Biomedical subjects
Publications and source records attributed to I Krc.
The authors review some historical data connected with the discovery of the enzyme l-asparaginase and then summarize the basic informations concerning the mechanism of action, clinical application, toxicity and future prospectives of this substance. From the clinical viewpoint, a broader use of l-asparaginase prepared from Erwinia carotovora instead of Escherichia coli seems to be warranted.
In a group of 116 patients with different types of malignant lymphoma, the frequency, type and clinical manifestations of heart involvement are evaluated. A case of primary cardiac lymphoma is demonstrated. Along with this, clinical and pathological findings in 15 further subjects with secondary lymphomatous infiltration of the heart are discussed; attention is being paid also to indirect factors causing cardiovascular damage, including radio- and chemotherapy.
Features of 10 patients with hypertrophic osteoarthropathy are described. Seven patients suffered from lung disease. Joint symptoms preceded the symptoms of primary disease in four cases. Symmetrical periosteal new bone formation was present in all patients. The theories of hypertrophic osteoarthropathy pathogenesis are discussed.
The authors refer about an unusual localization of haemorrhage to the adrenal glands, observed in two patients with acute leukaemia similar to the picture of Waterhouse-Friderichsen's syndrome. This complication causing serious and dramatic effects should be taken into differential diagnostic consideration. Literature data and own results are given in order to review the general characteristics of bleeding in acute leukaemia.
Bone marrow necrosis (BMN) is a rare intravitally recognized finding in acute leukaemia with an uncertain clinical significance. The clinical events in 4 patients with AML, ALL, AMoL and blastic transformation of CGL in whom bone marrow cytology and histology revealed BMN are reviewed. One patient with BMN at clinical presentation of AML entered complete, long lasting remission with marrow restoration after the standard DAT therapy. In the three remaining patients survival after BMN diagnosis was 6, 11, and 14 weeks. Clinical, haematological, histological and marrow scanning findings and their significance for early diagnosis and means to asses the extent and evaluation of BMN will be discussed. In contrast to the most earlier reports, BMN does not appear to confer a poor prognosis in all patients with blastic leukaemia.
In a series of 164 patients, a retrospective study has been carried out on the incidence, frequency and character of bleeding episodes occurring in chronic lymphocytic leukaemia. The authors stress the relatively high frequency of haemorrhagic complications which were causing death in a significant proportion of cases (5.5%). Thrombocytopenia was the main precipitating factor of bleeding on most occasions.
Balloon cell melanoma of the skin was found in the left axillary region of a 60-year-old man. The tumour was very large, grew local, not forming metastases. The histological picture corresponded to a solid alveolar pattern, in some places with pseudoglandular structures. The cytoplasm of the balloon cells proved to contain lipid vacuoles and glycogen. The Masson-Fontana reaction was negative: the Warthin-Starry method gave positive results in sporadic tumour cells. The DOPA oxidase reaction was negative. Histochemistry demonstrated remarkable presence of histiocytic elements among the neoplastic cells. Reaction for the proof of alpha mannosidase was positive both in the tumour cells and in the histiocytic elements. Reaction with the NKI-C3 antibody was found to be positive in many tumour cells, the anti S-100 protein antibody less positive. Most of the cells reacted strongly positively with anticytokeratin antibody CAM 5.2. Findings of anticytokeratin antibody positive melanomas have not been published yet. Electron microscopy showed rare melanosomes in some of the neoplastic cells, making it possible to include the tumour in melanomas.
Electron microscopy of an oligomelanotic balloon cell melanoma confirmed the considerable presence of lipid vacuoles in the cytoplasm of the tumours cells. There was also a marked finding of glycogen. The tumour consisted of little cohesive cells only with sporadic specialized intercellular junctions of the desmosome or hemidesmosome type. No tonofibrils could be seen. The cell cytoplasm contained a large amount of cell organelles, while stage II melanosomes were observed only exceptionally. Some of the melanosomes of stage III were difficult to distinguish from the regressively altered cell organelles, particularly from mitochondria and lysosomeś. The prevailing findings of stage II melanosomes rather indicated organelles sui generis than phagocyted organelles. There were infrequent neurosecretory granules in some places of the cytoplasm. Quite numerous Langerhans' cells with Bierbeck's granules were demonstrated among the tumour cells.
Angioimmunoblastic lymphadenopathy (AILD) is a relatively rare serious disorder of the lymphatic system with various organ manifestations and a significant immunological background. It probably represents an abnormal reaction to different chemical and biological antigens. The etiopathogenesis of the disease is still poorly understood. The authors report on findings in a series of five patients with AILD. Stress is laid on polymorphous symptomatology, unpredictable course and on resulting diagnostic as well as therapeutic difficulties in dealing with the disease.
The association between mediastinal teratoma and acute megakaryoblastic leukemia (AMKL) in a 15-year-old boy is described. The clinical course is compared with 20 previously reported cases of AMKL in children. Chromosome studies at diagnosis of the leukemia showed multiple leukemic stem lines with numerical and structural abnormalities.
Angioimmunoblastic lymphadenopathy is usually characterized as a lymphoproliferative disease caused by an abnormal reaction to various chemical and biological antigens and the possible development into malignant lymphomas of different types. The authors evaluate clinical and laboratory findings in a group of their patients suffering from the above disease. They emphasize the variable symptomatology, the frequently atypical course and the ensuing diagnostic difficulties.
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Richter's syndrome represents an acute transformation of chronic lymphocytic leukaemia or other type of low-grade malignant non-Hodgkin lymphoma to highly malignant anaplastic large-cell lymphoma. The prognosis of this complication is highly unfavourable. The authors report on eight cases of Richter's syndrome observed in a series of 198 patients with the clinical diagnosis of chronic lymphocytic leukaemia and evaluate some clinicopathological and immunological peculiarities of this disease.
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