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Biomedical subjects

A Wahlin

Publications and source records attributed to A Wahlin.

At least 55 records · Page 3Linked to original sources

Serum thymidine kinase in congenital dyserythropoietic anaemia type III.

Serum thymidine kinase (TK) was determined in a family with congenital dyserythropoietic anaemia type III (CDA, type III). 20 patients and 10 of their healthy siblings were investigated. Elevated TK was found in all 20 patients (median 56.2 U) but their healthy siblings had normal values (median 2.65 U). We suggest that determination of TK should be used for discrimination between healthy siblings and individuals affected by CDA type III when bone marrow examination is not suitable.

Anemia, Dyserythropoietic, Congenital↗

Intravascular haemolysis and increased prevalence of myeloma and monoclonal gammopathy in congenital dyserythropoietic anaemia, type III.

A family with congenital dyserythropoietic anaemia type III was studied. Twenty patients and 10 of their healthy siblings were clinically examined and questioned about their medical history. Blood sampling and bone marrow aspirations were also performed. Forty-five percent of the patients reported symptoms of anaemia and 35% regularly felt weakness, fatigue, or headache. However, the majority of the patients regarded themselves as healthy. The bone marrow showed a uniform picture of erythroid hyperplasia with multinuclear erythroblasts and gigantoblasts with up to 12 nuclei. There was laboratory evidence of intravascular haemolysis and mild anaemia. We also observed a high prevalence of monoclonal gammopathy of undetermined significance (3 cases) and myeloma (1 case) among the patients.

Anemia, Dyserythropoietic, Congenital↗

Prior knowledge and face recognition in a community-based sample of healthy, very old adults.

This experiment addressed the effects of prior knowledge on episodic face recognition in a community-based sample of healthy older adults from 75 to 96 years of age (N = 228). Subjects were presented with photographs of public Swedish individuals who attained their fame during the 1930s and 1940s, or during the 1980s. Results indicated an overall deterioration of face recognition performance across age. However, performance was higher with the dated than with the contemporary faces across the entire age range, suggesting that the ability to utilize prior knowledge in the context of face recognition is well preserved in very old age. Finally, a multiple regression analysis indicated that, among a variety of demographic, psychometric, and biological variables, age was the best predictor of face recognition performance.

Aged↗

Observations on two members of the Swedish family with congenital dyserythropoietic anaemia, type III.

Two affected individuals of the Swedish family with CDA, type III, in which the disease is transmitted as an autosomal dominant character, were studied. Both cases displayed features hitherto undescribed in this family but described in patients with CDA, type III, in whom the inheritance may have been as an autosomal recessive character. Such features were: (a) haemosiderinuria, (b) grossly disorganised erythroblast nuclei, (c) differences in the ultrastructural appearances of individual nuclei within the same multinucleate erythroblast and (d) intraerythroblastic inclusions resembling precipitated globin chains. In both cases the giant mononucleate erythroblasts and the multinucleate erythroblasts had total DNA contents up to 28c (1c = haploid DNA content) and 48c respectively, and some DNA synthesising bi- and multinucleate erythroblasts contained one or more nuclei which were unlabelled with 3H-thymidine. These findings are similar to those in patients with the autosomal recessive type of disease. Thus no major phenotypic differences are yet apparent between cases of CDA, type III, with different patterns of inheritance. Analysis of the surface erythrocyte proteins of the 2 Swedish CDA, type III, patients with monoclonal antibodies recognising Band 3, glycophorins A, B, C and D, Rh, CD44, CD47, CD55, CD58, CD59, Lutheran, Kell, LW and acetylcholinesterase did not reveal any gross abnormality of expression of these proteins. A slightly altered expression of blood group antigens A and H was revealed by the lectins Dolichos biflorus and Ulex europaeus and the Mr of Band 3 as judged by SDS polyacrylamide gel electrophoresis was also slightly reduced, suggesting that there may be minor alterations in the degree of N-glycosylation of some red cell membrane constituents.

ABO Blood-Group System↗

[Morning toilette].

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Activities of Daily Living↗

A pilot study of piperacillin and ciprofloxacin as initial therapy for fever in severely neutropenic leukemia patients.

We studied the efficacy of piperacillin and ciprofloxacin as initial parenteral therapy in 41 adult patients with leukemia who developed 47 febrile episodes during severe neutropenia following chemotherapy. 40 patients (98%) survived their febrile episode(s), whereas 1 patient died of infection. When assessed at 72 h after initiation of treatment (early evaluation), 24/47 episodes (51%) had been successfully treated. These 24 favourable responses were seen in 15/24 (63%) microbiologically documented infections and 9/19 (47%) fever of unknown origin (FUO). At the resolution of fever (late evaluation) 46 episodes were evaluable, and 28 (61%) had responded successfully to piperacillin and ciprofloxacin. Successful treatment was most frequently observed in microbiologically defined infections, 18/23 (78%). Three of 5 (60%) Gram-positive, 11/12 (92%) Gram-negative and 1 of 2 mixed bacteremias were successfully treated. In contrast, only 10/19 (53%) FUO and none of 4 clinically defined infections had responded. Thus, this pilot study indicates that piperacillin and ciprofloxacin may be a safe and effective combination for the treatment of febrile episodes in severely neutropenic leukemia patients, which merits further investigation in randomized trials.

Adult↗

Multiple DNA rearrangements in the BCL2 region in a patient with follicular lymphoma.

A 39-year-old male with follicular non-Hodgkin's lymphoma was repeatedly studied with respect to DNA rearrangements with the two probes pFL-1 and pFL-2, representing two segments of chromosome 18. The oncogene BCL2, detected by pFL-1, was as expected translocated to the J region of the immunoglobulin locus. The standard BCL2 translocation was found in three samples, one obtained at diagnosis, one ten months later, and one after 5 years. Another translocation was found with the probe pFL-2 hybridizing with a region located about 20 kb 3' from BCL2. This latter rearrangement was found only in the first biopsy, which was obtained at the time of diagnosis, but not in the two later samples, when the morphology of the lymphoma was unchanged. No cytotoxic therapy had been given in the interval from diagnosis to disappearance of the latter rearrangement. Thus, one of the observed translocations (pFL-2) was detected only in the first biopsy, while the other (pFL-1) was a clonal marker in all three biopsies. The finding suggests that clonal evolution does not necessarily mean clinical progression.

Adult↗

Mitoxantrone and cytarabine versus daunorubicin and cytarabine in previously untreated patients with acute myeloid leukemia.

A total of 44 adults aged 18-78 years were allocated to an open randomized study whose aim was to compare the efficacy and toxicity of mitoxantrone with those of daunorubicin in previously untreated patients presenting with acute myeloid leukemia. In one arm, induction treatment consisted of mitoxantrone plus cytarabine given on a 3- plus 7-day schedule. Post-induction treatment consisted of two courses of mitoxantrone plus cytarabine given on a 2- plus 5-day schedule. In the control arm, mitoxantrone was replaced by daunorubicin. In all, 14 of 21 eligible and evaluable patients in the mitoxantrone arm achieved a complete remission (CR). In the control arm, 14 of 20 subjects attained a CR. The median survival was 365 days for patients randomized to mitoxantrone-cytarabine and 401 days for those given daunorubicin-cytarabine. The efficacy and toxicity of mitoxantrone were similar to those of daunorubicin.

Acute Disease↗

Remission rate and survival in acute myeloid leukemia: impact of selection and chemotherapy.

113 patients with acute myelogenous leukemia (AML), representing 82% of the total cohort of AML patients within the geographical area of northern Sweden, were recorded. The total complete remission (CR) rate was 47.8%, and median survival was 4 months. The probability of long-term survival for all patients without exclusions was only 5%. Thus, the results from this study differ strongly from data on patient outcome in most therapy studies in AML, where the influence of patient selection on the results is larger. The median age in our patients was 63 years, which is also higher than in most other studies. Elderly patients had a low CR rate (24% in patients greater than or equal to 70 yr), but remission duration was similar in the different age groups. Patients treated according to "high-dose" protocols had a CR rate of 64%, while only 14% of less aggressively treated patients achieved remission. A better response rate after more aggressive chemotherapy was evident also in elderly patients. CR rate was 81% in patients below 60 yr of age who had no antecedent blood disorder and who had had symptoms for less than 3 months. Other variables with prognostic implications were: cytogenetic subgroup, antecedent hematological disease, and level of serum ferritin. High serum ferritin was associated with short CR duration. Ferritin is produced by the leukemic cells and could be regarded as a marker for leukemic activity.

Adolescent↗

Leukocyte interferon in the treatment of chronic myelogenous leukemia in second chronic phase.

Four patients with Philadelphia chromosome positive CML were treated with 18-42 x 10(6) IU of purified natural leukocyte IFN-alpha per week, after high-dose chemotherapy for blast phase and attainment of 2nd chronic phase. The second blast phase occurred within 3 months in 3 patients, but one patient is still in second chronic phase after 22 months of treatment. Treatment consisted of interferon only during the first year, and interferon in combination with hydroxyurea during the second year. During the second year suppression of the Philadelphia chromosome was seen in one patient, with 20% Philadelphia negative bone marrow metaphases. The toxicity of purified natural leukocyte IFN-alpha was similar to the toxicity of recombinant IFN-alpha. Antibodies to IFN-alpha were not detected in any patient.

Adult↗

Cytogenetic abnormalities and leukemic transformation in hydroxyurea-treated patients with Philadelphia chromosome negative chronic myeloproliferative disease.

Eighty-one consecutive hydroxyurea-treated patients with Philadelphia (Ph) chromosome negative chronic myeloproliferative disease were followed prospectively from 1981 to 1989; 35 of them had polycythemia vera, 32 had essential thrombocythemia, 12 had myelofibrosis, and 2 had myeloproliferative syndromes. The 81 patients were treated with hydroxyurea for a total of 3,804 months during the observation time. Only three patients had been treated with alkylating agents or 32P before start of hydroxyurea treatment. Four patients transformed into acute myeloid leukemia or myelodysplastic syndromes; three of these patients had essential thrombocythemia, and one had a myeloproliferative syndrome. Two patients died of solid cancers. Five out of 53 evaluable patients (9%) had pretreatment clonal cytogenetic abnormalities involving chromosomes 1, 9, 20, and 21. At follow-up, during or after hydroxyurea treatment, 15% had cytogenetic abnormalities, an unexpectedly low frequency compared to the previously reported frequency in patients with polycythemia vera treated with alkylating agents. None of our patients who developed cytogenetic clonal changes during hydroxyurea therapy had polycythemia vera. However, follow-up is too short to draw any conclusions about the mutagenic potential of hydroxyurea compared to alkylating agents.

Adult↗

Effect of interferon alpha-2b in advanced multiple myeloma.

The antitumour effect of recombinant human interferon (rh-IFN) alpha-2b was studied in 22 patients with advanced multiple myeloma (MM). Nine of 14 evaluable cases were refractory to cytostatic therapy; five were in relapse. rh-IFN was administered s.c. three times per week, in escalating doses starting with 2 x 10(6) IU m-2 and if possible up to 15 x 10(6) IU m-2. Two patients (one refractory, one relapsing) showed a partial response, defined as a 50% reduction of the serum M-component. Three further patients had a minor, significant but short-lived response. Subjective side-effects grade 1-2 were noted during rh-IFN therapy in all patients. In three cases thrombocytopenia necessitating platelet transfusions occurred. Although a fraction of patients with advanced MM obviously respond to rh-IFN, this type of therapy may be more effective, alone or in addition to chemotherapy, in patients with a low tumour cell burden.

Aged↗

Reversal of myelofibrosis by hydroxyurea.

Bone marrow morphology in 39 symptomatic patients with myeloproliferative disorders (polycythaemia vera 15, essential thrombocythaemia 14, idiopathic myelofibrosis 9, myeloproliferative syndrome 1) and elevated platelet counts was studied before and after a median of 18 months of continuous treatment with hydroxyurea. We found a significant reduction of bone marrow fibrosis, believed to be mediated by suppression of thrombopoiesis by hydroxyurea.

Bone Marrow↗

Amsacrine, cytarabine and etoposide in the treatment of bad prognosis acute myeloid leukemia.

Thirty-seven patients (median age 50 yr, range 17-82) with acute myeloid leukemia (AML) received intensive induction treatment with amsacrine, cytarabine and etoposide in combination. Nine of the patients were refractory to previous induction therapy, 15 relapsed during or after treatment with daunorubicin and cytarabine, 13 had AML after previous hematologic disorders. Eleven of the patients with AML after previous hematologic disorders had been treated with cytotoxic drugs. Toxicity was substantial, but complete remission (CR) was achieved in 33% of patients with refractory AML, 47% of patients with AML in relapse, 54% of patients with AML after antecedent blood disorder. CR duration was 15 weeks (median). Patients with AML of FAB types M4 and M5 entered remission more often (70%) than patients with other AML types (37%).

Adolescent↗

Philadelphia chromosome negative acute lymphoblastic leukemia preceding Philadelphia positive chronic myelogenous leukemia.

A patient with Philadelphia chromosome (Ph) negative acute lymphoblastic leukemia (ALL, FAB type L1) developed Ph-positive chronic myelogenous leukemia (CML) after more than 2 years in complete remission. Subsequently, Ph-positive lymphoblastic transformation occurred, which was again successfully treated. Thereafter, the CML state was interrupted twice more by blast crisis. The additional chromosomal abnormalities were atypical for Ph-positive CML. The course is interpreted as a possible example of the multistep development of CML. Blastic transformation occurring prior to the Ph chromosome has been reported in only two cases previously.

Adult↗