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J Nanosci Nanotechnol 2019 Jul;19(7):3772-3776

Universidade Estadual Paulista, Instituto de Química, Araraquara, SP 14800-060, Brazil.

The diagnostics of the autoimmune hemolytic anemia (AIHA), a rare disease caused by autoantibody-induced hemolysis, is still prone to false positives for it is based on visual observation in the so-called Direct Coombs test. In this study, we developed a specific IgG hemolysis immunosensor produced with layer-by-layer (LbL) films containing a monoclonal antibody against human immunoglobulin (mAbIMUG) deposited along with a layer of silk fibroin (SF) derived from cocoons. Adsorption of mAbIMUG on a SF layer was confirmed by the fluorescence emission band at 326 nm. Read More

Eur J Cancer 2019 Feb 7;110:21-23. Epub 2019 Feb 7.

APHP, Service de Médecine Interne Immunologie Clinique, Hôpitaux Universitaires Paris Sud, Le Kremlin Bicêtre, France. Electronic address:

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):197-201

Department of Blood Transfusion, The People, s Hospital of Weifang, Weifang 261041, Shandong Province, China.

Objective: To investigate the correlation of IgG subclasses with blood cell parameters in the patients with autoimmune hemolytic anemia (AIHA).

Methods: Thirty-four patients with AIHA (except C3d types) of immune complex type (IgG+C3d) and single IgG type, including 26 cases of primary AIHA and 8 cases of secondary AIHA from December 2010 to August 2016 in our hospital were selected and enrolled in AIHA group; 30 healthy persons were selected and enrolled in healthy control group. The levels of IgG subclasses in blood plasma were detected by double antibody sandwich ELISA in healthy persons and AIHA patients, at the same time. Read More

Case Rep Obstet Gynecol 2019 14;2019:2093612. Epub 2019 Jan 14.

Department of Obstetrics and Gynecology, Toyooka Public Hospital, 1094, Tobera, Toyooka City, Hyogo 6680065, Japan.

The simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and idiopathic thrombocytopenic purpura (ITP) is known as Evans syndrome. We experienced a case of Evans syndrome that developed AIHA during pregnancy and ITP long after delivery. The patient was a 35-year-old pregnant woman (gravida 2, para 1). Read More

Case Rep Hematol 2019 9;2019:2089359. Epub 2019 Jan 9.

Internal Medicine, Monmouth Medical Center, Long Branch, New Jersey, USA.

Internal watershed infarcts (WI) involve white matter between deep and superficial arterial systems of middle cerebral artery. These infarcts are considered to be either from low blood flow or microembolism. Anemia is an extremely rare cause of watershed infarcts. Read More

J Nucl Med Technol 2019 Jan 29. Epub 2019 Jan 29.

Walter Reed National Military Medical Center, United States.

Tc-red blood cells (RBCs) labeled multi-gated acquisition (MUGA) scan is a procedure in which the patient's red blood cells (RBCs) are radiolabeled and electrocardiograph (ECG)-gated cardiac scintigraphy is obtained to assess the heart's pumping efficiency. Cold agglutinin disease (CAD), or cold antibody autoimmune hemolytic anemia, is a rare form of autoimmune hemolytic anemia in which the body's immune system attacks and destroys its own red blood cells. This case addresses an altered biodistribution pattern of radiolabeled RBCs in the presence of suspected cold agglutinin disease observed during a MUGA scan. Read More

Asian J Transfus Sci 2018 Jul-Dec;12(2):99-104

Department of Transfusion Medicine, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India.

Background And Aim: Patients with warm autoimmune hemolytic anemia (AIHA) may carry immunoglobulin (Ig) M antibodies that react at room temperature and are nonpathological, but few may have cold agglutinins that react at or above 30°C and are referred to as "mixed" AIHA (MAIHA). Here, we present our experience on characterizing MAIHA both clinically and serologically.

Materials And Methods: Out of 134 AIHA patients, 13 diagnosed as MAIHA were subjected to detailed immunohematological characterization. Read More

Prostate Cancer Prostatic Dis 2019 Jan 28. Epub 2019 Jan 28.

Graduate Institute of Life Sciences, National Defense Medical Center, Taipei, Taiwan.

Background: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases.

Methods: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases. Read More

Medicine (Baltimore) 2019 Jan;98(4):e14201

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Rationale: Concomitant cryoglobulinemic vasculitis and cold agglutinin disease (CAD) is an extremely uncommon clinical scenario. The role of bortezomib in the treatment of cryoglobulinemic vasculitis needs further investigation.

Patient Concerns: A 72-year-old Chinese woman presented with a 25-year history of cyanosis of the extremities after cold exposure, which worsened and was accompanied with purpuric skin lesions and proteinuria in recent years. Read More

J Clin Immunol 2019 Jan 22. Epub 2019 Jan 22.

Centre de Référence National des Cytopénies Autoimmunes de l'Enfant (CEREVANCE), University Hospital of Bordeaux, Bordeaux, France.

Purpose: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. Read More

Blood 2019 Jan 22. Epub 2019 Jan 22.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano - UOC Ematologia, Italy;

BMC Infect Dis 2019 Jan 18;19(1):68. Epub 2019 Jan 18.

Sri Jayawardanapura General Hospital, Thalapathpitiya, Nugegoda, Western province, Sri Lanka.

Background: Infectious mononucleosis is one of the main manifestations of Epstein - Barr virus, which is characterized by fever, tonsillar-pharyngitis, lymphadenopathy and atypical lymphocytes. Although 60% of patients with IMN develop cold type antibodies, clinically significant hemolytic anemia with a high ferritin level is very rare and validity of serum ferritin as an important biomarker has not been used frequently.

Case Presentation: 18-year-old girl presented with fever, malaise and sore throat with asymptomatic anemia, generalized lymphadenopathy, splenomegaly and mild hepatitis. Read More

J Emerg Nurs 2019 Jan 14. Epub 2019 Jan 14.

Acta Haematol 2019 Jan 10;141(2):79-83. Epub 2019 Jan 10.

Autoimmune diseases, including autoimmune hemolytic anemia and immune thrombocytopenic purpura, have been described in patients with non-Hodgkin lymphoma (NHL) after immunochemotherapy. However, the underlying pathogenesis remains unclear. We examined NHL patients with autoimmune cytopenia and all patients were treated with rituximab-containing therapy. Read More

J Med Case Rep 2018 Dec 26;12(1):380. Epub 2018 Dec 26.

Department of Surgical Oncology, Mohamed I University, 30050, Oujda, Morocco.

Background: Gastric duplication cysts are uncommon congenital anomalies found primarily in children and rarely seen in the adult population. Accurate diagnosis of cysts before resection is difficult even using the most advanced imaging techniques.

Case Presentation: In this report, we describe a 28-year-old Moroccan patient with a history of autoimmune hemolytic anemia who presented with an asymptomatic abdominal cystic mass detected during abdominal computed tomography performed before splenectomy. Read More

Mol Med Rep 2019 Feb 12;19(2):1194-1202. Epub 2018 Dec 12.

Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.

A type of aplastic anemia (AA), non-severe aplastic anemia (NSAA) is defined as AA that does not meet the diagnostic criteria of severe aplastic anemia (SAA). Complement component 1q (C1q) has an important role in the pathogenesis of various autoimmune diseases; however, the role of C1q in the immune pathogenesis of NSAA is not clear. The current study aimed to determine whether C1q has an important role in the pathogenesis of NSAA. Read More

Intern Med 2018 Dec 18. Epub 2018 Dec 18.

Department of Internal Medicine, Ako City Hospital, Japan.

A 79-year-old man with Sjogren's syndrome and systemic lupus erythematosus developed acute impaired consciousness and hemolytic anemia. The patient's red blood cells agglutinated spontaneously at 25-37°C. The treatment of red blood cells with 2-mercaptoethanol resulted in the loss of spontaneous agglutination. Read More

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1108-1111

Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor VIII (FVIII) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FVIII, and some cases of AHA may act as the first manifestation of SLE. Read More

Blood 2018 Dec 17. Epub 2018 Dec 17.

Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria;

Cold agglutinin disease is a difficult-to-treat autoimmune hemolytic anemia in which immunoglobulin M antibodies bind to erythrocytes and fix complement, resulting in predominantly extravascular hemolysis. This trial tested the hypothesis that the anti-C1s antibody sutimlimab would ameliorate hemolytic anemia. Ten patients with cold agglutinin disease participated in the phase 1b component of a first-in-human trial. Read More

Clin Adv Hematol Oncol 2018 Oct;16(10):670-676

Mayo Clinic, Rochester, Minnesota.

Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) have been associated with B-cell lymphoproliferative disorders. Here, we review the epidemiology, pathogenesis, diagnosis, and treatment of these autoimmune disorders, specifically in the setting of B-cell malignancies. AIHA and ITP are classically associated with chronic lymphocytic leukemia (CLL) but have also been reported in plasmacytic and lymphoproliferative disorders. 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

Pediatr Blood Cancer 2018 Dec 10:e27569. Epub 2018 Dec 10.

Department of Pediatrics, Hematology/Oncology Section, Baylor College of Medicine, Houston, Texas.

Autoimmune cytopenias (AIC) post-hematopoietic stem cell transplant (HSCT) are rare but exceptionally challenging complication. We conducted a comprehensive literature review and identified a pooled incidence of post-HSCT autoimmune hemolytic anemia and/or immune thrombocytopenia of 2.66% (SE = 0. Read More

Ann Hematol 2019 Mar 10;98(3):589-594. Epub 2018 Dec 10.

Medical Intensive Care Unit, Saint-Louis Teaching Hospital, AP-HP, Paris, France.

Patients with autoimmune hemolytic anemia (AIHA) may require intensive care unit (ICU) admission. In order to describe the characteristics of AIHA patients in ICU and identify prognosis factors, clinical and biological data from 44 patients admitted in one ICU between 2002 and 2015 were retrospectively analyzed. The main reasons for ICU admission were profound anemia without any organ failure in 19 patients (either for safer transfusion or continuous monitoring only). Read More

Nursing 2019 Jan;49(1):22-29

Vincent M. Vacca, Jr., is an adjunct faculty member at Massachusetts College of Pharmacy and Health Sciences in Boston, Mass.

Acquired autoimmune thrombotic thrombocytopenic purpura (TTP)-the most common form of TTP-is a life-threatening hematologic disease characterized by hemolytic anemia and thrombocytopenia. Acquired autoimmune TTP can cause signs and symptoms of neurologic and other organ involvement, with mortality approaching 90% if the disease is not promptly recognized and treated. Since the introduction of plasma exchange in 1991, the acquired autoimmune TTP survival rate has increased to 78%. Read More

Eur J Intern Med 2018 12;58:77-83

UOC Oncoematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano; Università degli Studi di Milano, Italy.

The physiology of the immune system involves morphologic and functional changes occurring along ageing, with a decrease in immune response and an increase in autoimmune phenomena, even in the absence of overt disese. Autoimmune cytopenias, namely autoimmune hemolytic anemia (AIHA), chronic idiopathic neutropenia (CIN) and aplastic anemia (AA), show different epidemiologic predilection, but are increasingly diagnosed in the elderly, where complications and comorbidities are more frequent. A systematic review of recent literature, shows that comorbidities as well as underlying deficiencies, medications, neoplasms, and, pathophysiologic chronic organ failures, frequently challenge the differential diagnosis in this setting and should always be evaluated and excluded. Read More

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):382-389

Department of Haematology, Leeds Teaching Hospitals, Leeds, United Kingdom.

The diagnosis of autoimmune hemolytic anemia (AIHA) can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test. Once alternative causes for these findings have been excluded, AIHA is established, and the clinician must search for secondary causes, as well as identify the type of AIHA. Rituximab is now the preferred second-line treatment for primary warm AIHA and first-line treatment for primary cold agglutinin disease (CAD), either as monotherapy or combined with bendamustine. Read More

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1836-1840

Department of Hematology and Oncology，Beijing Children's Hospital，Capital Medical University，Beijing 100045，China

Immune-mediated hemocytopenia is a common cytopenic diseases without bone marrow hematopoietic abnormalities, the patient's quality of life is significantly reduced when first-line treatments are ineffective. Rapamycin, possesses a clear mechanism of targeting mTOR protein, can upregulate regulatory T cells and induces apoptosis of specific cells, by regulating the lymphocyte subsets, so as to treat various types of immune-mediated hemocytopenia with a certain therapeutic effect. In this reviews, the action mechanism and clinical application of rapamycin in immune thrombocytopenia（ITP）, autoimmune hemolytic anemia（AIHA）, acquired aplastic anemia and autoimmune lymphoproliferative syndrome（ALPS） etc. Read More

Case Rep Infect Dis 2018 25;2018:1030382. Epub 2018 Oct 25.

Department of Pediatrics, SN Medical College, Jodhpur, India.

Brucellosis is one of the most widespread zoonosis in the world. Hematological complications in brucellosis usually present as mild anemia, leukopenia, or pancytopenia. Autoimmune hemolytic anemia in brucellosis is rarely reported. Read More

J Oncol Pharm Pract 2018 Nov 28:1078155218816775. Epub 2018 Nov 28.

2 Eisenhower Lucy Curci Cancer Center, Rancho Mirage, CA, USA.

Castleman disease is a rare B-cell lymphoproliferative disorder characterized by lymph node enlargement with or without constitutional signs. Herein, we describe a unique patient with multicentric Castleman disease and retroviral infection who presented with a sudden onset of constitutional signs and was found to have severe warm-antibody autoimmune hemolytic anemia. Rituximab monotherapy yielded an excellent clinical response. Read More

Zhonghua Xue Ye Xue Za Zhi 2018 Nov;39(11):908-911

Department of Hematology, The First Affiliated Hospital of Guangxi Medial University, Nanning 530021, China.

To explore the diagnosis, treatment and prognosis of autoimmune hemolytic anemia (AIHA) after allo-HSCT in patients with thalassemia major (TM). A retrospective analysis of AIHA status after allo-HSCT in 291 TM patients from July 2007 to December 2017 was conducted. Five of the 291 TM patients (1. Read More

GE Port J Gastroenterol 2018 Nov 14;25(6):340-341. Epub 2018 Feb 14.

Gastroenterology Department, Centro Hospitalar Tondela-Viseu, Viseu, Portugal.

Clin Psychopharmacol Neurosci 2018 Nov;16(4):501-504

Departments of Child Hematology and Oncology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey.

Autoimmune hemolytic anemia is a disease characterized with destruction of erythrocytes as a result of antibody produce against patient's own erythrocytes and anemia. Autoimmune hemolytic anemia can be roughly stratified into two groups according to serological features and secondary causes including drugs induced hemolytic anemia. Drugs induced autoimmune hemolytic anemia is very rare in pediatric patients. Read More

PLoS One 2018 8;13(11):e0207218. Epub 2018 Nov 8.

Department of Internal Medicine and Clinical Immunology, Constitutive Referral Center for Autoimmune Cytopenias, University Hospital, Dijon, France.

Thrombotic manifestations are a hallmark of many auto-immune diseases (AID), specially of warm autoimmune hemolytic anemia (wAIHA), as 15 to 33% of adults with wAIHA experience venous thromboembolic events (VTE). However, beyond the presence of positive antiphospholipid antibodies and splenectomy, risk factors for developing a VTE during wAIHA have not been clearly identified. The aim of this retrospective study was to characterize VTEs during wAIHA and to identify risk factors for VTE. Read More

Front Immunol 2018 23;9:2442. Epub 2018 Oct 23.

Sanquin Research and Landsteiner Laboratory, Department of Blood Cell Research, Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands.

Intravenous immunoglobulins (IVIg) are used in the treatment of different autoimmune and inflammatory diseases, such as immune thrombocytopenia and hemolytic anemia. One of the modes of action of IVIg is preventing phagocytosis of autoantibody-opsonized blood cells by saturation of the Fc-gamma receptors of macrophages in spleen and liver. IgG contains a fixed glycan, which is in most cases biantennary, at the asparagine residue at position 297 in the Fc tail. Read More

Indian J Crit Care Med 2018 Oct;22(10):753-756

Department of Medicine, Military Hospital, Ranchi, Jharkhand, India.

Autoimmune hemolytic anemia (AIHA) is a very rare cause of anemia in a case of malaria and drug-induced AIHA is even rarer. A 50-year old patient with a history of fever for 8 days was diagnosed with a case of complicated malaria with mixed infection having initial parasite index of 45%. He showed good response on initial therapy with artesunate (parasite count reduced to <1%) but his haemoglobin (Hb) continued to drop from 12 g% to 4. Read More

Biomed Res Int 2018 30;2018:3139404. Epub 2018 Sep 30.

Biomedical Genomics and Oncogenetics Research Laboratory, Faculty of Sciences and Techniques of Tangier, University Abdelmalek Essaâdi, Tetouan, Morocco.

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with a high female predominance. To date, studies about SLE in Morocco are few. This retrospective study describes the clinical and immunological features in a series of 50 SLE Moroccan patients in University Hospital Center of Rabat, Morocco, between December 2011 and December 2013. Read More

Lupus 2018 Dec 25;27(14):2274-2278. Epub 2018 Oct 25.

Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.

We determined the clinical utility of the direct Coombs' test in the absence of hemolytic anemia as an indicator of disease activity and therapeutic response in systemic lupus erythematosus (SLE). SLE patients without hemolytic anemia who visited our hospital from January 2016 to November 2016 were retrospectively evaluated with a direct Coombs' test. Clinical features, including SLE disease activity index (SLEDAI), treatment and laboratory findings were analyzed. Read More

J Blood Med 2018 10;9:171-184. Epub 2018 Oct 10.

Department of Hematology, Internal Medicine Division, Dr José E González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México,

Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. Read More

Eur J Haematol 2019 Feb 29;102(2):157-162. Epub 2018 Nov 29.

Section of Hematology and Medical Oncology, Tulane University, New Orleans, Louisiana.

Background: Immune checkpoint inhibitors (CPI) are widely used in modern oncology and have improved the prognosis of lung cancer, malignant melanoma, and other malignancies. Unlike cytotoxic chemotherapy, drugs such as nivolumab, pembrolizumab, and ipilimumab are associated with immune-related adverse effects. We recently observed a patient with lung cancer who developed a fulminant warm antibody autoimmune hemolytic anemia (AIHA). Read More

Hematol Rep 2018 Sep 24;10(3):7394. Epub 2018 Sep 24.

Faculty of Medicine, Pelita Harapan University, Tangerang, Banten, Indonesia.

Vascular events are the most common clinical complication of essential thrombocythemia, leading to sign and symptoms of this disease. There are various sign and symptoms of essential thrombocythemia, such as thrombosis in artery or vein, and enlarged spleen. Portal hypertension and hepatic cirrhosis could be caused by essential thrombocythemia via intrahepatic thrombus. Read More

Front Pediatr 2018 1;6:272. Epub 2018 Oct 1.

Department of Pediatrics, University of Brescia, Spedali Civili Hospital, Brescia, Italy.

We describe a previously healthy 14-year-old girl with acute onset autoimmune hemolytic anemia, associated with severe but transient lymphopenia during corticosteroid therapy, without infectious episodes during follow-up. After detailed investigations to rule out an underlying immunodeficiency, we detected a heterozygous gene mutation. This was associated with slightly increased blood levels of adenosine and deoxyadenosine nucleotides and with reduced ADA activity in red blood cells, but within the normal range. Read More

Ann Hematol 2018 Oct 15. Epub 2018 Oct 15.

Department of Hematology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.

Rinsho Ketsueki 2018;59(10):2354-2361

Department of Hematology/Oncology, Transfusion and Hemapheresis Center, Day Chemotherapy Center, Kurashiki Central Hospital.

Autoimmune hemolytic anemia (AIHA) is caused by the generation of autoantibodies against red cells. Sometimes, the destruction of red cells occurs in the intravascular space following the autoantibody-mediated activation of the complement cascade. More frequently, extravascular hemolysis occurs when macrophages phagocytose autoantibodies and complements bearing red cells. Read More

Case Rep Med 2018 13;2018:3625139. Epub 2018 Sep 13.

Internal Medicine Department, Beaumont Hospital, Royal Oak, MI, USA.

Hepatitis A is a common viral infection with a benign course but in rare cases can progress to acute liver failure. It usually presents with abdominal pain, nausea, vomiting, diarrhea, jaundice, anorexia, or asymptomatically, but it can also present atypically with relapsing hepatitis and prolonged cholestasis. In addition, extrahepatic manifestations have been reported, including urticarial and maculopapular rash, acute kidney injury, autoimmune hemolytic anemia, aplastic anemia, acute pancreatitis, mononeuritis, reactive arthritis, glomerulonephritis, cryoglobulinemia, Guillain-Barre syndrome, and pleural or pericardial effusion. Read More

J Pediatr Hematol Oncol 2018 Oct 5. Epub 2018 Oct 5.

Department of Pediatrics, Division of Pediatric Hematology and Oncology, Children's Hospital of Michigan.

Evans syndrome is defined by bilineal autoimmune cytopenia, typically coombs positive hemolytic anemia and thrombocytopenia. Corticosteroids are the mainstay of treatment, with rituximab and/or mycophenolate mofetil often used in steroid-refractory cases. However, no treatment methodology has ever evaluated by a randomized clinical trial. Read More

Transfusion 2018 Dec 6;58(12):2777-2781. Epub 2018 Oct 6.

Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.

Background: Evans syndrome is a rare autoimmune disorder that is defined by the simultaneous or sequential presence of two or more cytopenias without an obvious underlying precipitating cause. Evans syndrome usually follows a chronic relapsing and remitting course and is quite rare, making it difficult to evaluate in clinical studies.

Case Report: A 66-year-old male patient with a 17-year history of Evans syndrome presented with fulminant autoimmune hemolytic anemia (AIHA). Read More

Blood Adv 2018 Oct;2(19):2550-2553

Division of Allergy, Immunology and Blood and Marrow Transplant, Benioff Children's Hospital, University of California, San Francisco, San Francisco, CA.

New-onset autoimmune hemolytic anemia (AIHA) occurs in 2% to 6% of pediatric patients post-hematopoietic stem cell transplantation (HSCT) and is a significant complication. Incomplete immune recovery following HSCT may predispose to immune dysregulation including autoimmune cytopenias. We describe an innovative therapy for AIHA refractory to proteasome inhibition. Read More

Blood Adv 2018 Oct;2(19):2543-2549

Department of Hematology, University Hospital, Essen, Germany.

Cold agglutinin disease (CAD) is a complement-dependent disorder, with extravascular and intravascular hemolysis resulting from initial or terminal complement activation, respectively. We tested the efficacy and safety of eculizumab, an inhibitor of the terminal complement pathway. Treatment-requiring patients received 600 mg eculizumab weekly for 4 weeks, followed 1 week later by 900 mg every other week through week 26. Read More

Medicine (Baltimore) 2018 Oct;97(40):e12716

Department of Rheumatology and Immunology, Xiangya Hospital.

Rationale: Relapsing polychondritis (RP) is a rare autoimmune-related disease and may be associated with other autoimmune diseases.

Patient Concerns: Here we reported a case of RP patients with mixed-type AIHA. The patient was diagnosed with RP in March 2008 which was treated and the patient was in stable condition. Read More

Rheum Dis Clin North Am 2018 11 7;44(4):635-649. Epub 2018 Sep 7.

Division of Renal Diseases and Hypertension, Department of Medicine, George Washington University, 2150 Pennsylvania Avenue, Washington, DC 20037, USA. Electronic address:

Thrombotic microangiopathies are heterogeneous disorders characterized by microangiopathic hemolytic anemia with thrombocytopenia and renal injury. There are a variety of causes, including metabolic disorders, infections, medications, complement disorders, pregnancy, malignancy, and autoimmune disorders. This review focuses on renal thrombotic microangiopathy in the setting of rheumatologic diseases. Read More