Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extramedullary hematopoiesis (EMH) is observed in people suffering from severe anemia of prolonged duration and appears to be a compensatory mechanism for disturbed medullary hematopoiesis. The hemoglobinopathies (such as thalassemia, spherocytosis, and sickle cell disease), neoplastic diseases such as leukemia and lymphoma, and others, including myelofibrosis and osteitis fibrosa cystica, are associated with EMH. These diseases and their resultant anemia have in common the ability to stimulate erythropoietin production, which in turn may stimulate hematopoiesis in organs of mesenchymal origin. The liver and spleen are the most common sites of EMH; however, other sites, including the falx cerebri, thoracic cavity, retroperitoneal area and pelvis have been reported. When present, intrathoracic EMH is most frequently associated with thalassemia. Spinal cord compression and hemothorax have also been reported as complications of intrathoracic EMH.
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PMID:Asymptomatic intrathoracic extramedullary hematopoiesis: a report of three cases. 262 Nov 1

On July 7h, 1988 all Italian groups practicing allogeneic bone marrow transplantation (AlloBMT) convened with the objectives of performing an analysis of their clinical material and designing some cooperative retrospective and prospective studies. It was felt that, although the great majority of the Institutions performing AlloBMT contribute data to the International Bone Marrow Transplant Registry (IBMTR) and to the European Group for Bone Marrow Transplantation (EBMT), it might still be of interest to pursue specific regional studies, establish a National Registry, and program workshops and educational meetings. The Italian Group for Bone Marrow Transplantation (GITMO) was accordingly founded. It has been determined that by December 31st, 1988, 1390 AlloBMTs have been performed in Italy by 19 Centres. Fourteen have been involved in BMT for adults and children, and 5 exclusively for children. The chief indication for AlloBMT in this clinical material was chronic myelogenous leukaemia, followed by homozygous beta thalassaemia and by the acute leukaemias; severe aplastic anaemia, malignant lymphomas and multiple myeloma have also been important indications. Overall crude survival was 58.3%; this was reduced to 50% for patients over 20, while it reached 62.5% for those under 20. Other studies are in preparation, and a similar survey for Auto BMT has been presented at the GITMO Meeting of June 28, 1989, which is currently in press.
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PMID:[Allogenic bone marrow transplant in Italy]. 269 Feb 21

Splenic abscess is an unusual disease and may be presented either as a localized area of infection in the spleen or as a part of generalized sepsis. Population-based autopsy studies have established the incidence of splenic abscess at between 0.2-0.7 per cent. An eleven-year retrospective study of cases of splenic abscess treated at Siriraj hospital, a total of 9 cases, is presented. Pseudomonas pseudomallei is the most frequent causative agent, found in one-third of the cases, especially if the patient is thalassemic or a resident in the Northeastern part of the country. Thalassemia is also the leading predisposing condition of this malady with the incidence of 33 per cent. There are some differences in the presenting clinical features in Thai patients compared with those reported in the literature. Splenectomy was performed in all but one who died of leukemia preoperatively. The mortality rate of this disease in this series is 11 per cent and we recommend splenectomy under antibiotic coverage as soon as the diagnosis of splenic abscess has been confirmed.
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PMID:Splenic abscess at Siriraj Hospital, Thailand. 280 52

Since freshly obtained acute lymphoblastic leukemia (ALL) cells rarely replicate spontaneously in vitro in a sustained way, development of a useful clonogenic assay for ALL blast progenitors is dependent on identifying the cellular growth requirements. Thus, marrows from 25 ALL cases were cultured in methylcellulose to determine the optimal conditions for cell growth. Blast colonies were confirmed as leukemic by morphology, cytochemistry, surface markers, and cytogenetics. Irradiated (7000 rads) normal peripheral blood feeder cells were an absolute requirement and produced number-dependent increases in ALL colonies; added growth factors enhanced the feeder cell effect. ALL cell-feeder cell contact was essential since their physical separation in a two-layer culture system drastically interfered with colony growth. Feeder cells from various donors, including new and relapsed cases of ALL, yielded colony numbers that differed widely when tested on the same marrow with and without added growth factor; thus, identification of a "good" feeder cell donor was key to an optimal assay. Neither recombinant interleukin-2 nor recombinant GM-CSF had ALL growth-promoting properties when tested alone or in combination but in the presence of feeder cells they moderately enhanced the feeder cell effect. The most effective growth factors were derived from cells exposed to phytohemagglutinin (PHA) for 72 h. In order of magnitude for colony growth-promoting activity, PHA-T cell conditioned medium (CM) was more stimulatory than PHA-blast cell CM followed by PHA-leukocyte CM; removal of PHA from CM by affinity chromotography did not alter the results. The most potent PHA-TCM was prepared from T-cells from a phlebotomized hemochromatosis patient; PHA-TCM from transfused thalassemia patients and normal donors were less active. Concanavalin-A blast cell CM had modest colony promoting properties whereas CM prepared with other B-cell mitogens and supernatants from ALL blasts in liquid culture had none. Our studies illustrate the complex and fastidious growth needs of ALL cells. The data have allowed us to refine a clonogenic blast progenitor assay that should facilitate study of proliferative properties of B and T lineage leukemias. The assay could be adapted further for detection of residual leukemia cells in marrow samples used for autologous transplantation, and in patients during complete hematological "remission."
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PMID:Growth requirements for human acute lymphoblastic leukemia cells: refinement of a clonogenic assay. 304 51

Various kinds of lesions exist which should be discriminate from malignant or premalignant or borderline lesions. If there were a morphologic technical procedure on detection of malignant transformation of the cells at the initiation stage, before the lesion would develop a definitely identical with malignant lesion, such method must be most highly applicable for pathologists. DNA diagnosis has realized a warning of diagnosis of certain diseases or genetical maldevelopment prior to develop their clinical manifestation. Gene analysis has introduced in ++phragmatical screening test for certain diseases such as diabetes mellitus, thalassemia, T-cell leukemia or lymphoma, neuroblastoma, muscular dystrophy of Duchenne or Becker type, Ph' chromosome and so on. Immunohistochemical technology has provided an intracellular oncogene detection in some neoplastic malignancies such as n-myc in neuroblastoma. Amplification of c-erb B2 (also referred as neu and HER-2/neu) has indicated a higher malignant mammary carcinoma with poor-prognosis, even their size small and early stage. Oncogene analysis is expected to be available sperimposing on pathological morphology.
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PMID:[Detection of early stage cancer: pathological aspect with special reference to differential diagnosis]. 317 85

A 64-year-old man was found to have a hemolytic anemia with a hematocrit of 0.28 (28%) during a routine evaluation. One month before this his hematocrit had been normal. Further studies revealed a myelodysplastic syndrome and acquired hemoglobin H disease. Eighteen months later this transformed into acute megakaryoblastic leukemia with disappearance of hemoglobin H, and shortly thereafter he had myelofibrosis develop. Acquired hemoglobin H disease, which is an alpha-thalassemia-like syndrome, results in the formation of an unstable hemoglobin composed of beta chain tetramers. This condition has been associated with preleukemia, sickle cell anemia, and hematologic malignancies. Although idiopathic myelofibrosis also has been described as a setting in which this thalassemic syndrome occurs, the present case is unusual in that the myelofibrosis was preceded by refractory anemia with leukemic transformation.
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PMID:Myelodysplastic syndrome with acquired hemoglobin H disease. Evolution through megakaryoblastic transformation into myelofibrosis. 327 51

This study reviews results of fetal liver transplantation in hematologic disorders including aplastic anemia, leukemia and thalassemia. One hundred and twenty two patients received transplants for aplastic anemia; engraftment was reported in 4 patients; graft-versus-host disease (GvHD) did not occur. Complete and partial responses were reported in one-half of patients, the majority of whom had no evidence of engraftment. Thirty-nine patients received transplants for leukemia. Transient engraftment was reported in 40% and two developed GvHD; survival extended to more than 2 years. The higher rate of engraftment in patients with leukemia suggests a role of pretransplant immune suppression. The risk of GvHD appears to be low despite complete HLA-mismatching. These data suggest a possible role for fetal liver transplantation in man. Future studies should probably be based on preclinical data obtained in large animal models.
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PMID:Fetal liver transplantation in aplastic anemia and leukemia. 332 6

A female patient with therapy-related acute non-lymphoblastic leukaemia occurring after intensive combined modality treatment for IIIB Hodgkin's disease was transplanted from her HLA-identical brother. No attempt was made to induce a remission and the transplant was performed with standard chemoradiotherapeutic conditioning. Three years after the transplant the patient is alive in complete remission with a 46,XY karyotype. The donor has a mild, heterozygous beta-thalassaemia ('thalassaemia minima'). The recipient has much more pronounced thalassaemic features, similar to so-called thalassaemia intermedia, with peripheral blood smears indistinguishable from thalassaemia major. The erythrocytic alterations are undoubtedly due to the splenectomy which was performed during a staging procedure, but the marked difference between donor and recipient haemoglobin values cannot be attributed solely to the asplenic state.
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PMID:Bone marrow transplantation for secondary (therapy-related) acute non-lymphoblastic leukaemia: report of a case associated with adoptive beta-thalassaemia. 333 61

Toxicity to the bone marrow is a frequent limiting factor in the use of high doses of chemotherapeutic agents. Bone marrow transplantation overcomes the marrow toxicity problem, but it is not protective to other organs. Extensive animal studies have been carried out in the mouse, the rat, rhesus monkeys, and dogs to delineate the dose-limiting toxicity of cyclophosphamide (Cytoxan) (CY) therapy. Studies in the dog have shown 100 mg/kg of CY to be lethal with supportive care alone. Dogs given this dose followed by stored autologous marrow recovered after a period of profound pancytopenia and severe gastrointestinal toxicity. This dose of CY also permitted allogeneic engraftment in the dog. Monkeys given up to 200 mg/kg of CY have uneventful hematopoietic recovery, but doses of 240 mg/kg were generally fatal even when stored autologous marrow was infused. Cardiac toxicity was the limiting factor. CY 180 mg/kg was not lethal and permitted successful allogeneic marrow engraftment. CY is successfully used for conditioning leukemia or aplastic anemia patients for bone marrow transplantation. Patients with severe aplastic anemia are conditioned with CY 50 mg/kg on each of four days followed by allogeneic marrow transplantation. Patients undergoing transplantation before transfusion have a long-term survival rate of about 80%. Patients with genetic disorders of the marrow generally have a normocellular or hypercellular marrow, and the preparative regimen must include destruction of the abnormal marrow as well as immunosuppression sufficient to permit engraftment. Patients with thalassemia are treated with dimethylbusulfan 5 mg/kg or busulfan 14 mg/kg followed by CY 50 mg/kg on each of four days. Approximately 100 thalassemia patients have been treated, with a survival rate of approximately 75%. For patients with leukemia, radiotherapy is generally added to the CY conditioning regimen. In the early Seattle studies, 1,000 rad total body irradiation was combined with CY 60 mg/kg on each of two days. There were many early deaths, but some long-term survivors are alive and well 5 to 13 years later. Current regimens involve fractionated total body irradiation and various post-grafting immunosuppressive regimens designed to prevent graft-v-host disease. Complications and problems of current regimens are discussed, and future goals for marrow transplantation are presented.
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PMID:High-dose therapy and bone marrow transplantation. 390 18

Bone marrow transplantation is increasingly used to treat a broad spectrum of human diseases including aplastic anemia, leukemia, solid tumors, immune and genetic disorders. In certain circumstances the role of transplantation is reasonably well established, such as aplastic anemia and resistant leukemia. In other circumstances there is controversey as to the role of transplantation such as leukemia in remission. An increasing number of genetic disorders including severe combined immunodeficiency, Wiskott-Aldrich syndrome, osteopetrosis, and Thalassemia have been cured by transplantation. Despite substantial progress, with transplantation that remain to be solved including graft-vs.-host disease, interstitial pneumonia, immune deficiency, and the lack of suitable donors for most potential recipients. These problems and potential approaches are discussed in detail Future direction of research include the application of transplantation to other diseases as well as the use of this approach either as a prelude to solid-organ grafts or as a vehicle for the introduction of new genetic information.
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PMID:Bone marrow transplantation. 391 31


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