Pharmaceutical care for rare ADR in a patient with ALK-positive non-small cell lung cancer induced by alectinib / 中国药房

OBJECTIVE To provide reference for safe drug use in patients with anaplastic lymphoma kinase （ALK）-positive non-small cell lung cancer （NSCLC）. METHODS Clinical pharmacists participated in the diagnosis and treatment of a patient with ALK-positive NSCLC who developed bilateral pleural effusion and hemolytic anemia after taking alectinib； regarding symptoms such as pleural effusion and hemolytic anemia in the patient， clinical pharmacists investigated the patient’s history of medication and disease， as well as potential drug interaction； to consider the correlation between the patient’s use of alectinib and the duration of pleural effusion and hemolytic anemia， clinical pharmacists suggested that clinical doctors discontinued alectinib and used reduced dose treatment after the pleural effusion improved， but the patient suffered from bilateral pleural effusion and hemolytic anemia again； after evaluating the correlation between alectinib and bilateral pleural effusion and hemolytic anemia using the Naranjo’s assessment scale， clinical pharmacists recommend permanent discontinuation of alectinib and jointly recommend replacement with ensartinib with clinical physicians. RESULTS Physicians adopted the suggestions of clinical pharmacists. The pleural effusion subsequently regressed and hemolytic anemia improved after replacing the drug. The correlation between alectinib and bilateral pleural effusion and hemolytic anemia was confirmed. CONCLUSIONS Clinical pharmacists participate in pharmaceutical monitoring of ALK-positive NSCLC patients， assist clinical doctors in developing personalized medication recommendations， and ensure the safety of patient medication.

Púrpura trombótica trombocitopénica en pediatría. Reporte de un caso / Thrombotic thrombocytopenic purpura in pediatrics. A case report

La púrpura trombótica trombocitopénica es una entidad poco frecuente en pediatría, pero de alta mortalidad sin tratamiento adecuado y oportuno. Se caracteriza por presentar anemia hemolítica microangiopática asociada a signos y síntomas neurológicos, cardíacos, abdominales y menos frecuentemente renales; puede estar acompañada de fiebre. En niños, el diagnóstico se basa en los hallazgos clínicos y de laboratorio. La actividad de ADAMTS13 <10 % apoya, pero no confirma el diagnóstico y, dada la gravedad de la patología, el resultado no debe retrasar el inicio del tratamiento. Se presenta una paciente de 15 años, previamente sana, con signos neurológicos asociados a anemia hemolítica y trombocitopenia. Durante su internación, se arribó al diagnóstico de púrpura trombótica trombocitopénica adquirida.

Thrombotic thrombocytopenic purpura is a rare disease in pediatrics, but it has a high mortality if not managed in an adequate and timely manner. It is characterized by microangiopathic hemolytic anemia associated with neurological, cardiac, abdominal, and less frequently, renal signs and symptoms; it may be accompanied by fever. In children, diagnosis is based on clinical and laboratory findings. ADAMTS13 activity < 10% supports the diagnosis but does not confirm it and, given its severity, the result should not delay treatment initiation. Here we describe the case of a previously healthy 15-year-old female patient with neurological signs associated with hemolytic anemia and thrombocytopenia. During hospitalization, she was diagnosed with acquired thrombotic thrombocytopenic purpura.

Cognitive Function in Children With ?-Thalassemia Major

Objectives: To assess intelligence Quotient (IQ) in transfusion dependent ?-thalassemia major patients using Malin Intelligence Scale for Indian Children (MISIC) and to correlate verbal IQ (VIQ), performance IQ (PIQ) and full scale IQ (FSIQ) with serum ferritin levels and annual blood transfusion requirements. Methods: Cross-sectional study design, enrolling 100 patients of transfusion-dependent ?-thalassemia aged 6 years to 15 years 11 months. IQ was assessed using MISIC. Results: Mean (SD) full scale IQ was 95.96 (7.23). IQ was ‘average’ in most of the patients. There was a significant negative correlation of serum ferritin levels with object assembly (r=-0.215, P=0.034) component of PIQ; annual blood requirement with general comprehension component of VIQ (r=-0.275, P=0.006) and age at diagnosis with PIQ (r=-0.273, P=0.006). There was a significant linear correlation of PIQ (r=0.280, P=0.005) and FSIQ (r=0.274, P=0.006) with pre-transfusion hemoglobin. Conclusion: IQ correlates with age at diagnosis and average annual pre-transfusion hemoglobin. This highlights the importance of early diagnosis and maintenance of satisfactory hemoglobin levels

Cold antibody autoimmune hemolytic anemia following varicella infection

Introduction: Autoimmune hemolytic anemia (AIHA) is a rare complication of chicken pox. In adults, such AIHA is due to warm antibodies. We report a case of cold antibody AIHA following chicken pox in a young female. Case Report: A 24-year-old female presented with clinical and laboratory features consistent with hemolytic anemia 5 days after the onset of chicken pox. Her hemoglobin levels dropped rapidly during the course of admission from 7.9 to 3.8 g/dL with evidence of ongoing haemolysis in the form of rising total and indirect bilirubin. Peripheral smear revealed red cell agglutinates and erythrophagocytosis. Direct Coomb's test (DCT) was positive for C3d suggesting a cold antibody AIHA. Since test for Donath Landsteiner antibody was negative, and all other tests for common causes of hemolytic anemia were noncontributory, it was presumed to be due to chicken pox. The fulminant course necessitated a short course of oral steroids to which she responded with rise in hemoglobin and no further hemolysis. Two weeks later, her peripheral smear was normal and DCT negative. Conclusion: In patients presenting with acute onset anemia following chicken pox, possibility of cold antibody AIHA must be considered and appropriate testing pursued. Despite lack of empiric evidence, short course of steroids may be beneficial if drop in hemoglobin is rapid with evidence of fulminant hemolysis, showing no abatement after first week.

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.

Progress in immune hemolytic anemia caused by drugs / 中国输血杂志

Drug-induced immune hemolytic anemia (DIIHA) is a rare cytopenia caused by damage to RBCs by drug-induced antibodies or non-immune protein adsorption (NIPA). The drugs associated with DIIHA and the mechanistic hypotheses that are thought to be involved have been controversial, with complex serological tests often required by specialized Immune Hematology laboratories for diagnosis. It is necessary to know the clinical manifestation and laboratory diagnosis of DIIHA in order to distinguish the immuno-hematological abnormality caused by drugs from other causes. How to improve the diagnostic ability of DIIHA and establish a scientific and reasonable idea of DIIHA serological examination is urgent to help clinical diagnosis and correct treatment.

Rare case of hereditary spherocytosis with rheumatic mitral stenosis

A rare case of hereditary spherocytosis (HS) and rheumatic mitral stenosis coexisting in a patient having severe stenosis, atrial fibrillation, and symptoms of the left ventricular dysfunction, along with hemolytic anemia attributed to HS. We present the case of a 58-year-old lady who presented to the emergency department with complaints of increasing shortness of breath for the past week. She was examined to have atrial fibrillation with a fast ventricular rate. On investigations, she was found to have severe rheumatic mitral stenosis with evidence of hemolytic anemia. Further, evaluation of the cause of her anemia revealed HS.This case highlights the importance of the evaluation of anemia in patients with valvular heart diseases. If a treatable cause is found, anemia can be treated to reduce the cardiac burden

Pancytopenia as a presentation of B12 deficiency

We present the case of a 16-year-old boy with hemolytic anemia with investigations showing pancytopenia, which turned out to be due to B12 deficiency. Whenever we see a teenager presenting with pancytopenia, the common causes that we think of are hematologic malignancies, drug-induced cytopenias, hypersplenism, etc. Anemia as a presentation is common in B12 deficiency, yet its occurrence with jaundice is a very uncommon and easily overlooked presentation. The same is the case of B12 presenting as pancytopenia which is a well-described entity, yet omitted initially due to its uncommon nature in this modern developed world.

Infección por virus de epstein barr y debut de lupus eritematoso sistémico (LES) en un adolescente / Epstein barr virus infection and systemic lupus erythematosus (SLE) debut in an adolescent / Infecção pelo vírus Epstein Barr e início de lúpus eritematoso sistêmico (LES) em adolescente

Introducción: El lupus eritematoso sistémico (LES), prototipo de enfermedad autoinmune, cursa con empujes y remisiones. Dada la diversidad de presentaciones posibles, su diagnóstico y tratamiento son un reto para el clínico, y se requiere tener un alto índice de sospecha. Objetivo: presentar el caso clínico de un adolescente que debuta con LES a forma de anemia hemolítica, probablemente gatillado por infección por virus de Epstein Barr. Caso clínico: Varón de 14 años, sin antecedentes a destacar. Consulta por fiebre de 7 días de evolución de hasta 39º C, odinofagia, astenia y adinamia. Al examen físico se constata palidez cutáneo mucosa, ictericia, adenopatías cervicales y hepatoesplenomegalia. El laboratorio muestra anemia severa regenerativa con aumento de las bilirrubinas a expensas de la indirecta sin hepatitis. Prueba de Coombs positiva. Anticuerpos específicos para Epstein Barr positivos, con lo que se diagnostica anemia hemolítica secundaria a mononucleosis y se inicia tratamiento corticoideo. En la evolución agrega eritema malar y limitación en flexión de codos y rodillas. Se reciben anticuerpos antinucleares y anti ADN nativo positivos con hipocomplementemia severa. Con diagnóstico de LES se inicia hidroxicloroquina y azatioprina, manteniéndose la prednisona. Conclusiones: Muchos virus (hepatitis C, Parvovirus B19, Epstein Barr y Citomegalovirus) se han descrito como posibles inductores o simuladores de LES. Es necesario mantener un alto índice de sospecha para realizar un diagnóstico oportuno y tratamiento precoz.

Introduction: Systemic lupus erythematosus (SLE), prototype of autoimmune disease, progresses with flares and remissions. Given the diversity of possible presentations, its diagnosis and treatment are a challenge for the clinician, and a high index of suspicion is required. Objective: To present the clinical case of an adolescent who debuted with SLE in the form of hemolytic anemia, probably triggered by Epstein Barr virus infection. Clinical case: 14 - year - old male, with no history to highlight. Consultation for fever of 7 days of evolution of up to 39º C, odynophagia, asthenia and adynamia. Physical examination revealed mucous skin pallor, jaundice, cervical lymphadenopathy, and hepatosplenomegaly. The laboratory shows severe regenerative anemia with increased bilirubin at the expense of indirect without hepatitis. Positive Coombs test. Specific antibodies for Epstein Barr were positive, with which hemolytic anemia secondary to mononucleosis was diagnosed and corticosteroid treatment was started. In the evolution, it adds malar erythema and limitation in flexion of the elbows and knees. Positive antinuclear and anti-native DNA antibodies are received with severe hypocomplementemia. With a diagnosis of SLE, hydroxychloroquine and azathioprine were started, maintaining prednisone. Conclusions: Many viruses (hepatitis C, Parvovirus B19, Epstein Barr and Cytomegalovirus) have been described as possible inducers or mimics of SLE. It is necessary to maintain a high index of suspicion for timely diagnosis and early treatment.

Introdução: O lúpus eritematoso sistêmico (LES), protótipo de doença autoimune, evolui com impulsos e remissões. Dada a diversidade de apresentações possíveis, seu diagnóstico e tratamento são um desafio para o clínico, sendo necessário um alto índice de suspeição. Objetivo: apresentar o caso clínico de uma adolescente que iniciou com LES na forma de anemia hemolítica, provavelmente desencadeada por infecção pelo vírus Epstein Barr. Caso clínico: Homem de 14 anos, sem antecedentes a destacar. Consulta por febre de 7 dias de evolução de até 39º C, odinofagia, astenia e adinamia. O exame físico revelou palidez cutânea mucosa, icterícia, linfadenopatia cervical e hepatoesplenomegalia. O laboratório mostra anemia regenerativa grave com aumento da bilirrubina em detrimento da indireta sem hepatite. Teste de Coombs positivo. Anticorpos específicos para Epstein Barr foram positivos, com o qual foi diagnosticada anemia hemolítica secundária à mononucleose e iniciado tratamento com corticosteróides. Na evolução, acrescenta eritema malar e limitação na flexão dos cotovelos e joelhos. Anticorpos antinucleares e anti-DNA nativos positivos são recebidos com hipocomplementemia grave. Com diagnóstico de LES, iniciou-se hidroxicloroquina e azatioprina, mantendo-se prednisona. Conclusões: Muitos vírus (hepatite C, Parvovírus B19, Epstein Barr e Citomegalovírus) têm sido descritos como possíveis indutores ou mimetizadores do LES. É necessário manter um alto índice de suspeição para diagnóstico oportuno e tratamento precoce.

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)

Síndrome del linfocito pasajero por anticuerpos anti-D en trasplante hepático. Reporte de caso / Passenger lymphocyte syndrome due to anti-D antibodies in liver transplantation. Case report

Resumen El síndrome del linfocito pasajero (PLS) es una complicación de injerto contra huésped que se presenta en el trasplante de órganos sólidos o en el trasplante de células progenitoras hematopoyéticas. Es una causa importante de hemólisis inmune después del trasplante causada por la producción de anticuerpos por parte de los clones específicos de linfocitos B viables transferidos a través del órgano del donante contra los antígenos de los glóbulos rojos del receptor. Generalmente ocurre en los trasplantes con discordancia menor ABO o Rh. Este estudio descriptivo describe el caso de un paciente de 54 años con grupo sanguíneo O/Rh(D) positivo, con cirrosis secundaria a enfermedad metabólica asociada al hígado graso (MAFLD) que fue llevado a trasplante hepático de donante O/Rh(D) negativo. A los 9 días del trasplante presentó una anemia hemolítica inmune por anticuerpos anti-D por efecto del linfocito B pasajero del donante sensibilizado. El paciente recibió medidas de soporte, transfusión de glóbulos rojos e inmunosupresión con esteroides, con lo que se logró la estabilización de los parámetros hemolíticos. En conclusión, esta es una entidad que se debe sospechar en caso de anemia hemolítica aguda en el período postrasplante.

Abstract Passenger lymphocyte syndrome (PLS) is a graft-versus-host complication in solid organ transplantation or hematopoietic stem cell transplantation. It is a major cause of immune hemolysis after transplantation caused by the production of antibodies by the specific clones of viable B lymphocytes transferred through the donor organ against the antigens of the recipient's red blood cells. It usually occurs in transplants with minor ABO or Rh mismatch. This descriptive study explains the case of a 54-year-old patient with O/Rh(D) positive blood group, with cirrhosis secondary to metabolic disease associated with fatty liver (NAFLD), who underwent liver transplantation from an O/Rh(D) negative donor. Nine days after the transplant, the patient presented with immune hemolytic anemia due to anti-D antibodies because of the transient B lymphocyte from the sensitized donor. The patient received support measures, transfusion of red blood cells, and immunosuppression with steroids, which stabilized the hemolytic parameters. In conclusion, this entity should be suspected in the case of acute hemolytic anemia in the post-transplant period.

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.

A case report of hereditary spherocytosis (HS): Approach to diagnosis and management of HS

Hereditary spherocytosis (HS) is a type of congenital hemolytic anemia, in which heterogeneous alterations in one of the six genes that encode for proteins involved in vertical associations which tie the red blood cell (RBC) membrane skeleton to the lipid bilayer causes dysfunction or deficiency of cell membrane protein resulting in spherical-shaped, hyper-dense, and poorly deformable RBCs with a shortened life span. We report a case of HS in a 2-month-old female who presented with severe anemia, jaundice, and hepatosplenomegaly. The peripheral blood smear showed spherocytosis and reticulocytosis. The osmotic fragility was positive and direct antiglobin test was negative. The osmotic fragility test and direct antiglobulin test were positive. She was managed with packed RBCs (PRBCs) transfusion and folic acid supplementation

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.

Síndrome hepatorrenal en asociación con hepatitis alcohólica y síndrome de Zieve / Hepatorenal syndrome in association with alcoholic hepatitis and Zieve's syndrome

RESUMEN La enfermedad hepática alcohólica tiene un amplio espectro de enfermedades, incluida la hepatitis alcohólica, que en sus formas graves puede conducir al síndrome hepatorrenal. La anemia es común en pacientes alcohólicos, pero una anemia hemolítica asociada con hiperlipidemia e ictericia se reconoce como síndrome de Zieve. Un varón de 42 años con consumo excesivo de alcohol fue admitido por ictericia y dolor abdominal. Durante su evolución presentó azoemia progresiva y anemia hemolítica. Se realizó el diagnóstico de síndrome hepatorrenal asociado a hepatitis alcohólica, así como un síndrome de Zieve. Fue tratado con corticoterapia y la combinación de albúmina y noradrenalina, además del retiro de alcohol, con resultados favorables.

ABSTRACT Alcoholic liver disease has a broad spectrum of diseases, including alcoholic hepatitis, which in its severe forms can lead to hepatorenal syndrome. Anemia is common in alcoholic patients, but a hemolytic anemia in association with hyperlipidemia and jaundice is recognized as Zieve's syndrome. A 42 year old man with heavy alcohol consumption was admitted for jaundice and abdominal pain. During his evolution, he presented progressive azotemia and hemolytic anemia. The diagnosis of hepatorenal syndrome associated with alcoholic hepatitis was made, as well as a Zieve's syndrome. He was treated with corticosteroid therapy and the combination of albumin and norepinephrine, in addition to alcohol withdrawal, with favorable results.

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.