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Blood 2021 Mar;137(10):1432

Shaare Zedek Medical Center.

Indian Pediatr 2021 Feb 25. Epub 2021 Feb 25.

Department of Pediatric Hematology Oncology and BMT, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi  Correspondence to: Dr Anupam Sachdeva, Director Pediatric Hematology Oncology and BMT, Institute for Child Health, Sir Ganga Ram Hospital, New Delhi, India.

Objective: To analyze clinical, laboratory parameters and treatment outcomes of children with autoimmune hemolytic anemia(AIHA).

Methods: Retrospective analysis of 50 children aged 0-18 years. Monospecific direct antiglobulin test (DAT) and investigations for secondary causes were performed. Read More

J Clin Med 2021 Jan 9;10(2). Epub 2021 Jan 9.

Division of Hematology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.

Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient's exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Read More

Cureus 2020 Nov 22;12(11):e11622. Epub 2020 Nov 22.

Internal Medicine, Government Medical College Kottayam, Kottayam, IND.

Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia (AIHA). PCH occurs in acute and chronic forms. The main risk factors for PCH include viral infections, vaccination, and syphilis. Read More

Immun Ageing 2020 Nov 20;17(1):38. Epub 2020 Nov 20.

Department of Hematology and Bone Marrow Transplantation, Poznan University of Medical Sciences, Poznań, Poland.

Autoimmune hemolytic anemia (AIHA) is an acquired, heterogeneous group of diseases which includes warm AIHA, cold agglutinin disease (CAD), mixed AIHA, paroxysmal cold hemoglobinuria and atypical AIHA. Currently CAD is defined as a chronic, clonal lymphoproliferative disorder, while the presence of cold agglutinins underlying other diseases is known as cold agglutinin syndrome. AIHA is mediated by autoantibodies directed against red blood cells (RBCs) causing premature erythrocyte destruction. Read More

Indian J Hematol Blood Transfus 2020 Nov 22:1-6. Epub 2020 Nov 22.

Department of Obstetrics & Gynaecology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr Ram Manohar Lohia Hospital Delhi, New Delhi, India.

Paroxysmal cold haemoglobinuria (PCH) is an acquired cause of haemolytic anaemia. It is caused by the biphasic IgG antibodies that sensitize and attach the red cells at lower temperature; detach and rebind on rewarming. Donath-Landsteiner antibody test is the diagnostic test for PCH. Read More

Asian J Transfus Sci 2020 Jan-Jun;14(1):57-59. Epub 2020 Jul 24.

Department of Transfusion Medicine, Medanta-The Medicity, Gurgaon, Haryana, India.

61-year old male patient was admitted to the hospital with clinical picture of hemolytic anemia with hemoglobinuria. Patient was suspected to have Infectious Mononucleosis (IM) with Auto Immune Hemolytic Anemia (AIHA). DAT was positive with anti-C3d specificity. Read More

Front Immunol 2020 4;11:1006. Epub 2020 Jun 4.

UO Ematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Autoimmune hemolytic anemia (AIHA) is characterized by immune mediated erythrocytes destruction by autoantibodies with or without complement activation. Additional pathologic mechanisms include cellular cytotoxicity, cytokline dysregulation, and inadequate bone marrow compensation with fibrosis/dyserythropoiesis. The latter resembles that of bone marrow failures, namely aplastic anemia and myelodysplastic syndromes. Read More

Clin Epidemiol 2020 22;12:497-508. Epub 2020 May 22.

Department of Clinical Research, University of Southern Denmark, Odense, Denmark.

Background: Acquired hemolytic disorders-autoimmune hemolytic anemia (AIHA), cold agglutinin disease (CAD), paroxysmal nocturnal hemoglobinuria (PNH), drug-induced hemolysis (DIHA), and acquired hemolysis not otherwise specified (AHNOS)-are considered rare. Despite their potentially major health implications, data regarding their incidence and prevalence are scarce.

Methods: To fill this gap we collected data regarding all patients with acquired hemolytic disorder diagnoses in 1977-2016 from the Danish National Patient Register. Read More

J Clin Invest 2020 05;130(5):2152-2163

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

The renaissance of complement diagnostics and therapeutics has introduced precision medicine into a widened field of complement-mediated diseases. In particular, complement-mediated diseases (or complementopathies) with ongoing or published clinical trials of complement inhibitors include paroxysmal nocturnal hemoglobinuria, cold agglutinin disease, hemolytic uremic syndrome, nephropathies, HELLP syndrome, transplant-associated thrombotic microangiopathy, antiphospholipid antibody syndrome, myasthenia gravis, and neuromyelitis optica. Recognizing that this field is rapidly expanding, we aim to provide a state-of-the-art review of (a) current understanding of complement biology for the clinician, (b) novel insights into complement with potential applicability to clinical practice, (c) complement in disease across various disciplines (hematology, nephrology, obstetrics, transplantation, rheumatology, and neurology), and (d) the potential future of precision medicine. Read More

Transfus Clin Biol 2020 Apr 18;27(2):83-86. Epub 2020 Mar 18.

Department of pretransfusion testing and hemovigilance, Institute for Blood Transfusion of Serbia, Svetog Save 39, 11000 Belgrade, Serbia. Electronic address:

Paroxysmal cold hemoglobinuria is a rare form of autoimmune hemolytic anemia caused by the Donath-Landsteiner autoantibody. The condition is characterized by the presence of an IgG biphasic hemolysin with specificity to the P blood group antigen. The antibody biphasic action may be demonstrated in the Donath-Landsteiner test. Read More

Blood 2020 01;135(5):393

Johns Hopkins University.

Blood Adv 2019 11;3(22):3575-3578

Department of Pediatrics, University of California, San Francisco, San Francisco, CA; and.

Indian Pediatr 2019 10;56(10):879-880

Department of Transfusion Medicine, Max Superspeciality Hospital, Saket, New Delhi, India.

Paroxysmal Cold Hemoglobinuria is a rare cause of intravascular hemolysis presenting in children following an acute viral illness. It is usually self-limiting in nature. We present the details of a 4-year-old boy who presented with rapid onset intravascular hemolysis. Read More

Ther Adv Hematol 2019 9;10:2040620719873321. Epub 2019 Sep 9.

Department of Medicine, Henri Mondor Hospital, Université Paris-Est, Assistance Publique Hôpitaux de Paris Creteil, Creteil, France.

Complement-mediated hemolytic anemias can either be caused by deficiencies in regulatory complement components or by autoimmune pathogenesis that triggers inappropriate complement activation. In paroxysmal nocturnal hemoglobinuria (PNH) hemolysis is entirely complement-driven. Hemolysis is also thought to be complement-dependent in cold agglutinin disease (CAD) and in paroxysmal cold hemoglobinuria (PCH), whereas warm antibody autoimmune hemolytic anemia (wAIHA) is a partially complement-mediated disorder, depending on the subtype of wAIHA and the extent of complement activation. Read More

Blood Adv 2019 06;3(12):1897-1906

Department of Research and Innovation, Haugesund Hospital, Haugesund, Norway.

The terminology applied to autoimmune hemolytic anemia (AIHA) seems inconsistent. We aimed to evaluate the consistency of definitions used for diagnosis and treatment. In this systematic review of literature from January 2006 to December 2015, we assessed heterogeneity in the definition of AIHA and its subtypes, refractory disease, disease phase, severity, criteria for treatment response, and response durability. Read More

Tidsskr Nor Laegeforen 2019 Mar 18;139(6). Epub 2019 Mar 18.

Immunohematology 2019 Jan;35(1):3-6

Medical Director of Donor Services, Associate Medical Director of Transfusion Medicine, Houston Methodist Hospital, Department of Pathology and Genomic Medicine, 6565 Fannin Street, MS 205, Houston, TX.

Conclusions: The Donath-Landsteiner (DL) test is a serologic test used to detect the presence of a biphasic hemolysin. This autoantibody is seen in patients with paroxysmal cold hemoglobinuria. The test relies on the characteristic cold binding of an IgG autoantibody with specificity to the P blood group antigen. Read More

Case Rep Hematol 2018 13;2018:7586719. Epub 2018 Nov 13.

Division of Hematology and Medical Oncology, Department of Medicine, Mount Sinai Beth Israel, Mount Sinai Hospital, New York, NY, USA.

Paroxysmal cold hemoglobinuria (PCH) is a rare form of cold autoimmune hemolytic anemia first discovered in the early 20th century in adults with tertiary syphilis. Today, it is more commonly seen in children as a life-threatening anemia during a viral upper respiratory tract infection (URI). Although respiratory syncytial virus (RSV) has previously been reported to cause PCH in a child, herein we present the first documented case in an adult. Read More

BMJ Case Rep 2018 Nov 8;2018. Epub 2018 Nov 8.

Medicine, Nassau University Medical Center, East Meadow, New York, USA.

Donath-Landsteiner haemolytic anaemia (DLHA), also known as paroxysmal cold haemoglobinuria, is a very rare and difficult condition to diagnose as well as treat. Here, we present a case of a 55-year-old Hispanic woman who presented with severe intravascular haemolytic anaemia in the setting of a viral illness 2 weeks prior to presentation. Direct antiglobulin testing revealed mixed results: positive for either complement, IgG or both on various occasions which led to a battery of tests including the Donath-Landsteiner antibody testing which turned out positive establishing the diagnosis of DLHA. Read More

Vnitr Lek Summer 2018;64(5):514-519

Autoimmune hemolytic anemia (AIHA) is caused by auto-antibodies directed against self red blood cell (RBC) surface antigens. A consequence may be an intravascular hemolysis mediated by activated complement or extravascular hemolysis caused by destruction of complex of RBC with autoantibody in spleen and liver. The basic classification subdivides AIHA in primary/idiopathic and secondary with known underlying disease. Read More

Semin Hematol 2018 07 10;55(3):141-149. Epub 2018 Apr 10.

Department of Research and Innovation, Haugesund Hospital, Helse Fonna HF, Haugesund, Norway. Electronic address:

The classical complement pathway and, to some extent, the terminal pathway, are involved in the immune pathogenesis of autoimmune hemolytic anemia (AIHA). In primary cold agglutinin disease (CAD), secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria, the hemolytic process is entirely complement dependent. Complement activation also plays an important pathogenetic role in some warm-antibody AIHAs, especially when immunoglobulin M is involved. Read More

Blood 2018 05;131(22):2506

Pathology Queensland.

Ugeskr Laeger 2018 Mar;180(10)

Epstein-Barr virus (EBV) is globally prevalent and in adolescents mostly observed as infectious mononucleosis. Abnormal liver blood tests are common, whereas more serious hepatitis is less prevalent. Autoimmune haemolytic anaemia may also occur in the course of this infection. Read More

Cent Eur J Immunol 2017 30;42(4):404-406. Epub 2017 Dec 30.

Department of Paediatrics and Nephrology, Medical University of Warsaw, Poland.

Paroxysmal cold haemoglobinuria (PCH) is a form of autoimmune haemolytic anaemia (AIHA) characterised by a sudden onset of haemoglobinuria, either spontaneously or following exposure to cold. In children, it is commonly seen following a viral illness or after immunisation. Diagnosis of PCH is confirmed by a positive Donath Landsteiner (DL) test in which biphasic haemolysins are detected. Read More

Am J Hematol 2018 05 30;93(5):718-721. Epub 2018 Jan 30.

Department of Haematology, Royal Infirmary of Edinburgh, United Kingdom.

J Investig Med High Impact Case Rep 2017 Oct-Dec;5(4):2324709617740905. Epub 2017 Nov 13.

East Tennessee State University, Johnson City, TN, USA.

Autoimmune hemolytic anemia (AIHA) is a disease process that involves the destruction of red blood cells mediated by the humoral immune system. It can be characterized as a cold agglutinin syndrome, paroxysmal cold hemoglobinuria, and warm, mixed type, and drug-induced AIHA. Although a well-established relationship exists between the presence of AIHA and lymphoproliferative malignancy, AIHA rarely presents in association with solid malignancies. Read More

Transfusion 2017 06;57(6):1332-1335

Department of Pathology, University of Michigan, Ann Arbor, MI.

Transfusion 2017 06 31;57(6):1401-1406. Epub 2017 Mar 31.

Division of Pediatric Hematology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Background: Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare. Read More

J Pediatr Hematol Oncol 2017 05;39(4):320-321

Department of Pediatrics, Musashino Red Cross Hospital, Musashino City, Tokyo, Japan.