Cases reported "Leukemia"

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1/20. Characterization of Epstein-Barr virus (EBV)-infected natural killer (NK) cell proliferation in patients with severe mosquito allergy; establishment of an IL-2-dependent NK-like cell line.

    The clinical evidence of a relationship between severe hypersensitivity to mosquito bite (HMB) and clonal expansion of EBV-infected NK cells has been accumulated. In order to clarify the mechanism of EBV-induced NK cell proliferation and its relationship with high incidence of leukaemias or lymphomas in HMB patients, we studied clonally expanded NK cells from three HMB patients and succeeded in establishing an EBV-infected NK-like cell line designated KAI3. immunoblotting and reverse transcriptase-polymerase chain reaction (RT-PCR) analyses revealed that KAI3 cells as well as infected NK cells exhibited an EBV latent infection type II, where EBV gene expression was limited to EBNA 1 and LMP1. As KAI3 was established by culture with IL-2, IL-2 responsiveness of peripheral blood NK cells from patients was examined. The results represented markedly augmented IL-2-induced IL-2R alpha expression in NK cells. This characteristic property may contribute to the persistent expansion of infected NK cells. However, KAI3 cells as well as the NK cells from patients were not protected from apoptosis induced by either an anti-Fas antibody or NK-sensitive k562 cells. Preserved sensitivity to apoptosis might explain the relatively regulated NK cell numbers in the peripheral blood of the patients. To our knowledge, KAI3 is the first reported NK-like cell line established from patients of severe chronic active EBV infection (SCAEBV) before the onset of leukaemias or lymphomas. KAI3 cells will contribute to the study of EBV persistency in the NK cell environment and its relationship with high incidence of leukaemias or lymphomas in HMB patients.
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2/20. Epidemiological investigation of ochrobactrum anthropi strains isolated from a haematology unit.

    ochrobactrum anthropi is an oxidase-producing gram-negative bacillus preferring aqueous environments. It is an opportunist of low pathogenicity with a wide and unpredictable antibiotic resistance. We observed bacteraemia caused by this organism in two immunocompromized patients hospitalized in the same haematology unit and catheter-associated sepsis was recognized within two days. Another isolate was obtained from the stools of a third patient of the same unit. Environmental investigations recovered an isolate from a tap-water sample of the unit. Pulsed-field gel electrophoresis analysis of these four isolates and two others isolates previously found in the same ward, showed identical restriction patterns for the two blood isolates and confirmed that the two bacteraemia were epidemiologically related.
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3/20. Two cases showing clonal progression with full evolution from aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome to myelodysplastic syndromes and leukemia.

    We report 2 paroxysmal nocturnal hemoglobinuria (PNH) patients who were initially diagnosed with aplastic anemia and sequentially developed PNH, myelodysplastic syndromes (MDS), and leukemia. flow cytometry and cytogenetic analysis showed the initial appearance and expansion of PNH clones, gradual replacement of PNH clones by MDS clones with monosomy 7, and then expansion of MDS clones or their subclones with additional chromosomal abnormalities. In relation to these developments, expression increased of the Wilms' tumor gene WT1, a marker for leukemic progression. These patients not only shared bone marrow failure but also might have harbored a hematopoietic environment favorable for the emergence of abnormal clones leading to leukemogenesis.
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4/20. Successful treatment of disseminated fusarium infection in an infant with leukemia.

    Disseminated fusarium infection in an immunocompromised host is intractable and results in high mortality. We provide the first full case report on successful treatment of a disseminated fusarium infection in an infant. The 6-month-old infant, whose family raised livestock, had infantile leukemia. During the neutropenic period after intensive chemotherapy, vomiting, diarrhea, fever, subcutaneous nodes, and coughing appeared. pneumonia was diagnosed, and fusarium moniliforme was isolated from blood culture. A central venous catheter was removed. granulocyte colony-stimulating factor (G-CSF) and amphotericin b (AMPH-B) (total dose, 65 mg/kg) were administered continuously for 8 weeks. The infection was resolved according to improvement of clinical and laboratory findings, and intensive chemotherapy was restarted for the leukemia. cord blood stem cell transplantation from an unrelated donor was performed. The fusarium infection did not recur, but after transplantation, leukemia relapsed. Treatment of neutrophils using G-CSF, AMPH-B, and local treatment induced resolution of the disseminated fusarium infection in this immunocompromised host with malignancy. We suggest caution for patients living in an environment conducive to the development of fusarium infection because of the particular risk of infection.
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5/20. Recurrent sphingomonas paucimobilis -bacteraemia associated with a multi-bacterial water-borne epidemic among neutropenic patients.

    A cluster of septicaemias due to several water-related species occurred in a haematological unit of a university hospital. In recurrent septicaemias of a leukaemic patient caused by sphingomonas paucimobilis, genotyping of the blood isolates by use of random amplified polymorphic dna-analysis verified the presence of two distinct S. paucimobilis strains during two of the separate episodes. A strain of S. paucimobilis identical to one of the patient's was isolated from tap water collected in the haematological unit. Thus S. paucimobilis present in blood cultures was directly linked to bacterial colonization of the hospital water system. Heterogeneous finger-printing patterns among the clinical and environmental isolates indicated the distribution of a variety of S. paucimobilis clones in the hospital environment. This link also explained the multi-microbial nature of the outbreak.
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6/20. The multiple tumor syndrome. Personal experience.

    The multiple tumor syndrome is an unusual pathologic condition, which consists in association of multiple malignancies in the same patient. Seven cases are discussed: two women, five men, aged 32-70 years. The period between the two neoplasias was 2-23 years (in 6 cases). In one case the two malignancies appeared concomitantly. The hematological malignancies were: multiple myeloma: 2 cases; chronic granulocytic leukemia: 2 cases; chronic lymphatic leukemia: 3 cases. In four cases, the solid tumor followed the hematological malignancy at variable periods (2 and 4 years). In other two cases, the solid tumors preceded the hematological malignancy with 2 years, 23 years respectively. The solid tumors were genital cancers, malignant melanoma, spino-cellular carcinoma, thyroid cancer, hemangiosarcoma. In a single case the second tumor was a hematological malignancy too (NHL-diffuse lymphocytic lymphoma). Possible implications of previous therapy and environmental factors are discussed.
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7/20. candida glabrata fungemia in transplant patients receiving voriconazole after fluconazole.

    The clinical impact of voriconazole resistance in candida glabrata is not well described. Five hematopoietic stem cell transplant recipients that developed breakthrough candida glabrata bloodstream infections while receiving voriconazole are described and the clinical management and susceptibility profiles of their isolates are reported. All patients were markedly immunosuppressed, and in all cases, voriconazole use was preceded by prolonged fluconazole exposure (median 60 days); median voriconazole exposure prior to candidemia was 48 days. Isolates from 4 patients were shown to be resistant to fluconazole and itraconazole when tested in vitro; these same isolates had MICs to voriconazole and posaconazole > or = 2 microg/ml. Clinical failure to voriconazole may result from deficits in host defense, retained infected foci, and adaptation of the organism to environmental pressures, the specific sequence and mechanisms of which warrant further study. Clinicians must maintain a high index of suspicion for these infections in highly susceptible hosts despite voriconazole therapy, particularly when voriconazole use is preceded by prolonged fluconazole exposure.
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8/20. Chromosomal changes in secondary leukemias of childhood and young adulthood.

    The increasing success of antineoplastic therapy has resulted in a growing number of long-term survivors. These people are at risk for complications of the therapy itself. Among these induced acute nonlymphoid leukemia (ANLL) has been both common and often lethal. We reviewed 72 recently reported patients under 30 years of age at the time of initial diagnosis who developed a secondary, karyotypically defined leukemia. Fifty-eight patients contracted ANLL a mean of 4 1/2 years from the initial diagnosis. In 25 patients, this was preceded by a preleukemic phase characterized by a hypercellular bone marrow with abnormal precursors, often accompanied by peripheral pancytopenia, that lasted a mean of 6 months. Three additional patients died in this preleukemic phase. In all 61, the most common chromosomal abnormalities were numerical errors. Twenty-four patients had a hypodiploid karyotype, most often in those in whom the primary diagnosis was lymphoma (22 of 43). The most common chromosomes missing in whole or in part were number 7 (18 patients), number 5 (8 patients), number 17 (5 patients), and number 21 (4 patients). The anomalies were frequently multiple and complex. monosomy 7 figured particularly strongly and may be similar to a karyotypically identical myeloproliferative disorder characterized by micromegakaryocytes, giant platelets, and abnormal granulocyte function arising de novo in children. These findings are similar to those in older patients with ANLL induced by environmental carcinogens or antineoplastic therapy. They are different from the karyotypic changes seen in de novo ANLL in children and young adults, suggesting a different etiology. Also, they reinforce the need to find less leukemogenic treatment programs.
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9/20. Translocation (6;9)(p23;q34) in smoldering leukemia and acute nonlymphocytic leukemia.

    Two further cases with myeloproliferative disorders (a child with smoldering leukemia and a young male with acute nonlymphocytic leukemia of FAB type M2) and a translocation t(6;9)(p23;q34) are described. Special attention is paid to environmental factors and the early age of onset of patients within the group of leukemias with this specific translocation. In one of our cases a secondary chromosomal anomaly has arisen, which is comparable with another case from the literature.
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10/20. Acute leukemia and preleukemia in eight males in a family: an X-linked disorder?

    Eight males in a family died of acute leukemia or a potentially preleukemic blood disease. Three patients were descendants of a man through his first wife, and the other five through his second wife. The pattern of disease in the family suggests X-linked inheritance through the two wives, although other mechanisms cannot be excluded. No detectable action of environmental leukemogens was observed in this family disorder.
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