Search PubMed⌕ Search

Biomedical subjects

H F Tien

Publications and source records attributed to H F Tien.

81 records · Page 5Linked to original sources

Immunopathological diagnosis of plasma-cell dyscrasia. Part I. "Primary" monoclonal gammopathy.

Two patients with "primary" plasma-cell dyscrasia, one with IgG kappa multiple myeloma and another with delta type light chain disease, are reported. Both of them presented extraosseous tumor mass on the forehead as an initial problem. They had moderate to severe anemia, accelerated ESR and multiple osteolytic lesions in the x-ray bone survey. They did not show prominent monoclonal spikes in serum electrophoregram and physiological immunoglobulins were not decreased. Repeated bone marrow aspirates on these two cases did not reveal definite plasmacytosis and pathological diagnosis was established by tumor biopsy. Immunopathological correlations were made, with an emphasis on rational analysis and interpretation of monoclonal proteins.

Biopsy, Needle↗

Immunopathological diagnosis of plasma-cell dyscrasia. Part II. "Secondary" monoclonal gammopathy.

Two cases of secondary plasma-cell dyscrasia are presented: one is of undetermined cause and the other is due to hepatic schistosomiasis. Both of the patients showed a prominent IgG monoclonal spikes of more than 3000 mg/dl. One of them had multiple osteolytic bone lesions and pathological fractures, and histopathological picture showed a chronic inflammatory process. An additional case of marked polyclonal gammopathy due to schistosomiasis is also presented to illustrate the immunochemical differential between the physiological and altered immune responses. Schistosomiasis is postulated to be an important immunopathological cause of plasma-cell dyscrasia.

Adult↗

Primary T cell leptomeningeal lymphoma--successful treatment with systemic chemotherapy.

Primary central nervous system lymphoma (PCNSL) is rare, accounting for only 1-2% of non-Hodgkin's lymphoma, and primary isolated leptomeningeal lymphoma is even rarer. It may create a diagnostic problem, particularly when the tumor cells are of T cell lineage. We herein report a patient with primary T cell leptomeningeal lymphoma. The final diagnosis was confirmed by the cytogenetic study which revealed clonal aberration, isochromosome of long arm of chromosome 7, in the lymphoid cells from CSF. She was treated with a novel protocol of systemic chemotherapy specifically designed for the CNS lymphoma. The regimen consisted of carmustine, vincristine, high-dose methotrexate, etoposide, and methylprednisolone. A total of 5 courses were given and she was still in complete remission at the time of reporting, 21 months after the establishment of diagnosis.

Adult↗

Treatment of refractory or relapsed adult acute leukemia by using mitoxantrone-containing regimens.

Twenty-eight adult patients with primary refractory or relapsed acute leukemia were treated. The regimens consisted of mitoxantrone plus cytosine arabinoside for 17 patients with acute non-lymphocytic leukemia (ANLL) and mitoxantrone accompanied with vincristine and prednisolone for 11 patients with acute lymphoblastic leukemia (ALL). In primary refractory patients, 1 of the 4 (25%) ANLL and 1 of the 3 (33%) ALL attained complete remission (CR). Excluding 2 patients who underwent bone marrow transplantation, 8 of the 13 (62%) relapsed ANLL and 4 of the 8 (50%) relapsed ALL achieved CR with a median duration of remission of 6.2 months and 3.8 months, respectively. Myelosuppression occurred in all treatment courses and was associated with pyrexia due to infections in 84% of the cases. Nausea, vomiting and stomatitis were mild. Abnormal liver function tests were observed in 8 (28%) patients. One patient, pretreated with 550 mg/m2 of doxorubicin, developed congestive heart failure. The results suggest that mitoxantrone is of value in the treatment of Chinese patients with refractory or relapsed acute leukemia.

Adult↗

Prognostic significance of immunophenotypes in adult lymphoblastic lymphomas.

Adult lymphoblastic lymphoma (LBL) can be of T-cell or B-cell lineage. However, the clinical significance of immunophenotypes is largely unknown. We conducted a retrospective study to compare T-cell LBL with its B-cell counterpart. Between 1983 and 1995, 50 adult patients were diagnosed as LBL at National Taiwan University Hospital. Twenty-seven patients (T-LBL:20 and B-LBL:7) had adequate clinical information and formed the basis of final analysis. Pertinent characteristics, including sex, age, and lymphoma stages of these two groups of patients were identical. Detailed clinical features were compared. Systemic involvements of lymphoma were similar except that T-cell LBL had significantly more mediastinal involvement (T:B = 70%:14.3%, p = 0.011). CNS involvement was high in both groups (T:B = 50%:28.6%, p = NS). B-cell LBL had a better overall survival than T-cell LBL, although the survival benefit became less significant after 30 months. The median survival of T- and B-cell LBL was 8 and 31 months, respectively. Both groups taken together, patients who had received prophylactic cranial irradiation had a better overall survival (p < 0.01). We suggest that: a) B-cell LBL has a relatively favorable prognosis than T-cell LBL, at least in the initial 2 to 3 years; b) except for mediastinal involvement, the clinical presentation of T- and B-cell LBL appears to be similar; c) treatment policy, such as the need of prophylactic cranial irradiation, of these two groups may also be similar.

Adult↗

Frequent p53 mutation in relapsed acute lymphoblastic leukemia with cytogenetic instability: a longitudinal analysis.

P53 mutation, through defects in repairing DNA damages, is associated with genomic instability. In 24 adult patients with relapsed acute lymphoblastic leukemia (ALL), the status of the p53 gene at different stages was serially followed by cytogenetic and polymerase-chain reaction/single strand conformation polymorphism (PCR/SSCP) analysis. P53 mutation was detected in 6 patients (25%) and all were at relapsed stages: 3 at first relapse, 2 at second relapse and one at refractory phase. Loss of the remaining p53 allele was demonstrated in 5 of them by cytogenetic and SSCP analysis. Furthermore, all of the p53 mutations were associated with appearance of complex cytogenetic changes (CCG), suggesting of cytogenetic instability (CGI). The frequency of acquiring p53 mutation during leukemic progression is significantly higher in patients with CGI than in patients without CGI (6/11 v 0/13, p = 0.003). P53 mutation was detected either concurrently (n = 2) or after (n = 4, 2(+)-18 months) appearance of CCG. The results suggest that p53 mutation is frequently involved during ALL relapse and is closely linked with cytogenetic instability.

Adult↗

P53 mutation in advanced stage of primary myelodysplastic syndrome.

Polymerase chain reaction and single strand conformation polymorphism (PCR-SSCP) analysis of the p53 tumor suppressor gene (from exon 2 to 9) was performed on samples from 47 adult patients with primary myelodysplastic syndrome (MDS). Point mutation was found in 5 (11%) patients: exon 7 in 3, exon 4 in 1 and intron 5 in 1. The frequency of p53 mutation was significantly higher at advanced stages (p = 0.048) and higher in patients with abnormal karyotypes (p = 0.023). Although all of the p53 mutations were detected at advanced stages, four of them were detected at initial diagnosis with very short survival. Sequential SSCP analysis in 20 transformed MDS patients revealed only one new p53 mutation during progression from early MDS phases. The results suggest that p53 mutation might occur as an early genetic event and is probably associated with rapid progression and poor survival in some MDS patients.

Adult↗