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Biomedical subjects

K H Lin

Publications and source records attributed to K H Lin.

At least 127 records · Page 7Linked to original sources
PubMed1993

Comparison of clinical and biologic features between myeloid and lymphoid transformation of Philadelphia chromosome positive chronic myeloid leukemia.

Analysis of clinical and biologic features of chronic myeloid leukemia (CML) in blast crisis (BC) was performed on 36 patients: 25 had myeloid and 11 had lymphoid transformation. The median duration from diagnosis to onset of BC was significantly shorter in patients with lymphoid BC (6 months) than in those with myeloid BC (41 months). Patients in lymphoid transformation showed better response to therapy and had a significantly longer median survival time after BC than patients with myeloid transformation (56% vs 0% and 10 months vs 4 months, respectively). The leukemic cells from all the patients with lymphoid BC showed B-cell immunophenotype, confirmed by the presence of immunoglobulin (Ig) heavy chain gene rearrangements in the five patients studied. Two of the eight patients with complete marker study expressed myeloid-associated antigens on the blasts. A high incidence of CD7 expression (7/17 or 41%) was found in patients with myeloid BC, but none of the patients who had DNA analysis showed rearrangement of T-cell receptor beta chain gene. Chromosomal abnormalities +8, +19, +21, and i(17q) were detected only in the patients with myeloid BC but not in those with lymphoid BC. Combined analysis of the patients in this series and those reported previously has revealed a statistically significant difference in the distribution of bcr breakpoints between myeloid and lymphoid BC: the bcr breakpoints in more than half of the patients with myeloid crisis were mapped to Zone 2 while those in patients with lymphoid crisis occurred most frequently in Zone 3.

Adolescent↗
PubMed1993

Molecular epidemiology of a variant of coxsackievirus A24 in Taiwan: two epidemics caused by phylogenetically distinct viruses from 1985 to 1989.

In order to know the phylogenetic relationship and the route of transmission of a variant of coxsackievirus A24 (CA24v), an agent that caused four sequential outbreaks of acute hemorrhagic conjunctivitis from 1985 to 1989 in Taiwan, the nucleotide sequence variations in the virus-encoded proteinase 3C region (549 nucleotides) were studied with 19 isolates. The prototype strain (EH24/70), four isolates from Japan, and two isolates from Hong Kong were used for comparison. The nucleotide sequences of the Taiwan strains from the 1985-1986 and 1988-1989 epidemics were closely related within each epidemic, while they were more distantly related between strains from two epidemics. Phylogenetic analysis by the unweighted pairwise grouping method of the arithmetic average revealed that the 19 Taiwan isolates had diverged into two groups, 1985-1986 and 1988-1989 groups. The time at which these two groups diverged was estimated to be around May 1982, more than 3 years prior to the first appearance of the CA24v epidemic in Taiwan. On each occasion, the viruses caused a 2-year epidemic and then disappeared. The Taiwan isolates from 1985 to 1986 were closely related to the Japan isolates from 1985 to 1986 and the Taiwan isolates from 1988 to 1989 were phylogenetically close to the 1989 Japan isolates, indicating that Taiwan and Japan had two common-source outbreaks. However, none of the 1988 Taiwan isolates were phylogenetically close to the 1988 Japan or Hong Kong isolates. The evidence revealed that Taiwan has had two repeated but discontinuous introductions of CA24v since its first appearance in Taiwan in 1985. None of the other CA24v strains have been detected so far.

Base Sequence↗
PubMed1993

Bone marrow transplantation for severe aplastic anemia.

Fifteen patients (nine male, six female) with severe aplastic anemia (SAA) undergoing HLA-identical allogeneic bone marrow transplantation (BMT) received preparative regimens consisting of cyclophosphamide and total lymphoid irradiation. Patients were aged from eight to 26 years (median 15 years). Prophylaxis of graft versus host disease (GVHD) including cyclosporine and short course methotrexate was administered. One early death occurred at day 8 post BMT. Among the other 14 patients, one died of sepsis at day 53 with no evidence of engraftment, 13 were engrafted despite the number of donors exposed in transfusions of previous blood components. Eleven patients have survived from six to 100 months (median, 54 months), post BMT. The remaining two patients died of acute GVHD-related infections on days 44 and 63. Acute GVHD occurred among eight of 13 engrafted patients, five of whom were grades II-IV clinically. Chronic GVHD developed among five patients, three of whom were clinically progressive and extensive. Two of three patients with extensive chronic GVHD had received transfusion of donor's buffy coat after BMT. Our data indicate an engraftment rate of 87% (13/15). The projected probability of disease-free survival was 73% (11/15) at 9.3 years after BMT according to the Kaplan-Meier model. Further efforts must be made to eliminate GVHD and to control fatal infections.

Adolescent↗
PubMed1993

Congenital factor VII deficiency complicated with hemoperitoneum and intracranial hemorrhage: report of a case.

Hypoproconvertinemia, or factor VII deficiency, is a rare congenital coagulopathy. We report on a female infant with congenital factor VII deficiency complicated by hemoperitoneum and intracranial hemorrhage. Most reports indicate that the bleeding of victims tends to be mild and confined to the superficial mucosa area. However, other reports and our experience with this patient suggest that it can result in fatal cerebral hemorrhage and necessitate early diagnosis, effective treatment and careful genetic counseling.

Cerebral Hemorrhage↗
PubMed1993

Characterization of acute myeloid leukemia (AML) coexpressing lymphoid markers: different biologic features between T-cell antigen positive and B-cell antigen positive AML.

The clinical and biologic characteristics of acute myeloid leukemia (AML) with coexpression of lymphoid-associated antigens (Lym+ AML) were studied from 39 cases who represented 24% of 161 newly diagnosed de novo AML. Twenty-seven cases (16.8%) were positive for the expression of T-cell markers (T+ AML) and 12 (7.5%) for B-cell markers (B+ AML). Chromosomal abnormalities t(9;22)(q34;q11) and t/del(11)(q23), which were considered to be associated with acute leukemia coexpressing markers of more than one cell lineage, were detected in five and in four patients, respectively. There was no prognostic significance of B-cell or T-cell antigen expression in AML. Of 12 T+ AML cases in which cells were available for gene analysis, all showed germline configuration of immunoglobulin heavy chain and T-cell receptor beta chain genes, while seven of nine B+ AML showed rearrangements of either or both of the genes. Double labeling of the cells with myeloperoxidase and lymphoid markers demonstrated that individual blasts in all the five T+ AML tested were simultaneously expressing myeloperoxidase activity and CD7; however, most blasts in the three B+ AML studied expressed either myeloperoxidase activity or CD10, but not both. In eight of the nine T+ AML tested, the T-cell antigen-positive leukemic blasts were significantly decreased to less than 10%, after in vitro culture with the differentiation-inducing agent phorbol ester. B-cell markers remained positive (> or = 20%) on the cells in the two B+ AML who had the same study. These findings suggested that T+ AML and B+ AML might have different biologic features. Further studies on more patients are needed to clarify this point.

Acute Disease↗
PubMed1992

Phosphorylation stimulates the transcriptional activity of the human beta 1 thyroid hormone nuclear receptor.

The role of phosphorylation on the gene activation activity of the human beta 1 thyroid hormone nuclear receptor (h-TR beta 1) was examined. h-TR beta 1 was found to be a phosphoprotein when expressed in COS-1 cells, with serine, threonine, and tyrosine (85:10:5) as the phosphorylation sites. Okadaic acid (a potent inhibitor of phosphatases 1 and 2A) at 0.1, 0.25, and 0.5 microM increased the phosphorylation of h-TR beta 1 by 3-, 7-, and 11-fold, respectively. The increase in phosphorylation was accompanied by a concomitant increase in phosphorylation was accompanied by a concomitant increase in receptor-mediated transcription in transient transfection assays. h-TR beta 1 purified from Escherichia coli was phosphorylated in vitro by the endogenous kinase from cellular extracts. Serine, threonine, and tyrosine were phosphorylated in a similar ratio to that found in COS-1 cells. The in vitro phosphorylation was stimulated by okadaic acid. Phosphorylation did not affect the binding of h-TR beta 1 to 3,3',5-triiodo-L-thyronine. However, phosphorylation of h-TR beta 1 resulted in an increase of its binding to DNA and conferred on it the ability to bind to nuclear accessory proteins. The results indicate that phosphorylation plays an important role in the transcriptional activity of h-TR beta 1.

Adenosine Triphosphate↗
PubMed1992

Peripheral blood hematopoietic progenitor cells in beta-thalassemia major.

Colony assays in methylcellulose (semisolid medium) of erythroid precursors and granulocyte-macrophage precursors from the peripheral blood of 34 patients with beta-thalassemia major and 31 age-matched normal controls were studied. In patients homozygous for beta-thalassemia, a significant increase of circulating erythroid progenitor cells to 3-5 times of normal controls was observed. Furthermore, the number of circulating colony forming units-granulocyte-macrophage (CFU-GM) and colony forming units-mixed (CFU-Mixed) also increased significantly. In these subjects, there was a linear relationship between the number of burst forming units-erythroid (BFU-E) and CFU-GM. The serum level of erythropoietin (EPO) was also significantly higher than that of normal controls (p < 0.05). The increased hematopoiesis, indicated by an increased number of circulating hematopoietic progenitor cells and an elevated serum level of EPO, suggests that the physiologic regulation of erythropoiesis still operated in patients with beta-thalassemia major.

Adolescent↗
PubMed1992

Phylogenetic analysis of a coxsackievirus A24 variant: the most recent worldwide pandemic was caused by progenies of a virus prevalent around 1981.

Nucleotide substitutions in the viral-encoded proteinase 3C (3Cpro) region (549 nucleotides) of the RNA genome of a coxsackievirus A24 variant (CA24v), one of the agents causing acute hemorrhagic conjunctivitis (AHC), were studied using 32 isolates collected from the Eastern hemisphere in 1970-1989. Based on regression analysis of nucleotide differences among isolates, the nucleotide substitution rate of CA24v 3Cpro was estimated to be 3.7 x 10(-3)/nucleotide/year. A phylogenetic tree constructed by the modified unweighted pair group method using arithmetic averages (UPGMA) indicated that CA24v had evolved from a common ancestor which appeared in one focal place in November 1963 +/- 21 months, about 7 years before the first isolation of CA24v in Singapore. The tree also revealed that all the recent epidemic isolates in 1985-1989 including Asian and Ghanian strains diverged from each other after 1981. This finding is consistent with the evidence that AHC due to CA24v had been confined to Southeast Asia and the Indian subcontinent until 1985, then suddenly and explosively spread to other areas where no CA24v isolations had been reported.

Amino Acid Sequence↗
PubMed1992

Cytogenetic study of acute lymphoblastic leukemia and its correlation with immunophenotype and genotype.

Among 72 Chinese patients with acute lymphoblastic leukemia (ALL), 50 had clonal chromosomal abnormalities. Structural abnormalities were detected in 42 patients: these included t(9;22) in 9, t(1;19) in 6, t(4;11) in 5, del(11)(q23) in 4, and del(6q) in 4. Adults had a higher incidence of t(9;22) and t(1;19) but a lower incidence of t(4;11) and hyperdiploid greater than 50 karyotype than children. A significant difference was also noted in white blood cell (WBC) count among various karyotypic groups. Patients with chromosomal abnormalities t(9;22), t(1;19), t(4;11) and del(11) (q23) had a shorter complete remission duration as compared with patients free of these abnormalities. Immunophenotyping was performed on 69 patients. All patients with t(9;22), t(1;19), and t(4;11) had B-lineage ALL restricted to certain stages of maturation: groups III and IV, groups IV and V, and group II, respectively (according to the classification of Foon and Tood). Among patients with t(9;22), t(4;11), and del(11)(q23), which have been considered to be associated with acute mixed-lineage leukemia, one each, respectively, showed myeloid antigen expression on the leukemic blasts (My+ ALL). No cross-lineage rearrangements of immunoglobulin (Ig) or T-cell receptor (TCR) genes were detected in these karyotypic subgroups of patients who underwent gene analysis.

Adolescent↗
PubMed1992

Characterization of Philadelphia-chromosome-positive acute leukemia by clinical, immunocytochemical, and gene analysis.

Philadelphia chromosome (Ph') was detected at presentation in 10 out of 110 patients with acute lymphoblastic leukemia (ALL) and five of 168 patients with acute myelogenous leukemia (AML). Two other ALL patients who had studies at relapse were also included in the analyses. One of the 12 Ph'-positive (Ph+) ALL patients had simultaneous expression of myeloid-associated antigen on the leukemic blasts, while all the five AML patients coexpressed markers of lymphoid cells. Double labeling of the cells with myeloperoxidase and CD10 on three Ph+ AML cases showed that most leukemic blasts expressed either myeloperoxidase activity or CD10 but not both. Cross-lineage gene rearrangements of T-cell receptor (TCR) beta-chain gene were detected in three of the eight Ph+ ALL patients tested. All the four Ph+ AML cases studied showed immunoglobulin heavy chain gene rearrangements, and three of them also had simultaneous rearrangements of TCR beta-chain gene. The results revealed that Ph+ acute leukemia in this study belonged either to ALL or mixed lineage leukemia, and none was pure AML. This finding is contrary to that of acute blast crisis of chronic myelogenous leukemia in which the majority of patients had myeloid transformation. Rearrangements of bcr were detected in four of the 10 Ph+ ALL and three of the four Ph+ AML patients tested. No significant difference was noted in the clinical or hematologic manifestations among Ph+ leukemia with or without bcr rearrangements.

Adult↗
PubMed1992

Infantile osteopetrosis: report of two cases.

Two cases of infantile osteopetrosis are reported. Both were males aged four and eight months at presentation. They presented with osteosclerotic change of the bone, leukoerythroblastic anemia, optic atrophy, hepatosplenomegaly and frequent infection. The histology of the bone showed thickened bone trabeculae with little osteoclastic activity, although in one patient the number of osteoclasts increased, while in the other they did not. One received a bone marrow transplant (BMT) but died from disseminated cytomegaloviral infection, pulmonary hemorrhage and sepsis. The post-transplant marrow histology showed evidence of engraftment and osteoclastic activity. The other only received a course of prednisolone, which was of little help. His condition has followed a natural course with progressive visual impairment and marrow failure. Our cases suggest that infantile osteopetrosis should be taken into consideration in dealing with infants who present with early marrow failure and that patients of infantile osteopetrosis should receive BMT. BMTs appear to be the only cure. They should be given as early as possible to avoid major consequences and severe infection.

Bone Marrow Transplantation↗
PubMed1992

Long-term observation of pediatric aplastic anemia.

Fifty-nine verified cases of acquired aplastic anemia (AA), diagnosed at the Pediatric Department of the National Taiwan University Hospital from 1977 to 1987, were reviewed and analyzed. The demographic features showed a high relative incidence (acute myelogenous leukemia/AA ratio, 2.2/1), a high percentage of non-severe AA (39%) and a high association with hepatitis (20.8%). No evidence of hepatitis A, B or C virus infection was found in five cases of hepatitis-associated AA. No sex preponderance was noted in this pediatric series. The 10-year projected survival rate of the total series approached 55%. The crude two-year-survival and two-year-transfusion-free-survival rates were 59% and 44%, respectively, in the conservative therapy group treated with androgens and steroids; 36% and 32%, respectively, in patients with severe AA in the conservative therapy group; and 73% and 64%, respectively, in the aggressive therapy group treated with cyclosporin, anti-lymphocyte globulin or bone marrow transplant. The major causes of death were hemorrhage (44%) and infection (56%) in the conservative therapy group; but in the aggressive therapy group, two out of three deaths were related to therapeutic complications. Multivariate analysis of prognostic factors revealed that severity and treatment modality were independent risk factors. Only two out of 31 patients who survived more than two years (long-term survivors) experienced late mortalities. At two, five, seven and 10 years after diagnosis, 61%, 55%, 41% and 40% of the long-term survivors had inadequate hematopoietic recovery.

Adolescent↗
PubMed1992

DNA fingerprinting in the Chinese with an oligonucleotide probe (GTG)5.

DNA fingerprinting is a very powerful tool that enables specific identification of individuals. In order to study fingerprinting patterns in the Chinese, the oligonucleotide probe designed by Epplen et al was used. Peripheral blood leukocyte DNA was digested with restriction enzyme Hinf I and probed by (GTG)5. Normal females and males were tested; all revealed very different DNA fingerprints. Most of the discernible bands in the gel were polymorphic; there was no specific association between any polymorphic band and the sex of the individual tested. In situ gel-hybridization was used with the oligonucleotide probe, and after electrophoresis, all procedures could be completed within one working day. Three patients who had received bone marrow transplants from their respective siblings had the same fingerprints as their donors. The inheritance of all bands from either the father or mother proved parents' authenticity. DNA fingerprinting with a oligonucleotide probe is obviously a useful technique. Clinical application may include engraftment monitoring, zygosity determination and paternity testing.

Base Sequence↗
PubMed1992

Results of therapy for beta-thalassemia major.

During the last 10 years, 92 transfusion-dependent beta-thalassemia patients have been encountered at the National Taiwan University Hospital and Provincial Taoyuan General Hospital. Seventy-seven of them were followed up regularly. Long-term results of conventional therapy in 63 cases and allogeneic bone marrow transplantation (BMT) in 14 cases are reported. The conventional therapy included regular red cell transfusion and desferrioxamine iron chelation therapy. Preliminary results of conventional therapy showed a mortality of 7/63 (11%). Of those who were alive, the morbidity was 56/56 (100%). There was no disease-free-survival (0/56; 0%). BMT was performed after preparatory regimens of busulfan, cyclophosphamide, and/or total body or lymphoid irradiation. Preliminary results of BMT showed a mortality of 5/14 (36%). For those who were alive, the morbidity was 3/9 (33%), and the disease-free-survival rate was 6/9 (67%) during a follow-up period of three to six years. It is concluded that the only way to cure beta-thalassemia major at present is BMT. However, the risks of BMT and donor non-availability make conventional therapy unavoidable. Further study is needed to decrease the risk of BMT and to improve the efficacy of conventional therapy.

Adolescent↗
PubMed1992

A new codon 31 (-C) mutant resulting in beta zero-thalassemia.

A new beta zero-thalassemia mutation, a frameshift mutation with deletion of a single cytosine nucleotide in codon 31, is described. The propositus, which is compound heterozygous for this mutation and the 17 beta A-T beta zero-thalassemia mutation, has the phenotype of severe beta-thalassemia major.

Base Sequence↗