[Malignant histiocytosis. Review of the entity apropos of 2 cases with seldom seen clinical manifestations].
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Biomedical subjects
Publications and source records attributed to A Domingo.
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A partial purification scheme applied to the Triton X-100 insoluble pellet of adult flies homogenates yielded a fraction which upon polymerization reproducibly produces filaments which in the electron microscope have all the typical properties of intermediate filaments. This is the first report of the presence of protein components with such properties in Drosophila. Thus, it is highly possible that insects and arthropodes, like other lower invertebrates, may contain the third extremely insoluble element of the cellular cytoskeleton.
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Ninety-six patients with advanced chronic lymphocytic leukemia (CLL) (Stage C; anemia and/or thrombocytopenia of nonimmune origin) were randomized to receive either chlorambucil (CLR) (0.4 mg/kg orally, day 6) plus prednisone (PDN) (60 mg/m2 orally, days 1-5) every 2 weeks or cyclophosphamide (600 mg/m2 intravenously, day 6), vincristine (1 mg/m2 intravenously, day 6) and prednisone (60 mg/m2 orally, days 1-5) (COP) each month for 5 months. Complete remission (CR) was defined as the total disappearance of signs and symptoms related to the disease. Partial remission (PR) was considered to be achieved when, after treatment, the clinical stage changed to a less advanced one. Thirty (59%) responses (8% CR) with CLR plus PDN and 14 (31%, 2% CR) with COP were observed (P less than 0.01). The survival was not significantly different for the two groups. Patients previously treated had a lower number of responses (11/35, 31%) than those with no previous treatment (33/61, 54%) (P less than 0.05). Patients who attained a CR or a good PR had longer survivals (median not reached) than those with a poor PR (median, 25.2 months) or those who did not respond to treatment (median, 11.5 months) (P less than 0.005).
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The pattern of protein kinase activity in leukemic cells from patients with chronic myelocytic leukemia, acute myeloblastic leukemia, acute monocytic leukemia, chronic lymphocytic leukemia, and acute lymphoblastic leukemia was studied and compared with normal peripheral blood granulocytes and lymphocytes. Our data showed that: (a) histone kinase activity was slightly lower in leukemic cells than in normal cells, whereas casein kinase activity was 2- to 3-fold higher in leukemic cells; (b) cyclic adenosine 3':5'-monophosphate stimulated 1.4- to 1.6-fold histone kinase activity of both normal and leukemic cells, whereas it did not stimulate casein kinase activity; (c) the ratio of histone kinase activities to casein kinase activities correlated directly with the maturation of the white blood cells; and (d) histone and casein kinase activities of extracts from normal and leukemic cells behaved similarly on chromatography on phosphocellulose and casein/Sepharose 4B. These results suggest that the increase in casein kinase activity is not due to the appearance of a new type of casein kinase but to an increase of the casein kinases 1 and 2 present in normal cells.
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Between 1959 and 1973 were analyzed the records of 798 patients with chronic myelocytic leukemia. Mean survival (MS) for the entire group is 42 months. 342 patients have been followed closely during and after development of blastic transformation. Presence of following symptoms at the time of diagnosis: asthenia, weight loss, bone pain, fever, sweats and digestive disorders is of poor prognosis significance (MS: 36 months, no sign: MS 75 months) (P less than 0.001). Spleen size is also a prognostic factor. MS are respectively 70, 52 and 35 months if initial splenomegaly is moderate (less than 3 cm), marked (less than 6 cm) or tumoral (greater than or equal to 6 cm). Thrombocytopenia (less than 15,000/mm3 or thrombocythemia (greater than 1 million/mm3) have a poor prognosis with median survival 22 months and 28 months. If peripheral blast cells (hemocytoblasts + myeloblasts) exceed 5%, the prognosis is worse; beyond 10% MS is 26 months. In contrast certain factors have better prognosis: hemoglobin greater than or equal to 14 g/100 ml, young age (less than 20 y.) MS: 62 months), female sex and an initial WBC count below 25 x 10(3)/mm3 (MS: 70 months).
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Several factors such as age, sex, cytological type, visceromegaly, blast cell count in peripheral blood and recently some immunological traits, have been incriminated by some authors as having a variable prognostic value. Hypothetical influence of some of these factors on the rate and duration of the complete remission in an homogeneous group of 61 children with acute lymphoblastic leukemia have been studied. All of them were treated with same schedule (C protocol) in order to eliminate therapy influence. Some differences, without statistical significance, have been found related to: A) Sex: Mean duration of complete remission has been longer in females. B) Blast cell count in peripheral blood: Patients with initial blast-cell values under 25.000/mm3 have shown an increased number of complete remissions and a longer duration of the complete remission. No relationship has been found between initial age or visceromegaly and duration of complete remission. Neither, no relationship between cytomorphologic type and rate and duration of the complete remission could be established in our series.
A decrease in glycogen content and glycogen-synthetase activity was observed in leukemic leucocytes compared to those of normal humans, but the decrease was lesser in chronic myeloid leukemic leucocytes than in those of acute leukemic patients. Incubation of crude leucocyte homogenates at 30 degrees C before assay of the enzyme activity, revealed a conversion of the glycogen-synthetase from the D to the I form. It took place if the enzyme source were enzyme extracts from acute myeloblastic, acute lymphoblastic, chronic lymphocytic, acute menoblastic and undifferenciate leucocytes. The conversion did not occur in crude homogenates from leucocytes of chronic myeloid leukemic patients. ATP-Mg caused a conversion of the glycogen-synthetase I form to the D form in acute myeloblastic, chronic lymphocytic and undifferenciate leukemic leucocytes. This conversion was not observed in chronic myelocytic leukemic leucocytes.
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