- Evans syndrome as initial presentation of COVID-19 infection: A case report and review of the literature [Journal Article]Ann Biol Clin (Paris). 2023 01 31 [Online ahead of print]AB
- Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by the concomitant or sequential association of auto-immune hemolytic anemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. Coronavirus disease 2019 (Covid-19) may cause various hematologic conditions. COVID-19 may also induce Evans syndrome via immune mechanisms. Here, we describe t…
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- Clinical and Epidemiological Features of Paroxysmal Cold Hemoglobinuria: A Systematic Review. [Journal Article]Blood Adv. 2023 Jan 30 [Online ahead of print]BA
- Paroxysmal cold hemoglobinuria (PCH) is a rare autoimmune hemolytic anemia often overlooked as a potential etiology of hemolysis, and is challenging to diagnose due to the complicated testing methods required. We performed a systematic review of all reported cases to better assess the clinical, immunohematologic, and therapeutic characteristics of PCH. We systematically analyzed PubMed, Medline, …
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- Life-Threatening Severe Thrombocytopenia and Mild Autoimmune Hemolytic Anemia Associated with Brucellosis. [Case Reports]
- CONCLUSIONS: This is our region's first published case of severe thrombocytopenia and mild autoimmune hemolytic anemia associated with brucellosis. It contributes to the literature on the successful use of rifampicin and doxycycline to treat hematological disorders associated with brucellosis.
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- AUTOIMMUNE HEMOLYTIC ANEMIA DURING PREGNANCY AND PUERPERIUM: AN INTERNATIONAL MULTI-CENTER EXPERIENCE. [Journal Article]Blood. 2023 Jan 27 [Online ahead of print]Blood
- Relapsing or occurring de novo autoimmune hemolytic anemia (AIHA) during pregnancy or puerperium is a poorly described condition. Here we report 45 pregnancies occurring in 33 women evaluated at 12 centers from 1997 to 2022. Among the 20 women with AIHA diagnosis before pregnancy, 10 had a relapse. Additional 13 patients developed de novo AIHA during gestation/puerperium (two patients had AIHA re…
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- Barcellini W, Fattizzo B. How I treat warm autoimmune hemolytic anemia. Blood. 2021;137(10):1283-1294. [Journal Article]Blood. 2023 Jan 26; 141(4):438-439.Blood
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- Rise of the planet of rare anemias: An update on emerging treatment strategies. [Review]Front Med (Lausanne). 2022; 9:1097426.FM
- Therapeutic options for rare congenital (hemoglobinopathies, membrane and enzyme defects, congenital dyserythropoietic anemia) and acquired anemias [warm autoimmune hemolytic anemia (wAIHA), cold agglutinin disease CAD, paroxysmal nocturnal hemoglobinuria (PNH), and aplastic anemia (AA)] are rapidly expanding. The use of luspatercept, mitapivat and etavopivat in beta-thalassemia and pyruvate kina…
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- Direct antibody test negative autoimmune hemolytic anemia with pulmonary tuberculosis: A diagnostic challenge. [Case Reports]
- Tuberculosis (TB) has varied manifestations, but autoimmune hemolytic anemia (AIHA) due to TB is rare. Direct antibody test (DAT) or Coombs negative AIHA is also rare. We report a case of a 14-year-old boy who presented with hemolytic anemia and pneumonia. The Coombs test was repeatedly negative. After ruling out the possible infectious and noninfectious causes by extensive investigations, he was…
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- Application of flow cytometry in transfusion medicine: The Sanjay Gandhi Post Graduate Institute of Medical Sciences, India experience. [Review]
- The application of flow cytometry (FC) is diverse and this powerful tool in used in multiple disciplines such as molecular biology, immunology, cancer biology, virology, and infectious disease screening. FC analyzes a single cell or a particle very rapidly as they flow past single or multiple lasers while suspended in buffered solution. FC has a great impact in the field of transfusion medicine (…
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- Chemical- and Drug-Induced Allergic, Inflammatory, and Autoimmune Diseases Via Haptenation. [Review]
- Haptens are small molecules that only elicit an immune response when bound to proteins. Haptens initially bind to self-proteins and activate innate immune responses by complex mechanisms via inflammatory cytokines and damage-associated molecular patterns and the subsequent upregulation of costimulatory signals such as cluster of differentiation 86 (CD86) on dendritic cells. Subsequent interaction…
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- Pseudo-thrombotic microangiopathy due to folate deficiency. [Case Reports]BMJ Case Rep. 2023 Jan 20; 16(1)BC
- Classically, deficiencies of vitamin B12 and folate are associated with megaloblastic anaemia. Additionally, vitamin B12 is able to cause a haemolytic anaemia in the form of pseudo-thrombotic microangiopathy (pseudo-TMA). Here, we present a case of a middle-aged woman with a history of Roux-en-Y gastric bypass who presented with dyspnoea and fatigue and was found to have thrombocytopenia and a no…
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- Cold antibody autoimmune hemolytic anemia following varicella infection. [Case Reports]Indian J Pathol Microbiol. 2023 Jan-Mar; 66(1):188-190.IJ
- CONCLUSIONS: 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.
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- Neutrophilic erythrophagocytosis in myelodysplastic syndrome and cold agglutinin disease co-occurrence. [Case Reports]
- In this article, we report the rare phenomenon of erythrophagocytosis by neutrophils in peripheral blood smear of a 56-year-old male patient with concurrent myelodysplastic syndrome and cold agglutinin disease.
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- Therapeutic Options in Refractory Evans Syndrome: A Case Report. [Case Reports]
- Evans syndrome is a rare autoimmune disease, characterized by at least two immune cytopenias, most frequently anemia and thrombocytopenia and rarely immune neutropenia. It has a variable clinical presentation and is rarely diagnosed in adults. It can be idiopathic or secondary to lymphoproliferative disease, infections, autoimmune diseases, drugs, and immunodeficiencies in about 50% of cases. It …
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- TLR7 and IgM: Dangerous Partners in Autoimmunity. [Review]
- The B cell antigen receptor (BCR)-repertoire is capable of recognizing a nearly unlimited number of antigens. Inevitably, the random nature of antibody gene segment rearrangement, needed in order to provide mature B cells, will generate autoreactive specificities. Once tolerance mechanisms fail to block the activation and differentiation of autoreactive B cells, harmful autoantibodies may get sec…
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- Warm autoimmune hemolytic anemia with anti-Jka specificity following babesiosis masquerading as a delayed hemolytic transfusion reaction. [Case Reports]Transfusion. 2023 Jan 17 [Online ahead of print]T
- CONCLUSIONS: Healthcare providers should be cognizant of the potential for babesiosis-induced WAIHA, particularly in individuals who continue to hemolyze despite undetectable parasitemia. Furthermore, this case highlights the possibility for warm autoantibodies to demonstrate Kidd antigen specificity. Though Kidd antigen variants are rare, antigen genotyping may be beneficial, particularly in the context of recent RBC transfusions, which typically preclude accurate serological phenotypic assessment.
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- Successful Management of Refractory Autoimmune Hemolytic Anemia with Cold Agglutinin Disease with Splenectomy: A Case Report with Review of Literature. [Case Reports]Kobe J Med Sci. 2023 Jan 11; 68(1):E30-E34.KJ
- CONCLUSIONS: Splenectomy significantly improved the patient's refractory hemolytic anemia due to primary CAD. Thus, it may be an effective treatment option in such cases, although further cases and studies are required to evaluate the effects of splenectomy.
- The Role of Complement in Autoimmune Disease-Associated Thrombotic Microangiopathy and the Potential for Therapeutics. [Journal Article]J Rheumatol. 2023 Jan 15 [Online ahead of print]JR
- The complement system is a tightly regulated, cascading protein network representing a key component linking the innate and humoral immune systems. However, if misdirected or dysregulated, it can be similarly damaging to host-tissue. The role of complement dysregulation on vascular endothelial cells has been well established in atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopat…
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- Autoimmune myositis and autoimmune hemolytic anemia in two sisters with DOCK8-deficient hyper-IgE syndrome. [Journal Article]Immunol Res. 2023 Jan 12 [Online ahead of print]IR
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- Thymoma With Triple Threat: Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia, and T-Cell Large Granular Lymphocytic Leukemia. [Case Reports]
- Thymomas are a rare neoplasm of the anterior mediastinum and often associated with paraneoplastic syndromes. Though myasthenia gravis is the most common and well-known, the list of reported paraneoplastic syndromes occurring with thymoma is extensive and ever-growing. Paraneoplastic syndromes can involve nearly every organ system, including hematologic abnormalities affecting any or all cell line…
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- Autoimmune Hemolytic Anemia After Cord Blood Transplantation: A Retrospective Single-Center Experience. [Journal Article]
- CONCLUSIONS: AIHA is a clinically significant common complication in recipients post-CBT. Corticosteroids combined with intravenous immunoglobulin (IvIg) is recommended for the treatment of warm antibody AIHA after CBT.
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- GeneReviews®: Wilson Disease [BOOK]GeneReviews®. University of Washington, Seattle: Seattle (WA)BOOK
- Wilson disease is a disorder of copper metabolism that, when untreated, can present with hepatic, neurologic, or psychiatric disturbances – or a combination of these – in individuals ages three years to older than 70 years. Manifestations in untreated individuals vary among and within families. Liver disease can include recurrent jaundice, simple acute self-limited hepatitis-like illness, autoimm…
- Not as "D"eadly as once thought - the risk of D-alloimmunization and hemolytic disease of the fetus and newborn following RhD-positive transfusion in trauma. [Review]Hematology. 2023 Dec; 28(1):2161215.H
- The use of blood products to resuscitate injured and massively bleeding patients in the prehospital and early in-hospital phase of the resuscitation is increasing. Using group O red blood cells (RBC) and low titer group O whole blood (LTOWB) avoids an immediate hemolytic reaction from recipient's naturally occurring anti-A and - B, but choosing the RhD type for these products is more nuanced and …
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- Perioperative Management of a Patient With Severe Cold Agglutinin Disease Undergoing Total Hip Arthroplasty With a Cemented Stem: A Case Report. [Case Reports]
- Patients with cold agglutinin disease who undergo total hip arthroplasty (THA) are rarely encountered. Patients with cold agglutinin disease are very sensitive to cold ambient temperatures and require scrupulous perioperative body-temperature management. However, THA requires a cementing procedure that exposes patients to cold temperatures during surgery and may result in autoimmune hemolytic ane…
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- Refractory, fatal autoimmune hemolytic anemia due to ineffective thymic-derived T-cell reconstitution following allogeneic hematopoietic cell transplantation for hypomorphic RAG1 deficiency. [Letter]Pediatr Blood Cancer. 2022 Dec 30 [Online ahead of print]PB
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- Scleroderma Renal Crisis With Thrombotic Microangiopathy Treated With Eculizumab. [Case Reports]
- We herein report the unusual case of a 52-year-old female with systemic scleroderma who was admitted to the emergency department (ED) with renal dysfunction and hypertension. Following a decline in hemoglobin (Hb) and platelet (Plt) count, the diagnosis of scleroderma renal crisis (SRC) with associated microangiopathic hemolytic anemia was made. Renal replacement therapy using hemodialysis was re…
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- STEREOTYPED CASES IN UKRAINIAN COHORT OF CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS DEPENDING ON THE IONIZING RADIATION EXPOSURE. [Journal Article]Probl Radiac Med Radiobiol. 2022 Dec; 27:307-323.PR
- CONCLUSIONS: The revealed differences in distribution of stereotyped CLL cases in Ukrainian cohort are most likely to reflect variations in the genetic background, environmental factors (including IR exposure), and their interactions in different geographic areas.
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- Use of steroids in the management of low-risk myelodysplastic syndromes with autoimmune features. [Journal Article]Blood Transfus. 2022 Dec 22 [Online ahead of print]BT
- CONCLUSIONS: This observation suggests a trial with a short course (2-3 weeks) of standard steroid doses to ascertain efficacy and properly silence the autoimmune pathogenic mechanism. Steroid-related adverse events (16% of cases) should be monitored carefully in this elderly, frail population. In conclusion, features of autoimmunity are present in more than two-thirds of low-risk MDS patients and a trial with prednisone 0.5-1 mg/kg day for 2-3 weeks, with proper monitoring of adverse events, may be useful to improve cytopenias in selected cases.
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- Iron metabolism abnormalities in autoimmune hemolytic anemia and Jianpishengxue keli can ameliorate hemolysis and improve iron metabolism in AIHA mouse models. [Journal Article]
- CONCLUSIONS: Iron metabolism abnormalities exists in AIHA patients and Jianpishengxue keli can ameliorate hemolysis and improve iron metabolism in AIHA mouse models.KEY MESSAGESIron metabolism abnormalities exists in hemolytic episode AIHA patients. Hepcidin and ferritin levels significantly elevated and also correlated with the severity of AIHA patients. Jianpishengxue keli can ameliorate hemolysis and prompt the recovery of AIHA.
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