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  • 1
    ISSN: 1569-8041
    Keywords: chronic lymphocytic leukemia ; hematopoietic stem cell transplantation ; minimal residual disease ; molecular remissions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: Stem-cell transplantation is a reasonable therapeutic approach for younger patients with high-risk CLL. Patients and methods: Twelve patients (seven males; median age 47 years, range 29–51) with high-risk CLL underwent transplantation (allo, n = 7; auto, n = 5). The conditioning regimen consisted of cyclophosphamide and total body irradiation in 11 patients, and BEAC in the remaining one. Minimal residual disease (MRD) was assessed by cytofluorometry and PCR. Results: All 11 evaluable patients engrafted. Of the seven allografted patients, two died of treatment-related causes; three patients developed acute GVHD. No transplant-related mortality was observed in autografted patients. After transplantation, 10 of 11 patients evaluable for response achieved CR (91%; 95% CI 59%–100%) which was molecular in nine patients (82%; 95% CI 48%–98%). One patient in CR but MRD+ relapsed nine months after transplantation and died. Seven patients remain in molecular CR for a median of 16 months (range 1–58). Estimated actuarial survival and disease-free survival at two years is 81% (95% CI 43%–100%) and 71% (95% CI 43%–99%), respectively. Relapse risk at two years is 12.5% (95% CI 0%–35.5%). Conclusions: Patients with high-risk CLL can achieve long-lasting molecular CR after SCT. The role of transplants in CLL management deserves investigation in controlled trials.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0584
    Keywords: Chlorambucil ; Alpha-2b interferon ; Chronic lymphocytic leukemia ; Treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Eleven patients with early CLL (two in Rai's stage 0, seven in stage I, and two in stage II) received aIFN (3 MU subcutaneously three times a week for a median of 8 months; range, 4–12) after their tumor mass had been reduced with intermittent chlorambucil. Following chlorambucil/aIFN administration, a significant reduction in blood lymphocyte counts (from 25.1±12.0×109/1 to 6.3±5.32×109/1;p〈0.001) and in CD 19-positive cells (from 21.0±12.0×109/1 to 3.8±2.3×109/1;p〈 0.001) was observed. Three of seven patients with stage-I and one of two with stage-II disease moved to stage 0, and a complete response (CR) was observed in two patients with stage-I at diagnosis. Overall, there were eight patients who, after treatment, had either a CR (2 cases) or stage-0 disease (6 cases), which compares favorably with two patients with stage-0 disease before entering the study (p=0.015). In five patients (including the two who achieved a CR) aIFN further improved the disease status achieved with chlorambucil.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0584
    Keywords: Granular lymphocytes ; Proliferative disorders ; T lymphocytes ; NK cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A series of 20 patients with granular lymphocyte proliferative disorders (GLPD) is reported. The criterion of inclusion was presence of persistent (≥6 months) granular lymphocytosis in the absence of any causative illness. Diagnoses made upon analytical control in half the patients of splenomegaly (25%) and hepatomegaly (25%) were infrequent. Clinical course was nonprogressive in 17/20 patients, but two developed high-grade NHL several years later and one showed progressive disease. Actuarial probability of survival at 5 years was 85%. Granular lymphocyte morphology was relatively homogeneous, and peripheral blood counts were preserved in the most patients. Bone marrow lymphocytic infiltration was low, as assessed by bone marrow aspiration and/or biopsy. Eosinophilia was an outstanding feature in eight cases. Ultrastructurally, all cases showed parallel tubular arrays; cytoplasmic granules and numerous short microvilli were noticed. The lymphoid phenotype was heterogeneous, the most common being CD2+CD3+CD4-CD8+, but six patients (30%) were CD4+ with variable expression of natural killer-associated antigens. Chromosomal analysis was abnormal in 4/10 patients [trisomy 19, t(5;6); inv(14) and inv(10)]. The study of Β-chain of the T-cell receptor revealed clonal rearrangements in 14 (78%), restricted to CD3+ patients (92%). In vitro culture of myeloid precursors showed decreased CFU-GM in 5/6 patients. Virological studies for HTLV-I and II were negative. In conclusion, the presence of a clonal proliferation was not correlated with the clinical course or an associated disease.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0584
    Keywords: Key words Anaplastic large-cell lymphoma ; leukemia ; ALK-1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Anaplastic large-cell lymphoma (ALCL) is a lymphoproliferative disorder that frequently presents with disseminated disease and extranodal involvement. Rare atypical cells have been detected in the peripheral blood in occasional cases. However, the presence of a prominent leukemic phase is extremely rare in these patients. We describe a patient with a small-cell variant of ALCL of T-cell phenotype, ALK-1 positive, who developed a rapid leukemic phase in association with the progression of the disease. Similar to the nodal biopsy, the predominant cells in bone marrow and peripheral blood were small atypical lymphoid cells. The large tumor cells expressed ALK immunoreactivity with a cytoplasmic and nuclear pattern, whereas some of the small cells showed only a nuclear-restricted pattern of staining. An RT-PCR study detected the NPM-ALK chimeric product in the nodal biopsy and in a peripheral blood sample in the early phase of the disease, but it became negative in a peripheral blood sample obtained after completion of the chemotherapy treatment, suggesting that this assay may be useful in the follow-up of these patients. This case indicates that a prominent leukemic phase may develop in ALCL as a manifestation of tumor dissemination and that it may be composed of a predominant small-cell atypical component.
    Type of Medium: Electronic Resource
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