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

Myelodysplastic syndrome (MDS) combined with monoclonal gammopathy or multiple myeloma has rarely been reported. In this article, two siblings, a brother and his sister who showed simultaneous occurrence of MDS and monoclonal gammopathy are reported. The first case, a 73-year-old male, was admitted to our hospital in November, 1987. Analysis of peripheral blood revealed pancytopenia without blast cells. Bone marrow was hypocellular with 14.9% of myeloblasts and 2.8% of plasma cells characterized by 2 to 4 nuclei. Serum IgA level was 635 mg/dl and serum immunoelectrophoresis revealed a monoclonal IgA lambda band. The second case, a 70-year-old female, younger sister of the first case, was admitted to our hospital in January, 1988. Bone marrow was normocellular with 23% of peroxidase-negative myeloblasts and 12.8% of atypical plasma cells. Serum IgG level was 1,901 mg/dl with monoclonal IgG kappa band. Hematological findings have remained unchanged for 12 months. The first case was regarded as hypoplastic MDS with monoclonal gammopathy and the second case was MDS with smoldering myeloma. These cases were very similar with in respect to age, time of onset, clinical course, hematological findings and especially, association with M-protein. There are no reports concerning the familial incidence of MDS with M-protein. These findings supported the hypothesis that an initial event selects a clone of stem cells which retain the capability to differentiate into mature myeloid and lymphoid cells, in these cases B-cells.
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PMID:[Familial occurrence of myelodysplastic syndrome concomitant with monoclonal gammapathy]. 163 65

In a phase I-II study, nine patients with myelodysplastic syndromes and concomitant severe transfusion-dependent cytopenias were treated with recombinant human interleukin-3 (rhIL-3) to improve hematopoietic function. Doses of rhIL-3 ranged from 250 micrograms/m2 to 500 micrograms/m2 and were given as daily subcutaneous bolus injections for 15 days. Blood leucocyte counts increased 1.3- to 3.6-fold in all nine patients, including neutrophils, eosinophils, lymphocytes, basophils, and monocytes. The mean absolute neutrophil counts increased from 1,350/microL (range, 150 to 2,420) to 2,660/microL (range, 300 to 9,380) (P less than .05) immediately after the end of rhIL-3 therapy and to a maximum count of 4,096/microL (range, 350 to 10,820) (P less than .01). Platelet responses were seen in two of four profoundly thrombocytopenic patients, resulting in discontinuation of platelet transfusion. The requirements for red blood cell transfusion temporarily improved in one patient. Stimulation of plasma cells was evident by a significant increase in serum IgM and IgA levels. Mild side effects (fever, headache, local erythema, and bone pain) were observed in some patients, while transient thrombocytopenia developed in two patients. Disease progression with an increase in blast cells was seen in one patient. These results suggest that rhIL-3 is effective in stimulating hematopoiesis of all lineages in patients with myelodysplastic syndromes and may produce at least short-term hematologic improvement.
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PMID:Effects of recombinant human interleukin-3 in patients with myelodysplastic syndromes. 237 79

Immunohaematological investigations were carried out in 46 patients with erythrocyte autoantibodies associated with myelodysplastic syndromes. Eight patients had refractory anaemia, 17 refractory anaemia with ring sideroblasts, 11 refractory anaemia with excess of blasts, four chronic myelomonocytic leukaemia, five refractory anaemia with excess of blasts in transformation and one could not be classified. Standard agglutination direct antiglobulin tests showed that the red cells were most often coated with IgG and C3d, though increased amounts of IgM or IgA were also found in 15 of 35 cases (43%) when the more sensitive enzyme linked method was used. The IgG antibodies were predominantly of IgG1 subclass. Clinically important autoimmune haemolysis occurred in 15 patients, and was of "warm", "cold," and "mixed" types in seven, four, and four cases, respectively: it is important to recognise its presence in view of the good response to treatment. The increased incidence of erythrocyte autoantibodies in myelodysplastic syndromes is thought to be one manifestation of disturbed immune homeostasis.
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PMID:Erythrocyte autoantibodies, autoimmune haemolysis, and myelodysplastic syndromes. 258 9

Immunocompetence was evaluated in 36 untreated and noninfected patients affected with myelodysplastic syndromes (MDS). T-cell number and activity were evaluated by counts of total T-cells and T-lymphocyte subsets, and by measure of DNA synthesis in response to phytohemagglutinin and Concanavalin A. B-cells were evaluated as surface immunoglobulin- (SIg+) bearing cells and by serum immunoglobulin levels. Granulocyte activities were evaluated by responses to chemotaxis and to nitroblue tetrazolium test. Complement activity was measured by classic hemolytic complement assay. In addition, circulating immune complexes were detected in serum. MDS were associated with a significant decrease in the absolute numbers of total T (E-rosetting and T3+) cells, T4+, and T8+ cells and a dramatic decrease of the responses to Concanavalin A. An impairment of either chemotaxis or of nitroblue tetrazolium (NBT) test was frequently encountered. An increase in the levels of IgG or IgA was also a frequent feature. The findings reveal that all patients with a high degree of T-cell impairment have refractory anemia associated with an excess of medullary blast cells. All in all, the data suggest that the counts of the absolute number of cells bearing the T3 and T8 phenotypes could be of prognostic value: the higher the number, the better the patient's survival.
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PMID:Immunologic indices in myelodysplastic syndromes. 325 9

Multiple myeloma (IgG kappa + IgA kappa type, clinical stage IA) was diagnosed in a 82-year-old woman in January 1986. Chemotherapy (melphalan, prednisolone, vindesine, cyclophosphamide), caused prolonged myelosuppression. Therefore she was given other treatment. In October 1992, her peripheral blood examination demonstrated 2% blastic cells and 12% eosinophils. Bone marrow aspiration showed dysplastic features of trilineage blood cells with 4.8% myeloblasts. The karyotype of bone marrow cells from this patients was 47, XX, +der(1)t(1;7) (p11;p11), -7, +8. A diagnosis of therapy-related myelodysplastic syndrome (refractory anemia) was established. Eleven months after diagnosis of myelodysplastic syndrome, she is alive without leukemic transformation.
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PMID:[Multiple myeloma preceding myelodysplastic syndrome with eosinophilia and der (1;7)]. 782 98

Over a follow-up period of ten years, nine of our 100 patients with multiple myeloma (MM), developed myelodysplastic syndrome (MDS, preleukaemia). MDS occurred 19-156 (median 35) months from the diagnosis of MM. Six patients presented with pancytopenia and no patients had active MM at the time of MDS diagnosis. Three patients were defined as having refractory anaemia (RA) and six as refractory anaemia with excess blasts (RAEB) or RAEB in transformation (RAEBT), according to the FAB classification. The clinical course is characterized by increasing red blood cell and platelet transfusion requirements, recurrent infections and bleeding episodes. All patients, except for one, died within 3 to 8 (median 5) months from MDS diagnosis. The causes of death were sepsis or bleeding; three patients underwent leukaemic transformation. Thus, the clinical course of this small group of myeloma patients who developed secondary MDS (sMDS), was similar to other series of patients with sMDS. Serial bone marrow examinations suggest an initial hypercellular phase, followed by a rapidly evolving preterminal hypocellular marrow. In an attempt to detect MM patients at risk of developing sMDS, the epidemiological (including ethnic), clinical and laboratory data of the 9 MDS patients at the time of the MM presentation were reviewed and compared to the other MM patients. No significant differences were observed between the two groups in most parameters, except for two. All MDS patients were Ashkenazi Jews and no patients of Sepharadic origin developed MDS. Also, no IgA-myeloma patient developed MDS. If these findings are confirmed in a larger series, it may point to subgroups at risk which may require a different approach.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Secondary myelodysplastic syndrome in multiple myeloma--a study of nine patients with an attempt to detect myeloma patients at risk. 789 Feb 64

The report describes a patient in whom myelodysplastic syndrome and multiple myeloma (MM) were simultaneously present. This patient manifested an IgA-lambda type of MM concurrent with a refractory anaemia with ringed sideroblasts (RARS) without prior therapy. His bicytopenia could not be improved by vitamin B6 regardless of a reduced serum vitamin B6 concentration. A review of the literature suggests that myelodysplastic syndrome (MDS), chronic neutrophilic leukaemia (CNL) and idiopathic myelofibrosis (IMF) are the most frequent disorders associated with MM. The IgA type seems to be associated more commonly with these disorders. The mechanisms responsible for the development of plasma cell proliferation are diverse; the neoplastic transformation of a common progenitor, the involvement of the lymphoplasmacytic system and/or chronic reticuloendothelial stimulation may play a role in the occurrence of such hybrid haematological disorders.
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PMID:Refractory anaemia with ringed sideroblasts concurrent with multiple myeloma--a brief review of the recent literature. 940 63

The expansion of myeloma cells is regulated by cytokines, among which IL-6 is a major growth factor. It has been recently suggested that serum transforming growth factor beta 1 (TGF beta 1), a cytokine found in large amounts in alpha-granules of platelets, might play a role in multiple myeloma (MM). It was the purpose of this study to determine serum TGF beta 1 levels in MM patients and to seek a correlation with disease parameters. Measurements were done by ELISA. We studied 35 MM patients (19 stage II, 16 stage III, 20 IgG, 8 IgA and 6 BJ, 1 IgD) in different phases of the disease, 27 healthy individuals and 17 thrombocytopenic patients with other haematological diseases (three MDS, three congenital thrombocytopenia, 11 ITP). Overall samples from MM patients were included: 10 at diagnosis, 18 in remission and 32 in relapse. In normal controls TGF beta 1 serum levels ranged from 1 to 33 ng/ml (median 16.5 ng/ml). In both thrombocytopenic controls with other diseases and thrombocytopenic MM patients (seven samples), TGF beta 1 serum levels were very low (median 3.2 and 4.5 ng/ml respectively). In MM patients with PLT > 100 x 10(9)/L (53 samples), TGF beta 1 serum levels were in the normal range in patients without immunoparesis (1 to 27 ng/ml, median 16.6 ng/ml), whereas they were higher in patients with immunoparesis (polyclonal immunoglobulins (Igs) below lower normal reference values) ranging from 10.2 to 45 ng/ml (median 26.8 ng/ml) (P < 0.01). Serum TGF beta 1 levels fluctuated in the same patient at different times but not according to relapse or remission. Correlation was found only between serum TGF beta 1 levels and immunoparesis and not between serum TGF beta 1 levels and disease stage or Ig subtype nor with prognostic factors for MM (serum CRP, beta 2M or IL-6). This finding suggests that the remaining normal plasma cells are sensitive to the inhibitory action of TGF beta 1 on Ig production. In conclusion TGF beta 1 serum levels are very low in thrombocytopenic patients confirming that platelets are the major source of this cytokine. Furthermore, a strong correlation was found between TGF beta 1 serum levels and immunoparesis in MM patients.
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PMID:Serum transforming growth factor-beta 1 is related to the degree of immunoparesis in patients with multiple myeloma. 978 21

A 59-year-old man with myelodysplastic syndrome exhibited unticaria, dyspnea, fever, and hypotension immediately after the start of platelet concentrate (PC) transfusion on the 15th day of hospitalization. On the 17th and 21st day of hospitalization, the patient exhibited severe unticaria resembling that of erythroderma after the start of PC transfusion. Anti-IgA IgG antibody was detected by enzyme-linked immunosorbent assay (ELISA) on the 28th day of hospitalization. Anti-IgA (purified from pooled human plasma) was detected by Western blotting. The patient's IgA level was normal. No antibodies against HLA or platelet-specific antigens were detected in serum. The patient had no reaction to administration of washed PC. These findings suggest that anti-IgA antibody might have been responsible for the nonhemolytic transfusion reaction in this patient.
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PMID:[Detection of anti-IgA alloantibody in a case of non-hemolytic transfusion reaction]. 1022 32

An unusual association of paroxysmal nocturnal haemoglobinuria (PNH), myelodysplastic syndrome (MDS), acute myeloid leukaemia (AML) and monoclonal gammopathy is reported. A 60-year old male, who had a history of IgA monoclonal gammopathy, presented with haemoglobinuria and colic pain. Flow cytometry showed CD55negative/59dim peripheral red cells, and bone marrow examination disclosed MDS. Eleven months, he developed later AML with disappearance of the PNH clones, although the monoclonal gammopathy persisted. The relationship between PNH and MDS has not fully been assessed, although our findings indicate that these triple clonal disorders, all coexisted in one patient.
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PMID:An unusual association of monoclonal gammopathy, paroxysmal nocturnal haemoglobinuria and myelodysplastic syndrome transformed into acute myeloid leukaemia: coexistence of triple clonal disorders. 1169 14


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