ABSTRACT
Autoimmune Haemolytic Anaemia (AIHA) is a decompensated acquired haemolysis caused by the host immune system producing autoantibodies that bind to the antigens on the surface of circulating erythrocytes, leading to haemolysis and decreased red cell survival. It requires efficient and advanced immunohaematological and transfusion support. Despite advances in medical field, simple test like Direct Antiglobulin Test (DAT) still remains the diagnostic hallmark. The sensitive column gel technology further helps to characterise these antibodies according to class, subclass and titre of antibodies. It is very important to characterize these autoantibodies as there is a relation between strength of DAT and in vivo haemolysis. Serologically, cases are divided into warm (mainly due to IgG), cold (mainly due to IgM) or mixed depending upon the thermal amplitude of the antibody. IgA and IgG antibodies causing warm type of AIHA are rare as monospecific gel cards are not available in all centres. We here report rare case series of warm AIHA caused by dual antibodies IgA and IgG.
ABSTRACT
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.
ABSTRACT
Multiple myeloma is a neoplastic plasma cell dyscrasia. Anaemiain multiple myeloma is usually related to many factors, of which the commonest cause being anaemiaof chronic disease. We reporteda 67 years old hypertensive male presented with low back pain and loss of appetite. Investigations revealed severe anaemiaand elevated total serum protein with albumin/globulin (A/G) reversal (1:4). Bone marrow aspiration revealed plasma cell dyscrasia. Skeletal survey evaluation showed multiple osteolytic lesions. Serum protein electrophoresis revealed M spike in gamma globulin region with immunofixation suggestive of IgG and kappa monoclonal gammopathy. He was diagnosed as a case of multiple myeloma.. On evaluating the cause of severe anaemia, interestingly various findings suggestive of Autoimmune hemolytic anaemia(AIHA) were found. Packed red blood cell transfusion along with steroids was done for correction of severe anaemia. Review of literature showed that only about 4% of AIHA patients had multiple myeloma. We reported a rare case of multiple myeloma who presented with AIHA.
ABSTRACT
@#Autoimmune haemolytic anaemia (AIHA) is a group of disorders wherein autoantibody causes decompensated acquired haemolysis. There has been no epidemiological study of autoimmune haemolytic anaemia (AIHA) in Malaysia. This study retrospectively analysed the epidemiology of AIHA including Evan’s Syndrome in a Tertiary Haematology Centre in Malaysia. Patients diagnosed with AIHA and Evan’s Syndrome at 18 years old and above between 1 January 1994 to 1 October 2020 at the out-patient Haematology Clinic of Hospital Raja Permaisuri Bainun, Ipoh were selected. Patients’ information was retrieved from the outpatient clinic records. A total of 71 patients were included of which predominantly female. The mean age for both genders were comparable. Ethnic stratification revealed AIHA was higher in Malays followed by Chinese and Indian. Warm AIHA was most prevalent at 40.8%, compared to cold AIHA and Evan’s Syndrome (both 23.9%), and mixed AIHA (11.3%). Primary was more common than secondary AIHA followed by Evan’s Syndrome. Approximately half of the secondary AIHA and secondary Evan’s Syndrome were due to SLE. Overall, 67.6% of patients received corticosteroid only and 28.2% combined with immunosuppressant. Individuals at higher age and females have higher risk of developing AIHA and Evan’s Syndrome. The highest prevalence was seen among the Malay ethnic. Primary warm AIHA is the most common type and majority of Evan’s syndrome are secondary to autoimmune diseases.
ABSTRACT
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.
ABSTRACT
【Objective】 To explore a method to accurately identify the specificity of alloantibodies or autoantibodies in autoimmune hemolytic anemia (AIHA)patients with both warm and cold antibodies, so as to provide guidance for the selection of blood components. 【Methods】 Blood samples of AIHA patients with both warm and cold antibodies were screened by the direct antiglobulin testing (DAT). The plasma of patients were treated with dilution or adsorption method and the erythrocyte was dispersed for specificity identification of alloantibodies or autoantibodies.According to the results of antibody identification, appropriate phenotype of red blood cells(RBCs) were transfused to patients, and the incidence of adverse reactions and efficacy of transfusion were observed. 【Results】 Alloantibodies or specific autoantibodies were detected in serum or elution in 14 of the 16 patients. 10 patients underwent blood transfusion during hospitalization, and all of them received RBCs with the same or compatible ABO/Rh (D) type as the patients and without any reaction to the alloantibodies and specific warm autoantibodies. No hemolytic reaction occurred, and anemia symptoms were improved after blood transfusion. 【Conclusion】 The selection of appropriate methods could eliminate the influence of autoantibodies on the identification of alloantibodies in AIHA patients with both warm and cold antibodies. Therefore, the selection of blood from compatible donors for transfusion could effectively avoid the occurrence of hemolytic reaction.
ABSTRACT
Pre-transfusion compatibility testing is complicated in autoimmune hemolytic anemia (AIHA) patients due to the presence of autoantibodies. Delays in blood transfusion or even life-threatening would occur if blood type, isoantibodies/ autoantibodies of these patients could not be correctly identified to choose the appropriate blood components. Knowing the detection and treatment countermeasures against blood transfusion compatibility in AIHA patients is of great significance to ensure the timeliness and safety of blood transfusion. Based on the research progress at home and abroad, this article summarizes the serological characteristics, autoantibody types, blood group identification methods, antibody screening and antibody identification methods, and blood transfusion strategies about AIHA patients, in order to eliminate the interference of autoantibodies and provide transfusion guidance for the staff of Blood Transfusion Department.
ABSTRACT
【Objective】 To investigate the effect of cold agglutination on blood group typing. 【Methods】 37℃ water bath, absorption elution test and 2-mercaptoethanol method were used to eliminate the influence of cold agglutination. Forward and reverse blood group typing, cross matching, DAT and IAT experiments were then performed on red blood cells and serum after treatment. 【Results】 Before treatment, obvious discrepancy in forward /reverse typing and nontypable cross matching in 16 blood samples were noticed due to cold agglutination. After corresponding treatments, all samples were consistent or negative in forward/reverse typing, cross matching and antibody screening. No adverse reactions to cross matching blood transfusion occurred in patients, and the increase of hemoglobin was in line with the effective standard of transfusion. 【Conclusion】 37℃ water bath, absorption elution test and 2-mercaptoethanol method can be used to eliminate the interference caused by cold agglutination to obtain correct typing results. The strong reactivity caused by cold agglutination in AIHA patients were different from other cases, which deserved our attention.
ABSTRACT
Background: Autoimmune haemolytic anaemia (AIHA) is relatively uncommon condition with grave consequences, if not diagnosed and treated early. The literature on the clinical outcome and response to treatment is relatively scarce. Aim was to study the clinic-pathological profile and the treatment outcomes in patients with AIHA.Methods: Around 25 patients with AIHA attending a tertiary care hospital over a period of one year were included in the study. The patients were divided based on severity of anaemia and etiology. All the patients data were analysed for the demographic data, clinico -pathological findings and the response to treatment. All the patients data were analysed using SPSS software (version 22).Results: Out of 25 patients, 76% were females and 24% were males. Based on severity of anaemia, 60%, 28% and 8% had severe, moderate and mild anaemia. Around 48% of the patients had thrombocytopenia along with anaemia. 8 (32%) and 17 (68%) patients have primary and secondary AIHAs respectively. In our study the commonest cause for the secondary AIHA was Systemic Lupus Erythematosus (SLE) followed by haematological malignancy, primary Sjogrens, Anti-phospholipid antibody (APLA) syndrome, carcinoma colon and Wilsons disease. Hepatosplenomegaly and lymphadenopathy were present in 36% and 4% respectively. Out of 22 (88%) patients on corticosteroid therapy, 15 (60%) patients responded to corticosteroids alone and 6(24%) patients required corticosteroid plus immunosuppressive therapy.Conclusions: AIHA has to be ruled out in all anaemia patients with indirect hyperbilirubinemia and abnormal peripheral smear. Secondary AIHA is more common than primary. Corticosteroids and immunosuppressive agents are the mainstay of treatment of AIHA.
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We report a case of a 51-year-old woman with Evans syndrome (autoimmune hemolytic anemia and primary immune thrombocytopenia) and hypothyroidism. She was previously diagnosed with Hashimoto's thyroiditis in 1994 (age, 35) and autoimmune hemolytic anemia (AIHA) 3 years ago. She was treated with oral prednisolone. After a period, in which the anemia waxed and waned, there was an abrupt development of thrombocytopenia (nadir 15x10(9)/L) that coincided with the tapering off of prednisolone after 3 years of administration. Because her thrombocytopenia was refractory to prednisolone, we administered rituximab (375 mg/m2 weekly) for 4 weeks. Two weeks after the completion of the rituximab treatment, her platelet count was up to 92x10(9)/L. No intermittent peaking of thyroid stimulating hormone occurred after rituximab treatment was initiated. Evans syndrome and autoimmune thyroiditis might share common pathophysiological mechanisms. This notion supports the use of rituximab in a patient suffering from these disorders.
Subject(s)
Female , Humans , Middle Aged , Anemia , Anemia, Hemolytic , Anemia, Hemolytic, Autoimmune , Antibodies, Monoclonal, Murine-Derived , Hypothyroidism , Platelet Count , Prednisolone , Stress, Psychological , Thrombocytopenia , Thyroid Gland , Thyroiditis , Thyroiditis, Autoimmune , Thyrotropin , RituximabABSTRACT
A 32-yr-old male diagnosed with myelodysplastic syndrome underwent an unmanipulated, unrelated, HLA matched, peripheral blood stem cell transplantation. The patient and donor were both blood type O, CcDEe. Twelve weeks post-transplantation, he developed acute autoimmune hemolytic anemia (AIHA). He was transfused multiple times with washed O red cells. High-dose steroid therapy was initiated and he underwent splenectomy; however, AIHA was refractory to therapy. The patient was further treated with combined treatment modalities including immunosuppressive therapy with mycophenolate mofetil and cyclosporine and three cycles of plasma exchange, and AIHA responded to treatment. This is the third case of AIHA complicating hematopoietic stem cell transplantation reported in Korea. Since AIHA is relatively common after hematopoietic stem cell transplantation, accurate and timely diagnosis of the disease and treatment strategies with multiple modalities are necessary.
Subject(s)
Adult , Humans , Male , Anemia, Hemolytic, Autoimmune/diagnosis , Combined Modality Therapy , Cyclosporine/therapeutic use , Hematopoietic Stem Cell Transplantation/adverse effects , Mycophenolic Acid/analogs & derivatives , Myelodysplastic Syndromes/complications , Plasma ExchangeABSTRACT
Cold agglutinin disease (CAD) is a most common autoimmune hemolytic anemia (AIHA) induced by cold antibody. CAD represents approximately 16-32% of AIHA cases and causative cold autoantibodies commonly show specificity against the I antigen. We report a case of cold agglutinin disease with anti-Pr cold autohemolysin. A 20 year old woman with a history of bone marrow transplantation was admitted with nausea, vomiting, and pallor. Direct antiglobulin tests were positive with IgG and C3d specific AHG reagents. Cold agglutinin titer was as high as 1:1024 at 4degrees C, 1:16 at room temperature, negative at 37degrees C. The agglutinin titer was diminished after treatment with protease, ficin and immunohematologic results of cold agglutinin was compatible with anti-Pr specificity. In unexpected antibody identification test, anti-M which showed reactivity at anti-human globulin phase was identified. Washed and prewarmed 16 units of A+, M antigen negative red blood cells were transfused. After two weeks, patient was improved with steroid therapy and experienced relief of fever and hemolysis, and she was discharged.
Subject(s)
Female , Humans , Young Adult , Anemia, Hemolytic, Autoimmune , Autoantibodies , Bone Marrow Transplantation , Bone Marrow , Coombs Test , Erythrocytes , Fever , Ficain , Hemolysis , Immunoglobulin G , Indicators and Reagents , Nausea , Pallor , Sensitivity and Specificity , VomitingABSTRACT
Autoimmune hemolytic anemia (AIHA) is characterized by the production of Coombs' antibodies, which are responsible for the destruction of RBCs. The antibody specificity of warm AIHA (WAIHA) is very complex while all cells tested are usually reactive in routine test. Although some autoantibodies have broad specificity to the Rh system, apparent specificity for simple Rh antigen (D, C, E, c, e) is rare. A 63 year-old farmer was admitted at Inha University Hospital for evaluation of anemia. He had no history of blood transfusion. He presented overt anemia with decreased Hb (6.6g/dL) and increased reticulocyte count (18.5%, corrected reticulocyte count 8.4%). The direct antiglobulin test (DAT) was strongly positive for IgG and negative for C3d while indirect antiglobulin test (IAT) was weakly positive. Both two different antibody identification test kits identified this antibody as anti-e. His Rh phenotype was CDe. Although the eluted antibody from his own RBCs failed to show specificity against e but agglutinated all the reagent RBCs, we diagnosed this case as WAIHA induced by IgG anti-Rh(e) because his serum agglutinated all e-positive RBCs while it was non-reactive with e-negative RBC in the cross-matching test. Drugs (loxoprofen, cimetidine) couldn't be ruled out as causative agent.
Subject(s)
Humans , Middle Aged , Anemia , Anemia, Hemolytic, Autoimmune , Antibodies , Antibody Specificity , Autoantibodies , Blood Transfusion , Coombs Test , Immunoglobulin G , Phenotype , Reticulocyte Count , Sensitivity and SpecificityABSTRACT
Autoimmune hemolytic anemia(AIHA) is characterized by the production of Coombs antibodies, which are responsible for the destruction of RBCs. The antibody specificity of warm A1HA(WAIHA) is very complex while all cells tested are usually reactive in routine test. Although some autoantibodies have broad specificity to the Rh system, apparent specificity for simple Rh antigen(D, C, E, c, e) is rare. A 63 year-old farmer was admitted at Inha University Hospital for evaluation of anemia. He had no history of blood transfusion. He presented overt anemia with decreased Hb (6.6g/dL) and increased reticulocyte count(18.5%, corrected reticulocyte count 8.4%). The direct antiglobulin test(DAT) was strongly positive for IgG and negative for C3d while indirect antiglobulin test(IAT) was weakly positive. Both two different antibody identification test kits identified this antibody as anti-e. His Rh phenotype was CDe. Although the eluted antibody from his own RBCs failed to show specificity against e but agglutinated all the reagent RBCs, we diagnosed this case as WAIHA induced by IgG anti-Rh(e) because his serum agglutinated all e-positive RBCs while it was non-reactive with e-negative RBC in the cross- matching test. Drugs(loxoprofen, cimetidine) couldn' t be ruled out as causative agent. (Korean J Blood Transfusion 10(1): 61-67, 1999)
Subject(s)
Humans , Middle Aged , Anemia , Anemia, Hemolytic, Autoimmune , Antibodies , Antibody Specificity , Autoantibodies , Blood Transfusion , Immunoglobulin G , Phenotype , Reticulocyte Count , Reticulocytes , Sensitivity and SpecificityABSTRACT
Hashimotos thyroiditis has been associated with a various autoimmune disorders. The immunologic mechanisms involved in the pathogenesis of these disorders have not always been thought to be the same. Although it was demonstrated that there were high prevalence of abnormal thyroid function and autoantibody in autoimmune hemolytic anemia(AIHA) and Fisher-Evans syndrome(FES), AIHA combined with Hashimotos thyroiditis is rare in Korean literature. It was suggested that a common immunologic mechanism may be involved in the pathogenesis of both disease and the possibility of multiple autoimmune syndrome might present in autoimmune hematologic disorders. We experienced a 74-year old woman with a 12-year history of a hypothyroidism due to Hashimotos thyroiditis was hospitalized with sudden development of warm AIHA with positive Direct & Indirect Coombs test and pericardial effusion. Her thyroid function test showed subclinical hypothyroidism with the maintenance dosage of levothyroxine(100pg/day). With glucocorticoid and plasmapheresis, AIHA and pericardial effusion were corrected successfully. It is suggested that the prudent immunologic study is needed for the anemia developed in patients with Hashimotos thyroiditis with or without hypothyroidism.