idiopathic Aplastic anemia

Aplastic anemia where the cause is not known. However, an increase in CD8-positive T-CELLS and increased expression of INTERFERON-GAMMA by mononuclear cells in the bone marrow and peripheral blood of patients have been observed. (See: Autoimmun Rev. 2014 Apr-May;13(4-5): 569-73)

Also Known As:

Networked: 66 relevant articles (5 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Aplastic Anemia (Anemia, Hypoplastic)
2.	Fanconi Anemia (Fanconi's Anemia)
3.	Chromosome Breakage
4.	Myelodysplastic Syndromes (Myelodysplastic Syndrome)
5.	Pancytopenia

Experts

1.	Dufour, Carlo: 5 articles (01/2022 - 08/2005)
2.	Aljurf, Mahmoud: 4 articles (01/2022 - 09/2009)
3.	Bacigalupo, Andrea: 4 articles (01/2022 - 08/2005)
4.	Risitano, Antonio M: 3 articles (01/2022 - 01/2019)
5.	Bae, Sung Hwa: 3 articles (01/2020 - 10/2013)
6.	Choi, Jung Hye: 3 articles (01/2020 - 10/2013)
7.	Kim, Hawk: 3 articles (01/2020 - 10/2013)
8.	Kim, Min Kyoung: 3 articles (01/2020 - 10/2013)
9.	Kwak, Jae-Yong: 3 articles (01/2020 - 10/2013)
10.	Lee, Kyoo-Hyung: 3 articles (01/2020 - 10/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to idiopathic Aplastic anemia:

1.	ErythropoietinFDA Link 12/01/1993 - "An immunologic crossreactant of erythropoietin seemed to develop and persist in serum samples from a patient during treatment and remission of idiopathic aplastic anemia. " 07/01/1996 - "Serum erythropoietin (EPO) concentrations were measured by radioimmunoassay in 46 patients with idiopathic aplastic anemia. "
2.	eltrombopagFDA Link 10/01/2018 - "Upfront eltrombopag monotherapy induces stable hematologic remission in pediatric patients with nonsevere idiopathic aplastic anemia." 01/01/2022 - "Response of Eltrombopag in immune thrombocytopenia and acquired idiopathic aplastic anemia: A single-center experience."
3.	Oxymetholone (Anadrol)FDA Link 08/01/1980 - "We have studied a 13-year-old boy with idiopathic aplastic anemia in whom oxymetholone treatment was associated with a partial hematological remission. "
4.	Mitomycin (Mitomycin-C)FDA LinkGeneric 01/01/1981 - "Mitomycin C test for diagnostic differentiation of idiopathic aplastic anemia and Fanconi anemia." 05/01/2004 - "This study was conducted to differentiate between Fanconi anemia (FA) and "idiopathic" aplastic anemia on the basis of induced chromosomal breakage study with mitomycin C (MMC). " 05/01/2004 - "Differentiation of Fanconi anemia from idiopathic aplastic anemia by induced chromosomal breakage study using mitomycin-C (MMC)."
5.	Conditioned Culture MediaIBA 11/01/1980 - "[Studies on erythroid burst forming unit in children--burst promoting activity in children with idiopathic aplastic anemia and human placental conditioned medium (author's transl)]."
6.	Granulocyte Colony-Stimulating Factor (G-CSF)IBA 12/01/1994 - "Herein, using FACS analysis, we examined the expression of surface CD48 on neutrophils activated in vivo by G-CSF administered for the treatment of idiopathic aplastic anemia. " 01/01/1989 - "In 49 of 56 normal healthy persons (88%), the levels were beneath the sensitivity of the assay (less than 30 pg/mL), while in the remaining seven healthy persons, the levels ranged from 33 to 163 pg/mL. On the other hand, nine of 11 patients (82%) with idiopathic aplastic anemia (AA), one patient with Fanconi's anemia, six of 12 patients (50%) with myelodysplastic syndrome (MDS), five of 12 patients (42%) with acute leukemia without any blast cells in the blood (M4: one, M5: one, L1: one, and L2: two), six of 18 patients (33%) with chronic myeloid leukemia (CML), one of two patients with chronic lymphoid leukemia (CLL), two of four patients with lung cancer, one patient with cyclic neutropenia, two of seven patients with malignant lymphoma, and four patients with acute infection had G-CSF levels ranging from 46 pg/mL to greater than 2,000 pg/mL. Interestingly, a reverse correlation between blood neutrophil count and serum G-CSF level was clearly demonstrated for aplastic anemia (r = -.8169, P less than .01). " 01/01/1994 - "We report on our experience with the application of recombinant human granulocyte colony-stimulating factor (G-CSF, Neupogen, Hoffmann La Roche) in patients with various types of hematological malignancies, who had chemotherapy-induced myelosuppression, and in patients with idiopathic aplastic anemia. " 01/01/1993 - "G-CSF at a daily dose of 5-8 micrograms/kg was used in four patients with severe pancytopenia in the course of acute or chronic lymphoid leukemia treated with cytotoxic agents, and in two patients with idiopathic aplastic anemia. "
7.	Antilymphocyte Serum (Anti-Thymocyte Globulin)IBA 12/01/2012 - "To assess the response to antithymocyte globulin based immunosuppressive therapy (IST) in pediatric patients with idiopathic aplastic anemia. " 10/01/2017 - "Long-term risk of cancer development in adult patients with idiopathic aplastic anemia after treatment with anti-thymocyte globulin." 08/01/2023 - "A primary diagnosis of idiopathic aplastic anemia was made, and she was treated with prednisolone, cyclosporine, and anti-thymocyte globulin (ATG), which did not respond. " 01/01/1980 - "A 29-year-old woman with severe idiopathic aplastic anemia was given immunosuppressive therapy with procarbazine, 37.5 mg/kg, antithymocyte globulin, 36 mg IgG/kg, and cyclophosphamide, 200 mg/kg. This was followed by a marrow transplant from her HLA identical sister, immunosuppressive therapy with intermittent methotrexate for 3 months postgrafting and ultimate restoration of hematopoiesis. "
8.	Cyclosporine (Ciclosporin)FDA LinkGeneric 03/21/1994 - "[Cyclosporin in the treatment of idiopathic aplastic anemia]." 01/01/1991 - "We present a patient with severe idiopathic aplastic anemia with no previous chromosomal abnormalities who developed trisomy 21 and monosomy 7 during treatment with intravenous (i.v.) cyclosporine. " 08/01/2023 - "A primary diagnosis of idiopathic aplastic anemia was made, and she was treated with prednisolone, cyclosporine, and anti-thymocyte globulin (ATG), which did not respond. "
9.	HLA Antigens (Human Leukocyte Antigens)IBA 12/02/2016 - "The preferred treatment of idiopathic aplastic anemia (AA) is allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen (HLA)-identical sibling donor. " 01/01/2023 - "Idiopathic aplastic anemia (IAA) pathophysiology is dominated by autoreactivity of human leukocyte antigen (HLA)-restricted T-cells against antigens presented by hematopoietic stem and progenitor cells (HSPCs). " 12/30/2021 - "Idiopathic aplastic anemia (IAA) is a rare autoimmune bone marrow failure (BMF) disorder initiated by a human leukocyte antigen (HLA)-restricted T-cell response to unknown antigens. "
10.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 03/01/1976 - "A 23-year-old man with severe idiopathic aplastic anemia was prepared for marrow transplantation by the administration of cyclophosphamide (CY) 50 mg/kg on each of 4 days. " 07/01/2014 - "Allogeneic hematopoietic cell transplantation without total body irradiation from unrelated donor in adult patients with idiopathic aplastic anemia: fludarabine versus cyclophosphamide-ATG." 01/01/1980 - "A 29-year-old woman with severe idiopathic aplastic anemia was given immunosuppressive therapy with procarbazine, 37.5 mg/kg, antithymocyte globulin, 36 mg IgG/kg, and cyclophosphamide, 200 mg/kg. This was followed by a marrow transplant from her HLA identical sister, immunosuppressive therapy with intermittent methotrexate for 3 months postgrafting and ultimate restoration of hematopoiesis. "

Therapies and Procedures

1.	Hematopoietic Stem Cell Transplantation 01/01/2023 - "Survival after allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe idiopathic aplastic anemia (SAA) has improved in recent years, approaching 75% at 5 years. " 01/01/2022 - "Idiopathic aplastic anemia is a rare and life-threatening disorder, and hematopoietic stem cell transplantation (HSCT) from a matched sibling donor (MSD) is the standard treatment strategy for young patients. " 09/01/2009 - "Older age is a limitation for HLA-identical sibling hematopoietic stem cell transplantation (HSCT) as first-line therapy for severe acquired idiopathic aplastic anemia (SAA). " 01/01/2020 - "Effect of Stem Cell Source and Dose on Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Idiopathic Aplastic Anemia: Data from the Korean Aplastic Anemia Trials." 01/01/2019 - "Impact of T-cell depletion strategies on outcomes following hematopoietic stem cell transplantation for idiopathic aplastic anemia: A study on behalf of the European blood and marrow transplant severe aplastic anemia working party."
2.	Transplantation 08/01/2018 - "This article summarizes the recent development in the field of front-line unrelated donor transplantation for idiopathic aplastic anemia. " 09/01/2015 - "A 7-year-old boy presented with a change in behavior, poor attention, cognitive deficits, and abnormal movements 15 months after undergoing transplantation for idiopathic aplastic anemia. " 10/01/2014 - "We analyzed the outcome of 537 adolescents (age 12-18 years) with idiopathic aplastic anemia included in the database of the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation comparing: i) matched family donor hematopoietic stem cell transplantation performed as first-line treatment with ii) front-line immunosuppressive therapy not followed by subsequent transplant given for failure and with iii) hematopoietic stem cell transplantation performed after failed front-line immunosuppressive therapy. " 03/01/1976 - "A 23-year-old man with severe idiopathic aplastic anemia was prepared for marrow transplantation by the administration of cyclophosphamide (CY) 50 mg/kg on each of 4 days. "
3.	Therapeutics 01/01/2022 - "These findings raise the possibility that STAT1 hyperactivition defines a subset of idiopathic aplastic anemia patients for whom JAK inhibition may be an efficacious therapy. " 09/01/2009 - "Older age is a limitation for HLA-identical sibling hematopoietic stem cell transplantation (HSCT) as first-line therapy for severe acquired idiopathic aplastic anemia (SAA). " 01/01/1976 - "The results of a long-term corticosteroid therapy performed in 55 patients with idiopathic aplastic anemia are reported. " 01/01/1976 - "Long-term corticosteroid therapy of idiopathic aplastic anemia." 02/01/2002 - "We describe a case of primary cutaneous mucormycosis (zygomycosis) in a patient with idiopathic aplastic anemia which responded to surgical debridement and therapy with liposomal amphotericin B. "
4.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 11/01/1985 - "Bone marrow transplantation for severe idiopathic aplastic anemia was undertaken in a patient, using his monozygotic twin brother as the donor. " 07/01/1998 - "The author describes circumstances under which a young man with a severe idiopathic aplastic anemia, pancytopenia and hemorrhagic syndrome had undergone the first successful bone marrow transplantation performed at the Department of Haematology of the Internal Clinic in Novi Sad in 1977. " 01/01/2022 - "Upfront unrelated donor hematopoietic stem cell transplantation in patients with idiopathic aplastic anemia: A retrospective study of the Severe Aplastic Anemia Working Party of European Bone Marrow Transplantation." 11/01/1977 - "A case of multiple small well-differentiated hepatomas with early peliosis hepatis, multiple pancreatic islet cell tumors, and a renal medullary interstitial cell tumor following five years of androgen and prednisone therapy for idiopathic aplastic anemia is reported in a patient who died shortly after allogeneic bone marrow transplantation. "
5.	Cell Transplantation 10/24/2023 - "During the last few years, the therapeutic landscape of idiopathic aplastic anemia (IAA) has been profoundly revolutionized by the increased use of alternative transplant procedures, such that today hematopoietic cell transplantation (HCT) from a matched unrelated donor (MUD) has been suggested as a possible first line strategy in pediatric patients with severe IAA, in the absence of a matched related donor. " 07/01/2014 - "Allogeneic hematopoietic cell transplantation without total body irradiation from unrelated donor in adult patients with idiopathic aplastic anemia: fludarabine versus cyclophosphamide-ATG."