Biomedical subjects
Publications and source records attributed to R Heinz.
In the period between 1978 to 1987 16 female and 11 male patients with a median age of 35 years (range: 14 years to 85 years) underwent splenectomy for ITP. There was no lethality in the postoperative period. 77.8% of all patients are in a complete remission, only 22.2% showed a recurrence and need further conservative therapy.
In a prospective multicenter therapy trial the remission-inducing potential of the COP-BLAM (5 courses) and the possibly not cross-resistant IMVP-16 (2 courses) regimen for advanced diffuse large cell lymphomas were investigated. Inadequately responding patients were switched early after 2-3 courses to the IMVP-16 protocol. Between January 1986 and August 1988 349 previously untreated patients were recruited who fulfilled the entry criteria: age 15-75 (mean 56 years), stage II-IV. A first restaging after 3 courses of chemotherapy was documented for 280 cases, a second restaging following 7 courses of chemotherapy or a final report was available for 221 patients, 130 of which where in CR (59%). These figures include 29 cases who died already during induction-chemotherapy of progressive disease (n = 17) as well as of therapy-related complications in CR (n = 12). Thirty-three patients were switched early to IMVP-16 due to incomplete response at the first restaging; 13 of those achieved CR by the new regimen (39%), 9 achieved a partial remission which was turned into CR by additional radiotherapy in 5 cases. Dose reductions and interval prolongations were necessary at increasing rates with subsequent chemotherapy courses (20-50% of the cycles). Relapses have occurred in 32 patients so far (14 early, 18 after stop of treatment). The median survival was 24 months (n = 309 patients). These data show that this treatment strategy is effective in a large unselected group of patients of relatively high age. However, patients showing only delayed response with this protocol probably need more aggressive regimens to obtain durable remissions.
Necrobiotic xanthogranuloma is a rare generalized dermatosis occurring exclusively in association with paraproteinemia. Clinically it is characterized by large, ulcerating, nodular and plaque-like tumors of the dermis and subcutis. Histologically the lesions are composed of a nonspecific, lymphocytic and plasmacellular infiltrate and highly characteristic granulomas with giant cells of the Touton and foreign body type. Ultrastructurally, tubular cystic organelles with a central lamella and a few ribosomes at their outer membranes are found. By means of histochemical studies we excluded the skin infiltrates as the site of the paraprotein release. The pathogenesis of the disease remains unclear; it is suggested that the paraprotein has functional features of a lipoprotein, which may bind to lipoprotein receptors of the histiocytes, thereby inducing xanthoma formation.
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In a multicenter prospective randomized therapeutic trial in advanced (stage II-IV disease, Ann Arbor classification) high-grade malignant non-Hodgkin's lymphomas (NHL, Kiel classification) a sequential combination of the COP-BLAM (5 cycles) and the IMVP-16 (2 cycles) protocols was employed. Response was first determined after 2-3 cycles. In a response-adapted manner the therapy was immediately switched to IMVP-16 if only a partial remission or no response was obtained as evidenced by the first restaging. The aim of the study is the investigation of the efficiency of this concept to induce stable remissions. In an additional randomized trial, involving all patients reaching complete remissions after chemotherapy (second restaging), the prognostic relevance of adjuvant radiotherapy as compared to therapy-free follow-up is evaluated. Eighty percent of the 191 recruited qualified patients have so far become evaluable. Complete clinical remissions were achieved in 76/148 (51%) of the patients up to the first, in 52/85 (61%) of the patients up to the second restaging. Only in a few cases did the expected toxicity of intensive polychemotherapy reach WHO grade 3-4, including nausea and diarrhea, infections, septic complications, myelotoxicity, and stomatitis. Four of the 29 deaths recorded so far occurred in complete remission due to treatment-related complications, whereas 22/29 (76%) died in progression and 3 of unrelated causes.
Recent progress in polychemotherapy employed in the treatment of gastrointestinal lymphomas has markedly improved prognosis. Between 1980 and 1986, 22 patients with malignant lymphomas of the gastrointestinal tract were treated at the Department of Surgery at the Hanusch Medical Center, in cooperation with the Department of Hematology and Oncology. 19 patients underwent surgery. The average observation time was 29 months. The rate of survival after 36 months was 64% in the entire patient population. Palliative-surgical interventions combined with chemotherapy equally produced satisfactory results.
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A 12-year-old boy had a hyperdense area corresponding to a gyral pattern on an enhanced CT brain scan within 12 hours of his last seizure. The hyperdense area disappeared on a subsequent enhanced CT scan after he was seizure free for about 48 hours. The hyperdense area was in a location (mesial frontal lobe) predicted by the interictal physical exam findings and the seizure type recorded on video-EEG monitoring. We postulate that the CT abnormality was due to transitory increase of regional cerebral blood flow and vascular permeability.
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46 patients suffering from various malignancies (17 non Hodgkin lymphomas, 12 Hodgkin's diseases, 11 acute leukaemias, 4 myelomas, 2 carcinomas), 6 patients with haematological disorders such as ITP, SAA, myeloproliferative disease, LAS and 3 patients without preexisting disease were treated with acyclovir for herpes virus infection diagnosed by clinical means. All but 7 patients had been given intensive treatment with various cytostatic agents and/or irradiation. Most patients were treated with 1500 mg acyclovir daily for 5 to 13 days. Dosage was adjusted according to renal function and clinical response in the remaining 10 cases. 11 patients received intravenous immunoglobulins in addition. Side effects were negligible (local irritation, minimal rise in serum creatinine levels in 5 patients). All patients responded to treatment; 6 patients complained of severe neuralgia lasting for more than one month; 5 patients relapsed.
The Kiel classification provides a new subdivision of non-Hodgkin lymphomas into distinct entities showing different clinical and prognostic properties. In comparison with earlier classifications this system defines additional types of lymphoma (e.g. CC lymphoma, LP immunocytoma) (for abbreviations see text) which are to be considered separate entities also from a clinical point of view. By data derived from a multicenter prospective observation study (1,127 patients recruited from 1975 to 1980, follow-up until 1985) a precise definition of the clinical features of each lymphoma entity (e.g. frequency, age and sex distribution, patterns of initial involvement and spread of disease) was possible. In addition, the effect of radio- and/or chemotherapeutic measures was evaluated. Strictly localized disease (stage I/IE according to the Ann Arbor classification) occurred in 1.5 to 8% of patients with NHL of low-grade malignancy (comprising 69.4% of cases studied) and in 8 to 17% of patients with high-grade malignant NHL (comprising 30.2% of cases studied). Loco-regional irradiation alone was able to induce complete remission in 86 to 89% (CB and IB lymphomas) and in 100% (LP immunocytoma, CB-CC and CC lymphomas), respectively, of stage I/IE patients. Only CC and IB lymphomas showed a relevant risk of relapse (40% and 50%, respectively). Total lymphoid irradiation as able to induce stable complete remissions in about 50% of patients with stage III of CB-CC lymphoma. Probabilities of survival of patients with initial stages III and IV treated by several types of chemotherapy reflect different prognostic features of individual lymphoma entities.(ABSTRACT TRUNCATED AT 250 WORDS)
After spinal cord injury cystic lesions of different types are known to develop. However, in a large group of patients radiologic examinations have not revealed abnormalities in spite of neurologic deficit symptoms. Magnetic resonance imaging in 6 such patients demonstrated subtle changes in the cervical spinal cord, confirming the diagnosis of myelomalacia.
Cryoprecipitate has been demonstrated to correct the bleeding abnormality of patients with some congenital (storage pool disease) and acquired (uremia) platelet abnormalities, but the reason for this effect is unknown. We found significant platelet contamination in plasma harvested to prepare fresh frozen plasma and cryoprecipitate. The platelet membrane microparticles produced by freezing and thawing of the plasma were highly concentrated in cryoprecipitate and may contribute to its therapeutic effect.
An analysis of 173 cases of non-Hodgkin's lymphoma (NHL) admitted to our hospital from January 1973 to January 1983 is presented. Of the 173 cases, 124 patients suffered from NHL of high grade malignancy according to the Kiel classification (37 centroblastic lymphoma (CB), 30 immunoblastic lymphoma (IB), 43 lymphoblastic lymphoma (LB), 14 NHL high grade malignancy unclassifiable). In addition, 26 patients with secondary high grade malignant NHL were included in the analysis (14 secondary CB, 10 secondary IB, 2 secondary LB). Also investigated were 23 patients with anaplastic centrocytic lymphoma (CC) (20 primary CC, 3 secondary CC), an entity originally classified as low grade malignant lymphoma, but showing a poor outcome and need for aggressive therapy. Symptoms at presentation of all patients are described. Of the 173 patients, 71% had an advanced stage of the disease at the time of diagnosis (Ann Arbor stage III or IV). B-symptoms were observed in 81%. Extranodal involvement, (exceptive bone marrow involvement), determined by clinical examination was seen in 55%. Survival of patients changed significantly after replacing initial radiotherapy with aggressive chemotherapy (P less than 0.001). Improvement of survival statistics was due to the better outcome of patients with localized stages (Ann Arbor stages I and II) as compared to those with generalized disease (P less than 0.002). Prognostic factors influencing survival were elevation of lactic dehydrogenase (P less than 0.0001) and response to therapy (P less than 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)
Case histories are presented of 2 patients in whom the occurrence of several signs indicated the presence of an immunocytoma. A 56 year-old man presented with marked splenomegaly and a lymphocytosis in peripheral blood picture. Lymphatic infiltrates were demonstrated in the bone marrow and liver. During the course of the disease the initial monoclonal gammopathy and the lymphocytosis disappeared. An extensive carcinoma of the stomach became clinically manifest one year later. In the second patient, a 74 year-old man, lymph node swelling, monoclonal gammopathy and bone-marrow cytology indicated lymphatic disease. There was no indication of carcinoma in spite of extensive investigations. The patients died three years later of a hypernephroma. Both patients demonstrated no sign of lymphatic disease at autopsy and, hence, a lymphatic reaction must be diagnosed retrospectively.
15 patients suffering from idiopathic thrombocytopenic purpura were treated in our department with high-dosage immunoglobulins. The daily dosage amounted to between 0.13 and 0.4 g/kg body weight, administered for 4 to 15 days consecutively. The platelet count in 10 patients increased within the first week of treatment, but this increase was maintained for more than 4 weeks in only 3 patients. The average age of these 10 patients amounted to 41.2 years and was significantly lower than that of the remaining 5 patients (66.6 years), who failed to respond. Only one of 4 splenectomized patients responded with an increase in platelet count. Two different immunoglobulin preparations were used. No difference in efficacy was found and both preparations were well tolerated.
58 NHL-patients (9 large cell centrocytic, 18 centroblastic, 16 immunoblastic, 15 lymphoblastic lymphomas) were treated immediately after diagnosis with CHOP-chemotherapy regardless of the extent of disease. Because of the advanced age of the majority of patients (median age 61 years, range 22-85 years) a reduced dose in the first two cycles was administered. Statistically significant prognostic variables influencing survival were the following: histologic subtypes according to the Kiel-classification (p less than 0,05), B-symptoms (p less than 0,001), blood sedimentation rate (p less than 0,02) and LDH (p less than 0,0005). With regard to prognosis there was no difference between patients over 60 years of age and younger ones (p less than 0,4). Patients achieving complete remission survived significantly longer (p less than 0,0001). Ann Arbor stages were of limited value, since patients with CS II disease and accumulation of risk factors (B-symptoms, abdominal disease, bulky tumor masses) showed a poorer outcome than patients with CS III who did not have these risk factors. A risk factor score summarizing features influencing prognosis is described and might be a useful tool in stratifying the heterogeneous group of NHL with unfavorable prognosis.