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J Clin Med Res 2020 May 8;12(5):286-292. Epub 2020 May 8.

Hospital das Clinicas, Federal University of Pernambuco, Recife-PE, Brazil.

Antiphospholipid antibody syndrome (APS) is a state of hypercoagulability secondary to an autoimmune disorder. It is associated with thrombotic events in venous and arterial vessels, obstetric complications characterized by recurrent fetal losses, and increased perinatal morbidity. APS is classified as primary, when not associated with other pathologies; or secondary, when associated with an underlying autoimmune disease with, solid tumor, or hematological disorder. Read More

Pharmacology 2020 Jun 2:1-9. Epub 2020 Jun 2.

Department of Medical Oncology/Hematology Section, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.

Background: Autoimmune hemolytic anemia (AIHA) might be associated with underlying hematological malignancies such as chronic lymphocytic leukemia. However, the association between AIHA and chronic myelogenous leukemia is extremely unusual.

Summary: We reviewed case reports and series of 54 patients with chronic myeloid leukemia (CML) who developed autoimmune hemolysis between 1952 and 2018. Read More

Ann Clin Transl Neurol 2020 Jun 1. Epub 2020 Jun 1.

Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.

Objectives: The data concerning the association between Tx and ADs remain unclear and are scarce. This study was undertaken to investigate whether people with Tx are more likely to develop ADs, compared to those without Tx.

Methods: Individuals who received Tx between 2002 and 2015 were identified and matched on age and sex with individuals without Tx. Read More

Dermatol Ther 2020 May 30:e13711. Epub 2020 May 30.

Department of Dermatology, Alicante University General Hospital, Alicante Institute for Health and Biomedical Research (ISABIAL), Alicante, Spain.

Transfusion 2020 May 29. Epub 2020 May 29.

Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota.

Background: The AABB Clinical Transfusion Medicine Committee (CTMC) compiles an annual synopsis of the published literature covering important developments in the field of transfusion medicine (TM) for the board of director's review. This synopsis is now made available as a manuscript published in TRANSFUSION.

Study Design And Methods: CTMC committee members review original manuscripts including TM-related topics published in different journals between late 2018 and 2019. Read More

Br J Haematol 2020 May 27. Epub 2020 May 27.

Reading Hospital/ Tower Health, Department of Internal Medicine, 420 S 5th Avenue, West Reading, Pennsylvania, USA, 19611.

Cold agglutinin syndrome (CAS) is a rare disorder associated with infection, autoimmune disorders, and lymphoid malignancies. We are present a case of CAS associated with SARS-CoV-2 that causes COVID-19. A 46-year-old female presented with severe anemia and positive SARS-CoV-2 RNA PCR. Read More

Br J Haematol 2020 May 26. Epub 2020 May 26.

University of Palermo, Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), Palermo, Italy.

This Journal recently published two papers describing autoimmune hemolytic anemia (AIHA) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection (1, 2). AIHA is characterized by the destruction of red cells by autoantibodies, but the mechanism underpinning autoimmunity in COVID-19 patients has yet to be elucidated. Read More

Rev Alerg Mex 2020 Jan-Mar;67(1):87-93

Hospital Pediátrico de Sinaloa, Departamento de Inmunología y Alergia, Sinaloa, México.

Background: The Wiskott-Aldrich syndrome is a combined immunodeficiency associated with a syndrome linked to the X chromosome, which is characterized by eczema, recurrent infections, and thrombocytopenia. Other manifestations include autoimmune disorders such as hemolytic anemia or thrombocytopenic purpura mediated by the immune system, increased susceptibility to malignant tumors, including lymphoma or leukemia.

Clinical Case: A 7-year-old male patient with a diagnosis of Wiskott-Aldrich syndrome who was treated with intravenous gamma globulin, antimicrobial prophylaxis with trimethoprim/sulfamethoxazole, and fluconazole, as well as with prednisone and cyclosporine due to hemolytic anemia and uveitis. Read More

Harefuah 2020 May;159(5):339-342

Department of Pediatrics, Division of Pediatric Hematology-Oncology, Laniado Medical Center, Netanya, Israel.

Indian J Hematol Blood Transfus 2020 Apr 26;36(2):444-445. Epub 2019 Oct 26.

1Department of Clinical Laboratory, Second Hospital of Hebei Medical University, Shijiazhuang, China.

Indian J Hematol Blood Transfus 2020 Apr 25;36(2):422-423. Epub 2019 Oct 25.

2Department of Pathology, Apollo Hospitals, Hyderabad, 500034 India.

Br J Haematol 2020 May 18. Epub 2020 May 18.

Baylor College of Medicine, Houston, Texas, United States of America.

Evans syndrome (ES) is a rare condition characterized by the combination of autoimmune hemolytic anemia and immune thrombocytopenia (ITP). While the precise pathophysiology is not entirely understood, it is believed that dysregulation of the immune system is a primary contributor to the condition. ES has been observed in viral infections including hepatitis C, cytomegalovirus, varicella-zoster, and Epstein-Barr viruses (1-4). Read More

Mol Genet Metab 2020 May 5. Epub 2020 May 5.

Department of Human Genetics, McGill University, Montreal, Quebec, Canada.; Division of Medical Genetics, Department of Specialized Medicine, McGill University Health Centre, Montreal, Quebec, Canada.; Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada.; Division of Medical Biochemistry, Department of Specialized Medicine, McGill University Health Centre, Montreal, Quebec, Canada.

MTHFD1 is a trifunctional protein containing 10-formyltetrahydrofolate synthetase, 5,10-methenyltetrahydrofolate cyclohydrolase and 5,10-methylenetetrahydrofolate dehydrogenase activities. It is encoded by MTHFD1 and functions in the cytoplasmic folate cycle where it is involved in de novo purine synthesis, synthesis of thymidylate and remethylation of homocysteine to methionine. Since the first reported case of severe combined immunodeficiency resulting from MTHFD1 mutations, seven additional patients ascertained through molecular analysis have been reported with variable phenotypes, including megaloblastic anemia, atypical hemolytic uremic syndrome, hyperhomocysteinemia, microangiopathy, infections and autoimmune diseases. Read More

Pediatr Transplant 2020 May 15:e13739. Epub 2020 May 15.

Division of Pediatric Nephrology, Department of Pediatrics, University of Florida, Gainesville, FL, USA.

BK virus encephalitis after HSCT is uncommon. Several reports of native kidney BKVN in patients with HSCT, hematologic malignancies, human immunodeficiency virus infection, and non-renal solid organ transplantation have been described. However, an uncommon combination of BK encephalitis and ESRD of native kidneys secondary to BK virus in a child with HSCT has not been described. Read More

Ann Hematol 2020 May 14. Epub 2020 May 14.

Department of Haematology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, 110029, India.

Zhonghua Er Ke Za Zhi 2020 May;58(5):413-417

Department of Pediatric Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders (Chongqing), China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Child Infection and Immunity, Chongqing 400014, China.

To analyze the clinical and immunological characteristics of a patient with activated phosphoinositide 3-kinase δ syndrome 2 (APDS2). A retrospective analysis of clinical data, immune-related gene sequencing, imaging and laboratory findings of a patient with APDS2 admitted to Children's Hospital of Chongqing Medical University was performed. The absolute and relative numbers of peripheral lymphocyte subsets, immune cell subsets and phenotypes were detected by flow cytometry with the age matched healthy child or the patient's father as a control. Read More

J Clin Apher 2020 May 8. Epub 2020 May 8.

Faculty of Applied Health Sciences, University of Waterloo, Waterloo, Ontario, Canada.

Objective: We performed a systematic review and meta-analysis to evaluate the efficacy and safety of therapeutic plasma exchange (TPE) in adult patients with autoimmune hemolytic anemia (AIHA).

Methods: A search of major English and Chinese databases for randomized controlled trials (RCTs) comparing the use of TPE against no TPE in adult AIHA patients was performed. Outcomes were remission incidence, hematological parameters (ie, hemoglobin count, red blood cell count, reticulocyte percentage, total bilirubin, and hematocrit) and adverse event incidence. Read More

Br J Haematol 2020 May 6. Epub 2020 May 6.

Hôpital Avicenne, Assistance Publique - Hôpitaux de Paris, Université Paris 13, Bobigny, France.

Blood 2020 May 6. Epub 2020 May 6.

KG Jebsen Centre for B-cell Malignancies, Institute of Clinical Medicine, University of Oslo, Norway.

We retrospectively studied 232 patients with cold agglutinin disease (CAD) at 24 centers in five countries. In Norway and a northern region of Italy, the study was close to being population-based. For the first time, we demonstrate 4-fold differences between cold and warmer climates regarding prevalence (20 versus 5 cases/million) and incidence (1. Read More

J Chin Med Assoc 2020 May 1. Epub 2020 May 1.

Division of Hematology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.

Background: Thrombotic microangiopathy (TMA) syndromes are potentially life-threatening complications and are defined as integrated syndromes of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and organ injury. Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect various organs, including the hematopoietic system. SLE can complicate with TMA and can be categorized into two distinct groups by chronological association: TMA occurring as the initial presentation and leading to a diagnosis of SLE concurrently (TMA-cSLE) or TMA developing in patients previously diagnosed as having SLE (TMA-pSLE). Read More

Ideggyogy Sz 2020 Mar;73(3-4):141-144

Department of Neurology, University of Szeged, Szeged.

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Read More

J Med Econ 2020 May 20:1-6. Epub 2020 May 20.

Beta6 Consulting Group, Los Angeles, CA, USA.

Cold agglutinin disease (CAD) is a rare subtype of autoimmune hemolytic anemia associated with increased thromboembolism risk and early mortality. Healthcare resource utilization (HRU) in CAD has not been reported. We aimed to compare HRU of patients with CAD with a matched non-CAD cohort in the United States. Read More

Am J Hematol 2020 May 1. Epub 2020 May 1.

Division of Hematology, Mayo Clinic, Rochester, MN, USA.

Haematologica 2020 Apr 30. Epub 2020 Apr 30.

Hematology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano and University of Milan, Milan, Italy;

Exp Hematol Oncol 2020 15;9. Epub 2020 Apr 15.

2Department of Pharmacy, Taipei Veterans General Hospital, Taipei, Taiwan.

Background: The efficacy and safety of rituximab (RTX) on hemolytic anemia (HA) is unknown. Therefore we retrospectively analyze the efficacy and safety of RTX in autoimmune hemolytic anemia (AIHA) and microangiopathic hemolytic anemia (MAHA) from the previous literature.

Methods: Data in clinical trials and observational studies were collected from PubMed, Cochrane, Embase, and Google Scholar until Oct 15, 2018. Read More

Front Immunol 2020 7;11:590. Epub 2020 Apr 7.

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

Autoimmune hemolytic anemias mediated by cold agglutinins can be divided into cold agglutinin disease (CAD), which is a well-defined clinicopathologic entity and a clonal lymphoproliferative disorder, and secondary cold agglutinin syndrome (CAS), in which a similar picture of cold-hemolytic anemia occurs secondary to another distinct clinical disease. Thus, the pathogenesis in CAD is quite different from that of polyclonal autoimmune diseases such as warm-antibody AIHA. In both CAD and CAS, hemolysis is mediated by the classical complement pathway and therefore can result in generation of anaphylotoxins, such as complement split product 3a (C3a) and, to some extent, C5a. Read More

Proc (Bayl Univ Med Cent) 2020 Apr 23;33(2):270-272. Epub 2020 Jan 23.

Department of Pathology, Baylor University Medical CenterDallasTexas.

Thrombotic thrombocytopenic purpura may be seen with several autoimmune disorders such as immune thrombocytopenia purpura, immune hemolytic anemia, and systemic lupus erythematosus, but it is rarely associated with Graves' disease. We report a patient with thrombotic thrombocytopenic purpura associated with Graves' disease. Read More

J Clin Invest 2020 May;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

Case Rep Oncol 2020 Jan-Apr;13(1):219-224. Epub 2020 Mar 16.

Department of Medical Oncology, Hematology Section, National Center for Cancer Care & Research, Hamad Medical Corporation, Doha, Qatar.

Iron overload is commonly seen in the context of hematological diseases in patients who require frequent transfusions. On the other hand, an association between autoimmune hemolytic anemia complicating chronic lymphocytic leukemia and iron overload is unusual. Here, we report 2 cases of iron overload in patients with a background of chronic lymphocytic leukemia complicated by autoimmune hemolytic anemia. Read More

CEN Case Rep 2020 Apr 18. Epub 2020 Apr 18.

Department of Lifetime Clinical Immunology, Tokyo Medical and Dental University, Tokyo, Japan.

Thrombotic microangiopathy (TMA) is generally diagnosed through clinical features characterized as microangiopathic hemolytic anemia, thrombocytopenia, and multiple organ injury, as well as by pathological findings such as vascular damage and endothelial cell injury. Rheumatic and autoimmune diseases could be accompanied by secondary TMA; in fact, systemic lupus erythematosus (SLE) is a common disease associated with secondary TMA, and SLE complicated with TMA has been reported to have a poor prognosis. Although TMA occurs rarely in pediatric SLE patients, it often leads to severe clinical conditions. Read More

BMC Pediatr 2020 Apr 17;20(1):169. Epub 2020 Apr 17.

Pediatric Nephrology Department, Shengjing Hospital of China Medical University, No.36 Sanhao Street Heping District, Shenyang City, 110004, Liaoning Province, China.

Background: Autoimmune thyroid disease (AITD) has a complex pathogenesis and is associated with the development of autoimmunity against the thyroid. Graves' disease and Hashimoto's thyroiditis are the two main types of AITD, and they are characterized by thyrotoxicosis and hypothyroidism, respectively. Atypical hemolytic uremic syndrome (aHUS) is a rare disease, presenting with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Read More

J Hematol 2019 Dec 25;8(4):168-170. Epub 2019 Dec 25.

Division of Hematology/Oncology, Lenox Hill Hospital, New York, NY, USA.

We report a case of isolated immune thrombocytopenic purpura (ITP) as a result of babesiosis infection. The patient initially presented with a history, physical exam and laboratory findings consistent with idiopathic thrombocytopenic purpura. She was treated with standard of care therapy without clinical response. Read More

J Hematol 2019 Jun 30;8(2):71-78. Epub 2019 Jun 30.

Department of Hematopathology, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA.

Immune checkpoint inhibitors have recently emerged as important and effective advanced cancer treatment options. Programmed cell death receptor-1 (PD-1) antagonists such as pembrolizumab and nivolumab have been approved by the US Food and Drug Administration for treatment of many advanced cancers. As anti-PD-1 checkpoint inhibitor use has been increasing, previously unreported rare side effects emerge. Read More

J Hematol 2019 Jun 30;8(2):64-67. Epub 2019 Jun 30.

Division of Hematology and Cellular Therapy, Western Pennsylvania Hospital, Pittsburgh, PA, USA.

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) which generally presents as a triad of thrombocytopenia, hemolytic anemia and renal failure. We present the case of a 69-year-old woman with ongoing fevers, arthralgias, diffuse rash and pharyngitis for 3 months. Investigation revealed an elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and ferritin; however, autoimmune and infectious studies were unremarkable, raising the suspicion for adult onset Still's disease (AOSD). Read More

J Hematol 2019 Mar 30;8(1):44-45. Epub 2019 Mar 30.

Department of Medicine, Jamaica Hospital Medical Center, D Building: Ground Floor, 8900 Van Wyck Expressway, Richmond Hill, NY 11418, USA.

is a multi-systemic disease primarily affecting the pulmonary, gastrointestinal, and to a lesser extent, renal systems. We present a case of pneumonia, which after resolution of respiratory compromise, was complicated by the development of autoimmune hemolytic anemia (AIHA) as determined by a positive Coombs test, and negative workup of other causes. Steroid immunosuppression was initiated, and red cell counts subsequently improved. Read More

Transfus Clin Biol 2020 Apr 30;27(2):61-64. Epub 2020 Mar 30.

Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.

Background And Aim: The aim of this study is to evaluate the clinical, biological and hematological profiles of autoimmune hemolytic anemia (AIHA) in children and to specify its etiologies, therapeutic modalities, and treatment responses.

Methods: This is a 14-year retrospective study of AIHA cases collected at the department of pediatric emergency and reanimation of Hedi Chaker University Hospital in Sfax. We included patients under 14 years old with clinical and biological features of hemolysis and a positive direct antiglobulin test (DAT). Read More

Expert Rev Hematol 2020 Apr 29:1-13. Epub 2020 Apr 29.

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

: Autoimmune hemolytic anemia (AIHA) is due to autoantibodies against erythrocytes that may arise either because of primary tolerance breakage or along with several associated conditions, including genetic predispositions, congenital syndromes, environmental triggers, autoimmune diseases, immunodeficiencies, and neoplasms.: This review evaluated the risk of AIHA development in associated conditions and summarized disease-intrinsic risk factors for relapse and outcome. Diagnostic procedures were analyzed to properly identify primary and secondary forms. Read More

J Clin Rheumatol 2020 Apr 2. Epub 2020 Apr 2.

Rheumatology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal Young Adult and Pediatric Rheumatology, Unit / Centro Hospitalar e Universitário, do Hospital de São João, Porto, Portugal Rheumatology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal.

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

J Cardiothorac Vasc Anesth 2020 Feb 28. Epub 2020 Feb 28.

Melbourne Cardiac Anaesthesia, Ivanhoe, Melbourne, Victoria, Australia; Centre for Integrated Critical Care, The University of Melbourne, Parkville, Melbourne, Victoria, Australia. Electronic address:

Am J Hematol 2020 Apr 3. Epub 2020 Apr 3.

Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.

Neurol Neuroimmunol Neuroinflamm 2020 May 2;7(3). Epub 2020 Apr 2.

From the 1st Department of Neurology (J.S.T., D.T., L.S. K.K.), Eginition Hospital, Medical School, National and Kapodistrian University of Athens (NKUA); Tzartos NeuroDiagnostics (J.S.T., C.S., M.D.); Blood Transfusion Department (S.V.), Aretaieion Hospital, Medical School, NKUA; Hematology Division (D.B.), Alexandra General Hospital; Department of Clinical Therapeutics (E.P.), Medical School, NKUA, and 2nd Department of Radiology (G.V.), Medical School, NKUA, Athens, Greece.

Autoimmun Rev 2020 Jun 29;19(6):102526. Epub 2020 Mar 29.

Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:

Background: Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a monogenic disorder characterized by early onset fatal multi-system autoimmunity due to loss-of-function mutations in the gene encoding the forkhead box P3 (FOXP3) transcription factor which is crucial for the development, maturation, and maintenance of CD4 regulatory T (T-reg) cells. Various autoimmune phenomena such as enteropathy, endocrinopathies, cytopenias, renal disease, and skin manifestations are characteristic findings in patients affected by IPEX syndrome.

Objectives: In this systematic review, we focus on both clinical and demographic characteristics of IPEX patients, highlighting possible genotype-phenotype correlations and address prognostic factors for disease outcome. Read More

BMC Pulm Med 2020 Mar 30;20(1):77. Epub 2020 Mar 30.

Department of Respiratory Medicine, Tokyo Women's Medical University School of Medicine, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Background: Sarcoidosis is a systemic granulomatous disease caused by CD4+ cell-dominant inflammation. Meanwhile, diffuse panbronchiolitis is a chronic inflammatory respiratory disease predominantly caused by CD8+ lymphocytes and neutrophils. Herein, we report a rare case of sarcoidosis in which the clinical presentation had become evident as diffuse panbronchiolitis after splenectomy for sarcoidosis. Read More

Rinsho Ketsueki 2020 ;61(3):251-256

Department of Hematology, Kin-ikyo Chuo Hospital.

Richter syndrome (RS) is the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Most cases of diffuse large B-cell lymphoma variant of RS are clonally related to the original CLL. Here, we present a case of mantle cell lymphoma (MCL) that developed sequentially during the clinical course of CLL. Read More

Blood Cells Mol Dis 2020 Jul 16;83:102427. Epub 2020 Mar 16.

Division of Hematology, Oncology and Stem Cell Transplantation, Ann & Robert H. Lurie Children's Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.

Pediatr Gastroenterol Hepatol Nutr 2020 Mar 4;23(2):180-187. Epub 2020 Mar 4.

Department of Pediatrics, Seoul National University Hospital, Seoul, Korea.

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Read More

J Pediatr Hematol Oncol 2020 Mar 20. Epub 2020 Mar 20.

Department of Pediatrics, Division of Pediatric Hematology Oncology & Pediatric HSCT Unit.

Various autoimmune diseases may be associated with primary immune deficiencies. We reported a case with a loss-of-function mutation in DNASE1L3, a gene described previously in families with systemic lupus erythematosus. In addition, the patient showed a novel homozygous missense variant in DOCK8, a gene known to be responsible for the hyper-IgE recurrent infection syndrome (HIES). Read More

Hematol Transfus Cell Ther 2020 Mar 6. Epub 2020 Mar 6.

ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.

Objectives: The purpose of this study was to compare data obtained from the reticulocyte channel (RET channel) heated to 41°C with those obtained from impedance channel (I-Channel) at room temperature in the samples with the mean corpuscular hemoglobin concentration (MCHC)<370g/L and in samples with the MCHC>370g/L, in the presence of cold agglutinins.

Methods: In this study, 60 blood samples (group 1) with the MCHC<370g/L (without cold agglutinins) and 78 blood samples (group 2) with the MCHC>370g/L (with cold agglutinins) were used to compare the two analytical channels of the XN-9000 analyzer in different preanalytical conditions. The parameters evaluated in both groups were the following: red blood cell (RBC), hemoglobin (HGB), hematocrit (HCT), mean cell volume (MCV), RBC-most frequent volume (R-MFV), mean hemoglobin concentration (MCH) and mean cellular hemoglobin concentration (MCHC). Read More

J Vet Emerg Crit Care (San Antonio) 2020 May 18;30(3):308-311. Epub 2020 Mar 18.

The Centre for Small Animal Studies, Animal Health Trust, Suffolk, UK.

Objective: To describe the clinical presentation, diagnostic investigations, and outcome of a dog with pregnancy-associated immune-mediated hemolytic anemia.

Case Summary: A 6-year-old, pregnant Bichon Frise was presented due to lethargy, inappetence, and progressive regenerative anemia with spherocytosis. The dog had been recently receiving ranitidine and amoxicillin/clavulanate. Read More