Polycythaemia vera is a clonal disorder of the haemopoietic stem cell causing a pathologic expansion of the erythroid and sometimes the megakaryocytic and myeloid elements. In order to avoid the possible mutagenic effects of radioactive phosphorus, alkylating agents and hydroxyurea, since 1988 alpha-IFN has been used for the treatment of PV and has been shown to induce and maintain haematological remission. We describe a 24-year-old PV patient with chromosomal abnormalities who achieved not only a reduction of the proliferation of erythroid elements and reticulin content in the bone marrow, but also a complete cytogenetic remission after IFN treatment.
Interferon alpha (alpha-IFN) is increasingly used for the treatment of patients affected by polycythemia vera (PV). As prior studies are difficult to interpret in view of the lack of appropriate controls, we undertook a randomized comparison of lymphoblastoid alpha-IFN (alpha n-1 IFN) treatment against venesection treatment alone. In a crossover trial, we treated 22 PV patients alternatively for 5 months each with 3 MU/day sc of alpha n-1 IFN and phlebotomy alone. During IFN treatment, red blood cell count and hematocrit level were well controlled in both trial groups, reducing or eliminating the need for phlebotomy in all patients; furthermore, platelet number and white blood cell count declined during alpha-IFN therapy. In addition, the number of symptomatic patients was greatly reduced, and in six patients a reduction in splenic size was observed. Finally, the only patient with chromosomal abnormalities showed a complete cytogenetic conversion after 5 months of alpha-IFN therapy. Thus, for the first time, our results provide the unequivocal demonstration that alpha-IFN is superior to phlebotomy in controlling the pathologic expansion of erythroid elements and all the clinical aspects of this disease.
In essential thrombocythemia patients alpha-IFN rapidly reduces platelet count, and it is also able to maintain a low count during long-term treatment. In order to verify if long-term IFN treatment can produce sustained remission in selected patients, we decided to suspend IFN treatment in two subsets of 21 patients on long-term alpha-IFN treatment: (a) all six patients who had shown a platelet count below 450 x 10(9)/l for at least 2 months with 3 MU once a week; (b) three patients who had shown the same platelet count for at least 2 months with 3 MU three times a week. After withdrawal of alpha-IFN treatment, a rapid increase in the platelet count was observed in all three patients requiring 3 MU three times a week. Three of the six patients treated with 3 MU once a week are still free of symptoms and have been in complete hematological remission (platelet count below 450 x 10(9)/l) for 9+, 13+, and 14+ months, respectively. As far as the three remaining cases are concerned, one was not assessable because of loss to follow-up, while the other two relapsed after 1 and 2 months. We believe that the three cases of sustained remission might be the result of a long-term tumor load reduction produced by the alpha-IFN treatment. Finally, the factor best able to predict sustained, unmaintained remission seems to be the clinical response to a low dose of alpha-IFN during the maintenance phase, rather than disease features prior to treatment.
Objective: Arthritis is often associated with comorbidities. For many of them, such as hypertension, cardiovascular disease, chronic pulmonary disease, and upper gastrointestinal disease, arthritis and its treatment may also represent a risk factor. This study is concerned with an evaluation of the frequency of comorbidities in a cohort of patients with rheumatoid arthritis (RA). Methods: The discharge diagnoses of patients with RA during the period 1 January 1997 to 31 December 2000 were retrieved from the database of the Department of Internal Medicine of the University of Genova, Italy. The diagnosis of RA was made if the patient’s discharge record contained the code 714 of the International Classification of Diseases, IX revision, as first 3 numbers. The other diagnoses were also recorded along with demographic data, type and duration of hospital stay, and performed procedures. Results: During the study period, 427 patients with RA were admitted to the hospital for a total number of 761 admissions, which represented 2.2% of total admissions. Ninety-one (21.3%) patients did not have comorbidities, whereas 336 (78.6%) had one or more comorbidities. The most frequently observed comorbidities were cardiovascular diseases (34.6%), including hypertension (14.5%) and angina (3.5%), followed by gastrointestinal (24.5%), genito-urinary (18.7%) and respiratory (17%) diseases. There was a male predominance (p=0.004) within patients with comorbidities, who were significantly older (64.2±3.2 years vs. 57.2±4.2 years; p<0.001) and required longer periods of hospital stay (22.7 days vs. 12.5 days; p<0.001). Conclusions: Comorbidities are present in nearly 80% of RA inpatients. Comorbidity is a good predictor of health outcome, health services utilization, and medical costs. Because RA comorbidity can act as confounder, it should be considered in epidemiologic studies and clinical trials
Using the APAAP technique, we assessed the reactivity of five monoclonal antibodies, JSB1, C219, Mab 57, 2F8 and MRK16, to gp 170 in 60 cases of de novo acute myeloid leukemia (AML) and 13 relapses. Reactivity, varied between the five antibodies, and positivity was obtained with 2F8 > JSB1 > MRK16 > Mab57 > C219. Sixteen of the 60 cases were also studied by flow cytometry. In 10 cases, the results with the two techniques corresponded, in the other 6 cases, flow cytometry proved more sensitive than APAAP in detecting small amounts of gp170. In the flow cytometry analysis, the cells fixed in methanol and paraformaldehyde were more fluorescent than unfixed samples or those fixed in paraformaldehyde or methanol alone. Our results thus reveal that positivity for gp 170 depends on various factors, including the specificity of the monoclonal antibodies, the techniques used and the preservation of the samples. This suggests the need for a clear standardization of the methods to detect gp170.
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