[Metastases from unknown primary tumor].
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Biomedical subjects
Publications and source records attributed to H F Hillen.
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OBJECTIVE: To investigate the incidence and presentation of acute pernicious or fulminating beriberi in a general district hospital. METHODS: All patients with a diagnosis of acute pernicious or fulminating beriberi heart disease made between 1978 and 1994 were identified, and their medical records were retrospectively examined. RESULTS: Six cases of acute pernicious or fulminating beriberi heart disease were recognized. The disease was characterized by circulatory shock and peripheral cyanosis. All patients had severe lactic acidosis, in 5 of them without hypoxaemia. Four patients were alcoholics. The most important diagnostic criterion was the impressive improvement after thiamine administration. CONCLUSIONS: Acute pernicious or fulminating beriberi heart disease can be recognized occasionally. Thiamine should be administered as soon as possible in suspected cases.
Patients with a monoclonal gammopathy without evidence of lymphoproliferative or plasma cell malignancy within a year are still at risk for malignant transformation to multiple myeloma, Waldenström's macroglobulinaemia or non-Hodgkin's lymphoma. In a prospective study performed at the Mayo Clinic, the cumulative incidence of malignant transformation was 29% in 14 years. We conducted a retrospective study to determine the frequency of malignant transformation among 334 unselected out-patients with monoclonal gammopathy of undetermined significance (MGUS) from a community hospital in Southeastern Netherlands. The cumulative incidence of malignant transformation was 11% in 14 years (95% confidence interval 6-17%). The long-term survival of patients with MGUS was slightly lower than that of the average regional population. In a nested case-control study, presence of a kappa light chain was found to be a risk factor for malignant transformation (70% of patients who developed malignant transformation compared to 30% of the control group, P < 0.01). Likewise, an initial high gamma globulin level was also found to be a risk factor (18.7 g/l v 13.7 g/l in the control group, P < 0.01). As neither risk factor has been described before, the significance of these factors for definition of a high-risk group among patients with monoclonal gammopathy remains to be determined.
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Up to 30% of patients with idiopathic thrombocytopenic purpura (ITP) are refractory to standard therapy with corticosteroids and splenectomy. The treatment of refractory ITP is barely effective, but promising results of interferon therapy were reported recently. In a prospective study 21 refractory ITP patients were treated with interferon alpha 2B at 3 million Units three times a week for a period of 4 weeks. Ten out of 21 patients had a response, of whom 2 had a complete response of 3.5 and 7 months duration, after which both relapsed. One other patient retreated after relapse has a complete response of 24+ months. The characteristics of the responding patients were not different from the non-responding patients. Adverse effects were minimal. Short treatment with interferon in refractory ITP patients may be justified and the results reported here challenge us to elucidate the mechanism of action of interferon.
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Retrospectively the data were examined of 69 patients with polymyalgia rheumatica (PMR) and giant-cell arteritis (GCA), of whom 62 were treated with corticosteroids. The clinical and laboratory data, and the outcome of temporal artery biopsy were compared. In addition the relation between the course of the disease and survival was investigated. PMR and GCA are closely related syndromes with a multiform clinical presentation. There even is a group of patients with merely systemic symptoms, without specific signs of PMR or GCA. Concerning the course of the disease patients can be divided into groups with a shorter and longer period of disease activity; patients with a smooth stable remission and a chronic disease. The former group shows a significantly better five-year survival. At the time of diagnosis no reliable prediction of the course of the disease is possible.
This study investigated whether high-dose dexamethasone pulse therapy could bring about a remission in adult chronic idiopathic thrombocytopenic purpura (ITP) patients who had not achieved a remission on the usual prednisone therapy. Newly diagnosed patients with ITP received the usual prednisone therapy, 1 mg/kg/day, for 3 weeks, after which period the dosage was tapered off. If after 6 weeks of treatment the platelets were less than 50 x 10(9)/l the prednisone therapy was considered a failure and 200 mg dexamethasone i.v. was given on 3 consecutive days. Six of the twenty-five patients with newly diagnosed ITP treated according to this protocol achieved a sufficient response with prednisone therapy. Nineteen patients were resistant to or relapsed during prednisone therapy and were treated with high-dose dexamethasone. Four of these nineteen patients showed a sufficient response. Three are still in remission. As no serious adverse effects were observed, high-dose dexamethasone therapy can be considered before splenectomy in prednisone- resistant ITP patients.
A new system is described for cooling the scalp with cold air in order to prevent chemotherapy-induced alopecia. Compressed air was cooled by means of a vortex tube built into a hair-drier cap. This system reduced the blood flow in the scalp to 35%, the surface temperature to 14.2 degrees C and the intradermal temperature at hair follicle level to 29.2 degrees C. The low temperature could be kept constant for at least one hour of cooling. By means of comparison, with cryogel packs the lowest epidermal temperature attained was 17.9 degrees C; moreover, once this was reached after 10 min, it rapidly rose again to 20.6 degrees C after 40 min. Forty-eight patients receiving cytostatic treatment for breast cancer were subjected to scalp cooling with the cold air system, starting 15 min before chemotherapy and lasting for 90 min. With the system set at an air temperature of -12 degrees C, the treatment was well tolerated. Of the 13 patients treated with 40 mg/m2 doxorubicin in combination with other cytostatics, 6 had hair loss less than WHO grade 3, in contrast to 1 of 4 patients given cryogel packs. However, patients treated with epirubicin at 75 mg/m2 all showed grade 3 hair loss in spite of air cooling. In view of the possibility of achieving and maintaining low scalp temperatures, the cold air system is to be preferred to cryogel packs. Whether better clinical results may be obtained with cooling for longer periods and/or to lower temperatures remains to be determined.
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Twenty patients (15 male, 5 female) with nonresectable gastric adenocarcinoma were treated with FAP (5-fluorouracil 300 mg/m2 IV on days 1-5, adriamycin 50 mg/m2 IV on day 1, cisplatin 20 mg/m2 IV on days 1-5). Each course was repeated every 21 days. Eighteen patients were evaluable for response. The median age was 51 years, the range extending from 34 to 68. None had undergone chemotherapy. The median Karnofsky performance score was 80%. Nine (50%) partial responses (PR) and eight (44%) cases of stable disease (SD) were observed. One patient showed progression of the disease and died after 6 months. The median duration of response was 6+ months for PR and 6 months for SD. The median survival was 12 months. FAP toxicity was moderate, with the median WBC nadir 3.2 X 10(9)/l (range 0.7-4.2). One patient in PR died of septicemia. Nausea and vomiting were not dose-limiting. Neuropathy was mild in four and moderate in two patients. This FAP combination appears to be as effective with respect to response rate and duration as reported for 5-fluorouracil, adriamycin and mitomycin C (FAM).
Retrospectively we observed an early-onset transient decrease in platelet counts in a group of patients treated with cisplatin, vinblastine and bleomycin. Though less pronounced, the decrease was also found in a prospectively studied group of patients treated with bleomycin, etoposide and cisplatin, but was absent after treatment with etoposide ad cisplatin. In a randomized cross-over study acetylsalicylic acid (ASA) appeared not to prevent the drop in platelet counts. Bleomycin appears to induce an early-onset transient decrease in platelet numbers, possibly by direct or indirect platelet lysis. Vinblastine may have a synergistic effect to this phenomenon.
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