[Chronic myelomonocytic leukemia and hypersensitivity vasculitis].
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Biomedical subjects
Publications and source records attributed to J M Ribera.
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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 x 10(9)/l to 6.3 +/- 5.32 x 10(9)/l; p less than 0.001) and in CD 19-positive cells (from 21.0 +/- 12.0 x 10(9)/l to 3.8 +/- 2.3 x 10(9)/l; p less than 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.
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The role of alpha interferon in patients with chronic lymphocytic leukaemia (CLL) has yet to be well established. In studies carried out to date, a significantly higher response rate has been observed in previously untreated patients compared to those who have received prior chemotherapy. Patients with early-stage CLL also respond better than patients with advanced disease. Responses to alpha interferon are transient and complete responses are rare. It is not yet known whether alpha interferon can induce clonal remission, and response is usually measured in terms of the reduction in peripheral blood lymphocyte levels. In one study, a normalization of immunoglobulin levels was observed, and in another there was an increase in the absolute number of granulocytes. Further studies are needed to investigate the role of combined therapy with alpha interferon and cytotoxic agents or other cytokines, and to assess the ability of alpha interferon to prolong response duration after remission induction with chemotherapy. Toxicity is tolerable when alpha interferon is given in a low dose (e.g., 2 million units (MU)/m2 three times a week) and low doses have been shown to be as effective as high doses in CLL patients.
Non-Hodgkin's lymphomas (NHL) are part of the spectrum of disease associated with HIV infection. However, there are only occasional reports of NHL of T-cell origin in HIV-infected patients. A previously asymptomatic HIV-infected man, who was seronegative for human T-lymphotropic virus type I antibodies, developed a high-grade peripheral T-cell lymphoma of anaplastic large-cell type which was Ki-1 + (CD30 +), HLA-DR+, epithelial membrane antigen +, CD25 +, CD71 +, CD2 + and CD5 +. Pan-B markers CD19 and CD22 and histiocytic marker CD68 were negative. At diagnosis the patient had 0.3 x 10(9)/l T-helper lymphocytes. The response to chemotherapy was dramatic and the patient is alive and disease-free 18 months after treatment. A review of previously described peripheral T-cell lymphomas in HIV-positive individuals is performed, and we conclude that the spectrum of neoplasms in such cases is probably broader than originally thought.
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One hundred ninety-five male and female patients over 65 years old presenting with urinary incontinence were evaluated by clinical and urodynamics. Urinary incontinence was in the form of urgency-incontinence in 68% of the cases, incontinence at cough in 26%, and urinary incontinence which the patient referred to no specific situation in 6%. Among the different urodynamics data, bladder instability was the most common (73%). Urinary stress incontinence was observed in 35% of the cases. Bladder instability was demonstrated in 77% of the cases with urgency-incontinence and in 69% of the cases with incontinence not referred to a specific situation. All the male patients with lower urinary tract obstruction had urgency-incontinence. Stress incontinence was demonstrated in 60% of the cases with incontinence at cough. Stress incontinence was not observed in male patients without associated diseases. The associated neurological disorder was accompanied by bladder hyperreflexia in 90% of the cases. A prior adenomectomy or hysterectomy was associated with a greater number of cases with stress urinary incontinence, accounting for 62% in both males and females.
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The presence of antibodies against HTLV-I was investigated with ELISA and Western blot techniques in 76 patients with lymphoproliferative syndromes (n = 53), idiopathic subacute myelopathies (n = 4), chronic T-lymphocytosis (n = 3) and collagen vascular diseases (n = 16) diagnosed in the Barcelona area. 9 HIV seropositive patients were included: 7 with lymphoma, 1 with chronic T-lymphocytosis and 1 with polymyositis. Although 3 samples were repeatedly positive with ELISA, none was confirmed with Western blot. These results suggest that in our country, contrasting with areas where HTLV-I infection is endemic, acute and chronic lymphoproliferative diseases, collagen vascular diseases and demyelinating neurologic diseases are not associated with HTLV-I infection. The development of lymphoma in patients seropositive for HIV also could not be related with HTLV-I infection.
Sepsis due to Candida parapsilosis with involvement of the joints and the lungs, respectively, is reported in two patients with acute leukemia. The first patient had ankle arthritis 72 days after an allogenic bone marrow transplant for acute lymphoblastic leukemia. The second patient had pneumonia with cavitation during pancytopenia after chemotherapy for acute monocytic leukemia. In both cases, C. parapsilosis sepsis responded to therapy with amphotericin B, associated with miconazole in the first patient and with 5-fluorocytosine in the second one. The rarity of septic foci during C. parapsilosis fungemia and the good outcome of both patients are emphasized. This good result was probably due to early antifungal therapy and the relatively rapid recovery of granulocytopenia.
The value of Y-body study for assessment of stromal cell engraftment was analyzed in 25 patients submitted to allogeneic bone marrow transplantation (BMT) (sex-matched in 12 cases and sex-mismatched in 13). The study was performed weekly on bone marrow smears until day +35, and the results were compared with those obtained in a control group of 20 patients submitted to autologous BMT (12 males and 8 females). Engraftment of haemopoietic cells was documented in all cases. The results of Y-body study on the recipients' fibroblast cells showed a pattern identical to that observed prior to BMT, independent of donor's sex. On the other hand, there were no differences between allogeneic and autologous BMT recipients in regard to percentage of Y-body positive cells. These results indicate that in allogeneic BMT there is no engraftment of the fibroblastic component of bone marrow stroma.
We have analysed the immunological characteristics of blasts from 89 acute lymphoblastic leukaemia (ALL) cases (62 adults and 27 children), by using a panel of antilymphoid and myeloid associated monoclonal antibodies (McAb) and the APAAP method, which detects membrane and cytoplasmic expression of antigens. The McAb CD19 was the marker most consistently expressed in B lineage ALL, being positive in 100% of cases, compared to CD24 and CD22 expressed in 82% and 79%, respectively. Similarly, for the T lymphoid lineage, the McAb CD3 was the most reliable and specific marker, being expressed in all T-ALL cases including those with an early thymic phenotype (CD7+, TdT+). Lymphoblasts from eight adults (12.9%) and three children (11.1%) expressed one to four myeloid associated antigens recognized by CD13, CD14, CD33 and anti-myeloperoxidase. There were no substantial clinical and morphological differences between the two ALL groups with or without myeloid associated markers. However, the presence of myeloid associated markers in adult ALL was associated with a significantly lower complete remission (CR) rate (P = 0.05) and with a shorter survival (P = 0.001); this variable was independent of advanced age and high WBC. It is concluded that immunophenotypic analysis in ALL should include myeloid markers for its probable prognostic implications.