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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a retrospective study we investigated the development of HLA antibodies in patients who received platelet concentrates from cell separators. 118 hematological/oncological patients from the Frankfurt University Clinics were investigated. They received between 4 and 66 platelet concentrates for the duration of 30 months. All patients had a negative antibody screening on admission. 31% developed either transient (15%) or permanent (16%) lymphocytotoxic antibodies. The increasing number of platelet transfusions did not correlate with the development of HLA antibodies, but the appearance of these antibodies seemed to be dependent on the disease. Permanent antibodies appeared in 8% of patients with acute leukemia, whereas 38% of patients suffering from CL, lymphoma, MDS and myeloma produced antibodies. Some patients (18) received granulocyte transfusions as well. It is striking that 11% of these patients developed permanent and 28% transient HLA antibodies. There exist no data about recent transfusions or previous pregnancies. To lower the rate of sensitization in patients with diseases such as CL, lymphoma, MDS and myeloma, it should be discussed whether leukocyte-depleted platelet concentrates should be given to these patients.
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PMID:[Development of HLA antibodies in thrombocyte substitution with cell separator products]. 128 49

Examples of patients are used as a basis for discussing the treatment of severe aplastic anaemia with clonal chromosomal aberrations in haematopoietic cells, the aplastic form of paroxysmal nocturnal haemoglobinuria and myelodysplasia, with allogeneic bone marrow transplantation. Transplantation with marrow from an HLA-identical sibling donor has curative potential in selected patients with these disorders. The procedure should preferably be carried out before the patients have received massive treatment for the complications of marrow failure. The use of matched unrelated bone marrow donors is still at the investigation stage.
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PMID:[New therapeutic possibilities for acquired clonal blood diseases and myelodysplasia]. 141 33

A 9-year-old boy was admitted with the diagnosis of myelodysplastic syndrome (FAB RAEB in T). The patient was treated with busulfan and cyclophosphamide and transplanted with bone marrow cells from an HLA identical sister. Cyclosporin A (CyA) and short term methotrexate (MTX) was given for prophylaxis against graft versus host disease (GvHD). The serum potassium value was observed to increase to 6.3 mEq/l during the period of CyA therapy. The serum potassium value returned to 4 mEq/l when CyA treatment was decreased to a serum concentration of less than 50 ng/ml (FPIA). On day 90 post transplantation the patient was diagnosed as relapsed. The patient was preconditioned with cyclophosphamide and total body irradiation and a second bone marrow transplantation was performed using cells from the same donor. He was treated again with CyA and short term MTX for the prevention of GvHD. Once again the patient became hyperkalemic with 6.8 mEq/l. The serum creatinine level was 0.9 mg/dl, the GFR was 52.1 ml/min, FEK was 7.1%. Pseudohypoaldosteronism or hyporeninemic hypoaldosteronism was suspected. To investigate this possibility a renin/aldosterone stimulation test was performed. We speculate that an idiosyncratic response to CyA resulted in pseudohypoaldosteronism and produced a defect in potassium secretion.
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PMID:[Hyperkalemia in a cyclosporine A-treated allogeneic bone marrow transplant recipient]. 154 16

For severe aplastic anemia and several malignant hemopathies allogeneic bone marrow transplantation is the only treatment with curative potential. This is the case for chronic myelogenous leukemia, the myelodysplastic syndromes and probably multiple myeloma and chronic lymphocytic leukemia. It seems also the best therapeutic option for young adults who suffer from acute leukemia and for whom an adequate family donor is available. We review here the main complications of the procedure. Their better knowledge and the way to prevent and to treat them has decreased the mortality and morbidity of this treatment which is mostly successful when applied on patients in the early phase of their disease. Recently, the availability of HLA typed registered volunteers has extended the applicability of allogeneic bone marrow transplantation for those patients who lack adequate familial donors.
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PMID:[Bone marrow allograft in adults hemopathies. The Team of the Sterile Unit]. 160 90

A patient with polyarthritis, peripheral mononeuritis multiplex with spatial and temporal fluctuation, and eczematous, ulcerative skin rash in the lower extremities was found to have myelodysplastic syndrome (MDS) in the bone marrow and concomitant large granular lymphocytosis in the peripheral blood. Histochemical study showed that cells with large granular lymphocyte markers (CD2+, 11b+, 16+, 57+, HLA-DR+) had infiltrated into the skin and around the nerve fibers. Both the bone marrow dyscrasia and rheumatic manifestations of this patient improved significantly after prednisolone therapy. The unusual rheumatologic manifestations of this patient appear to derive from a delicate balance between MDS and large granular lymphocytosis.
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PMID:Polyarthritis, mononeuritis multiplex and eczematous ulcerative skin rash in a patient with myelodysplastic syndrome and peripheral large granular lymphocytosis. 172 42

A 36-year-old, previously healthy woman was referred to our institution with pancytopenia and splenomegaly for suspected acute leukemia. Bone marrow aspiration showed marked dysplastic changes, excess of blasts, and only spurious red blood cell precursors. Action was taken to prepare allogeneic bone marrow transplantation from an HLA identical sibling for myelodysplastic syndrome. Repeat cytological examination of the bone marrow revealed striking hyperplasia of the red cell line with presence of abnormal giant proerythroblasts. Acute parvovirus B19 infection was suspected and confirmed by detection of anti-B19 IgM and B19 DNA. The underlying disease for this transient aplastic crisis was a formerly unknown hereditary spherocytosis.
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PMID:Acute parvovirus B19 infection mimicking myelodysplastic syndrome of the bone marrow. 173 58

A combination of cyclosporine (CSA) and methylprednisolone (MP) was used as graft-versus-host disease (GVHD) prophylaxis in 25 patients age 11-47 years (median 27 years) who received HLA-compatible sibling marrow transplants after myeloablative therapy for leukemia, myelodysplasia or lymphoma. CSA was initiated at 3 mg/kg/day in two divided doses, and the dose was adjusted to maintain a trough whole blood h.p.l.c. concentration between 200 and 800 ng/ml. While on i.v. CSA, the dose of CSA was increased for 10 of the 25 patients. The actuarial rate of grades II-IV acute GVHD was 37%. Those patients who developed moderate to severe GVHD had a significantly higher early mortality than those who did not (56% vs 12%, p = 0.02). There was a significant association between the development of acute GVHD and a mean week 2 CSA trough concentration less than 250 ng/ml. Life threatening regimen-related toxicities in the first 100 days included capillary leak syndrome, acute pancreatitis and small bowel perforation. Although the combination of CSA and MP in this dosing schedule was active in preventing acute GVHD, nephrotoxicity remained a problem, and outcome was limited by the inability to achieve the target CSA trough concentration in a substantial proportion of patients.
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PMID:Cyclosporine and methylprednisolone after allogeneic marrow transplantation: association between low cyclosporine concentration and risk of acute graft-versus-host disease. 187 93

Ineffective hematopoiesis in MDS and evolution of acute leukemia in these patients are serious consequences of a neoplastic transformation at the stem cell level. The development of one or more cell clones is associated with morphological, cytogenetical and immunological aberrations and with changes in the normal stem cell pattern. For young people with MDS having an HLA identical donor bone marrow transplantation as the only curative approach so far is the treatment of choice. Younger people (less than 50 years) have a good chance to achieve complete remission by aggressive chemotherapy. In the majority of older people suffering from MDS treatment will be restricted to supportive measures. Recombinant hematopoietic growth factors can improve cytopenia in MDS.
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PMID:[Myelodysplastic syndromes]. 194 44

Myelodysplastic syndromes are clonal haemopoietic stem-cell disorders often associated with peripheral cytopenia due to the ineffective haemopoiesis and, there is a risk of transformation to overt acute leukaemia. Purpose of the therapy of various myelodysplastic syndromes is to reduce the severity of peripheral cytopenia with correction of ineffective haemopoiesis and, elimination of the aberrant haemopoietic clon. No standard treatment can be recommended for patients with myelodysplasia. Most of older patients without an excess of blasts of bone marrow are carefully monitored, and cytopenia may be corrected by administration of blood components. In myelodysplastic syndromes characterized with an excess of blasts low-dose Ara-C therapy or an "agressive" antileukemical chemotherapy may be used in younger patients. Selected patients may be cured by HLA-identical allogeneic bone marrow transplantation. Authors discuss the effect of various therapeutic procedures is myelodysplastic syndromes on the basis of clinical experiences and review their own observations from the application of small dose Ara-C.
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PMID:[Therapeutic possibilities in the treatment of the myelodysplasia syndrome]. 202 74

Thirty-four patients received bone marrow transplants from unrelated donors. Donors and recipients were phenotypically matched for 6 of 6 HLA-A, B, and DR antigens in 27 cases and at 5 of 6 antigens in 7 cases. Twenty-three patients had leukemia, six had myelodysplasia, and five had aplastic anemia. Twenty-four patients had durable engraftment. Five died of sepsis prior to engraftment. Five patients failed to engraft; 2 of these patients had autologous bone marrow recovery. Seventeen patients developed grade greater than or equal to II acute graft-versus-host disease for an actuarial probability of 67 +/- 20%. The severity of acute graft-versus-host disease and its mortality appeared increased for recipients matched for 5 of 6 HLA-A, B, and DR antigens. Of the 34 patients, 13 (38%) are alive; actuarial survival beyond 6 months is 44 +/- 17%. None of the 25 leukemia and myelodysplasia patients achieving engraftment have relapsed. For leukemia and myelodysplasia recipients of 6 of 6 HLA-matched grafts, actuarial survival at 6 months was 55 +/- 21% compared with 14 +/- 26% for recipients matched for 5 of 6 HLA loci (P = 0.19). Infection and acute graft-versus-host disease were the primary causes of death in the engrafted patients. Survival for aplastic anemia patients was 20%. Late deaths due to pneumonia and bronchiolitis obliterans occurred after one year in 2 patients. Closely matched unrelated donor bone marrow transplants are associated with a higher incidence of graft failure and graft-versus-host disease than typically reported for transplants from HLA-identical siblings, but these preliminary data suggest a lower rate of relapse.
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PMID:Bone marrow transplantation using unrelated donors for patients with advanced leukemia or bone marrow failure. 214 25


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