- Predictors of autoimmune hemolytic anemia in beta-thalassemia patients with underlying red blood cells autoantibodies. [Journal Article]
- BCBlood Cells Mol Dis 2019 Jun 29; 79:102342
- In beta-thalassemia patients, erythrocyte autoantibodies can remain silent or lead to Autoimmune Hemolytic Anemia (AIHA).The aim of this study was to identify predictors of AIHA in beta-thalassemia p…
In beta-thalassemia patients, erythrocyte autoantibodies can remain silent or lead to Autoimmune Hemolytic Anemia (AIHA).The aim of this study was to identify predictors of AIHA in beta-thalassemia patients with positive Direct Antiglobulin Test (DAT), in Tunisia. This longitudinal prognosis study was carried out on beta-thalassemia patients with a positive confirmed DAT. Predictors of AIHA were identified the Kaplan-Meier method. A Cox model analysis was used to identify independent predictors. Among 385 beta thalassemia patients, 87 developed positive DAT (22.6%). Autoimmune hemolytic anemia was occurred in 25 patients. Multivariate analysis showed that AIHA was independently associated with beta-thalassemia intermedia and similar family history of AIHA. Splenectomy in patients with positive DAT was independently associated with an increased risk of AIHA (HR = 6.175, CI: 2.049-18.612, p < 0.001). The risk of developing AIHA was higher during the first 72 transfusions. Autoimmune hemolytic anemia was significantly associated with polyspecific DAT (anti-complement and anti-IgG), blood group AB and prior alloimmunization. Whereas transfusion by phenotypic and leukoreduced blood was a protective factor. In summary, splenectomy after autoimmunization, prior alloimmunization, DAT specificity (IgG with complement), thalassemia intermedia, AB blood group and family history of AIHA were strongly associated with AIHA. Leukoreduced blood transfusion had a proven preventive role.
- Red blood cell alloimmunizations in beta-thalassemia patients in Casablanca/Morocco: Prevalence and risk factors. [Journal Article]
- TCTransfus Clin Biol 2019 Jun 19
- CONCLUSIONS: This study proves the data of literature. The presence of red cell autoantibodies appears to be a major risk factors for alloimmunization in thalassemic children, and could advocate specific transfusion guidelines.
- Albumin-indirect antiglobulin test. [Journal Article]
- IImmunohematology 2019; 35(2):63-64
- CONCLUSIONS: Albumin was the first widely used additive solution for hemagglutination tests. Its major effect is to decrease the repulsive forces that keep red blood cells (RBCs) apart. This effect may enable some RBC antibodies, particularly those in the Rh blood group system, to directly agglutinate antigen-positive RBCs after 37°C incubation. The impact of albumin on antibody binding before detection by an indirect antiglobulin test (IAT) is minimal. Use of albumin in antibody identification may help with separation of RBC antibody specificities in a mixture when one or more antibodies demonstrate reactivity after 37°C incubation. Warm autoantibodies can show decreased reactivity in albumin IATs, allowing recognition of underlying alloantibodies.
- An update on the Scianna blood group system. [Journal Article]
- IImmunohematology 2019; 35(2):48-50
- CONCLUSIONS: This update of the Scianna blood group system (Brunker PA, Flegel WA. Scianna: the lucky 13th blood group system. Immunohematology 2011;27:41-57) provides the recent work on the genetic variation of ERMAP across more world populations, the elucidation of the molecular basis of an historical serologic case, new cases of antibodies in the system, the development of new serologic reagents, and new discoveries in the biology of the erythroid membrane associated protein (ERMAP). Although genetic variation in ERMAP has been extensively cataloged, nonsynonymous variants associated with alloantigens have remained limited, and no new antigens have been identified. The first case of a severe hemolytic transfusion reaction to anti-Sc2 has recently been reported, highlighting the importance of pursuing the possibility of antibodies to low-prevalence antigens via indirect antiglobulin testing as a routine component of all transfusion reaction investigations. The expanding use of molecular testing in blood centers and transfusion services has uncovered a wider population distribution of Scianna antigens and heightened the awareness of this blood group system. The International Society of Blood Transfusion recognizes seven antigens in the Scianna blood group system 13.
- Defining autoimmune hemolytic anemia: a systematic review of the terminology used for diagnosis and treatment. [Review]
- BABlood Adv 2019 Jun 25; 3(12):1897-1906
- 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 li…
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. A Medline search for anemia, hemolytic, autoimmune was supplemented with keyword searches. Main exclusions were conference abstracts, animal and non-English studies, and studies with <10 cases. Of 1371 articles retrieved, 1209 were excluded based on titles and abstracts. Two authors independently reviewed 10% and 16% of abstracts and full papers, respectively. After full-paper review, 84 studies were included. AIHA was most frequently (32 [52%] of 61) defined as hemolytic anemia with positive direct antiglobulin test (DAT) and exclusion of alternatives, but 10 of 32 also recognized DAT-negative AIHA. A lower threshold for diagnosis of DAT-negative AIHA was observed in literature on chronic lymphocytic leukemia. Definitions of anemia, hemolysis, and exclusion criteria showed substantial variation. Definitions of primary/secondary cold agglutinin disease/syndrome were not consistent. Forty-three studies provided criteria for treatment response, and other than studies from 1 center, these were almost entirely unique. Other criteria were rarely defined. Only 7, 0, 3, 2, 2, and 3 studies offered definitions of warm AIHA, paroxysmal cold hemoglobinuria, mixed AIHA, AIHA severity, disease phase, and refractory AIHA, respectively. Marked heterogeneity in the time period sampled indicates the need to standardize AIHA terminology.
- Anemic Disease of the Newborn With Little Increase in Hemolysis and Erythropoiesis Due to Maternal Anti-Jra: A Case Study and Review of the Literature. [Review]
- TMTransfus Med Rev 2019 Apr 26
- The severity of the hemolytic disease of the fetus and newborn (HDFN) due to Jra mismatch ranges from no symptoms to severe anemia that requires intrauterine and exchange transfusions. We encountered…
The severity of the hemolytic disease of the fetus and newborn (HDFN) due to Jra mismatch ranges from no symptoms to severe anemia that requires intrauterine and exchange transfusions. We encountered a newborn, born to a healthy mother having anti-Jra at 38 weeks of pregnancy, who had moderate anemia, a positive direct antiglobulin test (DAT) result, no increased erythropoiesis, and no jaundice at birth. Flow cytometry revealed that the Jra antigen of red cells in the infant was nearly negative at birth, biphasic at 5 weeks, and lowly expressed at 7 months of life. We searched online for previous case reports on HDFN due to Jra incompatibility. Among 63 reported cases, excluding 25 cases, 38 were included with the present case for analysis. Of 39 newborns, 10 developed clear anemia (hemoglobin <10.0 g/dL), and 1 died, 5 developed hydrops fetalis, 4 needed intrauterine transfusion and/or exchange transfusion, and 3 received red cell transfusion after birth; overlaps were included. Among 29 neonates with no anemia, 8 needed interventions including phototherapy and γ-globulin infusion, and the remaining 21 received conservative supports only. The maternal anti-Jra titer, ranging between 4 and 2048, did not correlate with the severity of anemia, levels of bilirubin, or any interventions required. The DAT of red cells was positive in 29 of 36 fetuses/newborns tested, whereas it was often negative among anemic neonates (4 of 9) (P < .05). Hematopoiesis did not increase effectively, as indicated by reticulocyte ratios between 1.7% and 22.3%, even with the increase in reticulocytes in anemic neonates compared with nonanemic neonates (P < .05). Total bilirubin levels ranged broadly between 0.2 and 14.3 mg/dL but were generally low. The maternal anti-Jra titer and IgG3 subclass did not correlate with the morbidity of the newborns. Being identical/compatible between mothers and their infants may possibly enhance infants' morbidity, as a weak tendency was observed (P = .053). Maternal anti-Jra may suppress erythropoiesis in fetuses via a mechanism different from the established HDFN, such as anti-D, as evidenced by the lower reticulocyte count and small increase in bilirubin in neonates. As the anti-Jra titer, IgG subclass, and DAT were not correlated with the severity, the mechanism of anti-Jra-induced HDFN remains to be elucidated.
- Complete Remission of Associative Immune-Mediated Hemolytic Anemia in a Dog Following Surgical Resection of Intestinal Leiomyosarcoma. [Case Reports]
- VSVet Sci 2019 Jun 13; 6(2)
- A twelve-year-old male castrated Chihuahua with a severe, microcytic, hypochromic, and nonregenerative direct antiglobulin test positive anemia characterized by marked spherocytosis was referred to t…
A twelve-year-old male castrated Chihuahua with a severe, microcytic, hypochromic, and nonregenerative direct antiglobulin test positive anemia characterized by marked spherocytosis was referred to the veterinary hospital. Abdominal ultrasound revealed a peritoneal mass of unclear origin. Transfusion, followed by mass resection, rapidly resolved the anemia without further immunosuppressive treatment. Histopathology confirmed extraluminal jejunal leiomyosarcoma. Multiple mechanisms, including immune-mediated destruction, likely contributed to the anemia. To the authors' knowledge, this is the first report that describes the resolution of immune-mediated hemolysis in a dog after the removal of an intestinal neoplasm.
- Evaluation of Rh-Hemolytic Disease in Neonates and Management with Early Intensive Phototherapy in the Neonatal Intensive Care Unit. [Journal Article]
- JTJ Trop Pediatr 2019 Jun 14
- CONCLUSIONS: With a mean SI of 56.260 ± 8.768 µW/cm2/nm on the trunk, TSB remained below 18 mg/dl in majority thereby avoiding exchange transfusion. Early or late PRBC transfusion requirement was 1 (1-2) (median ± interquartile range).
- Specific activation of pro-Infliximab enhances selectivity and safety of rheumatoid arthritis therapy. [Journal Article]
- PBPLoS Biol 2019; 17(6):e3000286
- During rheumatoid arthritis (RA) treatment, long-term injection of antitumor necrosis factor α antibodies (anti-TNFα Abs) may induce on-target toxicities, including severe infections (tuberculosis [T…
During rheumatoid arthritis (RA) treatment, long-term injection of antitumor necrosis factor α antibodies (anti-TNFα Abs) may induce on-target toxicities, including severe infections (tuberculosis [TB] or septic arthritis) and malignancy. Here, we used an immunoglobulin G1 (IgG1) hinge as an Ab lock to cover the TNFα-binding site of Infliximab by linking it with matrix metalloproteinase (MMP) -2/9 substrate to generate pro-Infliximab that can be specifically activated in the RA region to enhance the selectivity and safety of treatment. The Ab lock significantly inhibits the TNFα binding and reduces the anti-idiotypic (anti-Id) Ab binding to pro-Infliximab by 395-fold, 108-fold compared with Infliximab, respectively, and MMP-2/9 can completely restore the TNFα neutralizing ability of pro-Infliximab to block TNFα downstream signaling. Pro-Infliximab was only selectively activated in the disease site (mouse paws) and presented similar pharmacokinetics (PKs) and bio-distribution to Infliximab. Furthermore, pro-Infliximab not only provided equivalent therapeutic efficacy to Infliximab but also maintained mouse immunity against Listeria infection in the RA mouse model, leading to a significantly higher survival rate (71%) than that of the Infliximab treatment group (0%). The high-selectivity pro-Infliximab maintains host immunity and keeps the original therapeutic efficiency, providing a novel strategy for RA therapy.
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- Implementation of Molecular RHD Typing at Two Blood Transfusion Institutes from Southeastern Europe. [Journal Article]
- TMTransfus Med Hemother 2019; 46(2):114-120
- CONCLUSIONS: Rh molecular testing was successfully implemented in both blood transfusion institutes in Banja Luka and Belgrade. This study proved the efficiency of serological algorithms for weak D, as well as the presence of the RHD gene among serologically tested RhD-negative, C/E-positive samples.