Immune-hematology

INTRODUCTION: Immune checkpoint inhibitors (ICIs) are used in several advanced malignancies and may cause various immune-related adverse events (irAEs). Among them, hematological irAEs are less described. Acquired amegakaryocytic thrombocytopenia (AAT) is a rare immune hematologic disorder characterized by severe thrombocytopenia and complete absence of megakaryocytes in bone marrow. CASE PRESENTATION: Herein, we present the case of a patient in their 40s with metastatic melanoma who developed an AAT after 12 cycles of nivolumab (anti-PD1)...

Cytopenia is a common finding in patients admitted to internal medicine wards and the clinical workup may be long and time-consuming. In this single-center observational study, we analyzed a series of 151 inpatients who received hematologist referral due to cytopenia observed during hospital admission. Patients were mainly elderly (median 71 years, 15-96) and 87% had at least one comorbidity. Anemia was the most common cytopenia (91%), followed by thrombocytopenia (51%), and neutropenia (22%); 73 (48%) patients had a bicytopenia and 5 (3%) pancytopenia...

Biallelic KARS1 mutations cause KARS-related diseases, a rare syndromic condition encompassing central and peripheral nervous system impairment, heart and liver disease, and deafness. KARS1 encodes the t-RNA synthase of lysine, an aminoacyl-tRNA synthetase, involved in different physiological mechanisms (such as angiogenesis, post-translational modifications, translation initiation, autophagy and mitochondrial function). Although patients with immune-hematological abnormalities have been individually described, results have not been collectively discussed and functional studies investigating how KARS1 mutations affect B cells have not been performed...

Daratumumab is a first-in-class human anti-CD38 IgG1 monoclonal antibody approved for treating newly diagnosed and relapsed refractory multiple myeloma. Pre-clinical data supported daratumumab's ability to deplete autoantibodies producing plasma cells, B-cells, and NK cells. Those reports showed promising results on using daratumumab in autoimmune disorders that are refractory to multiple lines of therapies, which encouraged using daratumumab in various autoimmune conditions that are refractory to standard therapies...

INTRODUCTION: The direct antiglobulin test (DAT) or Coombs test is the cornerstone of the diagnosis of autoimmune hemolytic anemia (AIHA). It can be performed by several methods with different sensitivity and specificity and enables the distinction of warm, cold, and mixed forms, which require different therapies. AREAS COVERED: The review describes the different DAT methods, including the tube test with monospecific antisera, microcolumn and solid phase methods that are routinely accessible in most laboratories...

Drug-induced hemolytic anemia is rare and can occur either by an immune-mediated mechanism or a non-immune-mediated mechanism. The drugs most frequently associated with immune-mediated hemolysis are penicillins and cephalosporins. It is usually difficult to distinguish drug-induced hemolysis from other more common causes of hemolysis; therefore, a high index of clinical suspicion is required to make the diagnosis. In this case report, we present a case of vancomycin-induced immune hemolytic anemia in a 75-year-old patient who developed hemolytic anemia after starting vancomycin for joint infection...

INTRODUCTION: Autoimmune hemolytic anemia is characterized by increased red cell destruction and/or decreased red cell survival due to autoantibodies directed against self-antigens on red cells. Since autoantibodies react with self and nonself red blood cells (RBCs), they tend to mask the underlying clinically significant alloantibodies and many a times mimic a specific pattern like alloantibodies. MATERIALS AND METHODS: We discuss three immune hematological cases of warm autoantibodies...

Novel targeting agents for hematologic diseases often exert on- or off-target immunomodulatory effects, possibly impacting on response to anti-SARS-CoV-2 vaccinations and other vaccines. Agents that primarily affect B cells, particularly anti-CD20 monoclonal antibodies (MoAbs), Bruton tyrosine kinase inhibitors, and anti-CD19 chimeric antigen T-cells, have the strongest impact on seroconversion. JAK2, BCL-2 inhibitors and hypomethylating agents may hamper immunity but show a less prominent effect on humoral response to vaccines...

INTRODUCTION: - Prozone phenomenon due to excess unbound antibodies may sometimes lead to failure of detection of ABO incompatibility. . The case series describes immunohematology work up of blood group discrepancies of two blood donors. MATERIALS AND METHODS: - Blood grouping was performed by a fully automated immune hematology analyzer (FAIHA Diagast, Qwalys 3, France) based on the principle of erythrocyte magnetized technology. Further immunohematology workup was done by tube technique (at different temperatures and phases) and column agglutination technique (CAT)...

Restoring the adaptive potential of an athlete is of paramount importance not only for the implementation of his training and competitive activities, but also for maintaining health. One of the leading place in complex recovery programs in sports is given to full-fledged optimal nutrition, which provides for meeting the body's requirements not only in energy, macro- and micronutrients, but also in minor bioactive compounds. The use of anthocyanin-containing products is a promising strategy for the normalization of metabolic and immune disorders that develop as a result of intense physical and neuro-emotional stress not only in athletes, but also in other groups of people exposed to these factors, including military personnel undergoing training in conditions close to combat...

Antibodies to red blood cells (RBCs) may hemolyze erythrocytes via Fc-mediated phagocytosis or complement-dependent. Complement activation on RBCs can be detected by C3d-direct antiglobulin test (DAT), which is the only test in immune hematology that directly targets complement. However, a positive DAT with anti-C3d cannot distinguish between C3b-mediated extravascular hemolysis, C5b-C9-mediated intravascular hemolysis and C5b-C8-mediated eryptosis. Furthermore, DAT is not suitable to estimate the strength of hemolysis...

OBJECTIVES: The immune dysregulation during SARS-CoV-2 has the potential to worsen immune homeostasis after recovery. Patients with hematological malignancies with COVID-19 have changes both in the innate and adaptive immune responses. Little is known about the severity of immune dysfunction following recovery from COVID-19 in hematological patients. METHODS: Here, we performed a comprehensive analysis of the lymphocyte subsets in peripheral blood mononuclear cells by FACS Canto II in 55 patients, including 42 with hematological malignancies 4-6 weeks after COVID-19...

Immune thrombocytopenic purpura (ITP) is caused by alterations in the immune system resulting in platelet destruction. It often manifests clinically with bleeding or on routine lab work revealing thrombocytopenia in asymptomatic individuals. Neurologic manifestations of this condition are sparsely documented in the literature. Among the symptoms reported were case reports of ischemic strokes, transient ischemic attacks, mononeuropathy multiplex, and polyneuropathy as neurological complications from immune thrombocytopenic purpura...

In this report, we describe the case of a woman with suspected COVID-19 immunization-induced probable catastrophic antiphospholipid syndrome. The patient is a 35-year-old female with a past medical history significant for antiphospholipid syndrome, not on anticoagulation, who presented with a 5-day history of abdominal pain and distention, nausea, vomiting, and shortness of breath. She had received her first dose of the Pfizer COVID-19 vaccine one day prior to the onset of symptoms. After extensive workup at an outside hospital, she was found to be in acute heart failure exacerbated by severe mitral and tricuspid regurgitation...

CBL syndrome is a Noonan-like RASopathy with heterogeneous clinical phenotype and predisposition to juvenile myelomonocytic leukemia (JMML). Here we describe two patients with identical germline CBL mutation and clinical and immune-hematological overlapping features with autoimmune lymphoproliferative syndrome (ALPS) and B-cell expansion with NF-κB and T-cell anergy (BENTA) syndrome. Increased immature/transitional B cells can be depicted in CBL syndrome, ALPS, and BENTA. Nonetheless, our patients here described showed peculiar B-cell phenotype due to increased immature/transitional CD34+ B cells...

Autoimmune hemolytic anemia (AIHA) is a rare disease characterized by autoantibodies directed at red blood cells. Patients typically present with anemia and are diagnosed by positive direct antiglobulin (DAT) test. AIHA is subclassified into warm or cold based on antibodies involved and depending on their optimal temperature in which they react with RBC antigens. Warm AIHA can be either primary (idiopathic) or secondary depending on etiology. Secondary causes are associated with malignancy, connective tissue and inflammatory diseases, infections (typically viral infections), or drugs (e...

Genetic studies of familial forms of interstitial lung disease (ILD) have led to the discovery of telomere-related gene (TRG) mutations (TERT, TERC, RTEL1, PARN, DKC1, TINF2, NAF1, NOP10, NHP2, ACD, ZCCH8) in approximately 30% of familial ILD forms. ILD patients with TRG mutation are also subject to extra-pulmonary (immune-hematological, hepatic and/or mucosal-cutaneous) manifestations. TRG mutations may be associated not only with idiopathic pulmonary fibrosis (IPF), but also with non-IPF ILDs, including idiopathic and secondary ILDs, such as hypersensitivity pneumonitis (HP)...

OBJECTIVES: The HEMVACO study evaluated the humoral response after mRNA anti-SARS-CoV-2 vaccination in an hematological cohort. METHODS: HEMVACO was a prospective, multicentric study registered in ClinicalTrials.gov, number NCT04852796. Patients received two or three doses of BNT162b2 vaccine or mRNA-1273 vaccine. The SARS-CoV-2 TrimericS IgG titers were measured 1, 3, 6 and 12 months after the second dose. RESULTS: Only 16 patients (11.6%) were naive of hematological treatment and 77 patients (55...

Individuals with oncohematological diseases (OHD) may develop an impaired immune response against vaccines due to the characteristics of the disease or to its treatment. Humoral response against SARS-CoV-2 has been described to be suboptimal in these patients, but the quality and efficiency of the cellular immune response has not been yet completely characterized. In this study, we analyzed the early humoral and cellular immune responses in individuals with different OHD after receiving one dose of an authorized vaccine against SARS-CoV-2...

AIM: Splenectomy has been performed for various indications. In this study, we aimed to present the experience of a tertiary center on splenic surgery and analyze what has changed in the last 10 years. MATERIAL AND METHODS: Three hundred and sixteen patients who underwent splenic surgery were enrolled in the study. Demographic data, comorbidities, American Society of Anesthesiologists score, indications, operation type, postoperative complications, and mortality were analyzed retrospectively...