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Hematol., Transfus. Cell Ther. (Impr.) ; 43(4): 396-401, Oct.-Dec. 2021. tab, graf
Article in English | LILACS | ID: biblio-1350809


ABSTRACT CD28 null T helper (Th) cells are rare in healthy individuals, but they are increased in various inflammatory and immune-mediated diseases. In this study, we determined the size of the CD4+/CD28 null T lymphocyte compartment in the peripheral blood of 40 autoimmune hemolytic anemia (AIHA) patients (idiopathic and secondary) and 20 healthy control subjects, using tri-color flow cytometry. The frequency and absolute count of CD4+/CD28 null T helper (Th) cells was significantly higher in idiopathic AIHA patients, compared to healthy controls (p = 0.001 and 0.001, respectively) and to patients with secondary AIHA (p = 0.04 and 0.01, respectively). The percentage of CD4+/CD28 null Th cells was also negatively correlated to the hemoglobin (Hb) level (p = 0.03). These findings demonstrate, for the first time, the expansion of this phenotypically-defined population of T lymphocytes in patients with idiopathic AIHA and indicate that it likely plays an etiological role in the development of this disease. However, establishing the use of this marker for diagnosis or monitoring treatment of such patients needs further studies.

Humans , Male , Female , Adolescent , Adult , Middle Aged , T-Lymphocytes, Helper-Inducer , Anemia, Hemolytic, Autoimmune , T-Lymphocytes , CD4 Antigens , Autoimmunity , CD28 Antigens , Th1 Cells , Flow Cytometry
Hematol., Transfus. Cell Ther. (Impr.) ; 43(2): 147-155, Apr.-June 2021. tab, graf
Article in English | LILACS | ID: biblio-1286683


ABSTRACT Objectives The purpose of this study was to compare data obtained from the reticulocyte channel (RET channel) heated to 41 °C with those obtained from impedance channel (I-Channel) at room temperature in the samples with the mean corpuscular hemoglobin concentration (MCHC) < 370 g/L and in samples with the MCHC > 370 g/L, in the presence of cold agglutinins. Methods In this study, 60 blood samples (group 1) with the MCHC < 370 g/L (without cold agglutinins) and 78 blood samples (group 2) with the MCHC > 370 g/L (with cold agglutinins) were used to compare the two analytical channels of the XN-9000 analyzer in different preanalytical conditions. The parameters evaluated in both groups were the following: red blood cell (RBC), hemoglobin (HGB), hematocrit (HCT), mean cell volume (MCV), RBC-most frequent volume (R-MFV), mean hemoglobin concentration (MCH) and mean cellular hemoglobin concentration (MCHC). Results The results of this study showed an excellent correlation with both channels of the XN-9000 analyzer in samples with and without cold agglutinins, except for the MCHC. The bias between the values obtained in the I-channel and those obtained in the RET channel of both groups was insignificant and remained within the limits of acceptability, as reported by Ricos et al. for all considered parameters, except for MCHC. Conclusions The presence of cold agglutinins in blood samples can be detected by a spurious lowering of the RBC count and by a spurious increase in the MCHC. The RET channel represents a great opportunity to correct the RBC count in a rapid manner without preheating. However, neither methodology can completely solve the residual presence of cold agglutinins in all samples, despite the MCHC values being < 370 g/L.

Reticulocytes , Agglutinins , Anemia, Hemolytic, Autoimmune
Journal of Experimental Hematology ; (6): 1935-1939, 2021.
Article in Chinese | WPRIM | ID: wpr-922227


OBJECTIVE@#To analyze the causes of positive irregular antibody screening test and incompatibility of cross matching in one patient with autoimmune hemolytic anemia complicated with neonatal hemolytic disease, and to accurately identify the type of antibodies in patients, and to select a reasonable strategy for blood transfusion.@*METHODS@#One children was enrolled, blood group positive and reverse typing, Rh typing, direct anti-human globulin test, free test, dispersal test and cross matching test were carried out by test tube method and microcolumn gel card; irregular antibodies were identified by the reaction of DTT treatment and untreated panel cells with patients' plasma.@*RESULTS@#The blood group of the patient was RhD positive B and irregular antibody screening positive, while the blood group of the mother was RhD positive O and irregular anti-screening negative, the result showed that the anti-LW detected in the plasma of the patient was autoantibody and ABO neonatal hemolytic disease (ABO-HDN) was present. Both O type RhD positive washing RBCs and B type RhD negative RBCs were transfused effectively.@*CONCLUSION@#Irregular antibodies in patients are anti-LW antibodies, and transfusion of homotype RhD negative suspended erythrocytes after the exclusion of ABO-HDN shows a better effect.

Anemia, Hemolytic, Autoimmune , Autoantibodies , Blood Group Incompatibility , Blood Transfusion , Erythroblastosis, Fetal , Humans
Article in Chinese | WPRIM | ID: wpr-880148


OBJECTIVE@#To analyze one case of multiple myeloma (MM) initially presenting cold agglutinin syndrome (CAS), so as to improve clinical understanding and screening of this disease.@*METHODS@#The clinical data, laboratory examination, bone marrow result, diagnosis and treatment of the patient were analyzed and summarized to provide ideas and clinical experience for the early diagnosis and treatment of CAS secondary to MM.@*RESULTS@#The clinical manifestations of asthenia, hemolysis, jaundice and scattered livedo reticularis were caused by CAS secondary to MM, which was different from the general Raynaud's phenomenon. IgMκ type MM was definitely diagnosed according to the morphological features of bone marrow cells and immunofixation electrophoresis. After 3 courses of chemotherapy with BAD regimen and enhanced thermal support, anemia was corrected, M protein was decreased and the cold agglutinin phenomenon was significantly reduced. The evaluation of efficacy reached very good partial response.@*CONCLUSION@#There are very few MM patients with CAS as the initial presentation, so it is easy to misdiagnose. Early diagnosis and individual therapy are particularly important, which requires clinicians to observe and gain experience further.

Anemia, Hemolytic, Autoimmune/diagnosis , Cryoglobulins , Early Diagnosis , Humans , Multiple Myeloma/diagnosis
Med. lab ; 25(4): 735-742, 2021.
Article in Spanish | LILACS | ID: biblio-1370935


La enfermedad por crioaglutininas es una anemia hemolítica autoinmune que se caracteriza, en la gran mayoría de los casos, por la hemólisis mediada por autoanticuerpos de tipo IgM y complemento C3d, contra los antígenos de la membrana del eritrocito, que conduce a hemólisis extravascular con propensión a la trombosis, y que afecta principalmente al sexo femenino y personas mayores. Su diagnóstico se realiza con la prueba de Coombs directo y fraccionado, y la titulación de aglutininas frías >1:64 a 4 °C. Se describe el caso clínico de una mujer de 89 años con un síndrome constitucional y una anemia de 3 años de evolución, en quien se determinó el diagnóstico de enfermedad por aglutininas frías. Asimismo, se describe el abordaje diagnóstico, el tratamiento instaurado, y se hace una breve revisión de la literatura publicada

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia characterized in the vast majority of cases by hemolysis mediated by IgM autoantibodies and complement C3d against erythrocyte membrane antigens, leading to extravascular hemolysis with propensity to thrombosis, affecting mainly females and older individuals. It is diagnosed by direct and fractionated Coombs test and a cold agglutinin titer >1:64 at 4 °C. We describe the case of an 89-year-old woman with a constitutional syndrome and a 3-year history of anemia, who was diagnosed with cold agglutinin disease. Also, we include the diagnostic and treatment approach, and a brief review of the literature

Humans , Anemia, Hemolytic, Autoimmune , Raynaud Disease , Coombs Test , Complement C3d , Livedo Reticularis , Rituximab
Rev. cuba. hematol. inmunol. hemoter ; 36(4): e1283, oct.-dic. 2020. tab
Article in English | LILACS | ID: biblio-1289419


Introduction: Autoimmune hemolytic anemia is a rare disorder characterized by hemolysis mediated by autoantibodies directed against red blood cells. The demonstration of antibody specificity is a very difficult procedure since autoantibodies in general are nonspecific of antigens and react with all erythrocytes analyzed. Occasionally, specificity is observed against the Rh system antigens. Objective: To determinate the specificity of erythrocytes autoantibodies in DAT positive autoimmune hemolytic anemia by MAIEA technique. Methods: The specificity and isotype of erythrocyte autoantibodies were determined in the eluate of 109 blood samples from patients with warm autoimmune hemolytic anemia, by means of the MAIEA technique and the use of monoclonal antibodies that recognized 11 blood group systems and the protein CD47. Results: In 100 percent of cases autoantibodies against Rh system antigens were detected; in 24 cases we detected autoantibodies of IgA and IgM isotypes that recognized different antigens that were recognized by IgG isotype autoantibodies. For idiopathic and secondary warm autoimmune hemolytic anemias, predominance was observed against three or more specificities. IgG was detected in 99.09 percent of the eluates, IgA in 35.77 percent and IgM in 16.51 percent. The high degree of hemolysis was related to the presence of several isotype autoantibodies against four or more blood group specificities. Conclusions: The MAIEA technique is a sensitive method that can be used to determine the specificities and isotypes of autoantibodies in patients with warm autoimmune hemolytic anemia.

Introducción: La anemia hemolítica autoinmune es un trastorno poco común, caracterizado por hemólisis mediada por autoanticuerpos dirigidos contra los glóbulos rojos. La demostración de la especificidad de los anticuerpos es un procedimiento muy difícil, ya que los autoanticuerpos en general no son específicos de los antígenos y reaccionan con todos los eritrocitos analizados. Ocasionalmente, se observa especificidad contra los antígenos del sistema Rh. Objetivo: Determinar la especificidad de los autoanticuerpos eritrocitarios en pacientes con anemias hemolíticas autoinmunes PAD positivas con el empleo de la técnica MAIEA Métodos: Se determinó la especificidad e isotipo de los autoanticuerpos eritrocitarios en el eluido de 109 muestras de sangre de pacientes con anemia hemolítica autoinmune caliente, mediante la técnica de MAIEA y el uso de anticuerpos monoclonales que reconocieron 11 sistemas de grupos sanguíneos y la proteína CD47. Resultados: En el ciento por ciento de los casos se detectaron autoanticuerpos contra los antígenos del sistema Rh. En 24 casos se descubrió autoanticuerpos de isotipos IgA e IgM que reconocieron diferentes antígenos que fueron a su vez reconocidos por autoanticuerpos de isotipo IgG. Se observó para las anemias hemolíticas autoinmunes calientes idiopáticas y secundarias; predominio frente a tres o más especificidades. Se detectó IgG en el 99,09 por ciento de los eluidos, IgA en 35,77 por ciento e IgM en 16,51 por ciento. El alto grado de hemólisis se relacionó con la presencia de varios isotipos de autoanticuerpos contra cuatro o más especificidades de grupos sanguíneos. Conclusiones: La técnica MAIEA es un método sensible que puede usarse para determinar las especificidades e isotipos de autoanticuerpos en pacientes con anemia hemolítica autoinmune caliente.

Humans , Blood Group Antigens , Immunoglobulin G , Immunoglobulin M , Sensitivity and Specificity , Anemia, Hemolytic, Autoimmune , Antibodies, Monoclonal , Antibody Specificity
Rev. cuba. hematol. inmunol. hemoter ; 36(2): e1098, abr.-jun. 2020. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1149894


Introducción: La membrana de los eritrocitos, al igual que las membranas de otros tipos celulares, está compuesta por una bicapa lipídica que es estabilizada por proteínas específicas, glucolípidos y otras moléculas especializadas. Las mutaciones producidas en los genes que codifican y regulan estas proteínas y sus interacciones producen cambios en la forma de los eritrocitos y son causa de anemias hemolíticas hereditarias. Objetivo: Describir las peculiaridades moleculares, clínicas y el diagnóstico de laboratorio de las principales anemias hemolíticas hereditarias por defectos en la membrana de los eritrocitos. Métodos: Se realizó una revisión de la literatura, en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google académico de artículos publicados en los últimos 10 años. Se hizo un análisis y resumen de la bibliografía revisada. Análisis y síntesis de la información: Las mutaciones que afectan la membrana de los eritrocitos son variadas y heterogéneas. El efecto sobre el fenotipo puede ser clasificado en cinco categorías principales: esferocitosis hereditaria; eliptocitosis hereditaria y piropoiquilocitosis hereditaria; ovalocitosis del sureste asiático; acantocitosis hereditaria y estomatocitosis hereditaria. Conclusiones: La cuidadosa observación de la morfología de los eritrocitos en extendidos de sangre periférica y los estudios moleculares permiten realizar un diagnóstico certero, además de confirmar la correlación genotipo/fenotipo en estas enfermedades(AU)

Introduction: The erythrocyte membrane, like the membranes of other cell types, is composed of a lipid bilayer that is stabilized by specific proteins, glycolipids and other specialized molecules. Mutations in the genes that encode and regulate these proteins and their interactions cause changes in the shape of erythrocytes and are the cause of hereditary hemolytic anemias. Objective: To describe the molecular and clinical peculiarities and the laboratory diagnosis of the main hereditary hemolytic anemias due to defects in the erythrocyte membrane. Methods: A literature review was carried out, in English and in Spanish, through the PubMed website and the Google Scholar search engine, of articles published in the last ten years. An analysis and summary of the revised bibliography was made. Information analysis and synthesis: Mutations affecting the erythrocyte membrane are varied and heterogeneous. The effect on the phenotype can be classified into five main categories: hereditary spherocytosis, hereditary elliptocytosis and hereditary pyropoikilocytosis, Southeast Asian ovalocytosis, hereditary acantocytosis, and hereditary stomatocytosis. Conclusions: Careful observation of erythrocyte morphology in peripheral blood smears and molecular studies allow an accurate diagnosis, in addition to confirming the genotype-phenotype correlation in these diseases(AU)

Phenotype , Genotype , Anemia, Hemolytic, Autoimmune/diagnosis
Article in English | WPRIM | ID: wpr-827360


IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported. The patient has shortness of breath, cough, and sputum after physical activity. Physical examination showed appearance of anemia, yellow staining of skin and sclera, palpable neck and multiple swollen lymph nodes. Laboratory examination, bone marrow biopsy, and lymph node biopsy confirmed the diagnosis. Therefore, clinicians should develop ideas and raise awareness of such diseases.

Anemia, Hemolytic, Autoimmune , Diagnosis , Drug Therapy , Autoimmune Diseases , Biopsy , Humans , Immunoglobulin G , Immunoglobulin G4-Related Disease , Diagnosis
Article in Chinese | WPRIM | ID: wpr-827187


OBJECTIVE@#To evaluate the characteristics of autoimmune hemolytic anemia caused by salvianolate by antibody detection and clinical index monitoring.@*METHODS@#Micro-column gel anti-human globulin method was used for irregular antibody screening and antibody identification. Salvianolate, sodium creatine phosphate and levocarnitine were used to sensitize red blood cells that were compatible with the patient's plasma, and the RBCs were used to test drug antibody in patient plasma respectively. The patient's clinical examination of hemolysis index and blood transfusion effect were analyed retrospectively.@*RESULTS@#The patients were positive for irregular antibody screening, and there were antoanti-Ce antibodies in serum. The erythrocytes sensitized with salvianolate in the patient's serum were positive, while those sensitized with sodium creatine phosphate and levocarnitine were negative.@*CONCLUSION@#Salvianolate causes drug-induced autoimmune hemolytic anemia in this patient.

Anemia, Hemolytic, Autoimmune , Blood Transfusion , Erythrocytes , Humans , Plant Extracts , Retrospective Studies
Journal of Experimental Hematology ; (6): 1307-1311, 2020.
Article in Chinese | WPRIM | ID: wpr-827121


OBJECTIVE@#To evaluate the efficacy and safety of different types of red blood cell (RBC) transfusion and hormone therapy in patients with autoimmune hemolytic anemia (AIHA).@*METHODS@#The clinical data and serological characteristics of 40 patients with AIHA treated in our hospital from 2014 to 2018 were collected and analyzed retrospectively. The efficacy and safety of different type of RBC transfusion and hormone therapy were evaluated according to the principle of minimally incompatible RBC transfusion after cross-matching.@*RESULTS@#Among 40 patients with AIHA, the female cases were more than the male cases, the cases of secondary AIHA was more than cases of primary AIHA, and the warm autoantibodies were in the majority. 11 cases of AIHA underwent 26 times minimally incompatible red blood cell transfusions. The total effective rate was 46.2%, the partial efficiency was 23.1%, and total inefficiency was 30.8%. Among them, the same type of non-washing red blood cell group showed efficiency of 42.1%, partial effective rate of 21.1%, and inefficiency of 36.8%; the same type of washed red blood cell group showed efficiency of 57.1%, partial effective rate of 28.6%, and inefficiency of 14.3%. the infusion effects was not significanly different between the two groups, and no hemolytic transfusion reaction occurred. In the hormone-treated group, the complete remission rate was 15.2%, the partial remission rate was 63.6%, and the ineffective rate was 21.2%. Among them, the side effects appeared in 2 patients after using hormones.@*CONCLUSION@#When AIHA patients need blood transfusion, use the same type of non-washed red blood cells or homologous washed cells is relatively safe, and the difference in efficacy is not significant. The partial remission of patients received hormone therapy is much higher than that of red blood cell transfusion, but the side effects easily happen.

Anemia, Hemolytic, Autoimmune , Autoantibodies , Blood Transfusion , Erythrocyte Transfusion , Erythrocytes , Female , Humans , Male , Retrospective Studies
Article in English | WPRIM | ID: wpr-811412


Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

Anemia, Hemolytic , Anemia, Hemolytic, Autoimmune , Biopsy , Giant Cells , Hepatitis , Hepatocytes , Humans , Infant , Inflammation , Jaundice , Liver , Liver Diseases , Male , Pallor , Physical Examination , Rare Diseases , Recurrence , Rituximab , Splenomegaly
Iatreia ; 33(2): 123-132, 20200000. tab, graf
Article in Spanish | LILACS | ID: biblio-1114784


RESUMEN Introducción: la enfermedad por aglutininas frías (EAF) es un trastorno hematológico primario o secundario, caracterizado por la anemia hemolítica autoinmune causada por los anticuerpos IgM a bajas temperaturas. Clínicamente, presenta parestesias y acrocianosis inducidos por frío y fiebre, aunque también puede ser asintomática y solo identificarse por alteraciones en el hemograma. Objetivo: describir las manifestaciones clínicas y de laboratorio, las causas primarias y secundarias de la EAF y compararlas con series de casos descritos en la literatura. Materiales y métodos: análisis retrospectivo de datos clínicos de pacientes del Hospital Universitario San Vicente Fundación de Medellín con resultados positivos para aglutininas frías. Dichos análisis se realizaron en el laboratorio de hematología de la Universidad de Antioquia, consideramos como positivo título ≥ 1: 64 o con la prueba de Coombs directa y positiva para anticuerpos fríos. Resultados: se incluyen los títulos de crioaglutininas de 23 casos con EAF: 6 formas primarias, 4 asociadas con los linfomas no Hodgkin (LNH), 8 secundarias a enfermedades infecciosas y autoinmunes y, 5 asociados con enfermedades misceláneas. Discusión y conclusiones: esta es la primera serie de casos en Colombia de EAF. La edad y género fueron similares a los datos reportados en la literatura. Observamos un mayor número de pacientes que presentaban anemia hemolítica y con síntomas asociados al frío. La relación hemoglobina hematocrito fue 1:2. Dentro de las causas secundarias destacamos las vasculitis, el lupus y la malaria. De las causas primarias las más frecuentes fueron los LNH, específicamente, el linfoplasmocítico. El tratamiento más utilizado para pacientes con EAF primaria incluyo rituximab.

SUMMARY Introduction: Cold agglutinin disease (CAD) is a primary hematologic disorder or can be secondary to another disease. CAD is characterized by autoimmune hemolytic anemia associated with IgM type antibodies, at low temperatures. Clinically CAD is associated with cryoparesthesia and acrocyanosis induced by cold and fever, or it can be asymptomatic and can be detected by abnormalities on cell blood counts. Objective: To describe the clinical and laboratory data and the etiology of CAD. Comparison between this case series and those described in the literature. Materials and Methods: Retrospectively, we analyzed clinical data of patients from Hospital Universitario San Vicente Fundación with positive results for cold agglutinin assays made in the hematology lab from Universidad de Antioquia. We consider patients with titers ≥ 1:64 or Coombs test positive for cold antibodies. Results: We describe clinical and laboratory findings included crioagglutinin titers of 23 cases with CAD: 6 of them with primary CAD, 4 with non-Hodgkin Lymphoma (NHL), 8 patients with CAD associated with infectious and autoimmune disease and 5 with CAD miscellaneous diseases. Discussion and Conclusions: This is the first CAD case series described in Colombia. Age and gender were like others case series. Most of patients presented with hemolityc anemia and cold related symptoms. The hemoglobin/ hematocrit ratio was 1:2. Secondary causes were vasculitis, lupus and malaria. Primary CAD were related to NHL, specifically limphoplasmocytic Most of the treatments of primary CAD included rituximab.

Humans , Agglutinins , Hematologic Neoplasms , Rituximab , Anemia, Hemolytic, Autoimmune , Lymphoma
Rev. méd. Chile ; 147(7): 836-841, jul. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1058612


Background: Autoimmune hemolytic anemia (AIHA) is an uncommon disease. In its presentation, it can be severe and even lethal. There is only one clinical report concerning this pathology in Chile. Objective: To describe the clinical characteristics and evolution of adult AIHA inpatients. Materials and Methods: Retrospective review of clinical records of adult AIHA inpatients between January 2010 and June 2018 was done. Demographic, clinical, laboratory and therapeutic information was analyzed. A descriptive, analytical and survival analysis was performed. Results: Forty-three adult patients diagnosed with AHIA were hospitalized in a period of 8 years. Median age was 63 years (range 22-86 years), mostly women (72%). Warm antibodies were detected in 36 cases (84%) and cold antibodies in seven. Seventy two percent of the patients had an underlying cause, and 58% were secondary to lymphoproliferative neoplasms. All patients except two, received steroids as initial treatment, with response in 37 (90%) of them. Three refractory patients received rituximab, with response in all of them, and relapse in one. Median follow-up was 38 months (range 2-98 months). Five year overall survival was 72%. Conclusion: AHIA in adults inpatients is a heterogeneous disease, mainly due to warm antibodies, and to secondary etiology.

Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Young Adult , Anemia, Hemolytic, Autoimmune/diagnosis , Splenectomy , Azathioprine/administration & dosage , Survival Analysis , Retrospective Studies , Follow-Up Studies , Rituximab/administration & dosage , Anemia, Hemolytic, Autoimmune/mortality , Anemia, Hemolytic, Autoimmune/therapy
Rev. cuba. hematol. inmunol. hemoter ; 35(1): e944, ene.-mar. 2019. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1003892


La infección por parvovirus B19 humano, es la causa de la mayor parte de los casos de crisis aplásica transitoria que aparecen de forma brusca en pacientes con enfermedades hemolíticas crónicas, como es el caso de la drepanocitosis. Por otra parte, se han descrito unos pocos casos de infección aguda, por parvovirus B19 humano como causa de anemia hemolítica autoinmune, por medio de la formación de anticuerpos dirigidos contra los glóbulos rojos. La asociación entre drepanocitosis y anemias hemolíticas autoinmunes es poco frecuente. Se reporta un caso poco usual de una paciente adulta, con antecedentes de hemoglobinopatía S/C que presentó una crisis aplásica y posteriormente apareció una anemia hemolítica autoinmune diagnosticada en el Instituto de Hematología e Inmunología. Se trató con dosis inmunosupresoras de esteroide, con lo que se alcanzó la remisión de la anemia hemolítica autoinmune(AU)

Infection with human B19 parvovirus is the cause of most cases of transient aplastic crisis that appear in patients with chronic hemolytic diseases, as in the case of sickle cell disease. On the other hand, a few cases of acute infection by human parvovirus B19 have been described as a cause of autoimmune hemolytic anemia, through the formation of antibodies directed against red blood cells. The association between sickle cell disease and autoimmune hemolytic anemia is rare. We report an unusual case of an adult patient, with a history of S C hemoglobinopathy who presented an aplastic crisis and subsequently an autoimmune hemolytic anemia diagnosed at the Institute of Hematology and Immunology, treated with high steroids doses, reaching the remission of autoimmune hemolytic anemia and constitutes the first report in Cuba(AU)

Humans , Female , Middle Aged , Erythrocyte Transfusion/methods , Anemia, Hemolytic, Autoimmune/complications , Anemia, Hemolytic, Autoimmune/drug therapy , Prednisone/therapeutic use , Anemia, Sickle Cell/complications
Arq. Ciênc. Vet. Zool. UNIPAR (Online) ; 22(2): 71-75, abr-jun. 2019. ilus
Article in Portuguese | LILACS, VETINDEX | ID: biblio-1052619


A anemia hemolítica imunomediada (AHIM) é o distúrbio imunológico de maior prevalência em cães. Caracteriza-se como uma hipersensibilidade do tipo II, que leva a destruição prematura de hemácias. Dentre as principais complicações, o estado de hipercoagulabilidade predispondo a coagulação intravascular disseminada e tromboembolismo pulmonar é a mais importante, sendo a causa de óbito em mais de 80% dos casos. O diagnóstico é realizado a partir da exclusão de outras causas para anemia e por meio da constatação de um ou mais desses sinais: anemia moderada a grave (hematócrito <25-35%), evidências de hemólise (hemoglobinemia, hemoglobinúria ou hiperbilirrubinemia) e presença de anticorpos na hemácia (caracterizado a partir da auto-aglutinação, esferocitose, teste de Coombs positivo ou citometria de fluxo). O tratamento é direcionado à supressão da resposta imune, sendo os corticosteroides e os imunossupressores, os fármacos de predileção.(AU)

Immune-mediated hemolytic anemia (IMHA) is the most prevalent immune disorder among dogs. It is characterized as type II hypersensitivity, leading to premature destruction of red blood cells. Among the main complications, hypercoagulability predisposing to disseminated intravascular coagulation and pulmonary thromboembolism is the most important, being the cause of death in more than 80% of the cases. The diagnosis is made by excluding other causes for anemia and the presence of one or more of these signs: moderate to severe anemia (hematocrit <25-35%), evidence of hemolysis (hemoglobinemia, hemoglobinuria or hyperbilirubinemia) and presence of antibodies in the erythrocyte (characterized by self-agglutination, spherocytosis, positive Coombs test, or flow cytometry). Treatment is directed to suppression of the immune response, with corticosteroids and immunosuppressants the drugs of predilection.(AU)

La anemia hemolítica inmunomediada (AHIM) es el disturbio inmunológico con mayor prevalencia en perros. Es definido como una hipersensibilidad tipo II, que lleva a destrucción prematura de hematíes. Dentro de las principales complicaciones, el estado de hipercoagulabilidad que predispone a coagulación intravascular diseminada y tromboembolismo pulmonar es el más importante, siendo la causa de muerte en más de 80% de los casos. El diagnóstico se realiza excluyendo otras causas de anemia y confirmando una o más de las siguientes alteraciones: anemia moderada a grave (hematocrito <25-35%), evidencias de hemolisis (hemoglobinemia, hemoglobinuria o hiperbilirrubinemia) y presencia de anticuerpos en hematíes (caracterizado a partir de autoaglutinación, esferocitosis, test de Coombs positivo o citometría de flujo). El tratamiento se basa en la supresión de la respuesta inmune, siendo los cortico esteroides y los inmunosupresores los fármacos de elección.(AU)

Animals , Dogs , Dogs/immunology , Dogs/blood , Anemia, Hemolytic, Autoimmune/classification , Immunosuppression Therapy/veterinary
Article in Chinese | WPRIM | ID: wpr-774336


OBJECTIVE@#To investigate the correlation of IgG subclasses with blood cell parameters in the patients with autoimmune hemolytic anemia (AIHA).@*METHODS@#Thirty-four patients with AIHA (except C3d types) of immune complex type (IgG+C3d) and single IgG type, including 26 cases of primary AIHA and 8 cases of secondary AIHA from December 2010 to August 2016 in our hospital were selected and enrolled in AIHA group; 30 healthy persons were selected and enrolled in healthy control group. The levels of IgG subclasses in blood plasma were detected by double antibody sandwich ELISA in healthy persons and AIHA patients, at the same time. The levels of IgG subclasses in of RBC diffuse fluid were detected as well. The relation of IgG subclass level with some parameters of blood cells was analyzed in the hight of partial parameters of blood cells in patients. The independent sample test was used for comparison of data in 2 groups, the Spearman method was used for correlation analyziz.@*RESULTS@#The average value of IgG1-4 in AIHA group was higher than that in healthy control group, there was statisticad difference between 2 groups (IgG1: t=-4.88, P0.01) the ratio pf IgG1, IgG2 and IgG3 all had the statistical differences (IgG1: t=4.03, P<0.01; IgG2: t=7.38, P<0.01; IgG3: t=3.03, P<0.01). The spearmen analysis of corrclation of IgG subclass in blood plasma of patients with partial parameters of blood cells showed that the IgG4 positivety correlated with Hb level, the RBC count and HCT (Hb: r=0.358, P<0.05; RBC: r=0.426, P<0.05; HCT: r=0.363, P<0.05); the IgG1 and IgG2 negatively correlated with WBC count (IgG1: r=0.437, P<0.05; IgG2: r=-0.487, P<0.01); the IgG2 negatively correlated with count (r=-0.436, P<0.05). The comparison of IgG subclass ratio in plasma and RBC diffuse fluid of patients showed that in addition to IgG2 (t=1.544, P>0.05), the rest IgG1, 3 and 4 all had statistical differences (IgG1: t=6.528, P<0.01; IgG3: t=-9.488, P<0.05; IgG4: t=-9.434, P<0.05).@*CONCLUSIONS@#The AIHA relates with IgG1 and IgG3, the detection of IgG subclasses may have a certain significance for studying the diagnosis, treatment and pothogenesis of AIHA.

Anemia, Hemolytic, Autoimmune , Blood Cells , Enzyme-Linked Immunosorbent Assay , Erythrocyte Count , Humans , Immunoglobulin G
Article in Chinese | WPRIM | ID: wpr-771862


OBJECTIVE@#To explore the serological characteristics of patients with autoimmune hemolytic anemia(AIHA) and analyze its clinical efficacy and safety of incompatible red blood cell transfusion.@*METHODS@#Sixty AIHA patients admitted in our hospital from January 2014 to January 2018 were selected. The blood type serological characteristics of 60 patients were analyzed retrospectively. According to the type of autoantibody and the composition of different red blood cells, the efficacy and safety of erythrocyte infusions were evaluated respectively.@*RESULTS@#The screen results of irregular antibody in 60 AIHA patients were positive, and the direct anti-human globulin test also was positive, including 8 cases of cold autoantibodies (13.33%), 49 cases of IgG warm autoantibodies (81.67%), and 3 cases of warm cold double autoantibodies (5%). The irregular anti-body identification test confirmed the existence of homoantiboby in 17 cases (28.33%). Out of 60 cases 34 received incompatible red blood cell (RBC) infusion for 108 time including ABO homotype non washing RBC (81 tirnes) and O type washing RBC (27 times). The infusion results showed that the total [JP2]effective rate was 57.41(62/108), total partial effective rate was 14.81% (16/108) and total ineffective rate was 27.78% (30/108).The infusion of ABO homotype non-washing RBC for 81 time showed that the effective rate was 58.02%[JP] (47/81) , partial effective rate was 12.35 (10/81) and ineffective rate was 29.67% (24/81); the infusion of O type washing RBC for 27 times showed that the effective rate was 55.56% (15/27), partial effective rate was 22.22% (6/27) and ineffective rate was 22.22% (6/27), there was no significant difference in effective rate between 2 kinds of infusion (P>0.05). The comparison of different antibody type infusion showed that in the infusion of IgM cold autoantiboay for 12 times, the effective rate was 41.67% (5/12), partial effective rate was 33.33% (4/12) and ineffective rate was 25% (3/12); in the infusion of IgG warm antoantibody for 93 times. The effective rate was 58.06% (54/93),partial effective rate was 12.90% (12/93) and ineffective rale was 29.04% (27/93), there was also no significant difference in effective rate between 2 kinds of infusion(P>0.05). However, in infusion of cold/warm double autoantibody for 3 times, the effective rate was 100% (3/3), moreover, the hemotytic reaction of infusion was not observed during the treatment .@*CONCLUSION@#The infusion of ABO homotype non-washing RBC and O type washing RBC both possess the high safely and efficacy for treatment of patients with AIHA, but the use of ABO homotype non-washing RBC can effectively avoid the excessive use of O type washing RBC.

Anemia, Hemolytic, Autoimmune , Autoantibodies , Erythrocyte Count , Erythrocytes , Humans , Retrospective Studies , Tics
Blood Research ; : 242-242, 2019.
Article in English | WPRIM | ID: wpr-785544


No abstract available.

Anemia, Hemolytic, Autoimmune , Breast Neoplasms , Breast