A rare case of complicated malaria presenting as warm autoimmune hemolytic anemia

Autoimmune hemolytic anemia (AIHA) has been rarely reported worldwide or from India as the underlying cause of anemia in malaria. We hereby present a case of complicated Plasmodium falciparum malaria with concomitant warm AIHA in a 31-year-old male. Direct Antiglobulin Test (DAT) was positive and elution studies showed pan-agglutination reaction. Clinico-hematological and serological follow-up of the patient was done post artesunate treatment until day 9. We suggest that it is important to establish the immune basis of anemia in malaria patients for guiding the treatment plan for the clinicians and providing packed red blood cell transfusion if required.

Human adenovirus associated with severe cold agglutinin syndrome: a rare complication in Pediatrics / Adenovírus humano associado à síndrome da aglutinina fria grave: uma complicação rara em Pediatria

ABSTRACT Objective: The objectives of this study were to describe the first pediatric case of cold agglutinin syndrome (CAS) triggered by human adenovirus and review the literature. Case description: This case report involves a previously healthy, 2½-year-old female child with human adenovirus isolated in a nasal swab. At 72 h after admission, the patient progressed to a severe episode of anemia (hemoglobin level: 2.6 g/dL). The laboratory findings were consistent with CAS. The patient received blood transfusion, vitamin supplementation, adequate hydration, and thermal protection. At her last follow-up, 1 year after her initial presentation, she remains clinically well without signs of hemolysis. Comments: While severe CAS is extremely uncommon in the pediatric emergency department, human adenovirus infection is a common illness in pediatrics. Recently, the adenovirus has been associated with new complications (acute hepatitis and fulminant liver failure). Pediatric physicians and hematologists should be aware of unusual evolution, signs, and symptoms of this infection that warrant more urgent medical attention. In this case, the hematologic complication suspicion was the key to early diagnosis and adequate management.

RESUMO Objetivo: Descrever o primeiro caso pediátrico de síndrome da crioaglutinina desencadeado por adenovírus humano e revisar a literatura. Descrição do caso: Paciente do sexo feminino, dois anos e seis meses, previamente hígida e diagnosticada com adenovírus humano isolado em swab nasal. Após 72 horas da admissão, a paciente evoluiu com quadro de anemia grave (hemoglobina de 2,6 g/dL). Os achados laboratoriais foram compatíveis com síndrome da crioaglutinina. A paciente recebeu transfusão de concentrado de hemácias, suplementação vitamínica, hidratação adequada e proteção térmica. Em seu último retorno ambulatorial, um ano após a apresentação inicial, permanecia clinicamente bem, sem sinais de hemólise. Comentários: Enquanto a síndrome da crioaglutinina grave é extremamente incomum na emergência pediátrica, a infecção por adenovírus humano é um quadro comum na infância. Recentemente, o adenovírus tem sido associado a novas complicações, e pediatras e hematologistas devem ficar atentos à possibilidade de uma evolução incomum dessa infecção e dos sinais e sintomas que possam necessitar de atenção urgente. No caso apresentado, a suspeita da complicação hematológica foi a chave para o diagnóstico precoce e seu manejo adequado.

Pediatric Evans Syndrome: A 20-year experience from a tertiary center in Brazil

Abstract Introduction The Evans syndrome (ES) is a rare, often chronic, relapsing and treatment-refractory hematological disorder. We described the clinical features, diagnostic workup, treatment and outcome in patients with ES. Method We performed a retrospective chart review of patients aged < 18 years with ES admitted to a tertiary center in Brazil from 2001 to 2021. The analysis of the data was primarily descriptive, using median, interquartile range and categorical variables presented in absolute frequencies. Main results Twenty patients (12 female, 8 male) were evaluated in this study. The median age at the initial cytopenia was 4.98 years (1.30-12.57). The ES was secondary in nine cases (45%), of which six patients (30%) showed autoimmune disease (AID) or primary immunodeficiencies (PID) and one presented a spontaneous recovery. Steroids and intravenous immunoglobulin were first-line therapy in 19 cases. Twelve patients (63%) required second-line treatments (rituximab, cyclosporine, splenectomy, sirolimus, cyclophosphamide, mycophenolate mofetil, azathioprine and eltrombopag). The median follow-up period was 2.41 years (1.4 -7.52). One patient (5%) died of underlying neuroblastoma, one case (5%) was lost to follow-up and four patients (20%) received a medical discharge. The median age for the 14 remaining cases was 12.6 years. Twelve patients (85.7%) were in complete response (CR) with no therapies. Two patients (14.3%) were in CR with chronic therapy. Conclusion As ES may be a symptom of AID and PID, a thorough rheumatological, immunologic and genetic workup and a careful follow-up are essential. The second-line treatment remains a dilemma. Further prospective studies are needed to address the optimal therapeutic combinations, morbidity and mortality in this disorder.

Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia

Abstract Introduction Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder that can present in primary or secondary forms. The literature looking at impact of baseline fluorescent antinuclear antibody (FANA) positivity on outcomes of AIHA patients is infrequent. Objective To study the impact of baseline FANA positivity in patients with primary AIHA. Method A prospective cohort study involving 29 consecutive primary AIHA patients presenting to the Haematology department from 2013 to 2015 was analysed. After recording baseline investigations including fluorescent ANA, all patients were treated as per the standard therapeutic protocols. Clinical remission, disease free survival, relapse, mortality were compared between the FANA positive and FANA Negative AIHA groups. Results Baseline FANA positivity was found in 17 patients (58.62%). Both the groups were comparable in terms of age, sex, Hemoglobin, LDH at presentation, number of lines of treatment needed and duration of follow up. Evan's syndrome was seen in six of FANA positive patients which was statistically significant (0 v/s 6, p= 0.023). FANA positive patients had significantly higher rates of relapse per patient month follow up (1.22 v/s 3.57, p= 0.023) and lower rates of complete response (83.33% v/s 35.29%, p= 0.0118) and relapse free survival at five years. Morbidity and mortality were numerically higher in FANA positive patients. Conclusion Baseline FANA positivity among AIHA patients was found to be associated with lower complete response rates and higher relapse rates with possible higher rates of morbidity. Presence of FANA will give us prognostic value and help us in deciding the treatment options.

Anemia hemolítica autoinmune: mecanismos de inducción de autoinmunidad e inmunobiología / Autoimmune hemolytic anemia: mechanisms of autoimmunity induction and immunobiology

Introducción: La patogénesis de la anemia hemolítica autoinmune es un proceso complejo en el que muchos elementos tienen una función esencial que repercuten en la gran heterogeneidad clínica de la enfermedad, pero los mecanismos involucrados en su inducción se desconocen en gran medida. Objetivo: Explicar los principales mecanismos propuestos en el inicio y aparición de la anemia hemolítica autoinmune y su contribución a la fisiopatología de la enfermedad. Métodos: Se realizó una revisión de la literatura en los idiomas inglés y español, de artículos publicados en los últimos 10 años sobre mecanismos propuestos en el inicio de la anemia hemolítica autoinmune. Análisis y síntesis de la información: Los mecanismos propuestos en la inducción de la autoinmunidad contra los eritrocitos incluyen el mimetismo molecular entre antígenos endógenos y antígenos exógenos, el procesamiento desregulado de autoantígenos influenciado por factores adquiridos y la disfunción de los linfocitos B y T. Conclusiones: Los mecanismos propuestos en la aparición de la anemia hemolítica autoinmune brindan información valiosa para mejorar la comprensión de los mecanismos moleculares involucrados y subrayan la complejidad de los fenómenos involucrados en la perdida de la tolerancia hacia los eritrocitos autólogos y el delicado equilibrio entre factores genéticos y ambientales(AU)

Introduction: The pathogenesis of autoimmune hemolytic anemia is a complex process in which many elements play an essential role and have an impact on the great clinical heterogeneity of the disease, but the mechanisms involved in its induction are largely unknown. Objective: To explain the main mechanisms proposed in the initiation and occurrence of autoimmune hemolytic anemia and its contribution to the pathophysiology of the disease. Methods: A review of the literature, in English and Spanish languages, of articles published in the last 10 years on proposed mechanisms in the initiation of autoimmune hemolytic anemia was carried out. Analysis and synthesis of information: Proposed mechanisms for the induction of autoimmunity against erythrocytes include molecular mimicry between endogenous and exogenous antigens, deregulated processing of autoantigens influenced by acquired factors, and B and T cells dysfunction. Conclusions: The proposed mechanisms in the occurrence of autoimmune hemolytic anemia provide valuable information to improve the understanding of the mechanisms involved and underline the complexity of the phenomena involved in the loss of tolerance towards autologous erythrocytes and the delicate balance between genetic and environmental factors(AU)

Caracterización fisiopatológica de las anemias hemolíticas autoinmunes / Pathophysiological characterization of autoimmune hemolytic anemia

Introducción: La anemia hemolítica autoinmune se define como el aumento de la destrucción de los eritrocitos en presencia de autoanticuerpos dirigidos contra antígenos de grupos sanguíneos eritrocitarios. Objetivo: Caracterizar las anemias hemolíticas autoinmunes teniendo en cuenta las características fisiopatológicas, manifestaciones clínicas y el diagnóstico de laboratorio. Métodos: Se realizó una revisión de la literatura en inglés y español de artículos publicados en los últimos 10 años sobre anemia hemolítica autoinmune. Conclusiones: La anemia hemolítica autoinmune es una enfermedad muy heterogénea. El diagnóstico suele ser fácil, pero los casos difíciles pueden ser un desafío. La definición de cada tipo es fundamental ya que la terapia es diferente y se enfoca más con la comprensión de los mecanismos patogénicos(AU)

Introduction: Autoimmune hemolytic anemia is defined as increased destruction of red blood cells in the presence of autoantibodies directed against red cell blood group antigens. Objective: To characterize autoimmune hemolytic anemias, taking into account immunohematological, clinical, diagnostic and pathogenic mechanisms. Methods: A review of the literature, in English and Spanish, of articles published in the last 10 years on autoimmune hemolytic anemia was carried out. Conclusions: Autoimmune hemolytic anemia is a very heterogeneous disease. Diagnosis is usually easy, but difficult cases can be challenging. The definition of each type is fundamental since the therapy is different and focuses more on understanding the pathogenic mechanisms(AU)

Sepsis-induced autoimmune hemolytic anemia in the elderly

Autoimmune hemolytic anemia (AIHA) secondary to hematological malignancy is well-known and common in the elderly. AIHA associated with infection is less commonly reported in the elderly. We are reporting a case of AIHA in an elderly female with comorbidities, probably secondary to Gram-negative infection. The case was admitted and treated during the peak of the second wave of the COVID pandemic. The treatment of AIHA also had an impact on the progress and outcome of the underlying disease, leading to readmission in a short span of time. The patient also developed a thrombotic complication known to be associated with AIHA.

Síndrome de Evans y embarazo: reporte de un caso / Evans syndrome and pregnancy: a case report

Resumen El síndrome de Evans es una enfermedad conformada por la presencia simultánea o secuencial de trombocitopenia inmunitaria y anemia hemolítica autoinmunitaria, que puede ser primaria o secundaria a otra patología. Es una afección poco frecuente, por lo que es necesario tener una alta sospecha, y descartar otras patologías que cursan con dichas alteraciones hematológicas, para hacer el diagnóstico. Su manejo representa un desafío terapéutico dado su curso crónico y recidivante. La presentación durante el embarazo se asocia a morbilidad materna y fetal. A continuación presentamos el caso de una gestante en quien se pesquisó trombocitopenia severa aislada al ingreso al control prenatal, y que en el curso del embarazo desarrolló AHAI conformando un síndrome de Evans, que se consideró secundario a LES incompleto al realizar el estudio reumatológico. Debido a la pobre respuesta al tratamiento médico con corticoides e inmunosupresores, la mayor parte del embarazo se mantuvo hospitalizada para observación, ajuste y cambio de terapia, siendo necesario recurrir a manejo quirúrgico con esplenectomía.

Abstract Evans syndrome is a rare entity formed by the simultaneous or sequential presence of immune thrombocytopenia and autoimmune hemolytic anemia, which can be primary or secondary to another pathology. The presentation of this disease during pregnancy is associated with maternal and fetal morbidity. The syndrome's diagnosis requires a high suspicion and the ruling out of other pathologies that can happen with the same hematological alterations. The management represents a therapeutic challenge because of its chronic and recurrent course. Below we present the case of a pregnant woman in whom isolated severe thrombocytopenia was detected at admission for prenatal control, and who developed AIHA during the pregnancy, forming Evans syndrome, which was considered secondary to incomplete SLE when performing the rheumatological study. Due to the poor response to medical treatment with corticosteroids and immunosuppressants, the patient was hospitalized for most of her pregnancy for observation, adjustment and change of therapy, and even it was necessary resort to surgical management with splenectomy.

A Case of Overlapping Autoimmune Sclerosing Cholangitis and Autoimmune Hemolytic Anemia

This case study revolves around a 14-year old female patient, who was otherwise healthy and normal, but brought to the hospital for her condition. The patient presented with complaints of fever, sore throat, severe abdominal pain, and generalized body aches for the last two days. She appeared to be lethargic and weak. Her condition had made her fatigued and a yellowish tinge of the face and sclera was obvious, pointing towards a likely case of jaundice. Upon abdominal examination, the patient also had hepatomegaly and splenomegaly present, and the muscles appeared to be tender as well. The child was admitted to the hospital, where her lab investigations revealed that she was anemic. Other investigations revealed that her liver enzymes and bilirubin levels were significantly elevated. Her DAT was positive and there was a presence of high eosinophilia. However, her Hepatitis A, B, and C screening came out to be negative. She was kept as a suspected case of Autoimmune Hemolytic Anemia as it appeared to be themost probable diagnosis, given her condition, but following a biopsy she was also confirmed as a case of Autoimmune Sclerosing Cholangitis.Once the diagnosis was confirmed, the child was treated accordingly. This was a unique case because it involved a significant overlap in the presence of two diseases. Both of the diseases, although similar in appearance, could have caused great havoc if they were not separately diagnosed and treated accordingly. This case study deals with such an overlapping case that was brought to the hospital. With a myriad of confusing symptoms, it was obvious that the diagnosis could have been misdiagnosed or incorrectly diagnosed. However, the lab reports and examinations that were carried out smartly helped in excluding the other diagnoses which would have obviously lead to confusions and also in starting the wrong treatment. This case study deals with the examination, laboratory protocols, along with the association of symptoms, all of which help in reaching towards the final diagnosis in a timely manner and thus, helped treat the patient effectively. The case study also highlights how both of these diseases present in a patient of the younger population, and how they need to be managed effectively and efficiently to ensure that there are no complications that might alter the already deteriorating state of the patient.

Cold autoimmune hemolytic anemia with myelodysplastic syndrome: Not just an “Open and Shut case”

Myelodysplastic syndromes (MDSs) are clonal hematopoietic stem cell neoplasms characterized by bone marrow failure leading to ineffective hematopoiesis, dyspoiesis, and cytopenias with a risk of progression to acute leukemia. Immunological syndromes have been reported to occur along with MDS in literature. However, cold autoimmune hemolytic anemia (AIHA) has rarely been reported in association with MDS. Herein, we report a case of an elderly male who presented with fever and cytopenias. He was being treated as a case of megaloblastic anemia in the past with no response to therapy. At present admission, the peripheral blood smear examination revealed red cell agglutination, thrombocytopenia with 4% blasts. Cold agglutinin disease was confirmed by a thermal agglutination test and bone marrow evaluation showed adequate megakaryocytes with 10% blasts; consistent with the diagnosis of MDS with excess blasts (MDS-EB2). Cytogenetic studies revealed multiple abnormalities. This report is being discussed in view of its rarity of presentation of cold AIHA with MDS.

Anemia hemolítica autoimune na doença de Castleman multicêntrica: relato de caso / Autoimmune hemolytic anemia in multicentric Castleman's disease: case report

A doença de Castleman é um distúrbio linfoproliferativo raro, podendo se manifestar sob a forma de massas localizadas ou como doença multicêntrica. A doença de Castleman multicêntrica é caracterizada por adenopatias generalizadas, visceromegalias, manifestações autoimunes e infecções recorrentes. Este artigo apresenta o relato de caso de anemia hemolítica autoimune por anticorpos quentes em paciente com doença de Castleman multicêntrica. Resposta eficaz foi obtida com uso de corticoterapia sistêmica e tocilizumabe.

Castleman disease is a rare lymphoproliferative disorder that can manifest as localized masses or as multicentric disease. Multicentric Castleman disease is characterized by generalized adenopathies, visceromegaly, autoimmune manifestations, and recurrent infections. This article presents the case report of a patient with multicentric Castleman's disease and autoimmune hemolytic anemia by warm antibodies. Effective response was obtained with systemic corticotherapy and tocilizumab.

The expression and clinical significance of CD4+ CD69+ T cells in peripheral blood of patients with autoimmune hemolytic anemia / 中国输血杂志

【Objective】 To analyze the expression and clinical significance of CD4+ CD69+ T cells in peripheral blood of patients with autoimmune hemolytic anemia. 【Methods】 A total of 206 cases of autoimmune hemolytic anemia admitted to our hospital from March 2018 to March 2021 were selected as the observation group, and 206 cases, who came for healthy physical examination during the same period, were selected as the control group. The levels of CD4+ CD69+ T cells in peripheral blood, CD69 mRNA in CD4+ T cells and CD69 in plasma were detected. The relationship between CD4+ CD69+ T cell level and Hb, reticulocyte ratio (Ret), total bilirubin (TBIL) and indirect bilirubin (IBIL) was analyzed by Pearson correlation analysis to observe the occurrence of venous thromboembolism. The AUC of receiver operating characteristic was used to evaluate the predictive efficacy of CD4+ CD69+ T cell expression in peripheral blood for venous thromboembolism. 【Results】 The levels of CD4+ CD69+ T cells in peripheral blood, CD69 mRNA in CD4+ T cells and CD69 in plasma of observation group were significantly higher than those of control group (P<0.05). Among 206 patients in the observation group, the levels of CD4+ CD69+ T cells in peripheral blood, CD69 mRNA in CD4+ T cells and plasma CD69 in hemolytic attack group were significantly higher than those in remission group, with statistical significance(P<0.05). The Hb in hemolytic attack group was significantly lower while Ret, TBIL and IBIL levels were significantly higher than those in remission group, with statistical significance(P<0.05). Pearson correlation analysis showed that the levels of CD4+ CD69+ T cells in peripheral blood of patients with autoimmune hemolytic anemia were negatively correlated with Hb(P<0.05) while positively correlated with Ret, TBIL and IBIL(P<0.05). According to ROC curve analysis, the expression level of CD4+ CD69+ T cells in peripheral blood predicted that the AUC of patients with autoimmune hemolytic anemia complicated with venous thromboembolism was 0.915(SE: 0.068, OR: 0.002, 95%CI: 0.000~1.000). 【Conclusion】 The up-regulated expression level of CD4+ CD69+ T cells in peripheral blood of patients with autoimmune hemolytic anemia is closely related to disease evolution, and it has good efficacy in predicting venous thromboembolism and deserves clinical attention.

Efficacy of plasma exchange in the treatment of autoimmune hemolytic anemia in children / 中国小儿急救医学

Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.

Efficacy of plasma exchange in the treatment of autoimmune hemolytic anemia in children / 中国小儿急救医学

Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.

Anemia hemolítica mixta posterior a infección por SARS-CoV-2 en un paciente infectado por virus de hepatitis B

Resumen Varón de 36 años, diabético, con antecedente de 10 días de evolución de una neumonía por COVID-19. Fue trasladado por disnea, somnolencia y astenia de 24 horas de aparición. Presentaba taquicardia, taquipnea, palidez e ictericia generalizada. Se confirmó una anemia severa normocítica normocrómica, acompañado de hemólisis intravascular (test de Coombs directo positivo, LDH y bilirrubina indirecta aumentada, consumo de haptoglobina). Además, tenía un HBsAg positivo, con IgM anti-HBc negativo y transaminasas elevadas. El paciente inició tratamiento con tenofovir, además de metilprednisolona, inmunoglobulina humana IV y múltiples microtransfusiones, con buena evolución.

Abstract A 36-year-old male, diabetic, with 10-day history of inpatient care due to SARS-CoV-2 pneumonia. Dyspnea, drowsiness, and a 24-hour asthenia evolution were the main symptoms the patient manifested. He had tachycardia, tachypnea, pallor, and a generalized jaundice. Laboratory studies revealed severe normochromic normocytic anemia with an intravascular hemolysis (Coombs test direct positive, LDH and indirect bilirubin increased, haptoglobin decreased), HBsAg: positive, IgM anti-HBc: negative and transaminases increased. The patient started treatment with tenofovir, apart from that boluses of methylprednisolone, human immunoglobulin and multiple microtransfusions were also given, having a good clinical evolution.

First report of expansion of CD4+/CD28 null T-helper lymphocytes in adult patients with idiopathic autoimmune hemolytic anemia

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.

Síndrome de Evans como manifestación inicial de lupus eritematoso sistémico / Evans syndrome as an initial manifestation of systemic lupus erythematosus

El síndrome de Evans se caracteriza por la presentación simultánea de anemia hemolítica autoinmune y púrpura trombocitopénica inmune, puede presentarse como una patología aislada o como manifestación de una enfermedad sistémica. Caso Clínico: Preescolar masculino de 3 años, diagnosticado de síndrome de Evans, requirió tratamiento con corticoides e inmunoglobulina por mala respuesta inmunológica, tres meses después de su diagnóstico inicial presento afectación renal, además de presentar autoanticuerpos positivos, por lo que se estableció diagnóstico de lupus eritematoso sistémico. Conclusión: El síndrome de Evans es una entidad nosológica poco frecuente, ante su diagnóstico se debe descartar enfermedad sistémica subyacente.

Evans syndrome is characterized by the simultaneous presentation of autoimmune hemolytic anemia and immune thrombocytopenic purpura; it can be manifested as an isolated pathology or as a manifestation of a systemic disease. Clinical Case: 3-year-old preschool male, diagnosed with Evans syndrome, that required corticosteroids and immunoglobulin intravenous treatment due to poor immune response. Three months after his initial diagnosis he presents kidney affectation in addition to presenting positive auto-antibodies, with which it was established the diagnosis of systemic lupus erythematosus. Conclusion: Evans syndrome is a rare nosological entity, when the diagnosis is made an underlying systemic disease must be ruled out.

Primary Autoimmune Hemolytic Anemia Due To Warm Reactive Autoantibodies: A Rare Case Report

Autoimmune hemolytic anemia (AIHA) is an acquired form of hemolytic anemia in which autoantibodies target red blood cell (RBC) membrane antigens, inducing cell rupture (lysis). It affects both pediatric and adult populations, although its presentation in childhood is relatively rare, with the annual incidence [3]estimated to be approximately 0.8 per 100,000 individuals under 18 years old . Here we report one such a rare case of autoimmune hemolytic anemia due to primary warm reactive autoantibodies in a 3 year old female child. As there was presence of hemolysis in peripheral blood smear & other investigations also, Direct coomb's test was done & it came out to be positive which was suggestive of autoimmune hemolytic anemia, as following laboratory reports are suggestive of continuous destruction of RBC & after introduction of steroids parameters of hemolysis came out to be normal suggestive of warm reactive autoantibodies type of AIHA. Clinically also patient improved & her urine colour also became normal after when prednisolone started. Patient also did not have any features of secondary causes of warm autoantibody like Systemic lupus erythematous, immunodeficiency disorders, ulcerative colitis & lymphoproliferative disorders so it was considered primary or idiopathic. W-AIHAtends to have a chronic course and is not expected to subside without treatment. It can be a fatal disease, with a mortality rate of up to 4% in children, either because of the acuity of the presentation or because of being refractory to treatment and requiring multiple lines of therapy with frequently associated toxicity. Fortunately, our patient responded to steroid therapy.

Anemia hemolítica autoinmune en paciente pediátrico infectado por virus de inmunodeficiencia humana (VIH); reporte de caso / Autoimmune hemolytic anemia in a pediatric patient infected human immunodeficiency virus (HIV); a case report

Las personas que viven con el virus de inmunodeficiencia humana presentan complicaciones de tipo hematológica durante el curso de la enfermedad, pueden ser propias de su estado mórbido, subyacente a infecciones oportunistas o por el tratamiento antirretroviral. La anemia hemolítica autoinmune constituye una complicación rara y potencialmente letal, en el contexto de la infección por VIH. Presentamos el caso de una lactante mayor de un año nueve meses de edad, con infección perinatal por VIH, diagnosticada con Anemia hemolítica autoinmune por anticuerpos mixtos, con alta reacción inmunológica y mala respuesta al tratamiento clínico. Aunque la prueba de Coombs sigue siendo el estándar oro para el diagnóstico, la positividad de esta no establece el diagnostico per se, pues puede ser positiva entre el 18-43% de los pacientes infectados por VIH.

People living with human immunodeficiency virus present hematological complications during course of disease, they may be due to their morbid state, underlying opportunistic infections or due to antirretroviral treatment. Autoimmune hemolytic anemia is a rare and potentially fatal complication of HIV infection. We present case of an infant older than one year nine months, for perinatal HIV infection, diagnosed with autoimmune hemolytic anemia due to mixed antibodies, with high immunological reaction and poor response to clinical treatment. Although the Coombs test remains the gold standard for diagnosis, its positivity does not establish the diagnosis per se, it can positive in 18-43% of HIV-infected patients.

Use of the reticulocyte channel warmed to 41 °C of the XN-9000 analyzer in samples with the presence of cold agglutinins

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.