Guest Editor: Raffaella Origa IRON TOXICITY AND HEMOPOIETIC CELL TRANSPLANTATION: TIME TO CHANGE THE PARADIGM.

The issue of iron overload in hemopoietic cell transplantation has been first discussed in the field of transplantation for thalassemia. Thalassemia major is characterized by ineffective erythropoiesis and hemolysis leading to anemia in the majority of patients. Patients require regular blood transfusion therefore they develop iron overload causing organ damage and hematopoietic cell transplantation (HCT) is a consolidated reliably curative option.In this category of patients an important issue for transplant outcome is the iron burden before transplant and in the long-life post-transplant. Nevertheless, today the concept of the impact of iron overload / toxicity on the outcome of HCT) has been extended to other diseases characterized by periods of variable duration of transfusion dependenceRecent preclinical data has shown how increased production of reactive oxygen species (ROS) resulting under iron overload condition, could impair the stem cells clonality capacity, proliferation and maturation. Also, microenvironment cells could be affected through this mechanism. For this reason, iron overload is becoming an important issue also in the engraftment period post-transplantThe aim of this review is to update consolidated knowledge about the role of iron overload/iron toxicity in the HCT setting in non-malignant and in malignant diseases introducing the concept of exposition of free toxic iron forms and related cellular damage in the different stage of transplant.
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research

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Abstract Thalassemia syndromes are a group of inherited blood disorders caused by limitation or absence of alpha or beta- globin chain synthesis resulting in ineffective erythropoiesis and chronic hemolytic anemia. According to the clinical fact of thalassemia as recessive autosomal, thalassemia divided into alpha and beta thalassemia. The main complication of beta thalassemia is excessive red blood cells destruction, oxidative stress, extramedullary hematopoiesis pseudotumors, hemosiderosis-induced organ dysfunction, malignancy, polyneuropathy, myopathy, osteoporosis, and leg ulcers. Although, the first obligator...
Source: Current Drug Targets - Category: Drugs & Pharmacology Authors: Tags: Curr Drug Targets Source Type: research
AbstractImmunosuppressive therapy (IST), consisting of anti thymocyte globulin and cyclosporine, is the standard of care for elderly patients and in younger patients without matched donor for stem cell transplant. In this retrospective study of 60 patients, most had non severe AA (51.6%) followed by very severe AA (26.7%) and severe AA (21.7%). The response rate at 6 months was 68.3% (complete response-3.3% and partial response-65%). Of the 45 who completed 12 months follow up, RR was 54.7% (CR-7.5%, PR-47.2 %). Eight patients died during the study period. Also, Indian data on IST is briefly reviewed.
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research
Aplastic anemia (AA) is a hematologic disease characterized by pancytopenia and hypocellular bone marrow, potentially leading to chronic anemia, hemorrhage, and infection. The China Aplastic Anemia Committee and British Committee for Standards in Haematology guidelines recommend hematopoietic stem-cell transplantation (HSCT) or immunosuppressive therapy (IST) comprising antithymocyte globulin (ATG) with cyclosporine (CsA) as initial treatment for AA patients. With limited epidemiological data on the clinical management of AA in Asia, a prospective cohort registry study involving 22 AA treatment centers in China was conduct...
Source: Acta Haematologica - Category: Hematology Source Type: research
Abstract Aplastic anemia (AA) is a hematologic disease characterized by pancytopenia and hypocellular bone marrow, potentially leading to chronic anemia, hemorrhage, and infection. The China Aplastic Anemia Committee and British Committee for Standards in Haematology guidelines recommend hematopoietic stem-cell transplantation (HSCT) or immunosuppressive therapy (IST) comprising antithymocyte globulin (ATG) with cyclosporine (CsA) as initial treatment for AA patients. With limited epidemiological data on the clinical management of AA in Asia, a prospective cohort registry study involving 22 AA treatment centers in...
Source: Acta Haematologica - Category: Hematology Authors: Tags: Acta Haematol Source Type: research
The issue of iron overload in hemopoietic cell transplantation has been first discussed in the field of transplantation for thalassemia. Thalassemia major is characterized by ineffective erythropoiesis and hemolysis leading to anemia in the majority of patients. Patients require regular blood transfusion therefore they develop iron overload causing organ damage and hematopoietic cell transplantation (HCT) is a consolidated reliably curative option.In this category of patients an important issue for transplant outcome is the iron burden before transplant and in the long-life post-transplant. Nevertheless, today the concept ...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
Bone marrow harvesting is the preferred method of hematopoetic stem cell acquisition from allogeneic donors for many types of transplant recipients. Due to the vast amount of red blood cells located in the marrow, BM donors are at an increased risk for anemia following BM donation and therefore may require a blood transfusion. Blood transfusions pose an increased risk of complications.
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Tags: 641 Source Type: research
We present our experience in haploidentical stem cell transplantation (haplo SCT) in children with benign disorders. We performed a retrospective study where children aged up to 18  years diagnosed to have benign disorders and underwent haplo SCT from 2002 to September 2017 were included. Of the 54 children, the most common indications were Fanconi anaemia 12 (22%), severe aplastic anaemia 8 (14%) and primary immune deficiency disorders (PID) 25 (46%). Post-transplant cycloph osphamide (PTCy) was used in 41 (75.9%) and ex vivo T depletion in 13 (24.1%). Engraftment rates were 70% with acute graft versus host disease i...
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research
Conclusions: Amongst patients undergoing major-side or bidirectionally ABO incompatible stem cell transplants, the majority will have cleared their donor-specific isoagglutinins within 120 days of stem cell infusion, although this does not guarantee against later return of reactivity. Thus, the persistence of donor-specific isoagglutinins beyond 120 days post-transplant, or the subsequent return of previously cleared isoagglutinins, may be considered abnormal results which warrant notification of the clinical team by the hospital transfusion service.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 401. Basic Science and Clinical Practice in Blood Transfusion: Poster I Source Type: research
We describe a case of treatment-refractory pure red cell aplasia following a major ABO-mismatchedallogeneic stem cell transplant successfully treated with daratumumab.The 72-year-old patient with MDS received a matched unrelated donor transplant with a major ABO incompatibility, recipient blood group O from blood group A donor. Despite prompt engraftment with WBC and platelet recovery he remained anemic and RBC transfusion dependent. Bone marrow biopsy showed
Source: Blood - Category: Hematology Authors: Tags: 401. Basic Science and Clinical Practice in Blood Transfusion: Poster II Source Type: research
Conclusions: A novel founder mutation in SLC25A38 causing CSA among individuals of Canadian First Nations Northern Cree descent results in a severe transfusion-dependent anemia. Despite a common genetic etiology, phenotypic variability was noted, with one individual having marked tolerance to anemia. A partial transient response to pyridoxine was noted in two individuals, raising the question of an alternative role for SLC25A38 in heme biosynthesis. HSCT, when performed before significant iron overloading, was beneficial. Characterization of this phenotype and evidence of successful HSCT may assist clinicians in identifyin...
Source: Blood - Category: Hematology Authors: Tags: 101. Red Cells and Erythropoiesis, Structure and Function, Metabolism, and Survival, Excluding Iron: Poster I Source Type: research
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