, Kathleen Ashcraft, Greg Palmer, Marilyn J Telen, Mark W Dewhirst
Resistance of hypoxic solid tumor niches to chemotherapy and radiotherapy remains a major scientific challenge that calls for conceptually new approaches. Here we exploit a hitherto unrecognized ability of sickled erythrocytes (SSRBCs) but not normal RBCs (NLRBCs) to selectively target hypoxic tumor vascular microenviroment and induce diffuse vaso-occlusion. Within minutes after injection SSRBCs, but not NLRBCs, home and adhere to hypoxic 4T1 tumor vasculature with hemoglobin saturation levels at or below 10% that are distributed over 70% of the tumor space...
2013: PloS One
Patricia C Veiga, Robert J Schroth, Rosalina Guedes, Songeli Meneses Freire, Getulio Nogueira-Filho
OBJECTIVE: The aim of this study was to evaluate possible immunologic relationships between sickle cell anaemia (SCA) and periodontal inflammation and its impact on serum cytokines. DESIGN: Twenty-five Brazilian children of African descent were involved in this study and divided in two groups: SCA (n=10): confirmed diagnosis of homozygous anaemia; and CTR-control (n=15): no sickle anaemia. Clinical examination included comprehensive medical (routine physical evaluation) and periodontal exams: plaque index (PI), bleeding on probing (BoP), and haematological analysis...
May 2013: Archives of Oral Biology
Jean-Luc Wautier, Marie-Paule Wautier
Red blood cell (RBC) adhesion to endothelium can be studied in static and flow conditions. Increased RBC adhesion was first described in sickle cell disease. Several molecules were shown to be involved in this phenomenon: VCAM-1, α4β1, Lu/BCAM, ICAM-4. In malaria, Plasmodium falciparum erythrocyte membrane protein1 binds to ICAM-1, PECAM-1 and facilitates the parasite dissemination. In diabetes mellitus augmented RBC adhesion is correlated to the severity of vascular complications. Glycated RBC band3 reacts with the endothelial Receptor for Advanced Glycation End products (RAGE)...
2013: Clinical Hemorheology and Microcirculation
Mehdi Nouraie, Sergei Nekhai, Victor R Gordeuk
OBJECTIVE: Some studies suggest that HIV infection progresses slowly in patients with sickle cell disease (SCD). The authors aimed to determine the relationships between SCD and HIV infection. METHODS: National Hospital Discharge Survey data from adult African-Americans in the period of 1997-2009 were analysed. The comorbidities of SCD with HIV infections in hospital discharges were analysed. Multiple logistic regression was used to test the association between SCD and HIV...
November 2012: Sexually Transmitted Infections
A J Jolanda Lammers, Alexander P N A de Porto, Roelof J Bennink, Ester M M van Leeuwen, Bart J Biemond, J Carel Goslings, Jan van Marle, Ineke J M ten Berge, Peter Speelman, Joost B L Hoekstra
Asplenic patients are at risk for pneumococcal sepsis. Patients with hyposplenic function, such as associated with sickle cell disease (SCD), are also at risk. However, tests to assess splenic function are either unavailable or lacking standardization. The aim of this study was to compare different methods for determining splenic function. Eighteen patients with SCD (i.e., 10 heterozygous (SC) and 8 homozygous (SS) SCD patients), and eight splenectomized patients were compared to 10 controls. All subjects underwent spleen scintigraphy, after which functional splenic volumes (FSV) were calculated...
May 2012: American Journal of Hematology
Romuald Dassé Séry, Koffi N'guessan, Paul Akré Dagra, Rita Yao, François Sombo Mambo
Painful sickle cell crises are among the principal manifestations of sickle cell disease. Their treatment routinely requires the use of non-steroidal anti-inflammatory drugs (NSAIDS). These drugs also, however, inhibit the cyclooxygenase cycle in arachidonic acid metabolism, promoting the synthesis of leukotrienes, which have bronchoconstrictive effects. This study took place from March through August, 2007, and included 100 patients of both sexes, aged 2 to 59 years, with any sickle cell phenotype (SS, SC, AS, SFA2, or SAFA2) and treated by NSAIDs in the Immunology and Haematology department of the University Hospital of Cocody...
October 2011: Santé: Cahiers D'étude et de Recherches Francophones
N Win, E Lee, M Needs, L-W Chia, R Stasi
BACKGROUND: Hyperhaemolytic transfusion reaction (HHTR) has been well described in patients with sickle cell disease (SCD). It is characterised by a decrease in haemoglobin concentration to levels below those before transfusion and a fall in the absolute reticulocyte count. As red blood cells (RBC) alloantibodies are typically not detected in post-transfusion samples in acute forms of HHTR, we have previously proposed that both the transfused and autologous RBCs cells (HbSS/reticulocytes) are destroyed by activated macrophages...
April 2012: Transfusion Medicine
Claude Tayou Tagny, Maxime Diané Kouao, Hamane Touré, Jalel Gargouri, Ahamada Said Fazul, Siaka Ouattara, Ludovic Anani, Habiba Othmani, Lochina Feteke, Honorine Dahourou, Guy Olivier Mbensa, Simplice Molé, Yacouba Nébié, Madeleine Mbangue, Michel Toukam, Mahommed Ould Boulahi, Lalatiana Valisoa Andriambelo, Olivat Rakoto, Mounirou Baby, Rakia Yahaya, Amelia Bokilo, Florent Senyana, Dora Mbanya, Caroline Shiboski, Edward L Murphy, Jean Jacques Lefrère
BACKGROUND: The goal of selecting a healthy blood donor is to safeguard donors and reduce the risks of infections and immunologic complications for recipients. STUDY DESIGN AND METHODS: To evaluate the blood donor selection process, a survey was conducted in 28 blood transfusion centers located in 15 francophone African countries. Data collected included availability of blood products, risk factors for infection identified among blood donor candidates, the processing of the information collected before blood collection, the review process for the medical history of blood donor candidates, and deferral criteria for donor candidates...
January 2012: Transfusion
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