[Norwegian scabies in a patient with chronic adult T-cell leukemia].
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Biomedical subjects
Publications and source records attributed to Y Yawata.
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Since 1980 five human myeloma cell lines, KMM-1, KMS-5, KMS-11, KMS-12-PE and KMS-12-BM, have been established in Kawasaki Medical School. Histologically, all the cell lines resembled plasma cells and were EBNA negative. KMM-1, KMS-11, KMS-12-PE and KMS-12-BM reacted with PCA-1, while KMM-1, KMS-12-PE and KMS-12-BM with CD38. KMM-1 and KMS-11 secreted immunoglobulins into culture medium. Karyologically, all the cell lines were abnormal. Only KMS-5 was tumorigenic when transplanted subcutaneously into nude mice.
Clinical and experimental studies on hereditary spherocytosis (HS) and high red cell membrane phosphatidylcholine hemolytic anemia (HPCHA) were performed in relation to lipid metabolism in plasma and in red cells of these patients. In HS, red cell (RBC) membrane lipids (free cholesterol (FC) and phospholipids (PL) such as phosphatidylethanolamine, phosphatidylcholine (PC), sphingomyelin (SM) and lysophosphatidylcholine (LPC] were markedly decreased in unsplenectomized HS. Plasma lipids (total cholesterol, FC, HDL-cholesterol, PL) were also decreased in these patients. After splenectomy, substantial normalization of plasma and RBC lipids were observed. Concerning lipid kinetics, the extents of 14C-PC synthesis in RBC from 14C-LPC in medium and of 14C-PC exchange between RBC and the medium were almost identical to those in normal control in the in vitro incubation conditions. These observations indicate that the decreased RBC lipids may be induced by the shortage of lipid materials in plasma in the presence of the spleen. In unsplenectomized HPCHA, plasma lipids were also decreased same as in HS. In contrast, membrane lipids (FC and PC) were markedly increased. Even after splenectomy, increased PC contents were rather enhanced in their membranes concomitant to developing of hemolytic anemia, although plasma lipids were almost normalized. Thus RBC membrane lipids in HPCHA appeared not to be affected by plasma lipids. In experimental studies on HPCHA, 14C-PC synthesis from 14C-LPC and 14C-PC uptake in these RBC were increased, in spite that large amounts of PC were already accumulated in these RBC.(ABSTRACT TRUNCATED AT 250 WORDS)
A Japanese family of congenital high red cell membrane phosphatidylcholine hemolytic anemia (HPCHA) is reported. The propositus was a 48-year-old woman, who had been followed up as hemolytic anemia of unknown origin and undergone splenectomy. She showed no improvement after splenectomy. She also had primary biliary cirrhosis, of which a diagnosis was made based on laboratory data and liver biopsy. Red cell morphology demonstrated stomatocytosis with erythroid hyperplasia in the bone marrow. Abnormal hemoglobins and the red cell enzyme activities were not demonstrated. A marked abnormality was noted in red cell membrane lipids, specifically the elevation of phosphatidylcholine (PC) and free cholesterol (FC), despite of normal plasma lipids and lecithin cholesterol acyltransferase activity. Sodium transport, both influx and efflux, was increased. Therefore, the diagnosis of HPCHA was confirmed. The three additional cases were found in her pedigree. The data on the red cells of her mother and elder sister were similar to hers with respect to the red cell lipids and sodium transport. The propositus and her mother showed no improvement of anemia or icterus after splenectomy. There are only three reports of the family with HPCHA in the world.
We analyzed hemolytic reaction following vinca alkaloid administration in patients with acute lymphoblastic leukemia, blast crisis of chronic myelogenous leukemia and malignant lymphoma in the leukemic phase. During the course of chemotherapy in which vinca alkaloids were included, indirect-form dominant hyperbilirubinemia was observed in 21 of the 31 evaluable patients. In 8 patients with hyperbilirubinemia, reticulocytosis coincided with vinca alkaloid administration. Peripheral blood films showed prominent anisocytosis in 4 patients and increased stomatocytes in 10 patients. Red cell membrane studies in patients with hemolysis revealed increased sodium transport and increased activity of Na+, K+-ATPase. These findings were considered to represent the abnormal membrane "leakiness". Thus, vinca alkaloids might induce erythrocytotoxicity, which leads to acquired stomatocytosis with hemolysis in some patients.
We have experienced a case of cytohemolytic hereditary elliptocytosis (HE) in a six-year-old boy. Metabolisms of the erythrocytic membrane were investigated on the members from his pedigree. The results were as follows; 1) The presence or absence of ovalocytic HE were studied in his pedigree. 2) Failure in the process of spectrin dimer to tetramer conversion was found. 3) Although abnormality existed in conversion of D to T by the patient's alpha-chain spectrin and normal beta-chain spectrin, no abnormality was recognized when normal alpha-chain and the patient's beta-chain were combined. 4) Decrease of the alpha-chain (80 kd) domain and appearance of abnormal (74 kd) spot were found by two dimensional peptide mapping of spectrin. 5) In his pedigree, neither patients with hereditary pyropoikilocytosis (HPP) nor carrier states were recognized. In summary, this patient's pedigree was considered to be HE [SP alpha 1/74]. This case appears to be the first case in Japan and only few cases have been reported in the world literature.
The clinical features of hereditary elliptocytosis were studied in 25 cases and compared with 20 normal individuals. Based on morphological features, these patients were classified into two groups: those with a rod-shaped type of elliptocytosis (nine cases), and those with a non-rod type (16 cases). Most of the cases with overt haemolysis were detected among cases of the non-rod type. Overt haemolysis typically tended to be accompanied by stomatocytic changes, which appear to be superimposed on elliptic transformation of the red cells. Sodium influx increased in eight of nine HE patients with overt haemolysis, but in none of the HE with rod-shaped red cells. Significant quantitative and qualitative differences in red cell membrane lipids were observed between the two types of HE. No spectrin abnormalities in domain composition, dimer-dimer association, and thermal stability were observed in 11 of the HE patients studied, including four cases with overt haemolysis.
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Chromosome analysis was performed on 1 patient with diffuse lymphoma of mixed type by histologic diagnosis and on 7 patients with the acute type of adult T-cell leukemia (ATL). Specific abnormalities in chromosome 14 at break band q11 with the assigned locus of the alpha-chain gene of the T-cell antigen receptor were identified in 6 of 8 patients. Inv(14) (q11q32) was found in 2 patients and translocation of chromosome 14 at break band q11 was observed in 4. Donor chromosomes involved in translocation of the 14q11 varied, i.e., chromosomes 3, 7 or X, with the exception of one patient whose donor chromosome origin could not be determined. The breakpoint in chromosome 3 was in band p25, a region reported to include the locus of the c-raf-I oncogene. In chromosome 7, it was in band p11, a region reported to include the locus of the c-erb-B oncogene, and in the sex chromosome X, it was in band q11. One patient also had a chromosome 14 aberration at break band q32. Of the 2 remaining patients, one had lost chromosome 14 and the other had an isochromosome 14q. Our observation and other reported findings suggest that the rearrangement of chromosome 14 at break band q11 is specific for lymphoma-type or acute-type ATL patients, and aberrations of proto-oncogene expression or the coding sequence by recombination involving a T-cell antigen receptor gene due to chromosome inversion or chromosome translocation may play an important role in T-cell neoplasia including ATL.
Membrane transport--sodium (Na+) influx and calcium (Ca2+) uptake--was examined in human mature red cells treated with phospholipase A2 (PLase A2) from snake venom. PLase A2-induced conversion of phosphatidyl choline (PC) to lysophosphatidyl choline (L-PC) was associated with a marked increase in Na+ influx and Ca2+ uptake. After L-PC was removed from the cell membrane of the PLase A2-treated red cells in the presence of albumin, an additional increase in Ca2+ transport was observed. These results indicate that membrane lipid abnormalities, such as increased L-PC and/or a loss of total lipids, appear to induce increased membrane transport.
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