Case Of Autoimmune Hemolytic Anemia Research Articles

Mixed autoimmune hemolytic anemia as the initial presentation of systemic lupus erythematosus: A case report and review.

Autoimmune hemolytic anemia (AIHA) is an acquired condition caused by autoantibody mediated destruction of erythrocytes. AIHA is classified as warm or cold depending on whether the autoantibodies involved react optimally at or below body temperature (37°C), respectively. Mixed AIHA, with features of both, is rare and clinically more severe. We report a case of mixed AIHA that was found to be the presentation of systemic lupus erythematosus (SLE). Treatment with rituximab and prednisone resulted in good response. Although more commonly associated with warm AIHA, SLE can present with mixed AIHA.

Mycophenolate Mofetil for the Treatment of Warm Autoimmune Haemolytic Anaemia Post-Rituximab Therapy: A Case Series.

Warm autoimmune haemolytic anaemia (wAIHA) is an acquired haemolytic disorder most commonly treated with a combination of corticosteroids, rituximab and/or splenectomy. Third-line therapies for refractory cases include immunosuppressive agents. Mycophenolate mofetil is frequently used in these scenarios, although its use is supported by small studies and anecdotal evidence rather than large-scale data. We describe three cases of refractory warm autoimmune haemolytic anaemia successfully treated with mycophenolate mofetil. Case 1: A persistent case of autoimmune haemolytic anaemia in a 56-year-old was ultimately managed with mycophenolate mofetil, leading to successful steroid tapering and stable haemoglobin levels without relapse. Case 2: A woman with a complex oncological history, including lymphoma and breast cancer, achieved remission with mycophenolate therapy, maintaining stability post-steroid treatment. Case 3: Mycophenolate proved effective for a 63-year-old with cirrhosis after recurrent autoimmune anaemia and deep vein thrombosis, enabling cessation of steroids and maintaining remission. Management of this condition can be challenging and balancing the available treatments is crucial to reduce potential complications from long-term therapies that appear to be ineffective. Our case series demonstrates anecdotal experience on successful use of mycophenolate mofetil for complex refractory cases of wAIHA. Warm autoimmune haemolytic anaemia can be a challenging condition to manage. Refractory cases that are steroid-dependent can benefit from trialling steroid-sparing agents such as mycophenolate.Anti-CD20 agents such as rituximab can be very effective in refractory cases, however there is a small percentage of patients that might not be responsive to this monoclonal antibody.Autoimmune haemolytic anaemias can be frequently complicated by thrombotic events, and part of the backbone treatment is establishing good thromboprophylaxis.

Mixed autoimmune hemolytic anemia during recovery from Immune Thrombocytopenia (ITP): A Case Report

Mixed autoimmune hemolytic anemia (AIHA) is characterized by hemolysis with the coexistence of warm and cold autoantibodies. It is a relatively rare condition, accounting for 6.5-8.3% of AIHA cases. It is diagnosed by identifying IgG and C3d with cold agglutinins on monospecific direct antiglobulin testing. We present a unique case of a 69-year-old female who developed mixed hemolytic anemia while she was being treated for chronic idiopathic thrombocytopenic purpura (ITP).

A case of autoimmune haemolytic anaemia associated with mediastinal teratoma

A case of autoimmune haemolytic anaemia associated with mediastinal teratoma

A national survey of current immunohematologic testing practices for the diagnosis of autoimmune hemolytic anemia in India.

Autoimmune hemolytic anemia (AIHA) is a common term for several disorders that differ from one another in terms of etiology, pathogenesis, clinical features, and treatment. Management of patients with AIHA has become increasingly evidence-based in recent years. While this development has resulted in therapeutic improvements, it also carries increased requirements for optimal diagnosis using more advanced laboratory tests. Unfortunately, limited data are available from developing countries regarding the testing and transfusion management of patients with AIHA. The main objective of this survey was to explore the current immunohematologic testing practices for the diagnosis of AIHA in India. This online survey consisted of 30 questions, covering the place of work, the number of AIHA cases encountered in the 3 preceding years, testing method(s), transfusion management, and so forth. Individuals representing 89 laboratories completed the survey; only 78 of which responded that AIHA testing was performed in their facility's laboratory. The majority of respondents agreed that the most commonly affected age-group comprised individuals of older than 20 years, with a female preponderance. Regarding transfusion management, respondents indicated that transfusion with "best-match" red blood cell units remains the most common practice. Column-agglutination technology is used by 92 percent of respondents as the primary testing method. Although a monospecific direct antiglobulin test is available at 73 percent of the sites, most of them have limited access to other resources that could diagnose cold or mixed AIHA. Merely 49 percent of responding laboratories have the resources to perform adsorption studies for the detection of alloantibodies. Furthermore, three-cell antibody screening reagents are unavailable at 32 percent of laboratories. In 72 percent of centers, clinical hematologists would prefer to consult a transfusion medicine specialist before administering treatment to AIHA patients. There is unanimous agreement regarding the need for a national registry. The survey data indicate wide variability in testing practices for patients with AIHA in India. Future studies are needed to focus on the feasibility and cost-effectiveness of different testing strategies for developing countries.

Autoimmune Hemolytic Anemia Associated with Mature Ovarian Cystic Teratoma Containing Monoclonal Immunoglobulin G: A Case Report and Review of Literature

Background. Autoimmune hemolytic anemia (AIHA) associated with solid tumors such as mature cystic teratomas is rare and poorly understood. Here, we report a successfully treated case of secondary AIHA in a mature cystic teratoma containing antibodies against red blood cells. Case description. A 22-year-old woman was referred to our hospital with progressive anemia. Laboratory findings revealed hemolysis with a positive direct and indirect antiglobulin test. Imaging studies identified a left ovarian mass, suspected to be a mature cystic teratoma, which was later confirmed by histopathology after laparoscopic oophorocystectomy. The patient was treated with prednisolone, resulting in improved anemia. To examine the relationship between the tumor and AIHA, an indirect antiglobulin test was performed on the tumor contents. Stronger aggregations were observed at any concentration diluted by 10 times from 10 to 10,000 times of the tumor contents compared to the patient’s serum. Additionally, immunofixation electrophoresis of the tumor contents revealed the presence of monoclonal immunoglobulin G-κ. Conclusion. The presence of monoclonal IgG-κ in the tumor suggests intratumoral antibody production as a possible mechanism. Further research is necessary to elucidate the pathogenic relationship between such tumors and AIHA. The report also highlights the importance of considering secondary AIHA in patients with unexplained anemia and solid tumors.

Challenges in the diagnosis and management of autoimmune hemolytic anemia: A case-based approach. Experience from a tertiary care hospital in the Haryana region.

Autoimmune hemolytic anemia (AIHA) is a rare immune disorder which occurs when antibodies are directed against self red blood cells (RBCs) leading to hemolysis. AIHA is widely classified as warm autoimmune hemolytic anemia, cold agglutinin syndrome, mixed AIHA, paroxysmal cold hemoglobinuria and rarely drug induced AIHA. The pathogenesis of AIHA is complex interplay between genetic predisposition, immune dysregulation and enviornmental triggers. A direct antiglobulin test can be used to assess the immunological origin of the hemolysis in order to diagnose AIHA after identifying laboratory and clinical symptoms of hemolysis. The objective is to understand underlying mechanism in AIHAs, and usage of targeted therapies to modulate specific components of the immune response. We are hereby presenting a case series of 11 clinically suspected cases of AIHA in collaboration with their clinical features, immuno-hematological and other laboratory parameters, Flow cytometric analysis of lymphocyte subset in relevant cases, underlying etiology as well as serological subtype are also included. Majority of the patients were categorized as secondary AIHA (7/11, 63.63%). Out of 11 cases 7 were serologically subtyped as warm AIHA (7/11, 63.63%) ,2 cases were DaaT negative AIHA (2/11;18.18%), 2 cases were characterized as mixed AIHA subtype (2/11, 18.18%). Accurate subtyping of AIHA requires a systematic immunohematological approach coupled with comprehensive evaluations of clinical, hematological, and biochemical parameters.

Laparoscopic excision of accessory spleen for recurrent autoimmune hemolytic anemia after splenectomy: a case report

BackgroundSplenectomy is indicated in cases of autoimmune hemolytic anemia (AIHA), which are refractory to medical management. In post-splenectomy, there exists a theoretical risk of AIHA recurrence, especially if an accessory spleen undergoes compensatory hypertrophy. In this context, we present a unique case of recurrent AIHA managed through laparoscopic excision of the accessory spleen (LEAS).Case presentationA 60-year-old male underwent laparoscopic splenectomy (LS) for AIHA refractory to standard medical therapies. Following the surgery, there was a marked improvement in hemolytic anemia symptoms, and oral steroid therapy was terminated 7 months post-LS. Nonetheless, a year after the LS, the patient exhibited a marked decline in hemoglobin levels, dropping to a concerning 5.8 g/dl, necessitating the reintroduction of oral steroids. A subsequent contrast-enhanced computed tomography (CT) scan unveiled an enlarged accessory spleen. The patient then underwent LEAS, during which the accessory spleen, obscured within adipose tissue, proved challenging to visualize laparoscopically. This obstacle was surmounted utilizing intraoperative ultrasonography (US), enabling successful excision of the accessory spleen. The post-surgical period progressed without complications, and the steroid dosage was reduced to one-twelfth of its initial preoperative quantity.ConclusionsRecurrent AIHA can be instigated by post-splenectomy compensatory hypertrophy of the accessory spleen. Ensuring comprehensive splenic tissue excision is crucial in AIHA management to obviate recurrent stemming from hypertrophic remnants. In scenarios of AIHA recurrence tied to an enlarged accessory spleen, LEAS stands as a viable and effective therapeutic modality.

Mefenamic acid triggered autoimmune hemolytic anemia in a child: a rare case report

Autoimmune hemolytic anemia (AIHA) is characterized by the abnormal production of antibodies that bind to antigens on the surface of red blood cells (RBC), resulting in RBC destruction. Here we describe a rare case of mefenamic acid-induced AIHA in a 3-year-old boy who developed acute hemolysis after ingestion of mefenamic acid for febrile illness. The aim is to draw the attention of pediatricians to one of the side effects of mefenamic acid, the drug which is very commonly used as an antipyretic.

Immunohematological evaluation of red cell alloimmunization and statistical assessment of various adsorption techniques in warm autoimmune hemolytic anemia

Adsorption techniques are widely applied to detect underlying masked alloantibodies in warm autoimmune hemolytic anemia (WAIHA). We established various adsorption techniques with an aim to detect alloimmunization in WAIHA This study conducted over a period of nine years included 298 patients of WAIHA. Complete immunohematological evaluation was performed on these 298 samples following departmental protocols. Clinical and laboratory details of patients were obtained from patient files. Various adsorption methods were performed and statistically evaluated in the study. Out of 479 cases of autoimmune hemolytic anemia, WAIHA comprised of 62.2 % (N = 298). A total of 139 (46.6 %) serum samples revealed autoantibodies. Adsorption study was performed in 101 (72.7 %) indicated samples and 24 (23.8 %) of these showed 26 alloantibodies. Among the patients subjected to adsorption study hemolytic marker were significantly deranged in the alloimmunization group (p < 0.01). Polyethylene glycol (PEG) adsorption was the quickest (52.2–54.6 min) of all adsorption techniques with minimum (1.3–1.5) numbers of adsorptions needing for complete removal of serum antibodies. The LISS-papain (LP) technique was found to be more sensitive and specific compared to the other two techniques. The agreement between PEG adsorption and LP adsorption was found to be ‘perfect’ (96.4 %) with a Cohen’s kappa (k) value of 0.9. We conclude that identification of alloantibody specificities underlying a warm autoantibody is critical for a safe and effective transfusion. All WAIHA patients with history of blood transfusion, pregnancy or both should be subjected to adsorption study. Selection of a suitable adsorption technique depends on multiple important factors.

Utility of Dithiothreitol in a Case of Spontaneous Autoagglutination Due to Mixed Autoimmune Hemolytic Anemia in a Child – A Rare Scenario

Mixed autoimmune hemolytic anemia (AIHA) represents &lt;5% of pediatric AIHA cases. Spontaneous agglutination is known in patients with cold agglutinins reacting at room temperature. They often interfere with serological tests; generally, they can be dispersed by simple warming techniques. Rarely, in severe cases, as seen in the index case, dithiothreitol treatment of the red cells is needed to eliminate autoagglutination.

A Rare Case of Autoimmune Hemolytic Anemia after Implantation of a Drug Eluting Stent to Coronary Artery

A Rare Case of Autoimmune Hemolytic Anemia after Implantation of a Drug Eluting Stent to Coronary Artery

The choice of new treatments in autoimmune hemolytic anemia: how to pick from the basket?

Autoimmune hemolytic anemia (AIHA) is defined by increased erythrocyte turnover mediated by autoimmune mechanisms. While corticosteroids remain first-line therapy in most cases of warm-antibody AIHA, cold agglutinin disease is treated by targeting the underlying clonal B-cell proliferation or the classical complement activation pathway. Several new established or investigational drugs and treatment regimens have appeared during the last 1-2 decades, resulting in an improvement of therapy options but also raising challenges on how to select the best treatment in individual patients. In severe warm-antibody AIHA, there is evidence for the upfront addition of rituximab to prednisolone in the first line. Novel agents targeting B-cells, extravascular hemolysis, or removing IgG will offer further options in the acute and relapsed/refractory settings. In cold agglutinin disease, the development of complement inhibitors and B-cell targeting agents makes it possible to individualize therapy, based on the disease profile and patient characteristics. For most AIHAs, the optimal treatment remains to be found, and there is still a need for more evidence-based therapies. Therefore, prospective clinical trials should be encouraged.

Mixed Autoimmune Hemolytic Anemia in a 2-Year-Old Girl

Immune hemolytic anemia is diagnosed when it exhibits the clinical symptoms and laboratory findings of hemolytic anemia, such as pallor, jaundice, anemia, high indirect bilirubin, increased reticulocyte count, and a positive direct antiglobulin test. Depending on the type of antigenic stimulation, this condition can be divided into three distinct subtypes: (1) autoimmune hemolytic anemia (AIHA), (2) alloimmune hemolysis, and (3) drug-induced hemolysis. In addition, the thermal amplitude of autoantibodies is used to categorize AIHA as either a warm (most common), cold, or mixed (rare) subtype. Mixed autoimmune hemolytic anemia is diagnosed when both warm and cold autoantibodies are present. Here, we report a case of a mixed AIHA in a 2-year-old girl who responded well to corticosteroids. Due to the rarity of the condition and the lack of rigorous diagnostic criteria, it is crucial to report this case.

Acute kidney injury following autoimmune hemolytic anemia due to simultaneous use of ciprofloxacin and hydrochlorothiazide: A case report and review of the literature

AbstractIntroductionRare cases of autoimmune hemolytic anemia (AHA) associated with using ciprofloxacin or hydrochlorothiazide (HCTZ) alone have been reported before. However, simultaneous use of both drugs could lead to a severe clinical condition. This study presents the combination of acute kidney injury (AKI) and AHA following the simultaneous use of ciprofloxacin and HCTZ for three days.Case DescriptionA 42‐year‐old Iranian woman presented to the emergency department with symptoms of fatigue, lethargy, nausea and vomiting, ataxia, oliguria, dark urine, and jaundice. The patient reported using HCTZ due to high blood pressure and ciprofloxacin for a urinary tract infection three days before presentation. Early laboratory findings revealed hemolytic anemia with a hemoglobin of 7 g/dl, the strongly positive direct and indirect Coombs test, high level of lactate dehydrogenase (820 IU/L), and hyperbilirubinemia (total: 3 mg/dL and direct: 1.2 mg/dL). Furthermore, hyperkalemia (5.2 mEq/L), hyperphosphatemia (6.2 mg/dL), high levels of BUN (100 mg/dL), and creatinine rise (6.8 mg/dL) were found. Urine analysis showed 2+ blood, 4‐6 red blood cells, and cola‐colored urine. Based on the findings, drug‐induced AHA, followed by AKI, was diagnosed. Following, the drugs were stopped and steroid therapy was initiated. The patient underwent four sessions of hemodialysis to improve the AKI.ConclusionHealthcare providers should be aware of the life‐threatening adverse effects of commonly used drugs such as ciprofloxacin or HCTZ. The timely diagnosis of the offending drugs leads to avoiding the persistence of the risk factor and the deterioration of the patient's clinical condition.

Real-world data from India on clinical practices in the management of autoimmune haemolytic anaemia: A survey-based cross-sectional assessment.

Autoimmune haemolytic anaemia (AIHA) is a decompensated acquired haemolysis caused by the host's immune system acting against its own red cell antigens. The aim of this national survey is to capture real-world data of clinical practices in AIHA by collecting responses from clinical haematologists across India. In this cross-sectional study, a structured, 26-question online survey was conducted in India by few members of the special interest group in immunohaematology between January and March, 2022. The final survey consisted of questions covering place of work, amount of AIHA cases being evaluated by the haematologist over preceding years, basic demographic, clinical and laboratory features of the patients being treated under them etc. Descriptive statistical analysis was performed during the assessment. The survey response rate was 48.2% (53/110), 69.8% (37/53) have diagnosed and managed more than 10 AIHA cases in the last 3years with a female preponderance. There was considerable variability in response. While 56.6% (30/53) of respondents do have the access to the facilities to subtype AIHA cases; 32.1% (17/53) of clinicians would prefer administering high dose steroids for 6weeks or more in non-responding patients, and only 45.3% (24/53) would assess the risks of thrombosis in AIHA. There is unanimous agreement among the participants that health-related quality of life should be taken into consideration in patients and the need for a national registry of patients with AIHA in India. The current national survey showed that some aspects of AIHA management were consistent; others were less so, but also significant variations were observed in certain clinical practices, where the evidence base is limited. A joint effort is needed to establish a national patient registry by including both clinical haematologists and transfusion medicine specialists which could potentially standardise AIHA management and future research in India.

A case of Evans syndrome

Evans syndrome is an autoimmune disorder described by Robert Evans in 1951 specifying that a link exists between primary thrombocytopenic purpura and acquired haemolytic anaemia. It is a rare autoimmune disorder characterised by the simultaneous or sequential development of autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Approximately, Evans syndrome represent 5-10% of warm autoimmune haemolytic anaemia cases and 2-5% of ITP cases. It is basically a diagnosis of exclusion. We present a 50-year-old female presented with anaemia, jaundice and petechia. Patient indirect bilirubin was raised, Coombs test was positive, lactate dehydrogenase and reticulocyte count were raised. Based on the Coombs positive haemolytic anaemia and thrombocytopenia patient was diagnosed with Evans syndrome. ANA profile was positive for SS-A suggestive of underlying autoimmune disorder. Evans syndrome is difficult to diagnose and treat. Typically, corticosteroids and other immunosuppressive medications are used to manage the syndrome.

Autoimmune haemolytic anaemia and immune thrombocytopenia following SARS-CoV-2 and non-SARS-CoV-2 vaccination: 32 Years of passive surveillance data.

Autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) are two uncommon haematologic autoimmune conditions that can rarely arise secondary to vaccination. Prior studies using the US Centers for Disease Control's (CDC) Vaccine Adverse Event Reporting System (VAERS) have demonstrated this infrequency, but contemporary data as well as comparison with current information regarding SARS-CoV-2 vaccination has not been assessed. In this study, we reviewed VAERS database reports from 1990 to 2022 to characterize the incidence and clinical and laboratory findings of non-SARS-CoV-2-associated AIHA and ITP and SARS-CoV-2 vaccine-associated AIHA and ITP. We discovered a total of 863 AIHA and ITP reports following vaccination with 15 non-SARS-CoV-2 and four SARS-CoV-2 vaccines submitted to the CDC VAERS database. AIHA and ITP reporting was low for both groups, with a large proportion excluded due to a lack of clinical details. ITP was reported the most frequently in both groups and was significantly more common with measles-mumps-rubella (MMR) vaccination (p < 0.001) in the non-SARS-CoV-2 group. AIHA and ITP cases were higher in the SARS-CoV-2 vaccine group, though ultimately still very infrequent. Autoimmune haematologic disease is vanishingly rare after immunization and rates are lower than in the general population according to passive reporting.

Splenectomy as an effective treatment for macrothrombocytopenia in Takenouchi-Kosaki syndrome.

Takenouchi-Kosaki syndrome (TKS) is a rare congenital disease caused by a de novo heterozygous mutation in the CDC42 gene. Its characteristic clinical features are macrothrombocytopenia, developmental delay, dysmorphic facial features, and deafness. Splenectomy has been contraindicated for inherited thrombocytopenia, and there is little information on treatment of macrothrombocytopenia in TKS. In a previously reported case of autoimmune hemolytic anemia (AIHA) with TKS, we observed that AIHA initially resolved with prednisolone, but gradually became refractory to drug therapy. After splenectomy, both anemia and macrothrombocytopenia improved. This is a novel positive effect of splenectomy for thrombocytopenia in TKS, although further studies are required to assess the effectiveness and safety of splenectomy.

Autoimmune Hematological Toxicities of Immune Checkpoint Inhibitors: A Real-World Pharmacovigilance Database Analysis

BACKGROUND: Autoimmune hematological toxicities are rarely reported from the use of checkpoint inhibitors. However, there have been several cases of autoimmune hemolytic anemia, immune thrombocytopenia, hemophagocytic lymphohistiocytosis (HLH), and immune mediated cytopenias that have been reported in patients who were treated with checkpoint inhibitors. We performed a retrospective analysis of data of adverse effects of drugs that has been made available to public by the FDA. METHODS: The FDA has made data on adverse effects of various treatments available to the public through the FDA Adverse Events Reports System (FAERS) public dashboard. We investigated the autoimmune hematological toxicities of various checkpoint inhibitor therapies available at FAERS for the years 2020-2022. RESULTS: We reviewed the reported side effects of PD-1 inhibitors (pembrolizumab, nivolumab, cemiplimab, dostarlimab), PDL-1 inhibitors (atezolizumab, avelumab, durvalumab), and CTLA-4 inhibitors (ipilimumab) from FAERS. A total of 5,195 hematological toxicities from immunotherapies were reported from 2020-2022. Out of those, 483 (9.3%) were autoimmune hematological toxicities. The most common autoimmune complications were autoimmune hemolytic anemia (21.9%), immune thrombocytopenia (36%), autoimmune neutropenia (4.1%), HLH (36.2%) and thrombotic/idiopathic thrombocytopenic purpura (ITP/TTP) (1.8%). Nivolumab had the highest incidence of autoimmune hematological events out of all immune checkpoint inhibitors. Since cemiplimab and dostaralimab are relatively newly being utilized for treatment, there has not been much reported data. CONCLUSIONS: Out of the reported cases of hematological adverse reaction to check point inhibitor, 9.3% were autoimmune toxicities. There was no statistical difference in rate of autoimmune toxicities caused by PD1, PDL1 or CTLA 4 inhibitors. As utilization of newer immune checkpoint inhibitors increase, we would need to re-evaluate incidence of autoimmune hematological adverse reactions. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal