Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report three patients with pulmonary disorders associated with myelodysplastic syndromes (MDS). All three patients had symptoms of pyrexia and respiratory discomfort. One patient had pulmonary eosinophilia with bilateral pleural effusion, one had interstitial pneumonia, and one had bilateral pleural effusion caused by systemic vasculitis. Elevated C-reactive protein (CRP) levels, polyclonal hypergammaglobulinemia, and morphological abnormalities in peripheral blood were observed in all three patients. The bone marrow of these patients revealed trilineage dysplasia and eosinophilia. Cytogenetic analysis showed [46,XY,-7,+der(1q;7p)]. Antibiotic treatment was not effective. However, improvement was dramatic after corticosteroid treatment; CRP levels were reduced and the hypergammaglobulinemia was improved. These cases suggest that MDS with [-7,+der(1q;7p)] may be correlated with bone marrow eosinophilia and that an immunologic abnormality may be involved in the pulmonary disorders.
 ...
PMID:Steroid-responsive pulmonary disorders associated with myelodysplastic syndromes with der(1q;7p) chromosomal abnormality. 757 89

Several studies aiming to describe the immunological abnormalities occurring in patients affected by myelodysplastic syndromes (MDS) have been carried out in recent years. We report on the immunological abnormalities found in 22 myelodysplastic patients at the time of diagnosis (RA: 2 cases; ASIA: 4 cases; AREB: 6 cases; AREB-T: 6 cases. LMMC: 4 cases). Matched with similarly aged healthy people (controls) all our patients revealed a significant lymphocytopenia mainly due to a reduction both in number and percentage of T-helper series with decreased OK T4/OK T8 ratio as a result; even B-cells were reduced in number but their percentage still overlapped with the controls. Out of 22 patients, 13 showed hypergammaglobulinemia (polyclonal in 12 cases, monoclonal in the one left) and 2 read positive for Coomb's and Ana-test respectively. The involvement of T-cell immunity in the course of MDS can be explained if we consider the clonal origin of such diseases. Among myelodysplastic patients the ones affected by LMMC, AREB and AREB-T show the heaviest immunological abnormalities: in these cases the whole of T-cells subsets and NK cells as well are affected. Eventually, the mentioned abnormalities are of paramount importance to explain how easily these patients can develop both severe infectious diseases and abrupt acute leukemia.
 ...
PMID:[Immunological indices in myelodysplastic syndromes]. 802 41

Autoimmune syndromes are common in patients with myelodysplastic syndromes (MDS). Clinical manifestations include an acute systemic vasculitic syndrome (characterized by skin vasculitis, fever, arthritis and sometimes associated with pulmonary infiltrates and peripheral edema), chronic autoimmune disorders, including chronic cutaneous vasculitis, polyneuropathy, inflammatory bowel disease and glomerulonephritis, and classical connective tissue disorders, most notably relapsing polychondritis. Asymptomatic immunologic abnormalities are also common and include hypergammaglobulinemia and a positive FANA. Autoimmune syndromes may be the primary cause of death in some patients with MDS. However, these syndromes frequently respond to immunosuppressive agents and occasional dramatic hematologic responses to steroid therapy are seen. We review the incidence, nature, course and response to therapy of these manifestations and discuss potential pathogenic mechanisms.
 ...
PMID:Autoimmune phenomena in patients with myelodysplastic syndromes. 908 38

Immunological abnormalities (IA) are frequently observed in patients with myelodysplastic syndromes (MDS). Although there have been a number of analyses of the prognostic factors, there have been few studies, if any, to determine whether IA affects prognosis. We investigated the prognosis of 153 MDS patients with or without IA who were treated at a single Japanese institute for 10 years. Nineteen of 153 patients (12%) developed autoimmune disorders. One hundred of 153 patients (63%) had an abnormality in at least one immunological laboratory test. Hypergammaglobulinemia was found in 50 of 128 (39%) patients tested, hypogammaglobulinemia was observed in 10 of 128 (8%), positivities of antinuclear antibody, RA factor. DNA antibody, and direct antiglobulin test were observed in 30%, 14%, 7% and 12%, respectively, and a CD4/CD8 ratio < 1 was observed in 20%. There was no significance in the distribution of age, MDS subtype, or sex between patients with and without IA. The survival of MDS patients without IA was significantly better than that of patients with IA, and the survival of patients with a CD4/CD8 ratio > 1 was also significantly superior to the survival of those with an inverted CD4/CD8 ratio. Patients with IA tended to die of infection or leukemic progression in comparison with those without IA, suggesting that IA may be associated with susceptibility to opportunistic infection and disease progression.
 ...
PMID:Correlation between immunological abnormalities and prognosis in myelodysplastic syndrome patients. 2733 45

Myelodysplastic syndrome (MDS) is known to be associated with functional abnormalities of B cells, including hypergammaglobulinemia and monoclonal gammopathy (MG). However, the pathogenesis of these immunological disorders has not been clarified. We report a patient who developed donor-derived MDS followed by leukemic transformation after cord blood transplantation for MDS with MG. Interestingly, MG reappeared before development of donor-derived MDS. We analyzed the immunoglobulin allotype gene polymorphisms to determine whether the MG after cord blood transplantation was of recipient origin or donor origin. Results of genetic analysis and enzyme-linked immunosorbent assay of IgG1 allotype revealed that the MG after cord blood transplantation was of donor origin. Although the mechanism of donor-derived MG remains unclear, the persistent presence of recipient's antigen presenting cells might have induced the abnormal immunoglobulin production.
 ...
PMID:Recurrence of monoclonal gammopathy associated with donor-derived myelodysplastic syndrome after cord blood stem cell transplantation. 2191 Oct 93