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Biomedical subjects

H F Tien

Publications and source records attributed to H F Tien.

At least 19 recordsLinked to original sources
PubMed2001

Philadelphia chromosome-positive acute lymphoblastic leukemia in Taiwan.

From January 1986 to December 1998, 26 (23%) of 114 acute lymphoblastic leukemia (ALL) patients older than 15 years were found to have Philadelphia (Ph) chromosome. They accounted for 28% (26 of 94) of the patients with B-lineage ALL. Compared with the other 88 ALL patients, the leukemic cells from all but one Ph-positive ALL patients were early pre-B cells with a higher rate of CD34 expression (92% vs 50%, P<0.05). At presentation, the Ph-positive adult ALL patients had higher circulating blasts (mean 21.4 vs 3.66x10(4)/microl, P<0.05) and lower initial platelet counts (mean 4.47 vs 7.34x10(4)/microl, P<0.01) and showed a trend toward higher frequency of initial central nerve system (CNS) involvement (25% vs 11%, P=0.098) than the others. Among patients with adequate chemotherapy, 16 (64%) of the 25 Ph-positive ALL patients achieved complete remission (CR), an incidence marginally lower than that of Ph-negative ALL (62 of 76, 82%, P=0.06) and significantly lower than that of Ph-negative B-lineage ALL (50 of 58, 86%, P=0.0037). However, all patients relapsed except for those who received allogeneic hemopoietic stem cell transplantation (allo-HSCT). The probabilities of 5-year continuous CR and 5-year survival for Ph-positive adult ALL patients were significantly inferior to those for Ph-negative adult ALL patients (0% vs 12%, P=0.0001, and 7% vs 19%, P=0.047, respectively), and those for Ph-negative B-lineage ALL (0% vs 14%, P<0.0001, and 7% vs 23%, P=0.002, respectively). Prognostic factors were analyzed among the Ph-positive ALL patients including the 26 adults mentioned above and an additional 11 children. No clinical or biological characteristics such as age, sex, initial circulating blast count, extramedullary involvement, or CD34 expression had an impact on the disease outcome. Allo-HSCT in first CR may improve the probability of 5-year continuous CR (100% vs. 0% for those without allo-HSCT, P=0.0091) although only three patients received it in this study. In conclusion, Ph-positive ALL patients tended to have a poor prognosis, regardless of whether other possible risk factors were present or not. Aggressive treatment, such as high-dose chemotherapy with allo-HSCT, should be considered for these patients to improve survival.

Adolescent↗
PubMed2001

Methylation of the p15(INK4B) gene in myelodysplastic syndrome: it can be detected early at diagnosis or during disease progression and is highly associated with leukaemic transformation.

To investigate the time sequence of occurrence of p15(INK4B) gene methylation in myelodysplastic syndrome (MDS) and its correlation with leukaemic transformation and survival of patients, the methylation status of the p15(INK4B) promoter region was analysed in 50 patients and was serially studied in 22 of them. Of the 50 patients, 17 (34%) showed p15(INK4B) gene methylation, first demonstrated at diagnosis or during follow-up. When FAB subtypes at the time of study were used in the analysis, the incidence of (p15INK4B) methylation in each risk group of MDS remained stable throughout the course: 0% for low-risk MDS [refractory anaemia (RA) and RA with ring sideroblasts] and from 23% at diagnosis to 30% for high-risk MDS [RA with excess of blasts (RAEB), RAEB in transformation and chronic myelomonocytic leukaemia] respectively. The incidence of p15(INK4B) methylation rose to 60% at initial study and, finally, to 75% in cases of acute myeloid leukaemia (AML) evolved from MDS. Most patients (69%) with p15(INK4B) methylation showed disease progression to AML; it could be detected before, at the time or after the diagnosis of leukaemic transformation. p15(INK4B) methylation in MDS patients implicated a shorter survival time in univariate analyses, but its prognostic significance disappeared in multivariate analyses. In conclusion, p15(INK4B) methylation can be detected early at the diagnosis of MDS or acquired during disease progression. It may play an important role in the pathogenesis of some high-risk MDS and is related to leukaemic transformation of MDS.

Acute Disease↗
PubMed2000

Characterization of acute myeloid leukemia with MLL rearrangements--no increase in the incidence of coexpression of lymphoid-associated antigens on leukemic blasts.

MLL gene rearrangements are associated with coexpression of myeloid- and lymphoid-associated antigens on leukemic blasts and a dismal outcome in acute lymphoblastic leukemia (ALL). Whether the same conditions can apply to acute myeloid leukemia (AML) is not quite clear. Rearrangements of the MLL gene were analyzed on 113 patients with newly diagnosed de novo AML in a single institution. Sixteen (14%) of them showed rearranged bands by Southern blot analysis, including three (50%) of six infants, three (14%) of 21 children between 1 and 15 years and 10 (12%) of 86 adults. MLL rearrangements were not only detected in M5 (four of 12 patients, 33%) and M4 (six of 31, 19%) subtypes but also in other non-M4-M5 AML (six of 70, 9%), including M1, M2 and M7, but not M3 subtype. Seven patients had chromosomal abnormalities involving 11q23, but nine did not. The latter comprised three (6%) of 48 patients with normal karyotype, one with t(8;21), none with t(15;17), inv(16) or t(9;22), and four (15%) of 27 with cytogenetic aberrations other than those specific structural abnormalities. In contrast to ALL, AML patients with MLL rearrangements did not tend to coexpress lymphoid- and myeloid-associated antigens simultaneously on leukemic blasts and have similar outcome as those without the gene rearrangements.

Acute Disease↗
PubMed2000

Geographical differences in human herpesvirus 8 seroepidemiology: a survey of 1,201 individuals in Asia.

Since the discovery of human herpesvirus 8 (HHV8) as a contributory cause of Kaposis sarcoma, the clinical role of this virus has been actively investigated. An understanding of HHV8 seroepidemiology is critical for the study of its pathogenesis within a specific environment. A sero-survey is described in Taiwan of 1,201 individuals ranging in age from under 1 year to over 70. Indirect immunofluorescence assay was used to determine antibody titers against both latent and lytic antigens of HHV8. The results indicate that very few individuals (3-4%) were exposed to HHV8 before 10 years of age. Infection rate peaked (19.2%) between the ages of 21 to 40. Females showed a slightly higher seroprevalence for HHV8 than males, but the difference was not statistically significant. Pregnancy did not correlate with increased HHV8 infection rate nor with augmented HHV8 antibody titers. It is concluded that HHV8 in Taiwan is predominantly an infectious agent for adults. In this geographical locale, HHV8 is similar to herpes simplex virus type 2 in its likely transmission occurring presumptively through sexual routes. However, the study also indicates that a smaller portion of HHV8-transmission could occur through nonsexual contacts.

Adolescent↗
PubMed1999

Extramedullary relapse after all-trans retinoic acid treatment in acute promyelocytic leukemia--the occurrence of retinoic acid syndrome is a risk factor.

All-trans retinoic acid (ATRA) is now a standard agent for remission induction of acute promyelocytic leukemia (APL). Recently, extramedullary relapse, which was a rare condition in APL patients after chemotherapy alone, was reported with an increased frequency after ATRA treatment. However, it is not yet clear whether ATRA truly increases the risk of extramedullary recurrence and what are the risk factors. In this study, three of 13 patients with recurrent APL after prior treatment of ATRA were found to have extramedullary involvement, compared with none in 11 recurrent patients previously treated with chemotherapy alone (estimated relative risk 2.100, 95% confidence interval 1.341-3.289). Furthermore, in the former group of patients, the development of retinoic acid (RA) syndrome during prior induction treatment was significantly associated with extramedullary involvement at relapse (three in five patients with RA syndrome vs none in eight without the syndrome, estimated relative risk 5.000, 95% confidence interval 1.448-17.271). In conclusion, ATRA may predispose APL patients to extramedullary involvement at relapse and the occurrence of RA syndrome is a risk factor for it. Further studies are needed to confirm these findings. It also remains to be clarified whether treatment modification is necessary in patients who develop RA syndrome during ATRA treatment.

Adolescent↗
PubMed1999

Minimally differentiated acute myeloid leukemia in Taiwan: predominantly occurs in children less than 3 years and adults between 51 and 70 years.

Acute myeloid leukemia (AML) with minimal differentiation was usually referred to as acute undifferentiated leukemia in the past. With the help of immunophenotyping, this subtype of leukemia was shown to express myeloid antigens on the blasts and was designated AML-M0 by FAB Cooperative Study Group in 1991. Among the 423 consecutive newly diagnosed de novo AML at our institution, 12 (2.8%) were of M0 subtype. The proportion of M0 in AML was higher in children than in adults (8.2% vs 1.7%). Four other M0 patients referred from outside hospitals for immunophenotyping were also included in this study. There were two peaks in age distribution of these 16 patients: less than 3 years and between 51 and 70 years, respectively. Organomegaly was more common in patients with AML-M0 than in those with other subtypes (56.3% vs 29.2%, P = 0.025). The former patients had higher incidences of CD7 and CD34 expression on the leukemic cells than the latter ones (50% vs 16.9%, P = 0.003 and 69.2% vs 37.9%, P = 0.019, respectively). The patients with AML-M0 showed more frequent clonal chromosomal abnormalities in the leukemic cells than other AML patients (83.3% vs 53.9%, P = 0.039); the same is also true for complex cytogenetic aberrations (50% vs 11. 4%, P = 0.004). Adults with AML-M0 showed a lower complete remission (CR) rate and significantly poorer survival than those with non M0-AML. However there was no significant difference in outcome between the two groups of pediatric patients. In conclusion, AML-M0 is a unique subtype of leukemia that has distinct age distribution and shows different clinical and biological characteristics from other AML. Adult patients have poor prognosis. Whether pediatric patients had better outcome than adults needs to be clarified in further studies.

Acute Disease↗
PubMed1999

Allogeneic stem cell transplantation for patients with high-risk myelodysplastic syndrome.

Allogeneic stem cell transplantation (allo-SCT) is the only treatment with curative potential for patients with myelodysplastic syndrome (MDS). From June 1986 to April 1997, we treated 12 patients with primary MDS (5 men, 7 women, median age, 36.5 years) by allo-SCT. All patients had one or more of the following poor prognostic factors: intermediate-2 or high-risk categories according to the International Prognostic Scoring System; disease progression during follow-up; heavy transfusion requirements and recurrent infections. The median duration from diagnosis of MDS to allo-SCT was 6 months. The preconditioning regimen included total body irradiation combined with either high-dose cytarabine (n = 6), high-dose cyclophosphamide (n = 4), or other regimens (n = 2). Ten patients received bone marrow transplantations and two patients received peripheral blood stem cell transplantations. Prophylaxis for graft-versus-host disease (GVHD) consisted of standard cyclosporin and short-course methotrexate. Acute GVHD of grade 2 or above occurred in 10 patients, while chronic GVHD occurred in seven of the nine patients who survived longer than 6 months after allo-SCT. With a median follow-up of 50 months, all nine patients with human leukocyte antigen (HLA)-matched sibling donors survived. One patient had a relapse 6 months after transplantation and achieved complete remission again with low-dose cytarabine therapy. The three patients receiving allo-SCT from unrelated or HLA-mismatched donors died of grade 3 to 4 acute GVHD and infection within 5 months after transplantation. The estimated disease-free survival at 4 years was 67% (95% confidence interval, 40-93%), and the overall survival was 75% (95% confidence interval, 50-99%). Our data suggest that allo-SCT should be considered early in the clinical course for young MDS patients with a poor prognosis and a matched sibling donor.

Adult↗
PubMed1998

Jacobsen distal 11q deletion syndrome with a myelodysplastic change of hemopoietic cells.

We describe a male infant with unusual facial appearance, relative pancytopenia, bilateral simian creases, and an accessory nipple. Cytogenetic analysis showed deletion of the long arm of chromosome 11 [46,XY,del(11)(pter-->q23.2:)]. Bone-marrow study showed a myelodysplastic change of hemopoietic cells compatible with peripheral blood findings. Pachygyria of the temporal and frontal lobes was demonstrated by magnetic resonance image (MRI) of the brain. We present our findings in order to contribute to the information on 11q23 deletion.

Bone Marrow Cells↗
PubMed1998

Acute and chronic arsenic poisoning associated with treatment of acute promyelocytic leukaemia.

Seven relapsed and/or refractory acute promyelocytic leukaemia patients were treated by arsenic trioxide (As2O3). Four patients (4/7, 57%) achieved complete remission after one to three cycles of treatment and the most common acute side-effect was fluid retention (in six patients, 86%), including weight gains and pleuro-pericardial effusions. Evident polyneuropathy compatible with chronic arsenic toxicity was noted in two of the three patients who received As2O3 maintenance therapy and one of them had marked distal muscular atrophy. We suggest that As2O3 may be a useful salvage therapy for relapsed and refractory APL patients, but the acute or chronic arsenic toxicity should be carefully monitored.

Acute Disease↗
PubMed1998

Clonal disease of natural killer large granular lymphocytes in Taiwan.

Lymphoproliferative diseases of large granular lymphocytes (LDGL) may arise from either CD3+ T cells or CD3- natural killer (NK) cells. LDGL with clonal proliferation of large granular lymphocytes (LGL) is defined as LGL leukaemia. The number of patients with NK-LGL leukaemia reported is limited and the pathogenesis of the disease is not yet clear. From 1991 to 1998 six patients with cytogenetically proved clonal disease of NK-LGL were identified in our institute. All were seropositive for Epstein-Barr virus (EBV). EBV RNA or DNA could be detected in LGL from four patients by EBV in situ hybridization or Southern blot analysis. Most patients ran an aggressive clinical course and five died of the disease. Nonrandom clonal chromosomal abnormalities, including duplication of 1q, rearrangement at 3q and loss of chromosomes Y, 13 or 10, were noted in the six patients from this study and in eight from the literature. The implications of these recurrent cytogenetic aberrations in the development and progression of the disease deserve further studies.

Adolescent↗
PubMed1998

CD7 positive hematopoietic progenitors and acute myeloid leukemia and other minimally differentiated leukemia.

Acute leukemias are believed to arise from unregulated proliferation of hematopoietic cells and loss of the ability to differentiate. Studies on the immunophenotypes of leukemic cells are very helpful for the understanding of antigen expression during normal hematopoiesis. CD7 antigen has until recently been considered to be a T-cell marker but has been found to be expressed by leukemic cells from some acute myeloid leukemia (CD7+ AML) and the normal putative counterparts of blasts from CD7+ AML can be found in human fetal livers. These double CD7 and myeloid antigen (CD13 and/or CD33) positive progenitors tend to lose their CD7 expression, while retaining their myeloid characteristics, after in vitro culture with the differentiation-inducing agent phorbol ester (TPA). This suggests that the cells are probably committed to the myeloid cell lineage and that CD7 is only transiently expressed in the early differentiation stage. On the other hand, there is a subset of CD7+ hematopoietic precursors which lack mature myeloid and T-cell antigens and have the potential to differentiate to both T-lymphoid and myeloid cells. These cells may in fact be the common myeloid-T lymphoid progenitors and represent the normal counterparts of acute undifferentiated leukemia or minimally differentiated T-cell acute lymphoblastic leukemia.

Acute Disease↗
PubMed1997

Clinical and biological characteristics of acute promyelocytic leukemia in Taiwan: a high relapse rate in patients with high initial and peak white blood cell counts during all-trans retinoic acid treatment.

Acute promyelocytic leukemia (APL) patients treated with all-trans retinoic acid (ATRA) and chemotherapy have been shown to have better outcome than those treated with conventional chemotherapy alone. However, the biological characteristics of leukemic cells and their clinical implications in patients treated with ATRA have not been well established. In this study, the biological and clinical features of 30 APL patients were reported. The risk factors for relapse and for occurrence of retinoic acid (RA) syndrome, which might cause morbidity or mortality of patients after ATRA treatment, were also analyzed. All patients showed 15;17 translocation by cytogenetic and/or gene analysis. Patients in this study had higher white blood cell (WBC) counts and a higher incidence of additional abnormalities than those from other areas. The ratio of long (L) form to short (S) form PML-RAR alpha fusion transcript was 1.8:1, a value lower than that of Latino patients but higher than that of Italians. Leukemic cells from four patients showed coexpression of T cell-associated antigen CD2 which was highly correlated with S form fusion transcript. Nine (36%) of the 25 patients treated with ATRA developed RA syndrome; all but one were successfully controlled by corticosteroid. Complete remission (CR) rate was 84%. Patients with high WBC counts tended to develop RA syndrome and had increased risk of relapse. Isochromosome for the long arm of the derivative chromosome 17, ider(17q), as an additional chromosomal abnormality was also associated with poor outcome in this study. In conclusion, APL in this study showed some different biological characteristics compared with those reported in other areas. High WBC count was a risk factor for relapse and development of RA syndrome after ATRA treatment. The prognostic implication of the presence of ider(17q) needs further clarification.

Adolescent↗
PubMed1997

Clonal chromosomal abnormalities as direct evidence for clonality in nasal T/natural killer cell lymphomas.

Nasal T/natural killer (NK) cell lymphoma is a distinct clinicopathologic entity which is more prevalent in Asia than in America and Europe. The clonal nature of the infiltrating lymphoid cells is difficult to demonstrate because of the lack of immunologic markers for clonality and the absence of clonal T-cell receptor gene rearrangement in most cases. In this study, clonal chromosomal abnormalities were detected in the tumour cells from four patients with nasal T/NK cell lymphoma. This finding provided direct evidence for clonality of the disease. Moreover, nonrandom cytogenetic abnormalities, including isochromosome for the short arm (p) of chromosome 6, isochromosome for the long arm (q) of chromosome 1, partial deletion of 6q, and aberrations at 11q, were disclosed. Isochromosome 6p was the sole structural abnormality in one patient, which may be a pathognomonic change in nasal lymphoma.

Adult↗
PubMed1997

Clinical, haematological and molecular studies in patients with chromosome translocation t(7;11): a study of four Chinese patients in Taiwan.

Translocation t(7;11)(p 15;p15) is an uncommon but recurrent chromosome aberration in acute myeloid leukaemia (AML). which occurs mostly in oriental patients and in AML M2 or, occasionally, M4 subtype. Recently, a consistent chimaeric fusion transcript NUP98-HOXA9 was found in several cases of t(7;11). Four AML cases with the chromosome abnormality in Taiwan are described. They were all adults with ages ranging from 30 to 41 years (median 36 years). Three of them were diagnosed as having AML M2; the remaining one as M4. Marked dyserythropoiesis was demonstrated in two patients. All four patients showed pan-myeloid antigen CD13 on the leukaemic cells, but none coexpressed lymphocyte-associated antigens and neither of the two patients studied for CD34 expression had positive staining. NUP98-HOXA9 fusion transcript was detected in both patients who had molecular analysis and the breakpoints on chromosome 11 and 7 respectively were similar to those previously reported. They all received conventional induction chemotherapy, but only one achieved a complete remission (CR) with short duration. This study and others reported in the literature suggest a racial or geographical predisposition among oriental patients to AML with t(7;11) and that this is associated with a poor prognosis. The molecular detection of NUP98-HOXA9 fusion transcript would be a useful method for the diagnosis of t(7;11) and also for monitoring disease status after treatment.

Adult↗
PubMed1997

Ticlopidine-induced aplastic anemia: report of three Chinese patients and review of the literature.

In this study, three Chinese patients with ticlopidine-induced aplastic anemia were reported and another 13 patients in the English literature were reviewed. We attempted to find underlying similarities, evaluate the risk factors, and identify appropriate treatment for this complication. All but one of the patients were over 60 years old, and the 6 who died were all older than 65. Therefore, old age may be a risk factor for developing this complication. Agranulocytosis occurred 3-20 weeks after initiation of ticlopidine, so frequent examination of white cell count during treatment is recommended. There seemed to be no direct correlation between the dose or duration used and the severity of bone marrow suppression. Treatment for ticlopidine-induced aplastic anemia with colony-stimulating factors seemed to have little effect. The fact that 5 of the 6 patients who received concurrent calcium channel blockers died, should alert clinicians to be more cautious when using these two drugs simultaneously.

Aged↗
PubMed1996

Clinicopathologic, cytogenetic, and molecular studies of 13 Chinese Patients with Ki-1 anaplastic large cell lymphoma. Special emphasis on the tumor response to 13-cis retinoic acid.

BACKGROUND: The clinicopathologic and molecular features of the newly characterized Ki-1 lymphoma, although well studied in Western countries, are less well described in Asia. METHODS: Pathology material of lymphoma cases, consecutively diagnosed at our institution between 1986 and 1994, was reviewed. The cases fulfilling the diagnostic criteria of Ki-1 lymphoma were selected, and their clinicopathologic features were studied. Immunohistochemistry and cytogenetic studies of the lymphoma tissues, and molecular analysis for nucleophosmin (NPM) gene translocation were performed. RESULTS: A total of 13 cases (2.3%) of Ki-1 large cell lymphoma were identified from a total of 572 cases of non-Hodgkin's lymphoma diagnosed during this period of time. There were 10 men and 3 women with a median age of 43 years (range, 18 to 61 years). The initial presenting sites included the skin alone in five cases, lymph node alone in one case, both skin and lymph node in five cases, and the stomach in one case. All cases had large cell morphology, with the majority of the neoplastic cells expressing Ki-1 antigen (CD30). Except for the case with gastric involvement, all other cases were T-cell lymphomas. One of seven cases examined had chromosomal abnormality of t (2;5). Rearrangement of the NPM gene at chromosome 5 was detected by Southern blot analysis in three of nine cases. Two of seven cases tested by reverse-transcriptase polymerase chain reaction showed fusion of the NPM gene and anaplastic lymphoma kinase gene. Seven (78%) of 9 patients who had received systemic chemotherapy with a standard cyclophosphamide, doxorubicin, vincristine, and prednisolone regimen obtained a complete remission (CR). The median duration of remission was 33 months. Three (60%) of 5 patients, of whom 4 recurred from previous intensive chemotherapy, achieved a prolonged CR with treatment with 13-cis retinoic acid (RA). The median survival of the whole group has not yet been reached at a median follow-up of 40 months. CONCLUSIONS: The clinicopathologic and molecular features of Ki-1 lymphoma of Chinese patients are comparable to those reported from Western countries. 13-cis RA appears to be an effective treatment of Ki-1 lymphoma.

Adult↗
PubMed1996

Phenytoin-induced pseudolymphoma: reevaluation using modern molecular biology techniques.

Patients receiving phenytoin (PHT) may develop pseudolymphoma or, rare ly, malignant lymphoma. Previously, distinguishing the two diseases based solely on histopathology has been difficult. The recent introduction of molecular biologic techniques has provided a powerful tool to reassess this problem. A 17-year-old girl developed systemic lymphadenopathy after receiving PHT for 1 year for generalized tonic-clonic seizures (GTCS). Biopsy of a cervical lymph node showed diffuse proliferation of large lymphoid cells mimicking a large cell lymphoma. Immunophenotypic, immunoglobulin gene rearrangement, and cytogenic studies, however, showed polyclonal B-cell proliferation, consistent with PHT-induced pseudolymphoma. After PHT discontinuation, lymphadenopathy resolved in 1 month and no recurrence developed in the subsequent 10 months. Obtaining a history of drug use is crucial to recognizing this group of patients. Molecular biology and chromosome studies have become the definitive basis differentiating pseudolymphoma from malignant lymphoma in patients receiving chronic PHT therapy.

Adolescent↗