Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of acute Mast-cell leukemia was studied. A 39 years old female presenting with a brief history of abdominal pain and attacks of flushing; peripheral blood and bone marrow contained up to 60% of poorly differentiated blasts with clumping of deep purpule granules. Peroxydase reaction stains were negative, chloroacetate esterase were strongly positive. Toluidine blue revealed metachromatic stain. Histamine content of the cells was highly greater than normal but nos heparinoid activity could be demonstrated. These abnormal mast-cells have been investigated with the electron microscope; only the dense particular type of granule substructure was found, without any lamellae component. The cells were temptatively classified as "immature" mast-cell. The disease was interpretated as an acute leukemic variety of systemic mastocytosis.
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PMID:[Acute mast-cell leukemia. Cytochemical and ultrastructural study, about a particular case (author's transl)]. 5

It was shown by Pincus and Klebanoff that a correlation existed between leukocytic iodination measured in vivo and microbicidal leukocytic activity. We have analyzed the results of this test in relation to time and in the presence of variable quantities of polymorphonuclear leukocytes (PMN). The values observed per time and PMN unit proved to be equivalent in the presence of 2.5 X 105 PMN or 5.0 x 105 PMN per 0.5 ml of incubation medium, measured after 10, 20 and 30 minutes or in the presence of 1.0 x 106 PMN, measured after 10 minutes. That is to say iodination is proportional to leukocyte concentration and incubation time. Increase of either the quantity of cells or the incubation time, beyond the area we defined, reduced iodination per cell and per unit of time. Concerning the patients with an insufficient iodination, we have studied 2 parameters in the presence of 5.0 x 105 PMN: 1) initial iodination measured after 10 and 20 minutes and 2) stability of iodination measured after 60 minutes. These two parameters were equally affected in two cases with myelofi-rosis, 3 patients with acquired refractory anaemia, one with chronic lymphoid leukaemia, one with erythroleukaemia, one with hairy cell leukaemia, one with systemic mastocytosis and almost complete myeloperoxidase dificiency, one with sickle cell disease, two with liver diseases and two with chronic myeloid leukaemia. The iodination at the 60th minute was more affected than at the 10th minute with a patient with myelofibrosis and 4 other patients with acquired refractory anaemias. The significance of these differences is not well understood; however the meaning of the decrease in the iodination of whatever type is that a PMN anomaly exists directly related to the myeloperoxidase H2O2 halogenation system, or to one of the stages of engulfment and/or metabolic events preceeding it and leading to the production of H2O2. This test, with the alterations we introduced, is suggested as a test for detection of functional PMN abnormalities.
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PMID:Quantitative iodination of human blood polymorphonuclear leukocytes. 16 86

Characteristic mononuclear cells with distorted nuclei and abundant pale-staining cytoplasms together with osteoclast-like giant cells are the cytologic hallmarks of histiocytosis X. The demonstration of acid phosphatase within giant cells in paraffin-embedded sections is a valuable aid to diagnosis. The histologic differential diagnosis of histiocytosis X includes some allergic granulomas, Hodgkin's disease, myelomonocytic leukemia, mastocytosis, and malignant histiocytosis. Some technical prerequisites for accurate diagnosis of histiocytosis X are considered briefly.
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PMID:Subtle clues to diagnosis by histochemistry. Histiocytosis X. 31 20

Sixty-one cases of the Zollinger-Ellison syndrome, encountered over a 2-year period, have been treated with cimetidine, half of them for over 1 year. Two-thirds of the patients responded to 300 mg of the drug every 6 hr by mouth. Others required up to 600 mg every 6 hr. In adequate doses the drug was highly effective: it controlled pain and dyspepsia, restored weight, abolished diarrhea, and allowed healing of ulcers and other inflammatory conditions. Missed or reduced doses led to rapid return of symptoms. Progression of the basic neoplastic process, with associated secretory drive, was unimpeded. Patient acceptance of the drug was 100 percent, and apart from minor transient abnormalities, gynecomastia (5 cases) and liver dysfunction (3 cases), which resolved while treatment continued, no serious adverse effects were seen. Of 61 patients 48 are still on the drug, 3 who were well controlled were treated surgically, 5 died for reasons unrelated to therapy, and 5 had significant problems. The drug provides an alternative to total gastrectomy and can be recommended with confidence for the suitably selected patients. The drug was also beneficial in some cases of the short bowel syndrome, systemic mastocytosis, and endogenous hyperhistaminemia due to leukemia.
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PMID:Report on the United States experience with cimetidine in Zollinger-Ellision syndrome and other hypersecretory states. 62 Sep 13

The authors report the case of a 68-year old woman with systemic mastocytosis revealed by spleen enlargement and portal hypertension, and associated with chronic myelomonocytic leukaemia. Based on their review of the literature, they describe the general characteristics of systemic mastocytosis and underline the frequent association of this disease with malignant haematological disorders, notably in the myeloid series.
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PMID:[Systemic mastocytosis and malignant hemopathies. Review of the literature apropos of a case]. 177 13

A 51-year-old woman presented with concurrent systemic mastocytosis and acute myeloid leukemia associated with a clonal karyotypic anomaly of t(8;21). Cytochemical, histologic, and cytogenetic studies helped in the distinction from a recently described entity: acute myeloid leukemia with marrow basophilia. Although the leukemia responded well to aggressive chemotherapy, the mastocytosis component worsened, illustrating the inherent resistance of mastocytosis to chemotherapy.
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PMID:Concurrent acute myeloid leukemia and systemic mastocytosis. 849 2

Mastocytoses are diseases caused by proliferating mast cells infiltrating one or more organs. The spectrum of mastocytosis includes the cutaneous forms urticaria pigmentosa and solitary mastocytoma (about 90% of mastocytoses) and systemic forms affecting other organs. Infiltrates are most often found in the bone marrow, spleen, lymph nodes and liver, but any organ may be affected. Patients with systemic mastocytosis may or may not have urticaria pigmentosa. About 35% of patients without urticaria pigmentosa have an associated malignant haematological disease and a poor prognosis. Symptoms caused by mast cell mediator release are best treated with antihistamines, but several other drugs may be used if the response is unsatisfactory. Many antineoplastic drugs have been tried to combat aggressive mastocytoses and mast cell leukaemia, but the results have been disappointing.
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PMID:[Mastocytosis]. 195 56

A 52-year-old Japanese man manifested various clinical signs and symptoms such as vomiting, high fever, dyspnea, cough, sweating, palpitation, eosinophilic leukocytosis and hepatosplenomegaly. These histamine-related clinical manifestations showed a dramatic response to steroid therapy. After 10 months of hospitalization, he suddenly succumbed to candidal septicemia at the end of the third cycle of steroid therapy. Autopsy revealed neoplastic proliferation of immature basophils in various internal organs without involvement of the skin. The neoplastic cells, positive immunohistochemically for leukocyte common antigen, possessed lobulated nuclei and weakly metachromatic cytoplasmic granules, predominantly of the basophil type, which exhibited weak naphthol ASD-chloroacetate esterase activity. Mast cell-type granules were also observed ultrastructurally. The neoplastic infiltration was associated with fibrosis in the liver, spleen and bone marrow and with extramedullary hematopoiesis in the liver, spleen, lymph nodes and perihypophyseal tissue. The bone marrow showed uneven and multifocal involvement. Despite the lack of leukemic manifestations and the results of chromosomal analysis, the most suitable diagnosis was aleukemic basophilic leukemia within the category of chronic myeloproliferative disorder. Kinship of this neoplasia to systemic mastocytosis is discussed.
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PMID:An unusual form of chronic myeloproliferative disorder. Aleukemic basophilic leukemia. 203 58

Blood findings in 61 cases of generalized mastocytosis (GM) were evaluated. The cases were divided into two major variants: Systemic mastocytosis (SM; n = 34) with urticaria pigmentosa-like skin lesions, and malignant mastocytosis (MM; n = 27), without skin involvement. The following results were obtained: (1) Significant differences between MM and SM were found in the main haematological parameters (erythrocyte, platelet and leucocyte counts and haemoglobin level); normal values were found in 16 of the SM cases, but never in MM. (2) The main pathological findings were: in SM, anaemia (9/34) and leucocytosis (5/34); and in MM, leucocytosis (19/27), monocytosis (14/27), eosinophilia (12/27), bicytopenia (12/27, mostly anaemia with thrombocytopenia), basophilia (10/27) and isolated anaemia (7/27). (3) The major finding was a significant difference between MM and SM in the incidence of myeloproliferative disorders (MPD), myelodysplasia and mast cell leukaemia (MCL): these disorders occurred in 23 (92%) MM patients, but only in two (6%) SM patients (P less than 0.001). The four instances of MCL and two of myelodysplasia all occurred with MM. Of the 19 cases of MPD, six (SM, 1; MM, 5) were acute variants (acute myeloid and myelomonocytic leukaemias) and 13 (SM, 1; MM, 12) were chronic variants. No case of malignant lymphoma was noted. (4) The blood picture in 10 of 13 chronic MPD cases represented an atypical chronic myeloid leukaemia for which the preliminary descriptive term 'mastocytosis-associated MPD' is proposed. (5) A survey of 103 published cases (SM, 77; MM, 26) yielded similar findings, including a high incidence of MPD and MCL in MM. These findings add further weight to the argument for recognizing SM and MM as two separate entities.
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PMID:Blood findings in generalized mastocytosis: evidence of frequent simultaneous occurrence of myeloproliferative disorders. 201 71

A 22-year-old female was admitted to our hospital because of general fatigue. The lymph nodes, liver and spleen were not palpable. She was without cutaneous lesions. Haematological examinations revealed leukocytes 3,200/microliters with 44% blasts of myelomonocytic origin, and platelets 15,000/microliters. Bone marrow smears were hypercellular marrow with 51% blasts of myelomonocytic origin and focal involvement of mast cells. Serum histamine and vitamin B12 level was high. Mast cells were round with rounded or segmented nuclei. The nucleoli were inconspicuous and the cytoplasm contained a number of metachromatic granules. Cytochemically, mast cells stained positive for alpha-naphthol-AS.D-chloroacetate esterase and acid phosphatase, and negative for peroxidase, Sudan black B and alpha-naphthyl butylate esterase. In toluidine blue staining, mast cells had stained similarly with pH values from 2.5 to 6.5. She was diagnosed as acute myelomonocytic leukemia with benign mastocytosis, and treated with BH.AC-DNP. A complete remission was obtained, but mast cells in the marrow did not decrease. Relationship between leukemia and mastocytosis was not known, but it was suggested that mast cells responded to the proliferation of the leukemic cells.
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PMID:[Acute myelomonocytic leukemia with mastocytosis in bone marrow]. 232 87


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