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Anemia is a decrease in the total amount of red blood cells (RBCs) or hemoglobin in the blood, or a lowered ability of the blood to carry oxygen. When anemia comes on slowly, the symptoms are often vague and may include feeling tired, weakness, shortness of breath, and a poor ability to exercise. When the anemia comes on quickly, symptoms may include confusion, feeling like one is going to pass out, loss of consciousness, and increased thirst. Anemia must be significant before a person becomes noticeably pale. Additional symptoms may occur depending on the underlying cause. For people who require surgery, pre-operatve anemia can increase the risk of requiring a blood transfusion following surgery.
Myelodysplastic syndromes (MDS) are a group of cancers in which immature blood cells in the bone marrow do not mature, so do not become healthy blood cells. Early on, no symptoms typically are seen. Later, symptoms may include feeling tired, shortness of breath, bleeding disorders, anemia, or frequent infections. Some types may develop into acute myeloid leukemia.
Aplastic anemia is a disease in which the body fails to produce blood cells in sufficient numbers. Blood cells are produced in the bone marrow by stem cells that reside there. Aplastic anaemia causes a deficiency of all blood cell types: red blood cells, white blood cells, and platelets.
Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications are drugs that inhibit or prevent activity of the immune system.
Fanconi anaemia (FA) is a rare genetic disease resulting in impaired response to DNA damage. Although it is a very rare disorder, study of this and other bone marrow failure syndromes has improved scientific understanding of the mechanisms of normal bone marrow function and development of cancer. Among those affected, the majority develop cancer, most often acute myelogenous leukemia, and 90% develop bone marrow failure by age 40. About 60–75% of people have congenital defects, commonly short stature, abnormalities of the skin, arms, head, eyes, kidneys, and ears, and developmental disabilities. Around 75% of people have some form of endocrine problems, with varying degrees of severity.
Transplant rejection occurs when transplanted tissue is rejected by the recipient's immune system, which destroys the transplanted tissue. Transplant rejection can be lessened by determining the molecular similitude between donor and recipient and by use of immunosuppressant drugs after transplant.
Anti-thymocyte globulin (ATG) is an infusion of horse or rabbit-derived antibodies against human T cells, which is used in the prevention and treatment of acute rejection in organ transplantation and therapy of aplastic anemia.
Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood. It may be autologous, allogeneic or syngeneic.
Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. These immune cytopenias may occur simultaneously or sequentially.
Cytopenia is a reduction in the number of mature blood cells. It is common in cancer patients being treated with radiation and/or chemotherapy.
Chicken anemia virus, or CAV, is currently a member of the Anelloviridae family which is found worldwide. The virus only affects chickens. CAV is a non-enveloped icosahedral single stranded DNA virus, which causes bone marrow atrophy, anemia, and severe immunosuppression. Clinical signs of CAV infection are predominantly found in young chicks due to vertical transmission from the breeder hens whose maternal antibodies have not yet formed following exposure. Clinical disease is rare today because of the widespread practice of vaccinating breeders, but the subclinical form of the disease—which normally affects birds more than two weeks of age following horizontal transmission of the virus via the fecal-oral route—is ubiquitous. The virus is very resistant in the environment, making elimination very difficult.
Diamond–Blackfan anemia (DBA) is a congenital erythroid aplasia that usually presents in infancy. DBA causes low red blood cell counts (anemia), without substantially affecting the other blood components, which are usually normal. This is in contrast to Shwachman–Bodian–Diamond syndrome, in which the bone marrow defect results primarily in neutropenia, and Fanconi anemia, where all cell lines are affected resulting in pancytopenia.
Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells. There are multiple etiologies that can cause PRCA. The condition has been first described by Paul Kaznelson in 1922.
Revesz syndrome is a fatal disease that causes exudative retinopathy and bone marrow failure. Other symptoms include severe aplastic anemia, intrauterine growth retardation, fine sparse hair, fine reticulate skin pigmentation, ataxia due to cerebellar hypoplasia, and cerebral calcifications. Its effects are similar to that of Hoyeraal-Hreidarsson syndrome. It is a variant of dyskeratosis congenita.
Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain, anemia, swelling in the hands and feet, bacterial infections and stroke. Long-term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years.
Bone marrow failure occurs in individuals who produce an insufficient amount of red blood cells, white blood cells or platelets. Red blood cells transport oxygen to be distributed throughout the body’s tissue. White blood cells fight off infections that enter the body. Bone marrow also contains platelets, which trigger clotting, and thus help stop the blood flow when a wound occurs.
Guo Mei is a hematologist and associate director of 307th Hospital of Chinese People’s Liberation Army and deputy director of Radiation Research Institute.
Thymoglobulin is an anti-human thymocyte immunoglobulin preparation made of purified polyclonal antibodies derived from rabbits. While these antibodies have a variety of specificities, their main mechanism of immunosuppression is through depletion of T cells. Thymoglobulin is currently approved for clinical use in Europe and the United States for renal allograft rejection, prevention of graft-vs.-host disease, and conditions involving bone marrow failure, including aplastic anemia and has additional off-label uses.
Hoyeraal–Hreidasson syndrome) is a very rare multisystem X-linked recessive disorder characterized by excessively short telomeres and is considered a severe form of dyskeratosis congenita. Being an X-linked disorder, Hoyeraal–Hreidasson syndrome primarily affects males. Patients typically present in early childhood with cerebellar hypoplasia, immunodeficiency, progressive bone marrow failure, and intrauterine growth restriction. The primary cause of death in Hoyeraal–Hreidasson syndrome is bone marrow failure, but mortality from cancer and pulmonary fibrosis is also significant.
Shimon Slavin, M.D., is an Israeli professor of medicine. Slavin pioneered the use of immunotherapy mediated by allogeneic donor lymphocytes and innovative methods for stem cell transplantation for the cure of hematological malignancies and solid tumors, and using hematopoietic stem cells for induction of transplantation tolerance to bone marrow and donor allografts.
References
- ↑ Hakim, Nadey; Danovitch, Gabriel (2013). Transplantation Surgery. Springer Science & Business Media. p. 11. ISBN 9781447136897.
- ↑ Verghese, PS; Dunn, TB; Chinnakotla, S; Gillingham, KJ; Matas, AJ; Mauer, MS (January 2014). "Calcineurin inhibitors in HLA-identical living related donor kidney transplantation". Nephrology, Dialysis, Transplantation. 29 (1): 209–18. doi:10.1093/ndt/gft447. PMC 3888312 . PMID 24414376.
- 1 2 Frickhofen, N; Kaltwasser, JP; Schrezenmeier, H; Raghavachar, A; Vogt, HG; Herrmann, F; Freund, M; Meusers, P; Salama, A; Heimpel, H (9 May 1991). "Treatment of aplastic anemia with antilymphocyte globulin and methylprednisolone with or without cyclosporine. The German Aplastic Anemia Study Group". The New England Journal of Medicine. 324 (19): 1297–304. doi:10.1056/NEJM199105093241901. PMID 2017225.
- ↑ Kaya B, Davies CE, Oakervee HE, Silver NC, Gawler J, Cavenagh JD (September 2005). "Guillain Barré syndrome precipitated by the use of antilymphocyte globulin in the treatment of severe aplastic anaemia". J. Clin. Pathol. 58 (9): 994–5. doi:10.1136/jcp.2004.020354. PMC 1770826 . PMID 16126887.
- ↑ Lichtman, Marshall A.; Spivak, Jerry L.; Boxer, Laurence A.; Shattil, Sanford J.; Henderson, Edward S., eds. (2000). "Commentary on and reprint of Speck B, Gluckman E, Haak HL, van Rood JJ, Treatment of aplastic anaemia by antilymphocyte glubulin with and without allogeneic bone marrow infusions, in Lancet (1977) 2:1145–1148". Hematology landmark papers of the twentieth century. San Diego: Academic Press. pp. 709–714. doi:10.1016/B978-012448510-5.50159-X. ISBN 978-0-12-448510-5.
- ↑ Speck, B; Gratwohl, A; Nissen, C; Osterwalder, B; Würsch, A; Tichelli, A; Lori, A; Reusser, P; Jeannet, M; Signer, E (1987). "A comparison between ALG and bone marrow transplantation in treatment of severe aplastic anemia". Thymus. 10 (1–2): 147–58. PMID 3324403.