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

Out of 2,474 bone marrow biopsies we have observed 330 cases (13.3%) with presence of lymphoid nodules (LN). LN were frequent in old age (24.6% over 80 years), in females (17%) and in some diseases, such as rheumatoid arthritis and systemic lupus erythematosus (73.7% of the cases), partial aplasia (34%), hypersplenism (30.4%), hemopoietic dysplasia (25%), chronic renal failure (20.4%), polycythemia vera (20.2%), idiopathic thrombocytopenic purpura (18.8%), acute leukemia (17.7%). Nodular lymphoid hyperplasia of the bone marrow was found especially in systemic autoimmune diseases (26.3%), hypersplenism (9.8%), preleukemia (7.3%) and acute leukemia (4.2%). The presence of excessive medullary LN could indicate a bone marrow microenvironment damage, possibly of autoimmune origin.
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PMID:Lymphoid nodules and nodular lymphoid hyperplasia in bone marrow biopsies. 393 2

The presenting clinical pictures and courses of seven patients with thrombocytopenia, decreased megakaryocytes in the marrow, and minimal changes in other hematopoietic cell lines are described. Little information exists in the literature on such patients. Initial bone marrow aspiration and biopsy in all patients showed decreased megakaryocytes with an otherwise normal marrow. Erythrocyte mean corpuscular volume was elevated in five of seven patients. Bone marrow karyotypes of six of the seven patients were normal. Chromium-51 platelet survival studies with platelet sizing, done in five of the seven patients, showed normal results. In two patients the course progressed to aplastic anemia. One of these died 9 months after presentation, and one responded dramatically to lithium. One patient developed preleukemia and died. The other four patients have remained thrombocytopenic but clinically stable. No useful therapy was identified. The differential diagnosis of such patients should include idiopathic thrombocytopenic purpura with misinterpretation of morphologic findings, hereditary and acquired aplastic anemia, preleukemia, and systemic lupus erythematosus.
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PMID:Thrombocytopenia with decreased megakaryocytes. Evaluation and prognosis. 719 25

3 patients with preleukaemia (idiopathic acquired myelodysplasia) presenting with isolated peripheral thrombocytopenia and an increased number of megakaryocytes are described. The diagnosis of preleukaemia, however, was strongly suspected on bone marrow morphology, revealing an abnormal megakaryocytic series. Megakaryoblasts with characteristic nuclei and basophilic pseudopodes, and immature megakaryocytes were frequent. These last cells had 1 or 2 nuclei and a basophilic poorly granulated cytoplasm, often containing large vacuoles, corresponding to dilated interconnecting demarcation membranes in electron microscopy. Megakaryocytes were not considered able to produce a normal amount of platelets, explaining the peripheral thrombocytopenia; platelet survival (51Cr) was indeed too long for ITP. Cytogenetic analysis confirmed the diagnosis of an abnormal haematopoietic clone. Ferrokinetics, in vitro agar cultures, bone marrow biopsy and platelet function tests were helpful additional examinations for establishing the correct diagnosis. It is important to differentiate preleukaemia from ITP. Prognosis is totally different and the treatment is not only ineffective in the great majority of the preleukaemic patients, but may be hazardous.
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PMID:Thrombocytopenia as presenting symptom of preleukaemia in 3 patients. 720 Nov 57

A morphometric analysis was performed on aspirate clots of bone marrow to identify the presence of atypical megakaryocytes after immunohistological staining with a monoclonal antibody against Factor VIII. This study included cases of myelodysplastic syndrome (MDS), myeloproliferative disorder (MPD), aplastic anaemia (AA), idiopathic thrombocytopenic purpura (ITP), chronic myelogenous leukaemia (CML), and control cases free from any haematological disease. Quantitative and qualitative abnormalities of megakaryocytes were assessed using an image analyser and a personal computer to perform a morphometric analysis of the number (per mm2), arrangement (microns), nuclear size (microns2), cell size (microns2), nuclear size/cell size (N/C) ratio, and nuclear contour index (NCI). Micromononuclear megakaryocytes were detected in MDS, while in MPD, large over-mature magakaryocytes were observed to increase in number. In AA, the megakaryocytes decreased dramatically in number without showing any morphological abnormality. In CML, the megakaryocytes also increased without any remarkable morphological abnormality. In ITP, only the NCI increased. The above results show that MDS is characterized by the presence of atypical micromegakaryocytes, while MPD is characterized by atypical large over-mature megakaryocytes.
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PMID:A megakaryocyte analysis of the bone marrow in patients with myelodysplastic syndrome, myeloproliferative disorder and allied disorders. 749 Jun 85

A 29-year-old Chinese woman developed pyrexia, multiple skin abscesses and bilateral fine nodular lung infiltrates about 3 months after the commencement of therapy for idiopathic thrombocytopenic purpura (ITP). Pseudomonas aeroginosa was isolated from the abscesses but multiple blood and sputum cultures, as well as a broncho-alveolar lavage did not yield any microorganisms. The persistence of fever and pulmonary infiltrates warranted an open lung biopsy which provided a definitive diagnosis of tuberculous-aspergillus granulomatous lung disease. Bone marrow re-examination revised the primary haematological disorder to that of a trisomy 8 associated myelodysplastic syndrome.
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PMID:Tuberculosis and invasive pulmonary aspergillosis in a young woman with a myelodysplastic syndrome. 757 Jan 23

In order to distinguish various types of MDS, such as RA/AA, RA/ITP or RA/HA, from AA, ITP or HA, bone marrow (BM) cells were studied by using cytogenetic techniques including R-banding karyotypic analysis and sister chromatid differentiation (SCD) assay in 334 cases of hematological diseases (160 MDS, 54 RA/AA, RA/ITP or RA/HA; 60 AA, 3 other known anemias, 38 PNH and 19 ITP). The results showed: (1) karyotypes and SCD values were both normal in more than 90% of AA, PNH, ITP and other known anemias, but they were both abnormal in about 35.6% of MDS and only 13.0% of RA/AA, RA/ITP or RA/HA. These results indicated that cytogenetic techniques were useful in hematological clinic and that RA/AA, or RA/ITP or RA/HA might be pre-RA or atypic RA. This was supported by the results of following up on some RA/AA, RA/ITP or RA/HA cases, (2) clonal abnormal karyotypes were found in 64.4% of MDS. The recurrent chromosomal alterations were +8, 20q-, -5/5q-, -7/7q-, similar to those reported in literatures. (3) 16 MDS cases were followed up and 15 MDS with SCD negative, but one with SCD positive developed leukemia in our hospital. It is suggested that change from SCD positive to negative was indicative of malignant transformation of BM cells. This was supported by the results of cytogenetic analysis in RA/AA, RA/ITP, RA, RAEB, RAEBT and leukemias. (4) Because more structural chromosome alterations occur in SCD negative than SCD positive MDS, the numerous chromosome alterations (monosomy) might occur in earliest development of MDS into leukemias.
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PMID:[Cytogenetic studies on 334 myelodysplastic syndrome (MDS), aplastic anemia (AA) and other hematological diseases]. 760 Aug 65

The sensitivity and specificity of 2 antibody tests for diagnosis of idiopathic thrombocytopenic purpura (ITP) in dogs were investigated prospectively. An ELISA to detect antibodies bound to the surface of platelets from affected dogs (direct test) was performed in 34 dogs with a clinical diagnosis of ITP and in 21 dogs with thrombocytopenia attributable to other causes. An ELISA to detect platelet-bindable antibodies in serum from affected dogs (indirect test) was performed in 32 dogs with ITP and in 15 dogs with other causes of thrombocytopenia. The direct test was positive in 32 of 34 dogs with ITP (sensitivity, 94%) and negative in 13 of 21 dogs with other causes of thrombocytopenia (specificity, 62%). Positive direct test results were obtained in 2 dogs with systemic lupus erythematosus, and in 1 dog each with concurrent Ehrlichia canis and Babesia canis infections, dirofilariasis, myelodysplasia, disseminated intravascular coagulation (of unknown cause), and thrombocytopenia subsequent to administration of trimethoprim/sulfadiazine, as well as in 1 dog with thrombocytopenia 14 days after a whole blood transfusion. The indirect test had positive results in 11 of 32 dogs with ITP (sensitivity, 34%) and negative results in 12 of 15 dogs with other causes of thrombocytopenia (specificity, 80%). Positive indirect test results were obtained in 1 dog each with systemic lupus erythematosus, concurrent E canis and B canis infections, and thrombocytopenia subsequent to administration of trimethoprim/sulfadiazine. Detection of platelet-bound antibodies was more sensitive than detection of serum-platelet bindable antibodies in confirming a diagnosis of ITP in dogs.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of platelet-bound and serum platelet-bindable antibodies for diagnosis of idiopathic thrombocytopenic purpura in dogs. 774 62

A 5-year-old girl was diagnosed as having idiopathic thrombocytopenic purpura (ITP) based on symptoms of nasal bleeding and purpura. The platelet count was 35,000/microliters without anemia or leukopenia. Micromegakaryocytes were observed in normocellular bone marrow without dyserythropoiesis or dysgranulopoiesis. She had periosteal fibroma of the rib and atopic dermatitis with elevated serum IgE. Prednisolone and azathioprine were administered but with no response. The cumulative dose of azathioprine was 20 g for 28 months. Nine years after the diagnosis of ITP, she was admitted because of dyspnea and anemia. The white cell count was 26,900/microliters with 17% monocytes. The hemoglobin was 3.9 g/dl and the platelet count was 9,000/microliters. Dyserythropoiesis, dysgranulopoiesis and micromegakaryocytes were observed in hypercellular bone marrow. The chromosome analysis demonstrated 47, XX, +21. She was diagnosed as having chronic myelomonocytic leukemia (CMMoL) and received bone marrow transplantation (BMT) from an HLA-identical sibling conditioned with high-dose busulfan and melphalan. After 17 months of remission, the disease recurred with an abnormal karyotype of 47, XX, +21, 7q+. Despite a second BMT conditioned with high-dose etoposide, cyclophosphamide and total body irradiation, she died of the disease. Refractory thrombocytopenia as a subgroup of myelodysplastic syndrome, rather than ITP, might have preceded the development of CMMoL, with the possibility of azathioprine-induced leukemia.
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PMID:[Chronic myelomonocytic leukemia developed 9 years after the diagnosis of idiopathic thrombocytopenic purpura in a child]. 807 97

To aid in the rapid differential diagnosis of thrombocytopenia, the authors developed a latex agglutination test for glycocalicin, a proteolytic fragment of platelet membrane glycoprotein Ib. Plasma glycocalicin determinations were performed for 34 patients with thrombocytopenia. Plasma samples from four patients with aplastic anemia and ten patients with myelodysplastic syndromes, all with glycocalicin levels less than 0.6 mg/L by an enzyme-linked immunosorbent assay, all had negative results by the latex test. In contrast, positive latex agglutination titers were obtained for all 12 patients with idiopathic thrombocytopenic purpura. Eight patients with liver cirrhosis and splenomegaly had elevated levels of plasma glycocalicin, and all of their plasma samples produced agglutination. This latex agglutination test for glycocalicin allows a rapid discrimination of thrombocytopenia caused by impaired platelet production from that caused by increased platelet destruction; thus, it is suitable for use as a screening test in a routine clinical laboratory.
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PMID:Rapid detection of plasma glycocalicin by a latex agglutination test. A useful adjunct in the differential diagnosis of thrombocytopenia. 824 98

Using enzyme-linked immunosorbent assays, we measured the concentrations of serum thrombopoietin (TPO) and plasma glycocalicin, a proteolytic fragment of platelet glycoprotien Ib alpha, in 13 patients with myelodysplastic syndrome (MDS), aplastic anaemia (AA) or idiopathic thrombocytopenic purpura (ITP). In the patients with AA or MDS, the TPO concentrations were remarkably increased, and their glycocalicin concentrations were decreased compared with the normal control individuals. In the patients with ITP, however, the TPO and glycocalicin levels were not changed as much as in the AA/MDS patients in spite of the same degree of thrombocytopenia. During immunosuppressive treatment of ITP patients, there was an inverse relationship between the level of TPO and the platelet count. Thus, measurements of TPO and glycocalicin levels are useful for the diagnosis of thrombocytopenia, and our results from ITP patients did not support the model which suggested the simple feedback regulation of TPO in thrombocytopenia.
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PMID:Serum thrombopoietin and plasma glycocalicin concentrations as useful diagnostic markers in thrombocytopenic disorders. 869 34


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