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)

In mast cell (MC) disorders (mastocytosis), clinical symptoms are caused by the release of chemical mediators from MCs, the pathologic infiltration of neoplastic MCs in tissues, or both. Cutaneous mastocytosis is a benign disease in which MC infiltration is confined to the skin. In pediatric cases cutaneous mastocytosis might regress spontaneously. Systemic mastocytosis (SM) is more frequently diagnosed in adults and is a persistent (clonal) disease of bone marrow-derived myelomastocytic progenitors. The somatic c-kit mutation D816V is found in the majority of such patients. The natural clinical course in SM is variable. Whereas most patients remain at the indolent stage for many years, some have aggressive SM (ASM) at diagnosis. Other patients have an associated clonal hematologic non-MC lineage disease (AHNMD). MC leukemia (MCL) is a rare disease variant characterized by circulating MCs and fatal disease progression. The diagnoses of ASM, SM-AHNMD, and MCL might be confused with a variety of endocrinologic, vascular, or immunologic disorders. It is therefore of particular importance to be aware of the possibility of an underlying (malignant) MC disease in patients with unexplained vascular instability, unexplained (anaphylactoid) shock, idiopathic flushing, diarrhea, headache, and other symptoms that might be mediator related. An important diagnostic clue in such cases is an increased serum tryptase level. The current review provides an overview of mastocytosis and its subvariants and a practical guide that might help to delineate mastocytosis from unrelated systemic disorders.
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PMID:Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. 1524 37

Granulocytic sarcomas are extramedullary tumors (EMD) of malignant myeloid precursor cells. EMD or granulocytic sarcoma of ovary is rare disease. A 15-year-old girl had complaints of abdominal pain and weight loss for 3 months. On physical examination, there were hepatosplenomegaly and a painless mass under the umbilicus. Breast development was grade II. There was no clitoris hypertrophy. Her labia majora were separate and vagina hypoplastic. Hemoglobin level was 9.3 g/dl, white blood cells count 2.8 x 10(6)/1, platelet count 31.6 x 10(9)/1. There were dysplastic features in the blood and bone marrow cells. There were 10 and 22% blasts in the peripheral blood smear and bone marrow, respectively. The levels of serum follicle stimulating and luteinizing hormones were high. An inguinal mass (diameter 9.5 x 7.6) cm was detected on computed tomography. The histopathological diagnosis of this was obtained from laporascopy was composed of ovotestis and there was marked blastic infiltration in this ovotestis which had myeloid markers on flow cytometry. In the immunohistochemical analyses of ovotestis and bone marrow, blasts were positive for LCA, CD-13, CD-33 and CD 68. The cytogenetic analysis of the bone marrow shaved 46 XY karyotype. No response was achieved with combination chemotherapy and the patient died from progressive leukemia. Here we report a rare patient with myelodysplastic syndrome, EMD and hermaphroditism. To our knowledge this is the first case of MDS, EMD and hermaphroditism.
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PMID:Granulocytic sarcoma of the ovotestis: an association of myelodysplastic syndrome and hermaphroditism. 1536 14

Disseminated infection with Prototheca zopfii is a rare disease in immunosuppressed patients. We here report the first case of lethal infection with P. zopfii following unrelated stem cell transplantation for leukemia. Breakthrough protothecosis occurred during long-term administration of voriconazole in the case of pulmonary aspergillosis.
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PMID:Disseminated infection with Prototheca zopfii after unrelated stem cell transplantation for leukemia. 1547 79

(1) Acute promyelocytic leukaemia is a rare disease. There is a high remission rate after combination treatment with tretinoin and anthracycline, but there is no established treatment for refractory or relapsed disease. Further treatment with tretinoin, combined with intensive cytotoxic chemotherapy, seems to give the best results in patients who qualify for this treatment, but assessment is limited. (2) Arsenic trioxide has now been approved for induction of remission and consolidation in patients with refractory or relapsed acute promyelocytic leukaemia. (3) The clinical evaluation dossier that supported the application contains data from two non comparative trials including 12 and 40 patients. A complete haematological response was obtained in 45 (87%) of the 52 patients, and the survival rate among patients in first relapse was 77% after a median follow-up of two years. These results are similar to those previously obtained with tretinoin plus intensive cytotoxic chemotherapy. (4) All the patients treated with arsenic trioxide experienced adverse events. This was to be expected given the acute and chronic toxicity of arsenicals. Most events included fatigue, gastrointestinal disturbances, peripheral neuropathies, prolongation of the QT interval; and biochemical disturbances (hypokalaemia, hyperglycaemia, elevated transaminase activity). (5) Like tretinoin, arsenic trioxide can provoke a potentially severe leukocyte activation syndrome. (6) In practice, these encouraging data justify further assessment of arsenic trioxide. This drug is already an option for patients with refractory or relapsed disease who cannot receive tretinoin plus intensive chemotherapy.
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PMID:Arsenic trioxide: new preparation. Acute promyelocytic leukaemia: encouraging results but persistent doubts. 1553 37

In mast cell (MC) disorders (mastocytosis), clinical symptoms are caused by the release of chemical mediators from MCs, the pathologic infiltration of neoplastic MCs in tissues, or both. Cutaneous mastocytosis is a benign disease in which MC infiltration is confined to the skin. In pediatric cases cutaneous mastocytosis might regress spontaneously. Systemic mastocytosis (SM) is more frequently diagnosed in adults and is a persistent (clonal) disease of bone marrow-derived myelomastocytic progenitors. The somatic c-kit mutation D816V is found in the majority of such patients. The natural clinical course in SM is variable. Whereas most patients remain at the indolent stage for many years, some have aggressive SM (ASM) at diagnosis. Other patients have an associated clonal hematologic none MC lineage disease (AHNMD). MC leukemia (MCL) is a rare disease variant characterized by circulating MCs and fatal disease progression. Two important diagnostic clues in SM are an increased serum tryptase level and the presence of abnormal mast cells in the bone marrow. The current review provides an overview of mastocytosis and its subvariants, the new classification of these diseases, a practical guide for the biological diagnosis and advances and future directions in therapy of these pathologies.
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PMID:[Mastocytosis, classification, biological diagnosis and therapy]. 1556 24

Necrotizing enterocolitis in adults is a rare disease and, in the past, has been associated with nearly uniform mortality. In recent years, necrotizing enterocolitis, now termed neutropenic enterocolitis, in adults has become more prevalent as a complication of aggressive systemic chemotherapy. In this report, we discuss two cases of neutropenic enterocolitis secondary to the administration of systemic chemotherapy in adult cancer patients: one with lung carcinoma, the other with leukemia. Both patients were successfully treated with early surgical intervention for resection of all necrotizing enteric lesions, and subsequent aggressive critical care support. Our experience suggests that early surgical intervention in adult patients with intestinal necrosis due to chemotherapy is essential to avoid mortality from this condition. Given the widespread, aggressive use of systemic chemotherapy in the neoadjuvant setting, patients at risk for this potentially lethal complication of neutropenic enterocolitis are increasingly common.
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PMID:Neutropenic enterocolitis in adults: case series and review of the literature. 1574 75

Phlegmonous gastritis is an acute infection of the stomach wall by pyogenic bacteria. It represents an extremely rare disease with a fulminating course and a high mortality rate. A precise lifetime diagnosis is generally unsuccessful. The inflammation, most often caused by alpha-hemolytic streptococci, is most frequently expressed in patients who are more susceptible to infection. Among these are elderly patients, women, patients with chronic gastritis, chronic peptic ulcer, hepatic cirrhosis and decreased immune tolerance, T-cell leukemia, patients with a low socio-economic status and alcoholics. In our paper we are describing the case of a 66-year old female patient, who had received many years of treatment for rheumatoid arthritis. She died due to phlegmonous gastritis, which was only established post-mortem. The authors share the opinion that the occurrence of the phlegmonous form of gastritis was influenced significantly by the treatment with nonsteroidal antirheumatics and corticosteroids, which she had received for many years and also immediately prior to the complication.
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PMID:Phlegmonous gastritis in a patient with rheumatoid arthritis. 1598 17

Some myeloproliferative disorders (MPD) result from a reciprocal translocation that involves the FGFR1 gene and a partner gene. The event creates a chimeric gene that encodes a fusion protein with constitutive FGFR1 tyrosine kinase activity. FGFR1-MPD is a rare disease, but its study may provide interesting clues on different processes such as cell signalling, oncogenesis and stem cell renewal. Some partners of FGFR1 are centrosomal proteins. The corresponding oncogenic fusion kinases are targeted to the centrosome. Constitutive phosphorylation at this site may perturbate centrosome function and the cell cycle. Direct attack at this small organelle may be an efficient way for oncogenes to alter regulation of signalling for proliferation and survival and get rid of checkpoints in cell cycle progression. The same effect might be triggered by other fusion kinases in other MPD and non-MPD malignancies.
Leukemia 2005 Oct
PMID:Myeloproliferative disorders: the centrosome connection. 1610 84

B-prolymphocytic leukemia (B-PLL) is a rare disease with poor prognosis. To further characterize the biological features of this disease, we analyzed immunoglobulin heavy chain (IgVH) mutations, ZAP-70 and CD38 in 19 cases with de novo B-PLL. Immunoglobulin heavy chain genes analysis showed an unmutated pattern (>98% homology to germ line) in 9/17 cases (53%), with 100% homology in eight. In the remaining, it ranged from 90 to 97.4%, with three cases slightly mutated (98-95%) and five heavily mutated (<95%). All B-PLL utilized members of VH3 (11/17) and VH4 (6/17) families, with V3-23, V4-59 and V4-34 gene accounting for more than half of them, regardless of mutational status. ZAP-70, assessed by flow cytometry, ranged from 1 to 91% cells, being > or =20% in 57% of cases. CD38 ranged from 1 to 99% (median 21%). There was no correlation between IgVH status and ZAP-70 or CD38 expression, but male gender and del(17p) were more common in the unmutated group. Neither IgVH mutations, CD38 expression nor del(17p) influenced patients' outcome. Unexpectedly, ZAP-70+ B-PLL patients survived longer (40 months) than ZAP-70- B-PLL (8 months). B-PLL appears biologically heterogeneous regarding IgVH mutations, ZAP-70 and CD38 expression, showing a pattern distinct from that of other lymphoproliferative disorders.
Leukemia 2006 Jul
PMID:IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL). 1664 47

Adult cutaneous Langerhans cell histiocytosis (LCH) is a rare disease. We report two cases illustrating the variability of the clinical presentation and the response to treatment. In both cases a remission was achieved: in one case a partial remission with psoralen plus UVA irradiation (PUVA) and methotrexate plus topical corticosteroid ointment; in the other case by treatment with thalidomide. Despite a therapeutic response, both patients later developed haematological malignancies: a chronic myelo-monocytic leukaemia and an acute lymphatic leukaemia. In conclusion, patients with adult cutaneous LCH should be monitored carefully so that a secondary malignancy is not overlooked.
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PMID:Cutaneous Langerhans cell histiocytosis with subsequent development of haematological malignancies. Report of two cases. 1699 7


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