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Cureus 2022 Apr 16;14(4):e24184. Epub 2022 Apr 16.

Internal Medicine, St. Joseph Mercy Oakland Hospital, Pontiac, USA.

Coombs-positive hemolytic anemia induced by cytomegalovirus (CMV) infection is a rare phenomenon, often not life-threatening in immunocompetent young adults. To date, the pathogenesis of CMV-induced severe hemolysis is still unknown. Here, we discuss a case of a 22-year-old male without significant past medical history who presented with severe hemolytic anemia that required four units of packed red blood cells. Read More

Transfusion 2022 May 19. Epub 2022 May 19.

University of Chicago, Department of Pathology, 5741 S Maryland Avenue, Chicago, IL.

Background: Hyperhemolysis syndrome (HHS) is a severe delayed hemolytic transfusion reaction seen in sickle cell disease (SCD) patients, characterized by destruction of donor and recipient RBCs. It results in a drop in hemoglobin to below pretransfusion levels and frequently reticulocytopenia.

Case Report: We report a case of a man in his 30s with SCD with a recent hospitalization two weeks prior for COVID-19. Read More

Ther Adv Hematol 2022 14;13:20406207221097780. Epub 2022 May 14.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan, Milan, Italy.

Background: Immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) show good responses to frontline steroids. About two-third of cases relapse and require second-line treatment, including rituximab, mainly effective in AIHA, and thrombopoietin-receptor agonists (TPO-RAs) in ITP, while the use of splenectomy progressively decreased due to concerns for infectious/thrombotic complications. For those failing second line, immunosuppressants may be considered. Read More

Clin Med (Lond) 2022 May;22(3):210-213

King's College Hospital, London, UK.

Haemolytic anaemia can seem like a complicated topic. The constellation of reticulocytosis, increased lactate dehydrogenase levels, increased unconjugated bilirubin levels and decreased haptoglobin levels should prompt general physicians to consider haemolysis as a differential diagnosis. When further approaching haemolytic anaemia, subdividing patients into those who are 'direct antiglobulin test (DAT) positive' (immune) or 'DAT negative' (non-immune) is a simple and clinically relevant way to start to formulate a cause for the haemolytic anaemia. Read More

Int J Lab Hematol 2022 May 15. Epub 2022 May 15.

Department of Clinical Pathology, School of Medicine, Kyungpook National University, Daegu, Republic of Korea.

The author report an interesting case of relapsed diffuse large B-cell lymphoma (DLBCL) with bone marrow (BM) and peripheral blood (PB) involvement after prior cold agglutinin disease (CAD). A minority of patients with DLBCL present with CAD, and BM or PB involvement with CAD are quite rare. Differential diagnosis of patients with CAD should include DLBCL, considering the possibility of BM or even PB involvement. Read More

Front Immunol 2022 25;13:851316. Epub 2022 Apr 25.

Department of Rheumatology and Immunology, The Sixth Affiliated Hospital of Guangzhou Medical University, Qingyuan People's Hospital, Guangdong, China.

For patients with autoimmune diseases, vaccination is controversial. The use of vaccination in patients with autoimmune diseases is controversial. There are many reports of secondary thrombotic thrombocytopenic purpura cases after various vaccinations. Read More

J Thromb Haemost 2022 May 12. Epub 2022 May 12.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico - Haematology Unit, Milan, Italy.

Background: Thrombosis may complicate autoimmune hemolytic anemia (AIHA), but its predictors are still lacking, and no clearcut indications for anticoagulant prophylaxis are available.

Objectives: To characterize frequency and severity of thromboses in AIHA patients and identify risk factors for thrombosis that may advise primary anticoagulant prophylaxis.

Patients/methods: 287 consecutive AIHA patients diagnosed and followed from 1978 at a tertiary Italian center were retrospectively studied; 174 of them were prospectively evaluated from January 2020 until December 2021. Read More

Sci Rep 2022 May 11;12(1):7743. Epub 2022 May 11.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza 35, 20100, Milan, Italy.

Data concerning the efficacy of SARS-CoV-2 vaccines in patients with non-oncological hematologic conditions are lacking. These include autoimmune cytopenias (autoimmune hemolytic anemia AIHA, immune thrombocytopenia ITP, and autoimmune neutropenia), and bone marrow failure syndromes (aplastic anemia, low risk myelodysplastic syndromes, and paroxysmal nocturnal hemoglobinuria). These conditions may relapse/reactivate after COVID-19 infection and vaccine. Read More

Hematology 2022 Dec;27(1):543-554

Division of Infectious Diseases, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, MO, USA.

Objectives: Describe the development of warm autoimmune hemolytic anemia warm (AIHA) secondary to a brown recluse spider () bite is known as systemic loxoscelism; and review epidemiology, clinical manifestations, diagnostic work-up, pathophysiology, and treatment options associated with warm AIHA secondary to systemic loxoscelism.

Methods: Cases series of two cases of warm AIHA due to systemic loxoscelism and a review of the current literature: epidemiology, clinical manifestations, diagnostic work-up, pathophysiology, and treatment options associated with warm AIHA secondary to systemic loxoscelism.

Results: Presented here are two cases of warm AIHA due to systemic loxoscelism. Read More

Blood Transfus 2022 Apr 19. Epub 2022 Apr 19.

Department of Microbiology, Immunology and Biopharmaceuticals, National Chiayi University, Chiayi City, Taiwan.

Rev Esp Anestesiol Reanim (Engl Ed) 2022 May 7. Epub 2022 May 7.

Anestesiología y Reanimación, Complejo Hospitalario Universitario de Albacete, Albacete, Spain.

Atypical uremic haemolytic syndrome is a variant of thrombotic micro-andiopathy characterized by non-autoimmune hemolytic anemia, thrombocytopenia and acute renal failure as a result of excessive activation of the complement. Up to 60% of patients have mutations in the genes that encode the complement system. A disensing factor is required for its manifestation, including gestation. Read More

Transfusion 2022 May 9. Epub 2022 May 9.

Department of Hematology, Odense University Hospital, Odense, Denmark.

J Family Med Prim Care 2022 Apr 18;11(4):1542-1544. Epub 2022 Mar 18.

Department of Medicine, Tata Main Hospital, Jamshedpur, Jharkhand, India.

Systemic lupus erythematosus (SLE) is an autoimmune disease associated with several autoantibodies targeted to nuclear and cytoplasmic antigens. Positivity of the serum antinuclear antibody (ANA), double stranded (ds) DNA, anti-smith (sm) antibody are essential for the diagnosis of SLE. Anti-histone antibody is usually present in drug-induced SLE. Read More

Invest New Drugs 2022 May 3. Epub 2022 May 3.

Department of Intensive Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Hankou, 430030, Wuhan, China.

In August 2021, penpulimab, an anti-programmed cell death 1 (PD-1) monoclonal antibody, was approved in China for the treatment of adult patients with relapsed or refractory classic Hodgkin's lymphoma who completed at least second-line chemotherapy. Penpulimab is currently in clinical trials in China and Australia for the treatment of nasopharyngeal cancer and non-small cell lung cancer. Several clinical studies have shown that penpulimab is safe and effective, and no immune-related adverse events (irAEs) above grade 3 were observed. Read More

J Vet Emerg Crit Care (San Antonio) 2022 May 2;32(3):289-314. Epub 2022 May 2.

School of Veterinary Medicine, Murdoch University, Murdoch, Australia.

Objectives: To expand the number of conditions and interventions explored for their associations with thrombosis in the veterinary literature and to provide the basis for prescribing recommendations.

Design: A population exposure comparison outcome format was used to represent patient, exposure, comparison, and outcome. Population Exposure Comparison Outcome questions were distributed to worksheet authors who performed comprehensive searches, summarized the evidence, and created guideline recommendations that were reviewed by domain chairs. Read More

Case Rep Hematol 2022 21;2022:6423143. Epub 2022 Apr 21.

HCA HealthONE, Lone Tree, CO, USA.

Herein, we present a unique case of a Coombs-negative, steroid-refractory autoimmune hemolytic anemia (AIHA) complicated by pseudoreticulopenia, describe its clinical presentation, histopathologic findings, and management, and review the salient literature. Coombs-negative, steroid-refractory AIHAs represent fewer than 1% of all AIHAs. Diagnosis of the disease is difficult and often delayed due to the pursuit of alternate diagnoses following a negative Coombs test. Read More

Rinsho Ketsueki 2022 ;63(4):254-259

Department of Hematology, Sasebo City General Hospital.

In about half of the cases, autoimmune hemolytic anemia (AIHA) is secondary to an underlying disease, often due to paraneoplastic syndromes. Recently, the number of patients developing metachronous multiple primary malignant tumors (MPMTs) has been increasing due to the aging of the population and the longer survival times of those with malignant tumors. A 78-year-old woman was diagnosed with sigmoid colon cancer in May 2017 and with warm AIHA in October 2017. Read More

BMJ Case Rep 2022 Apr 29;15(4). Epub 2022 Apr 29.

Intensive Medicine, Hôpital Louis-Mourier, Colombes, Île-de-France, France.

Impaired immune response with uncontrolled inflammation and various immunological disorders have been reported during SARS-CoV-2 infection. Here, we report a case of cold agglutinin disease occurring during a severe coronavirus disease 2019 (COVID-19) in a French intensive care unit. A patient was presented with acute respiratory distress syndrome, acute renal failure and haemolytic anaemia. Read More

Hong Kong Med J 2022 Apr 27. Epub 2022 Apr 27.

Department of Medicine, Hospital Sultanah Aminah, Johor Bahru, Johor, Malaysia.

Front Immunol 2022 6;13:846660. Epub 2022 Apr 6.

Paediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, ASST- Spedali Civili of Brescia, University of Brescia, Brescia, Italy.

Autoimmune diseases are usually associated with environmental triggers and genetic predisposition. However, a few number of autoimmune diseases has a monogenic cause, mostly in children. These diseases may be the expression, isolated or associated with other symptoms, of an underlying inborn error of immunity (IEI). Read More

J Formos Med Assoc 2022 Apr 19. Epub 2022 Apr 19.

Division of Nephrology, Department of Internal Medicine, Kaohsiung Veteran General Hospital, Kaohsiung, Taiwan. Electronic address:

Pseudo-pseudo Meigs' syndrome (PPMS) exhibits patients with ascites, pleural effusion, elevated CA-125, and diagnosed of systemic lupus erythematosus (SLE) eventually without evidence of ovarian or pelvic tumor. It's a rare diagnosis but it has a good response to treatment. We here present an 82-year-old female, who was found to have ascites, pleural effusion, and elevated CA-125. Read More

Ann Hematol 2022 Apr 21. Epub 2022 Apr 21.

Katholisches Klinikum Koblenz-Montabaur, Klinik Für Neurologie Und Stroke Unit, Kardinal-Krementz-Str. 1-5, 56073, Koblenz, Germany.

Cureus 2022 Mar 15;14(3):e23172. Epub 2022 Mar 15.

Internal Medicine, Stony Brook University Hospital, Brookhaven, USA.

Cold agglutinin disease (CAD) is a type of hemolytic anemia in which cold agglutinins can cause agglutination of red blood cells in cold parts of the body and hemolytic anemia. Cold agglutinin-mediated hemolytic anemia can occur in the setting of an underlying viral infection, autoimmune disorder, or lymphoid malignancy, referred to as a secondary cold agglutinin syndrome, or without one of these underlying disorders, referred to as primary CAD (also known as idiopathic CAD). We present a case of a 71-year-old female with hemolytic anemia due to primary CAD. Read More

J Assoc Physicians India 2022 Apr;70(4):11-12

Shyam Shah Medical College, Rewa.

The liver is the largest organ of the body weighing 1-1.5 kg, which is 1.5-2. Read More

Blood 2022 Apr 20. Epub 2022 Apr 20.

Hôpital Robert-Debré - APHP, Paris Cedex 19, France.

Splenectomy is effective in ~70-80% of pediatric chronic immune thrombocytopenia (cITP) and few data exist in autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES). Because of the irreversibility of the procedure and the lack of predictor of long-term outcomes, splenectomy decision is difficult to take in children. We report here factors associated with splenectomy outcomes from the OBS'CEREVANCE cohort, which prospectively includes French children with autoimmune cytopenia (AIC) since 2004. Read More

Cardiovasc Hematol Disord Drug Targets 2022 Apr 18. Epub 2022 Apr 18.

All India Institute of Medical Sciences, Jodhpur.

Background: Haematological manifestations in Hepatitis C virus (HCV) infection has been uncommon since the advent of direct-acting antiviral drugs (DAAs). However, primary HCV disease can cause significant haematological disease in the form of various autoimmune cytopenias.

Case Presentation: We herein discuss a 68-years-old female with chronic HCV infection for the last 15 years (not on the treatment), presented with complaints of progressive fatigue, exertional dyspnea, and increased abdominal distention over the previous 20 days. Read More

Arthritis Care Res (Hoboken) 2022 Apr 19. Epub 2022 Apr 19.

University of Washington, Division of Rheumatology.

Objective: The objective of this study was to evaluate independent risk factors for readmission and determine the major reasons for readmission in a nationally representative sample of patients with systemic lupus erythematosus (SLE).

Methods: We used the Nationwide Readmissions Database (NRD) to identify adults with SLE who were discharged from hospital to home during January-November of 2016 and 2017. Thirty-day all-cause readmissions were identified. Read More

J Assoc Physicians India 2022 Mar;70(3):11-12

Associate Professor and Incharge, Rheumatology Clinic, TNMC and BYL Nair Charitable Hospital, Mumbai, Maharashtra.

Background: SLE is a common connective tissue disease in Indians (mostly women) which is frequently underdiagnosed due to limited awareness and knowledge regarding the disease.

Methods: This is a retrospective observational study conducted in a tertiary care hospital in Western India among patients of SLE attending outpatient Rheumatology Clinic and inpatient admissions of Topiwala Medical College and BYL Nair Charitable Hospital, Mumbai. Sixty patients were recruited based on inclusion and exclusion criteria. Read More

Indian J Pathol Microbiol 2022 Apr-Jun;65(2):507-509

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Front Immunol 2022 28;13:867837. Epub 2022 Mar 28.

Department of Experimental Pathology, Immunology, and Microbiology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon.

The interleukin-7 receptor (IL-7R) is expressed on lymphoid cells and plays an important role in the development, homeostasis, survival, and proliferation of T cells. Bi-allelic mutations in the IL-7Rα chain abolish T cell development and function resulting in severe combined immunodeficiency disease. In this manuscript, we investigate a 1 year-old patient born to consanguineous parents, who suffered from autoimmune hemolytic anemia since birth associated with recurrent severe infections. Read More