Biomedical subjects

H F Tien

Publications and source records attributed to H F Tien.

At least 37 records · Page 2Linked to original sources

PubMed1996

Leptomeningeal seeding with acute hydrocephalus--unusual central nervous system presentation during chemotherapy in Ki-1-positive anaplastic large-cell lymphoma.

An unusual central nervous system (CNS) manifestation in a 18-year-old male with Ki-1- positive anaplastic large-cell lymphoma is presented. The diagnosis of Ki-1 lymphoma was first confirmed by the distinct pleomorphic morphology, expression of Ki-1 (CD30) antigen on neoplastic cells and the specific chromosome translocation, t(2;5)(p23;q35). Although young age is thought to be a good prognostic factor in this disease, the course in our patient was very aggressive. At presentation, there was already extensive extranodal involvement, with malignant cell found in the pleural cavity and bone marrow. In spite of rapid shrinkage of whole-body lymph nodes and a decrease in malignant pleural effusion soon after starting chemotherapy, headaches and vomiting ensued in the following days. A computerized tomography scan of the brain showed poor corticomedullary differentiation without definite mass lesions, and numerous malignant cells were found in the cerebrospinal fluid (CSF). Although intrathecal methotrexate was given, the patient died following the sudden onset of acute hydrocephalus. CNS involvement in Ki-1 anaplastic large-cell lymphoma is very rare and most cases present as focal mass lesions in the brain parenchyma. Leptomeningeal seeding of the lymphoma cells with acute hydrocephalus contributing directly to death has never been reported. The experience from this case suggests that CNS involvement may present in variable forms in Ki-1 lymphoma and may be an important cause of mortality in young patients, especially those in advanced stages of the disease. Early detection of CNS involvement by CSF investigation or even prophylactic CNS therapy may be mandatory in these patients.

Adolescent↗

PubMed1996

Clinical and hematological characteristics of hepatosplenic T gamma/delta lymphoma with isochromosome for long arm of chromosome 7.

Hepatosplenic T gamma/delta lymphoma is a rare entity of peripheral T cell lymphoma. Three of 386 patients with non-Hodgkin's lymphoma in our institute were found to have this subtype of lymphoma. All had chromosomal abnormalities of isochromosome 7q and trisomy 8. The clinical and hematological features of these three patients are reported. All were males with ages ranging from 23 to 29 years. Initial presentation comprised purpura and variable degree of hepatosplenomegaly. None had superficial lymphadenopathy. Hematologically, they showed pictures resembling immune related thrombocytopenia and/or hemolytic anemia. Examination of the bone marrows revealed hypercellularity with increased number of megakaryocytes and erythroid cells and various degrees of abnormal lymphoid cell infiltration. The histopathologic section of the spleen from one patient who underwent splenectomy revealed abnormal cell infiltration in the sinusoids of the red pulp. Lymphoma cells showed T gamma/delta lymphoid immunophenotype (CD3+ CD2+ CD4- CD8-, TCR delta-1+, and beta F1-). The platelet counts were elevated transiently after initial treatment with corticosteroids, but the condition soon deteriorated. All died of refractory lymphoma five to nine months after diagnosis. Review of the literature, showed that only four other cases have been reported until now and although no cytogenetic data were available for these patients, they had very similar clinical pictures as those in this series. It is suggested that hepatosplenic T gamma/delta lymphoma represents a rare, but distinct, clinicopathological and cytogenetic entity.

Adolescent↗

PubMed1996

Putative normal counterparts of leukaemic cells from CD7-positive acute myeloid leukaemia can be demonstrated in human haemopoietic tissues.

CD7-positive acute myeloid leukaemias (CD7+AML) represent a distinct biological and clinical subtype of AML. The results of previous studies suggested that CD7 expression on myeloid leukaemic blasts might result from leukaemic transformation and maturation arrest of haemopoietic precursors at the stage of early myeloid differentiation when CD7 was transiently expressed. However, CD7+ myeloid progenitors have not yet been directly documented in normal human haemopoietic tissues. In this study, haemopoietic cells from 16 human fetal livers with a gestational age of 16-28 weeks were studied. Double myeloperoxidase (MPO) and CD7-positive cells could be demonstrated on 0-4% (mean 1.8%) of total fetal liver mononuclear cells (FLMC) by double cytochemical reaction of MPO and immunocytochemical staining of CD7. Simultaneous expression of CD7 and myeloid antigens (CD13 and/or CD33) could also be detected on 2.2-15.6% (mean 9.3%) of FLMC by dual-colour immunofluorescence flow cytometry analyses. CD13 and/or CD33 positive (CD13/33+) myeloid cells were positively selected by immunomagnetic bead separation system to a purity of 86.5-99.1% (mean 96.0%) of which < or = 3.3% were CD3+ cells and < or = 1.2% were CD19+ cells. Coexpression of CD7 was detected on 8.7-34.5% (mean 17.3%) of this CD 13/33+ cell population, but it was induced to decrease significantly after short-term in vitro culture with the differentiation-inducing agent phorbol ester (TPA). Coexpression of CD7 and CD13/33 could also be shown on a minor population of adult bone marrow and cord blood mononuclear cells (mean 3.9% and 1% respectively). In conclusion, the normal putative counterparts of blasts from CD7+ AML could be demonstrated in human haemopoietic tissues. The fact that CD7 expression tended to be lost after TPA stimulation suggested that CD7 was transiently expressed in early myeloid differentiation.

Acute Disease↗

PubMed1995

Correlation of cytogenetic results with immunophenotype, genotype, clinical features, and ras mutation in acute myeloid leukemia. A study of 235 Chinese patients in Taiwan.

Of 235 consecutive patients with de novo acute myeloid leukemia (AML), clonal chromosomal abnormalities were detected in 151 (64%) of them. Twenty-four of the 71 patients with M2 AML had t(8;21), 35 of the 36 M3 patients had t(15;17), and 11 of the 45 M4 leukemia disclosed inv(16). Six of the eight patients with 11q23 abnormality had M4 or M5 subtype of leukemia. The incidence of t(15;17) and t(8;21) was higher in our patients than in patients from most Western countries. Immunophenotyping was performed on 197 patients. Patients with t(15;17) were associated with negativity to HLA-DR, CD11b, and CD34. Patients with t(8;21) expressed CD13 and CD33 less frequently than other patients, but all showed CD15 positivity. Coexpression of lymphoid-associated antigens on the leukemic blasts was detected in 52 patients (26%), including all 7 patients with t(9;22), 3 of the 8 patients with t/del(11)(q23), 2 of the 25 patients with t(15;17), and 2 of the 22 patients with t(8;21). Seven (35%) of the 20 patients coexpressing lymphoid markers showed immunoglobulin heavy chain or T-cell receptor beta-chain gene rearrangements, while only 2 (4%) of the 53 patients without lymphoid antigen expression did so. Patients with inv(16), t(8;21), and t(15;17) had a better prognosis than other patients. Of all surface antigens tested, only CD15, CD11b, and HLA-DR were of prognostic value: CD15 with a higher complete remission (CR) rate and CD11b or HLA-DR with a shorter CR duration. N-ras mutations were detected in 7 (18%) of the 40 patients in the study, including two of the three patients with inv(16). This study demonstrated differences in clinical features, immunophenotypes, and genotypes among different cytogenetic subgroups.

Adolescent↗

PubMed1995

Consistent presence of isochromosome 7q in hepatosplenic T gamma/delta lymphoma: a new cytogenetic-clinicopathologic entity.

Peripheral T-cell lymphoma (PTL), which is characterized by hepatosplenic presentation and the gamma/delta T-cell receptor (TCR) phenotype on the malignant cells, is a rare but distinct subtype of non-Hodgkin's lymphomas. Little is known about the chromosomal changes in these lymphomas. We report the cytogenetic analysis of three patients who had neoplastic proliferation of T gamma/delta cells in the spleen, bone marrow, and liver, but not in lymph nodes or skin. Isochromosome 7q and trisomy 8 were observed in all three patients. Isochromosome 7q as the sole abnormality has been previously reported in one patient with similar clinicopathologic features. It is suggested that i(7q) is a primary, nonrandom chromosomal abnormality in hepatosplenic T gamma/delta PTL.

Adult↗

PubMed1995

Detection of dural involvement by magnetic resonance imaging in adult patients with acute leukemias--preliminary experience.

Retrospectively, the dura in 18 adult patients with acute leukemia were evaluated by gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI). Abnormal dural enhancements were detected in three of four patients with positive cerebrospinal fluid (CSF) cytology, in one of five with suspicious central nervous system (CNS) disease, and in two of nine asymptomatic patients. Computed tomography failed to demonstrate any dural abnormality in these six patients. The abnormal dural enhancement was found in either (a) the brain and the spine, (b) the thoracolumbar spine, or (c) the area adjacent to the parenchymal lesions. Three of the patients were in hematological remission stage; disappearance of the abnormal dural enhancement was observed 1-2 months after radiotherapy and high-dose systemic chemotherapy. The results suggest that MRI is a sensitive and noninvasive imaging modality and superior to CT in detecting dural disease in leukemic patients.

Adolescent↗

PubMed1995

Acute leukemic transformation of myelodysplastic syndrome--immunophenotypic, genotypic, and cytogenetic studies.

The clinical and biological characteristics of myelodysplastic syndrome (MDS) in acute leukemic transformation were studied in 23 patients. All had myeloid transformation according to FAB criteria, but coexpression of lymphoid-associated antigens was detected in five of the 20 patients who underwent an immunophenotypic study. Rearrangement of the immunoglobulin heavy chain gene was also observed in one of the five patients who coexpressed lymphoid markers and that of the T-cell receptor beta chain gene in another one. None had pure lymphoid transformation. Clonal chromosomal abnormalities were noted in 12 (63%) of the 19 patients who underwent cytogenetic study, most commonly - 7 (six patients or 32%). In the 18 patients who underwent serial analyses both at MDS diagnosis and at acute transformation, seven (39%) underwent karyotypic evolution. The most common new or additional aberrations were +8 and +21. N-ras gene mutation was detected in two of the nine patients at acute leukemic transformation. The median interval from diagnosis of MDS to onset of acute transformation was 10 months (1-36 months). Patients with a normal karyotype at diagnosis had a significantly longer chronic phase duration than those with chromosomal abnormalities (median of 20 months vs. 5 months). However, all had a short survival time after diagnosis of acute leukemia, whether chromosomal anomalies were present or not.

Adolescent↗

PubMed1995

Hairy cell leukemia variant.

A 59-year-old man presented with lymphocytosis with huge splenomegaly. The abnormal lymphocytes had a high nucleoplasm:cytoplasm ratio, a prominent nucleolus and hairy cytoplasmic projections. Immunophenotyping revealed B-cell leukemia with negative reactions to CD5 and CD25. Cytogenetic study showed 46,XY,der(5)t(5;6)(q35;p21), del(7)(p13)/46,idem,add(22)(q13). The patient did not respond to chlorambucil and a combination of cyclophosphamide, vincristine and prednisolone. Splenic irradiation induced partial remission. He developed progressive anemia and thrombocytopenia and died of Escherichia coli septicemia 33 months after the diagnosis of hairy cell leukemia variant.

Fatal Outcome↗

PubMed1995

Trichosporon beigelii fungemia in patients with acute leukemia: report of three cases.

Trichosporon beigelii fungemia with localized visceral involvement or dissemination is a life-threatening infection in patients with granulocytopenia. We report three cases of T. beigelii fungemia in patients with acute leukemia seen at the National Taiwan University Hospital. Two patients were in the granulocytopenic state after cytoreductive chemotherapy, while one was in complete remission. Fever persisted or recurred despite conventional antibiotic treatment when fungemia developed. Cutaneous trichosporonosis was noted in one patient, which was misdiagnosed as candidiasis. An indwelling catheter constituted the likely portal of entry in two patients. Therapeutic response to amphotericin B is generally not achieved in patients without recovery of neutrophil counts and in those with retained catheters.

Adolescent↗

PubMed1995

Richter's syndrome: report of a case.

A 42-year-old male presented with generalized massive lymphadenopathy, fever, weight loss and numerous cutaneous nodules. Peripheral blood examination showed lymphocytosis with small lymphocytes, and immunophenotyping revealed B-cell chronic lymphocytic leukemia (CLL). Cytogenetic analysis of bone marrow aspirate revealed a clonal abnormality of chromosome 11. Lymph node biopsies showed a B-immunoblastic lymphoma. A diagnosis of Richter's syndrome (RS) was made. The patient did not respond to doxorubicin, cyclophosphamide, vincristine and prednisolone (CHOP) and died of progressive disease with pneumonia and respiratory failure 9 months later. CLL is not common among Chinese people and RS is extremely rare.

Adult↗

PubMed1995

Severe aplastic anemia induced by ticlopidine: report of a case.

Ticlopidine is a powerful antiplatelet activator that inhibits adenosine diphosphate (ADP)-induced platelet aggregation. Its most common side-effects are skin rashes, diarrhea and neutropenia. Aplastic anemia is rare. This paper reports a patient with severe aplastic anemia that developed after the use of ticlopidine. The 85-year-old woman developed fever, chills and chest pain 5 weeks after starting ticlopidine 250 mg twice daily. Severe aplastic anemia was proved by blood examination, bone marrow aspiration and biopsy. In spite of the recovery of absolute neutrophil count to more than 1,000/mm3, 16 days after ticlopidine was stopped and administration of strong antibiotics, the patient died from candidal sepsis.

Aged↗

PubMed1994

Characteristic clinicopathologic features of adult B-cell lymphoblastic lymphoma with special emphasis on differential diagnosis with an atypical form probably of blastic lymphocytic lymphoma of intermediate differentiation origin.

BACKGROUND: Lymphoblastic lymphoma is typically of thymic T-cell phenotype. Lymphoblastic lymphoma of B-cell origin (B-lymphoblastic lymphoma) has been relatively poorly described. Whether B-lymphoblastic lymphoma should be managed like its T-cell counterpart remains to be clarified. METHODS: From 1983 to 1991, 10 adult patients were diagnosed as having B-lymphoblastic lymphoma at National Taiwan University Hospital by using the histomorphologic criteria of international working formulation. B-cell phenotype was determined by the immunohistochemistry method. Clinicopathologic features of these 10 patients were reviewed. RESULTS: Seven patients were grouped as typical type and were characterized by an aggressive clinical course with lymph node (7 of 7), bone marrow (6 of 7), liver (3 of 7), spleen (3 of 7), and central nervous system (2 of 7) involvement. The median survival time was 8 months. In contrast, three patients had an atypical clinical picture. They were older patients (64-73 years) and were characterized by a relatively less aggressive course with predominantly bulky nodal involvement. Two of these three patients are alive (31 and 49 months, respectively) and well at this report, with one of them being repeatedly experiencing disease remission with the use of simple salvage chemotherapeutic regimens. Further studies revealed that tumor tissues of these three atypical cases had strong expression of CD5 (Leu-1) marker. CONCLUSION: B-lymphoblastic lymphoma diagnosed by histomorphologic criteria should be further distinguished from a relatively favorable subtype, which probably represents a variant of blastic lymphocytic lymphoma of intermediate differentiation as described by Lardelli et al. Clinical features of typical B-lymphoblastic lymphoma, except for the lack of mediastinal involvement, is similar to its T-cell counterpart.

Adolescent↗

PubMed1994

Peripheral T-cell lymphoma in childhood: a report of five cases in Taiwan.

We encountered five children with peripheral T-cell lymphoma (PTL) at National Taiwan University Hospital (NTUH) from 1985-1989. The patients were four boys and one girl, aged between 5 and 13 years. The duration of prediagnostic symptoms varied from 1 month to 5 years. All had pyrexia and lymphadenopathy; one had a prolonged history of granulomatosis with repeated infection. Four had hepatosplenomegaly. One patient presented with diffuse pulmonary infiltration and impending respiratory failure. All patients were negative for human T-cell leukemia virus (HTLV)-I antibody, and positive for HBsAg. Four patients who had EBV-viral capsid antigen (VCA) IgG and who were IgM tested were positive for EBV-VCA IgG, but only two had evidence of active EBV infection. Tumor cell markers were examined and showed the following phenotypes: all patients were CD2, CD3, and CD7 positive but CD19 and CD20 negative; three patients were CD4 positive and CD8 negative; the other two patients were CD4 negative and CD8 positive. Four patients died 2-7 months after diagnosis. The remaining patient received allogeneic bone marrow transplantation and has survived free of disease for more than 22 months after transplant. Our five cases reconfirm the high frequency of diagnostic delay, the heterogenous immunophenotypes, high mortality, and poor responsiveness to conventional therapy for PTL. Bone marrow transplantation in the early stage might be a possible cure of this disease.

Adolescent↗

PubMed1994

Cytogenetic studies, ras mutation, and clinical characteristics in primary myelodysplastic syndrome. A study on 68 Chinese patients in Taiwan.

Cytogenetics and clinical features were studied for 68 Chinese patients with primary myelodysplastic syndrome (MDS). Ras mutation was analyzed in 25 of them. Thirty-four patients (50%) had clonal chromosomal abnormalities at initial analysis. The most common cytogenetic aberrations were -7, +8, 5q-, and 20q-, which occurred in 11 (16.2%), seven (10.3%), five (7.4%) and three patients, respectively. The incidence of -7 was higher and that of 5q- lower in our patients than in patients from most other geographic areas. The 17 patients with multiple chromosomal abnormalities had a significantly shorter median survival (9 months) than the 34 patients with normal karyotype (33 months) and the 17 patients with patients with single anomalies (26 months). Evolution to acute leukemia occurred in 20 patients (29%) after a median interval of 8 months following the diagnosis. Patients with multiple cytogenetic changes at initial analysis or in subsequent studies had a significantly higher frequency of acute transformation than others (55% vs. 18.6%, p = 0.007); the same was not true if only the data of initial study were considered. Serial cytogenetic studies are important in patient follow-up. N-ras mutation was detected in 5 (20%) of 25 patients within the study. There was no correlation between the gene mutation and acute transformation. But combing the data of N-ras mutation and cytogenetics, patients with either the N-ras mutation or clonal chromosomal abnormalities were at significantly higher risk for developing acute leukemia than those with neither of the changes (77% vs. 25%).

Adolescent↗

PubMed1994

Epstein-Barr virus-associated lymphoproliferative disorder of granular lymphocytes presenting initially as cutaneous vasculitis.

Lymphoproliferative disorders of granular lymphocytes (LDGL) represent a family of diseases characterized by persistent granular lymphocytosis with variable prognosis. The Epstein-Barr virus (EBV) has been occasionally linked with the development of LDGL. However, cutaneous manifestations of LDGL have rarely been reported. One patient had cutaneous vasculitis for 10 years before a definite diagnosis of LDGL was made. Chronic EBV infection was documented serologically and EBV DNA was detected in the peripheral blood lymphocytes. EBV RNA was detected in the nuclei of infiltrating lymphoid cells expressing CD43 in a skin biopsy specimen. A cytogenetic study showed clonal chromosomal abnormalities. This is the first report of EBV-associated LDGL of natural killer cells with cutaneous manifestations.

Child↗

PubMed1994

Clinicopathological spectrum of haemophagocytic syndrome in Epstein-Barr virus-associated peripheral T-cell lymphoma.

Haemophagocytic syndrome (HS) is frequently observed in Epstein-Barr virus-associated peripheral T-cell lymphoma (EBV-PTCL) and represents a major cause of death. In this communication we have further analysed the spectrum of HS in 12 patients with EBV-PTCL. The patients could be divided into three groups according to the time of onset of HS during the clinical course of PTCL. Group I patients (four cases) had HS as the initial clinical manifestation. All four patients were initially suspected to have malignant histiocytosis (MH) but a MH-like PTCL was later diagnosed. Group II patients (six cases) developed HS at the time of lymphoma relapse. Four of them belonged to the angioinvasive type PTCL. Group III patients (two cases) developed HS at clinical remission; both were angioinvasive type PTCL. Nine patients had serological evidence suggesting active EBV infection. The clinical course after the onset of HS was generally fulminant in each group with a median survival of only 44d despite combination chemotherapy and/or empirical therapy with high-dose immunoglobulin and corticosteroids in six patients. In conclusion, HS represents a severe complication of EBV-PTCL. Although most patients develop HS at a time of active lymphoma, the syndrome may occur when the lymphoma is in remission. Because of the poor outcome, early diagnosis and a new modality of treatment for HS associated with EBV-PTCL should be pursued in future.

Adult↗

PubMed1994

Use of retinoic acids in the treatment of peripheral T-cell lymphoma: a pilot study.

PURPOSE: We have systemically analyzed, both in vitro and in vivo, the effect of 13-cis-retinoic acids (RA) on non-Hodgkin's lymphoma (NHL). METHODS: The in vitro growth-inhibitory effect of 13-cis-RA was examined in 11 (T cell, five; B cell, six) lymphoma cell lines by a tetrazolium colorimetric assay. A pilot clinical trial with oral 13-cis-RA 1 mg/kg/d was conducted in a selected group of 18 lymphoma patients, of whom 16 had failed to respond to at least one regimen of intensive chemotherapy. The in vitro and in vivo effects of 13-cis-RA were correlated with immunophenotypes, RA-induced changes of morphology, and patterns of DNA fragmentation of the lymphoma cells. RESULTS: Four of five T-lymphoma cell lines and none of six B-lymphoma cell lines were sensitive (concentration of 50% growth inhibition [IC50] < 1.5 microns) to 13-cis-RA (P = .015). In the clinical trial, five (two Ki-1, one angioinvasive type, one diffuse mixed cell, and one diffuse large cell) complete remissions and one (Ki1) partial remission were observed in 12 patients with peripheral T-cell lymphoma (PTCL), while none of six patients with B-cell lymphoma responded to 13-cis-RA. 13-cis-RA-induced cellular differentiation and apoptosis, as evidenced by the more mature morphology, characteristic nuclear condensation, and DNA ladder pattern signifying internucleosomal fragmentation, were demonstrated in the sensitive cell lines, as well as in the remitting lymphoma tissues. CONCLUSION: The 13-cis-RA appears to be active on lymphomas of T-lineage and their therapeutic indication may be extended to include some subtypes of PTCL. The mechanisms of action are related to differentiation and apoptosis of lymphoma cells. There appears to be no cross-resistance between 13-cis-RA and conventional chemotherapy.

Adult↗

PubMed1993

Lymphoblast colony-culture assay in acute lymphoblastic leukemia: a quantitative approach.

Lymphoblast colony-culture of adult acute lymphoblastic leukemia (ALL) was studied to explore its clinical implication. Among 13 marrow cultures from ALL patients with full-blown disease, 11 developed leukemic colonies. A type of colony, very similar to a lymphoblastic colony and possibly T-cell in origin, could be found in four cultures of the six control marrows. To minimize the difficulty in differentiating a leukemic blast colony and a normal lymphocyte colony, based solely on morphology, a quantitative approach was used. Since both the mean of blast colony count and the mean of blast percentage of leukemic marrow were significantly higher than those of the control group, mean value plus two standard deviations of the control group were defined arbitrarily as upper normal limits. The defined normal range was then used to examine the relationship between results of the cultures and clinical outcome for the ALL patients. Early relapse or incomplete remission following chemotherapy could be predicted in four patients by these quantitative colony-culture assays 0.5-2 months before full-blown disease. The low colony count and low blast percentage in the colony-culture assay of the fifth patient is compatible with the clinical observation of continuous remission. One culture, growing clusters only, had an increased blast percentage; this correlated well with cytogenetic relapse two months later. In summary, the quantitative colony-culture assay could detect morphologically unidentifiable leukemic cells in ALL patients with early relapse or incomplete remission. This quantitative colony-culture system, though not ultrasensitive in the detection of minimal residual leukemic cells, was of potential value as a prognostic assay.

Adolescent↗