Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023467 (acute myeloid leukemia)
 35,200 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is characterized by pyrexia, neutrophilia, and the abrupt appearance of erythematous, painful, cutaneous plaques, primarily on the upper extremities, head, and neck. Histologically, the salient feature is a dense dermal infiltrate of neutrophils. Approximately 10 to 15 percent of published cases of Sweet's syndrome occurred in patients with cancer. This report reviews the 39 patients with malignancy-associated Sweet's syndrome described in the world literature and compares Sweet's syndrome in cancer patients with the idiopathic form of the disease. The most common associated malignancy was acute myelogenous leukemia. However, other myeloproliferative disorders, lymphoproliferative disorders, myelodysplastic syndrome, and carcinomas have been observed. Importantly, the diagnosis of Sweet's syndrome was often the presenting sign of a new or recurrent tumor. The presence of anemia, abnormal platelet counts, immature cells in the differential, and/or severe vesiculobullous or ulcerative cutaneous lesions is infrequent in idiopathic Sweet's syndrome and should alert physicians to the possibility of a more serious underlying disease. Extracutaneous manifestations may occur and most often involve the musculoskeletal system. Response to systemic steroids is dramatic in virtually all patients, regardless of the presence of malignancy.
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PMID:Sweet's syndrome and malignancy. 330 Mar 6

We analyzed the natural history of idiopathic refractory sideroblastic anemia (IRSA) in 37 patients studied between 1969 and 1986. Although erythroid abnormalities were prominent in all, 12 patients also showed involvement of the granulocytic and/or megakaryocytic cell lines, and nonrandom chromosomal aberrations were observed in five of 23 patients studied for such defects. Measurements of erythroid marrow function showed in most cases erythroid expansion with ineffective erythropoiesis. In seven patients, however, the erythroid activity was found to be inappropriately low for the degree of anemia. Transfusion dependence occurred in 26 of 37 cases. Iron overload was a common feature at presentation but produced clinical manifestations of hemochromatosis only in those patients who subsequently had a regular need for blood transfusions. Five patients progressed to bone marrow failure, and another five patients (two of whom had monosomy 7) evolved into acute nonlymphocytic leukemia (ANLL). The median survival was 72 months, with a high transfusion requirement, multilineage defects, and inappropriately low erythroid proliferation being associated with a poor prognosis. The most common causes of death were complications of iron overload and evolution into ANLL. We conclude that (a) the natural history of IRSA is characterized by an initial phase of erythroid hyperplasia and ineffective erythropoiesis, which is usually stable for many years but in a subset of patients may be followed by a phase of marrow failure with or without the later emergence of leukemic blasts; (b) peripheral blood counts, measurement of erythroid marrow function, and chromosomal analysis are useful for identifying subjects at risk of evolution into marrow failure or ANLL; and (c) IRSA patients with no need for blood transfusions are very likely to be long survivors, whereas those who become transfusion dependent are at risk of death from the complications of secondary hemochromatosis.
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PMID:Natural history of idiopathic refractory sideroblastic anemia. 333 99

Between 1980 and 1986, we diagnosed refractory anaemia (RA), according to the FAB classification, in 69 patients, who constituted 22% of the 312 cases of myelodysplastic syndromes (MDS) seen over that period. The haematological features were variable, with pancytopenia in 14 cases (20%), bicytopenia in 24 (36%) and mono-cytopenia in the remaining patients, including 21 (30%) cases of anemia alone, 8 (12%) cases of refractory neutropenia and 2 (3%) cases of refractory thrombocytopenia. Myelodysplastic features were also quite variable, involving one, two or all three lineages. In patients with a single cytopenia or only one dysplastic lineage, FAB criteria appeared insufficient for adequate inclusion among RA and we suggest more precise diagnostic criteria, resulting from the utilization of cytogenetics, ferrokinetics, progenitor cultures and perhaps molecular biology, in such cases. Median survival was 42 months. 12 patients (17%) progressed to RAEB (of whom 7 finally developed ANLL) and 4 patients (6%) to CMML. In spite of the heterogeneity of haematological features, only two factors were associated with poor prognosis, namely age greater than 70 yr at diagnosis and haemoglobin less than 10 g/dl, whereas, to a lesser extent, neutropenia was associated with progression to RAEB.
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PMID:Refractory anaemia according to the FAB classification: a report on 69 cases. 336 22

The role of peripheral blood mononuclear cells (PB-MNC) on the growth of bone marrow (BM) CFU-GM was investigated in refractory anaemia (RA) patients. Whereas normal donor PB-MNC were found to inhibit autologous day 7 CFU-GM, PB-MNC from RA patients exhibited little modulatory effect on autologous or allogeneic day 7 CFU-GM. In contrast, patient PB-MNC inhibited autologous CFU-GM at day 10 at a time where no significant inhibition was seen in the PB-MNC/RA CFU-GM combination. The identity of the inhibitory cells was investigated using anti-T8+ and anti-N901+ subsets purified by immune-rosette depletion with a panel of monoclonal antibodies. The activity of these subsets was tested on immature myeloid cells enriched for MY7+ cells, and it was found that cells highly enriched for NK cells were responsible for the inhibition. Further support for NK cells as the inhibitory cells was obtained in experiments where a positive correlation between the level of PB NK cytotoxicity against K562 cells and the degree of CFU-GM inhibition was demonstrated. Thus, these data suggest the presence of a specialized subset of NK cells with a capacity to inhibit autologous CFU-GM. Since RA is a potentially premalignant disease, in which a significant number of cases transform into AML, these findings also suggest a physiological role for NK cells in suppression of newly arisen clonogenic cells at least in early stages of the disease.
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PMID:Natural killer cell-mediated inhibition of bone marrow colony formation (CFU-GM) in refractory anaemia (preleukaemia): evidence for patient-specific cell populations. 340 83

In 27 patients initially diagnosed as refractory anaemia (RA) or RA with sideroblasts (RA-S) according to the FAB-classification a number of clinical, morphological and cytogenetic parameters were correlated for prognostic significance. From these correlations it emerged that severe cytopenia is centrally positioned with regard to clinical course in RA and RA-S. Positive correlations were found to initial diagnosis, clonal cytogenetic abnormalities, progression to RA with an excess of blasts (RAEB) or acute myeloid leukaemia (AML), the percentage of bone marrow blast cells and prolonged half life for radioactively labeled iron. The degree of peripheral blood granulocytopenia, alone, was correlated to bone marrow hypoplasia. Moreover, the frequency of abnormal karyotypes was inversely correlated to bone marrow cellularity and proportional to the frequency of bone marrow blast cells. From these relationships it may be proposed that chromosome abnormalities are associated with prolonged blast cell generation times and inhibition of blast cell maturation resulting in reduced marrow cellularity and blast cell accumulation, and, in the peripheral blood, falling percentages of neutrophil granulocytes. With the blast cell accumulation the bone marrow cellularity again becomes hyperplastic and the preleukaemic condition is transformed into RAEB or AML.
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PMID:Prognostic significance of some clinical, morphological and cytogenetic findings in refractory anaemia (RA) and RA with sideroblasts. 345 30

A 27-year-old patient manifested severe hemolytic anemia during a preleukemia phase. Low levels of adenosine triphosphate and decreased activity of pyruvate kinase were found. Acute myeloblastic leukemia developed in this patient 20 months later. The case suggests that acquired enzyme deficiency may play a role in the development of anemia in the preleukemic state.
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PMID:Preleukemia manifested by hemolytic anemia with pyruvate-kinase deficiency. 345 56

Atypical bullous pyoderma gangrenosum was diagnosed during the course of a myeloid malignancy in three patients. One patient had chronic myeloid leukaemia, one acute myeloid leukaemia, and the third, refractory anaemia with excess of blasts. This atypical form of pyoderma gangrenosum has been specifically associated with myeloid malignancies. The atypical appearance of the skin lesions and the clinical context in which they arose caused the true diagnosis to be delayed in all cases. Treatment with steroids was associated with rapid healing of the skin lesion. The histopathological changes in the skin biopsy specimens from these cases were non-specific, and although the histology was considered to be atypical of pyoderma gangrenosum in one case, the unusual features could be attributed to the patient's neutropenia. (Skin biopsy was performed to exclude other specific pathology). Atypical bullous pyoderma gangrenosum is an uncommon association of the myeloid malignancies. It may remain unrecognised and should be considered more often.
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PMID:Atypical bullous pyoderma gangrenosum associated with myeloid malignancies. 347 88

A 79-year-old patient with acute myeloblastic leukemia (M2 type in FAB), who has survived more than 5 years, is reported. She was admitted because of fever and anemia. Her white blood cell count was 6200/mm3 with 58% blasts. Bone marrow aspiration showed a nucleated cell count of 26 X 10(4)/mm with 84% blasts, Complete remission was achieved within one month by DCMP two-step method therapy. She relapsed in the third and fifth years after initial therapy. Because leukemic change is atypical, she was treated with a low dose of Ara-C therapy, resulting in complete remission. In cases of acute myelobastic leukemia in elderly patients, long-term survival is rare. However in this case, follow-up has succeeded for 5 years. This patient is the oldest case of acute myeloblastic leukemia ever reported in Japan.
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PMID:[Long-term survival in an elderly patient with acute myeloblastic leukemia]. 347 33

The evolution of leukaemia was studied prospectively in 29 patients with myelodysplastic syndrome (MDS) followed for 2-6 years by sequential blast counts, cell kinetics derived from quantitative 14C-autoradiography and karyotype analysis. Overt leukaemia developed in seven patients. Two distinct patterns of leukaemic evolution were identified. The first was characterized by a gradual increase in blast cell count and in the frequency of labelled blasts, and a corresponding reduction in myeloid maturation index indicating increased intracompartmental myeloblast divisions and premature myeloid cell death. A second pattern of leukaemic evolution was marked by a sudden rise in the blast cell population in a previously stable MDS. This rise was attributed both to an increased rate of blast proliferation, and the accumulation of non-proliferating blasts. In an additional patient with smouldering ANLL and multiple karyotype abnormalities, transient clinical remission took place following prednisone and oxymetholone therapy, characterized by a sideroblastic morphology, normal karyotype, and persistence of a highly abnormal myeloid maturation index. The sudden emergence of overt leukaemia in previously stable MDS in some of our patients and the temporary reversal of overt leukaemia into sideroblastic anaemia in one case, lend support to the notion of leukaemic evolution by several steps of transformation. On the other hand, the gradual transition of MDS into overt leukaemia in other patients is compatible with a single step leukaemia transformation, although the possibility of clonal disease prior to the development of MDS cannot be excluded with certainty.
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PMID:Myelodysplastic syndromes: evolution of overt leukaemia by one or several steps of transformation. 367 2

Fifteen patients with the initial diagnosis of myelodysplastic syndrome (MDS) received aggressive chemotherapy with high dose cytarabine or with a standard acute myeloid leukaemia (AML) regime. Cases treated with aggressive chemotherapy were either younger individuals with refractory anaemia with excess of blasts (RAEB) or patients, irrespective of age in advanced stages of MDS (RAEB in transformation or after evolution to frank AML), who did not have a major infection at the time of presentation. Age seemed to be the most important factor in determining the outcome of aggressive remission induction chemotherapy in MDS: 86% of the patients less than 50 years entered complete remission, compared to only 25% in the older age group. In spite of intensive consolidation therapy the duration of complete remission was short. We conclude that young patients (less than 50 years) with excess of bone marrow blasts should be treated with aggressive chemotherapy even in the early stages of the disease. Elderly patients in advanced stages of MDS should be treated with less aggressive chemotherapy.
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PMID:The role of aggressive chemotherapy in the treatment of the myelodysplastic syndromes. 373 Feb 85


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