Who really discovered aplastic anemia?
نویسنده
چکیده
Andrea Bacigalupo has recently published very useful guidelines for the treatment of severe aplastic anemia. As in the most recent and authoritative monograph on this condition, «the empty bone marrow and its devastating consequences for the patient have rightly fascinated hematologists for a century». The term itself is somewhat of a misnomer since the disease consists of a hypo-aplastic pancytopenia rather than a monophyletic marrow failure such as pure red cell aplasia (PRCA). Paul Ehrlich is often credited with the first desciption of aplastic anemia, but I believe that we should now accept the idea of a stepwise historical identification of the disease. In 1888 Ehrlich, then a young physician, described the case of a young woman with extremely severe and ultimately fatal pancytopenia, in whom an autopsy performed by Dr. Israel disclosed a yellow femoral marrow, in contrast with the reddish appearance that one was used to seeing in most anemias. The anemia was considered pernicious, since that term was originally employed to describe fatal evolution. Probably at my instigation, Young and Alter credit Chauffard with introducing the term aplastic; however, Chauffard’s patient had hepatosplenomegaly, with a spleen 14 cm in diameter, and the author himself utilizes the term aplastic as a quotation from a former paper, a masterful publication by Vaquez. The patient died after a progressive loss of red cells from 0.85310 /L (on the day of admittance) to 0.3310/L. Histologic examination revealed that the bone marrow was fatty throughout, with a few lymphocytes. The marrow was «as stricken by sterility» (frappée de stérilité). The term aplastic was utilized to define this condition. Thus one can reasonably state that Paul Ehrlich described perhaps the first case of this condition, and that Vaquez and Aubertin were the first to analyze it in depth and confer on it the name which we still use today.
منابع مشابه
Abnormal quantity and function of Th17 cells in patients with severe aplastic anemia combined with infection
Severe aplastic anemia (SAA) is a hematologic disease characterized by peripheral pancytopenia with bone marrow failure. Fatal bacterial or invasive fungal infections are the most frequent cause of mortality in SAA. Th17 cells are a recently discovered CD4+ T-helper subset that secrete cytokines such as interleukin 17A (IL-17A) and subsequently provide protective immunity against infections and...
متن کاملOutcome of pregnancy and disease course among women with aplastic anemia treated with immunosuppression.
BACKGROUND Aplastic anemia may develop during pregnancy and sometimes improves spontaneously after delivery. The effects of pregnancy on aplastic anemia after immunosuppressive treatment and of aplastic anemia on the outcome of pregnancy have not been described. OBJECTIVE To determine the outcome of pregnancy and the disease course among women with aplastic anemia who received immunosuppressi...
متن کاملALLOGENIC BONE MARROW TRANSPLANTATION IN APLASTIC ANEMIA
Eighteen patients, twelve men and six women, with aplastic anemia underwent allogenic bone marrow transplantation (BMT) from HLA-matched siblings during the period of 1990 to 1996. The conditioning regimen was cyclophosphamide with or without busulfan, depending on the cause of aplasia. Antilymphocyte globulin (ALG) and cyclosporine were used for rejection and acute GVHD prophylaxis, respe...
متن کامل5-year survival after bone marrow transplantation for aplastic anemia.
The International Bone Marrow Transplant Registry reported experience from 179 transplant teams worldwide on patients treated with bone marrow transplantation for severe aplastic anemia. The cohort consisted of 470 patients (283 male, 187 female), mean age 20 years, who received an HLA-identical sibling bone marrow transplant for aplastic anemia between 1988 and 1992. The etiology of aplastic a...
متن کاملMyelodysplastic syndrome evolving from aplastic anemia treated with immunosuppressive therapy: efficacy of hematopoietic stem cell transplantation.
A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including post-aplastic anemia myelodysplastic syndrome. Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic hematopoie...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Haematologica
دوره 80 3 شماره
صفحات -
تاریخ انتشار 1995