[Our experiences in treating patients with Hodgkin disease in the last decade]. / Hodgkin-lymphomás betegeink kezelése során szerzett tapasztalatok az utóbbi évtizedben.

INTRODUCTION: Recently, in the diagnostics and treatment of Hodgkin's disease significant developments have occurred. AIM: To summarize the clinical and histological data of patients with Hodgkin's disease, treated at the 3rd Department of Internal Medicine, University of Debrecen between 1995-2004. In 2006 January, the mean follow-up was 69 (12-132) months. METHODS: Patients data was analyzed by using SPSS statistical software. RESULTS: The mean age of the 163 patients at the diagnosis was 36 years (14-75), with bimodal age distribution, the most frequent disease subtype was mixed-cell Hodgkin's disease (48.5%). 41.1% of the patients was at early stage, 15.7% had the worst prognosis, while 28.8% had bulky tumor. 7 patients had radiotherapy, 63 had chemotherapy, while at 92 patients combined modality treatment was used. 61.6% of radiotherapies were involved field, 61 patients received cyclophosphamide, vincristine, procarbazine, prednisolone, adriamycine, bleomycin, vinblastine, 87 adriamycine, bleomycin, vinblastine, 7 had other chemotherapies. As the response to the primary treatment 146 complete, 10 partial remission occurred, while 6 patients showed no response. 10 patients with partial remission and 5 non-responders were continually treated. 27 patients with complete remission had relapse, while 15 had high dose treatment with autologous peripheral stem cell transplantation. During the follow-up 18 patients died, 11 due to the lymphoma progression, or as the result of treatment, 6 had secondary malignancies, 1 due to other reasons. The 10-year prognosed overall survival was 83% (in details: early, advanced, favourable vs. unfavourable: 100% vs. 87.8%, 88.9% vs. 41.6%), the event free survival was 70% (82.6% vs. 70.8%, 64.5% vs. 0%). CONCLUSION: The treatment results of our Hodgkin's disease patients improved, additionally we showed that patients with early stage favourable disease the treatment toxicity should be reduced, while patients with advanced, unfavourable prognosis (10% of all patients) aggressive primary treatment should be used even with more severe side effects and complications.

Mediastinal bulky tumour in Hodgkin's disease and prognostic value of positron emission tomography in the evaluation of post-treatment residual masses.

Among the 193 patients (82 female, 111 male) treated primarily for Hodgkin's disease at our clinic between 1990 and 2001 and followed up until 2003, 42 (22%) had mediastinal bulky tumours (MBTs) by the Cotswolds criteria. The rate of MBT diagnosis was significantly greater in the early stage of the disease, these patients were younger and--in contrast to the other group--they all received combined therapy. No significant differences were found in the overall and relapse-free survival rate in the two groups, but relapse and death rates were lower in the patients with bulky tumours. Of the total number of patients, 27 underwent a total of 31 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) examinations, mainly for the evaluation of post-treatment residual mass viability. In the 12 positive cases, the majority of patients received further therapy. During the mean follow-up time of 58 months (range 5-98 months) after obtaining negative results, progression of the disease was found in 2 cases 14 and 23 months later, respectively. Based on our results, we conclude that FDG-PET examinations show a good correlation with clinical follow-up results.

Successful treatment of B cell chronic lymphocytic leukemia-associated severe paraneoplastic pemphigus with cyclosporin A.

Since the first description of paraneoplastic pemphigus, several cases have been described in the literature. However, curative therapy is usually a challenge to the physicians treating this disease. Several publications are available discussing the efficacy of steroids, cyclophosphamide and cyclosporin A. Recently, a report of the successful use of rituximab was also published. However, the use of cyclosporin A is controversial in the case of B cell malignancies, as there are reports showing the cytotoxic effect of this drug on B cells. However, other authors report no effect, or even unwanted effects resulting in B cell proliferation. We report the case of a 50-year-old Caucasian male. He developed a B cell lymphoma consisting of CD5/CD20-double-positive cells, and 2 months later, it was followed by a very severe paraneoplastic pemphigus affecting the mucosa and the skin. The lymphoma was well managed with CHOP and CVP polychemotherapy, followed by oral chlorambucil; however, the bullous eruptions did not disappear. Oral steroids, cyclophosphamide, plasmapheresis and IVIG therapy were only partially successful, so we decided to use oral cyclosporin A. Starting with 7 mg/kg and maintaining a steady plasma level of no less then 110 ng/l, the bullae completely disappeared within 6 weeks, and the patient has been in remission for 17 months now, taking the oral cyclosporin A continuously. The underlying B cell disorder did not relapse during the therapy.

[Incidence of valvulopathy in Hodgkin-disease patients in long-term complete remission]. / Valvulopathia elôfordulása tartósan komplett remisszióban lévô Hodgkin-kóros betegeknél.

AIM: To study the occurrence of valvulopathies after treatment in Hodgkin's disease patients. PATIENTS AND METHODS: 124 Hodgkin's disease patients in complete remission for at least 1 year were echocardiographically examined. RESULTS: Abnormal finding was observed in 48/124 (38.4%) of patients, all of them presented with regurgitation, no stenosis was observed. Regurgitation of grade I or II was recorded in most cases. We have found single valvulopathy in 25/48 (52.1%) of patients, and multiple valvulopathy in 23/48 (47.9%) of patients. In most cases (78.7%) the valvulopathy was detected in left heart. Among these 48 patients the ratio of females was significantly higher than those of males, and also the ratio of the patients in early phase compared with those in late phase. We could detect vitium mostly in those patients who had mediastinal irradiation. The combined treatment, including anthracycline therapy, did not increase the frequency of vitium. CONCLUSION: The occurrence of valvulopathy is frequent in Hodgkin's disease patients, particularly in patients treated by radiation. This is the reason why Hodgkin's disease patients should be examined regularly with echocardiography. These patients with valvulopathy need treatment adjusted to their state of health, and where possible, the complications should be prevented. In the future, when planning of the radiotherapy mediastinal irradiation of the Hodgkin's disease patients their heart protection from radiation should be taken into account.