Biomedical subjects

L Y Shih

Publications and source records attributed to L Y Shih.

At least 37 records · Page 2Linked to original sources

PubMed1998

End-stage renal disease following polycythemia vera: in vitro and in vivo response of erythroid progenitors to erythropoietin and effects of sera on normal erythropoiesis.

We investigated the in vitro erythroid progenitor growth and the effects of sera on normal-marrow CFU-E (colony-forming units - erythroid) growth in 2 patients with renal failure on regular hemodialysis following a prior history of polycythemia vera (PV). PV was diagnosed 3 and 11 years, respectively, before the development of terminal renal failure. One of the patients had entered a spent phase of PV as characterized by diffuse extensive myelofibrosis and anemia; the other also had mild myelofibrosis. The serum erythropoietin (EPO) levels were low or normal on serial measurements by radioimmunoassay. There was no correlation between the hematocrit values and serum EPO levels. EPO-independent erythroid colonies were present in the cultures of bone marrow and peripheral blood cells from both patients after renal failure in the anemic state. With the addition of various concentrations of EPO, the number of erythroid colonies increased as the concentrations of EPO increased which was in accordance with the clinical observation that 1 patient with postpolycythemic myeloid metaplasia partially responded to recombinant human EPO therapy. In the EPO-dependent CFU-E assay, normal-marrow CFU-E numbers supported by 10% of the patient sera were less than those by normal sera. In the absence of EPO in cultures, no erythropoietic activity was found in the patients' sera. Our study on uremic patients with underlying PV showed that the biologic characteristics of autonomous erythroid progenitor growth for PV persisted during the spent phase and after the development of terminal renal failure with anemia. The erythroid progenitors responded to EPO both in vitro and in vivo. Their sera exhibited an inhibiting effect on the growth of normal-marrow erythroid progenitors.

Adult↗

PubMed1998

Granulocytic sarcoma presenting as a giant breast tumor in a pregnant woman: a case report.

We report a case of granulocytic sarcoma (chloroma) presenting as a giant breast tumor in a pregnant woman with no history of leukemia. The case was initially diagnosed as medullary carcinoma on a biopsy specimen and a modified radical mastectomy was performed. The diagnosis of granulocytic sarcoma requires the pathologist's high index of suspicion. The presence of immature eosinophils was an important clue. Leder's chloroacetate esterase stain; immunostaining for myeloperoxidase, CD34, CD43, CD68, and lysozyme; and ultrastructural finding of cytoplasmic lysosomal granules and Auer bodies all aided in confirming the diagnosis. It is imperative to recognize granulocytic sarcoma to avoid unnecessary surgery. Granulocytic sarcoma should be included in the differential diagnosis of breast tumors, especially in tumors with diffuse proliferation of small tumor cells.

Adult↗

PubMed1997

Effect of cisplatin chemotherapy on extracortical tissue formation in canine diaphyseal segmental replacement.

The reconstruction of large bone and joint defects after the resection of malignant tumors remains a major challenge. Chemotherapy has significantly lowered the risk of metastasic disease, but complications associated with reconstructive techniques continue to result in late morbidity. In the present study, biomechanical torsion testing, gait analysis, and histomorphometric and scanning electron microscopic evaluations of 24 dogs were used to examine the effects of preoperative and postoperative administration of cisplatin on the biologic fixation of a porous-coated segmental replacement prosthesis. The chemotherapy consisted of four cycles of cisplatin administered at a dosage of 75 mg/m2 preoperatively or postoperatively. The healing was enhanced by use of an autogenous corticocancellous bone graft. The graft was placed evenly around the prosthesis and the adjacent femoral cortex. Mechanical analyses of torsional stiffness, yield strength, and maximum strength revealed no statistically significant differences between the groups at 12 weeks. Such lack of difference was mainly due to the penetration of highly organized fibrous tissue into the porous surface; this provided strong fixation of the implant to bone even in the absence of bone ingrowth. Although bone ingrowth into the prostheses was not affected, electron microscopic, histomorphometric, and radiologic analyses showed a clear difference in the formation of new bone around the prosthesis. Preoperative chemotherapy did not alter the formation of new bone, but specimens from animals treated postoperatively with cisplatin showed significantly less bone graft resorption and less new bone formation. Hence, the effect of cisplatin administration caused only a temporary delay, not a permanent effect, on extracortical capsule formation. The formation of extracortical bone and soft tissue might prevent debris-incised osteolysis and, therefore, prevent late complications by forming a tight capsule around the bone-prosthetic interface.

Animals↗

PubMed1997

Prediction of clinical course in patients with idiopathic erythrocytosis by endogenous erythroid colony assay but not by serum erythropoietin levels.

We studied the in vitro culture growth of bone marrow and blood erythroid progenitors and serum erythropoietin (EPO) levels by radioimmunoassay in 24 patients with idiopathic erythrocytosis (IE). All patients had an increased red blood cell (RBC) mass and lacked a cause of secondary polycythemia, but did not fulfill the diagnostic criteria of polycythemia vera (PV). Marrow and blood cultures were obtained simultaneously; the results of endogenous (EPO-independent) erythroid colony (EEC) growth were parallel in both cultures. EECs were present in five patients, all of them developed PV 3 to 48 months later. The EEC number did not correlate with the time to the progression of PV. In contrast, none of the 19 EEC-negative patients had PV evolution during a median follow-up period of 38 months. Three of the five IE patients in whom EECs formed displayed vascular complications during their clinical course compared with three of 19 patients who did not have EEC. The serum EPO levels were variable: low in five, normal in 14, and high in five patients. Serial measurements of serum EPO levels in three of five patients who had high initial levels showed persistently elevated values; the underlying cause of the increased EPO production could not be defined during a follow-up period of more than 36 months. Of the five patients who subsequently developed PV, two had low serum EPO levels and three had normal values at initial evaluation. Serum EPO levels did not correlate with the occurrence of thrombotic complications. Our results show that serum EPO levels have limited value in determining the underlying cause of IE and cannot predict the clinical course of patients with IE, whereas the assessment of EEC in bone marrow or blood can identify IE patients who will have PV evolution.

Adult↗

PubMed1997

The double-blind test of sodium hyaluronate (ARTZ) on osteoarthritis knee.

BACKGROUND: At this time, no definite treatment exists for osteoarthritis disease. Hyaluronate (ARTZ) is one of the most important components of synovial fluid. It is generally accepted that hyaluronic acid protects the articular cartilage and soft tissue surfaces from trauma during joint function. METHODS: Ninety patients with 116 knees diagnosed as early arthritis (mild to moderate) by four senior orthopaedic surgeons were selected to join this study. The trial design was applied with a double-blind model. The selected patients were randomly injected with 2.5 ml drugs (ARTZ or placebo) intra-articularly once a week for five consecutive weeks without the use of local anesthetic drugs. Evaluation results included grading of subjective and objective symptoms and daily activities. The follow-up period was up to six months after initial injection. RESULTS: According to the results of clinical evaluation and statistical analysis, SPH (ARTZ) is quite effective for osteoarthritis knees, and significantly better than the placebo. The effective peak was one week after five injections of ARTZ. The effective period could last up to three months without additional treatment. The efficacy of ARTZ on osteoarthritis knees was more prominent for relief of motion pain and improvement of knee movement. No side effects developed during a six month period. CONCLUSIONS: Based on clinical results here, SPH is a safe drug for administration as an alternative approach to treat the osteoarthritis knee.

Activities of Daily Living↗

PubMed1996

Enhanced vesiculation exacerbates complement-dependent hemolysis in glucose-6-phosphate dehydrogenase deficient red blood cells.

Glucose-6-phosphate dehydrogenase (G6PD) deficient red blood cells (RBCs) are known to be more susceptible to oxidant-induced hemolysis. Erythrocytes from G6PD-deficient individuals are significantly more susceptible to Ca(2+)-induced vesiculation than normal control cells. The enhanced susceptibility of G6PD-deficient RBCs to Ca(2+)-induced vesiculation is not due to ATP depletion. The remnant G6PD-deficient RBCs following vesiculation are more sensitive to complement-mediated hemolysis than control normal RBCs. A strong positive correlation exists between the level of Ca(2+)-induced vesiculation and the extent of complement mediated hemolysis.

Calcium↗

PubMed1996

High incidence of TEL/AML1 fusion resulting from a cryptic t(12;21) in childhood B-lineage acute lymphoblastic leukemia in Taiwan.

Despite its rarity by routine karyotypic analysis, cryptic t(12;21)(p12-13;q22) translocation leading to TEL/AML1 fusion has been recognized as the most frequent genetic rearrangement in childhood acute lymphoblastic leukemia (ALL) in two recent studies, one from France and the other from the United States. To estimate the frequency of this abnormality in the Chinese population, we studied 41 children with ALL and 17 with acute myeloid leukemia (AML) in two medical centers in Taiwan, using the reverse transcriptase polymerase chain reaction (RT-PCR) assay. Results of this analysis demonstrated a 17% frequency of this translocation in the ALL population overall and 19% in patients with B-lineage ALL, similar to previous findings in Caucasian children. None of the patients with AML had TEL/AML1 fusion transcripts. In addition to its association with the B-lineage immunophenotype, TEL/AML1 was also correlated with a low presenting leukocyte count and favorable age (1-10 years). These findings, combined with earlier reports, indicate that TEL/AML1 fusion is the most frequent genetic abnormality in childhood ALL, regardless of race. Molecular diagnosis of t(12;21)-positive ALL may identify a subgroup of patients who do not require intensive treatment for cure.

Adolescent↗

PubMed1996

Lack of TEL-AML1 fusion transcript resulting from a cryptic t(12;21) in adult B lineage acute lymphoblastic leukemia in Taiwan.

Cryptic t(12;21)(p12-13;q22) leading to TEL-AML1 fusion has recently been recognized as the most frequent genetic rearrangement in childhood acute lymphoblastic leukemia (ALL) in Western countries. More recently, we found a similar frequency of this abnormality in Chinese children with ALL in Taiwan. In this study, we assessed further the frequency of TEL-AML1 fusion as well as that of BCR-ABL in Chinese adults with ALL, using reverse transcriptase-polymerase chain reaction assays. Among the 81 cases with newly diagnosed B lineage ALL studied, none had the TEL-AML1 fusion whereas 30 had the BCR-ABL fusion. The lack of cases with the TEL-AML1 fusion together with the high frequency of BCR-ABL fusion could largely account for the poorer outcome of adult ALL as compared with childhood ALL.

Adolescent↗

PubMed1995

Spinocerebellar degeneration and cerebral hypomyelination in a family.

The proband is a 24-year-old woman who developed symptoms of a spinocerebellar degeneration in early childhood. Neurological examination revealed normal cognitive function, optic atrophy, dysarthria, titubation, action tremors, increased deep tendon reflexes, Babinski's signs, and a spastic scissoring gait. The magnetic resonance imaging (MRI) showed an abnormal increased signal on long TR images involving white matter throughout the cerebral hemispheres, most striking in the subcortical white matter, and to a lesser degree in the brainstem, compatible with diffuse hypomyelinating or dysmyelinating diseases. Metabolic and chromosomal studies were normal. Her 49-year-old mother developed similar symptoms in her 20s and is now wheelchair-bound. Findings on neurological examination and MRI were similar to her daughter but more severe. The proband's maternal grandfather had a female cousin who had a neurological illness beginning in her 20s with similar symptoms and signs and died at the age of 44 years. Spinocerebellar degenerations are a group of syndromes with similar clinical manifestations but heterogeneous etiology. We report a family with spinocerebellar degeneration with distinct MRI findings compatible with hypomyelination or dysmyelination which has not heretofore been described. This family may represent a new spinocerebellar syndrome due to an abnormality of as yet an undetermined gene.

Adult↗

PubMed1995

Primary plasma cell leukemia with extensive dense osteosclerosis: complete remission following combination chemotherapy.

Diffuse osteosclerotic myeloma is very rare, and primary plasma cell leukemia with extensive osteosclerosis is even more rare. We describe a 71-year-old man who presented with severe anemia and dense widespread osteosclerosis similar to the X-ray finding of myelosclerosis. His peripheral blood showed 40% plasma cells. Bone marrow examination revealed heavy plasma cell infiltration with marked myelofibrosis and myelosclerosis. Protein electrophoresis and immunoelectrophoresis demonstrated an M-protein of IgG-lambda type. He was treated with cyclophosphamide, vincristine, and prednisolone for 10 months. A complete remission was obtained, with disappearance of M-protein and circulating plasma cells and normalization of complete blood counts, bone marrow picture, and biochemical parameters, as well as complete regression of myelofibrosis and osteosclerotic lesions. Unmaintained complete remission lasted for more than 1 year and he survived for more than 22 months. Our case indicated that one must include in the differential diagnosis of an osteosclerotic lesion the possibility of multiple myeloma, and that combination chemotherapy can induce a complete remission in this disease.

Aged↗

PubMed1995

Localised plasmacytomas in Taiwan: comparison between extramedullary plasmacytoma and solitary plasmacytoma of bone.

The clinical features and response to therapy of 32 Chinese patients with localised plasmacytoma are presented, and a comparison between extramedullary plasmacytoma (EMP) and solitary plasmacytoma of bone (SPB) is made. Twenty-two patients had SPB and ten had EMP, accounting for 9% of all of our plasma cell neoplasms. Both groups had a male predominance with a median age of 54 years for SPB and 63 years for EMP. The common sites of SPB included vertebral bodies (15) and the skull (4). Most EMPs occurred in the oronasopharynx (6) and paranasal sinuses (2). An M-protein was detected in eight patients with SPB and in six with EMP. Seventeen patients with SPB and seven with EMP received radiation therapy, and all achieved initial local control. The pattern of failure in 22 patients with SPB manifested as local recurrence in two, multiple bone metastases without bone marrow plasmacytosis in two, multiple EMP progression in two, and development of multiple myeloma (MM) in one. There were two local recurrences, one further solitary bone involvement and one MM conversion in the EMP group. Local recurrence or dissemination was associated with the appearance of M-protein or an increase in the M-protein level in both groups. There was no significant difference in M-protein status or incidence and patterns of failure between the two groups. Patients with EMP had a more favourable overall survival than those with SPB (P = 0.03). The 5 year disease-free survival rate was 79% for EMP and 58% for SPB (P = 0.53). Patients aged less than 60 years had a better overall survival in the SPB group, but location of tumour, presence of M-protein, radiation dose and chemotherapy did not influence prognosis in either group. Our results indicate that adequate local therapy can result in long-term survival with a low frequency of MM progression for patients with localised plasmacytomas, and both EMP and SPB appear to be similar in terms of frequency and patterns of failure.

Adult↗

PubMed1995

Microangiopathic hemolytic anemia as an initial presentation of metastatic cancer of unknown primary origin.

Microangiopathic hemolytic anemia (MAHA) is a well-documented but rare complication of disseminated cancer; it usually occurs in the late or terminal stage of cancer. We describe a case of metastatic carcinoma of unknown origin in which MAHA was the initial presentation. A 36-year-old woman came to our hospital with lower back pain and progressive exertional dyspnea for 8 weeks. Hemolytic anemia, thrombocytopenia, and leukoerythroblastosis were found on admission. The peripheral blood smear revealed polychromasia, poikilocytosis, and many schistocytes. Bone marrow biopsy disclosed metastatic carcinoma. After careful workup, we failed to find the primary site of cancer. The anemia and thrombocytopenia responded dramatically to combination chemotherapy with 5-fluorouracil, mitomycin C, and cisplatin. This case indicates that metastatic carcinoma should be included in the differential diagnosis in previously healthy patients with MAHA.

Adenocarcinoma↗

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

Plasmacytoid lymphocytic lymphoma presenting with IgG hyperviscosity syndrome and peritoneal lymphomatosis.

We reported a 74-year-old woman with plasmacytoid lymphocytic lymphoma who initially presented with exertional dyspnea, conscious disturbance, ascites, and lytic skull lesions. Protein electrophoresis and immunoelectrophoresis showed monoclonal IgG-lambda gammopathy with IgG level of 13300 mg/dl and marked suppression of the residual normal immunoglobulins. Abdominal computed tomography (CT) revealed a pattern mimicked cancerous peritonitis. She responded to plasmapheresis with much clinical improvement of the hyperviscosity syndrome but died of H. influenza sepsis 3 weeks after diagnosis. The unusual and interesting features of this case included: (1) IgG hyperviscosity syndrome and diffuse peritoneal involvement as the initial manifestations in plasmacytoid lymphocytic lymphoma, and (2) presence of lytic bone lesions in conjunction with high level of M--component and marked suppression of normal residual immunoglobulins in a patient with lymphoma rather than multiple myeloma.

Aged↗

PubMed1995

Total hip arthroplasty in patients with ankylosing spondylitis: longterm followup.

OBJECTIVE: To evaluate the longterm functional effects of total hip arthroplasty (THA) on 46 patients. METHODS: Clinical and radiographic examinations were performed on 46 patients (74 THA) with ankylosing spondylitis (AS). The mean followup period was 100 months (range 37-174 months); 32 hips were followed for more than 10 years. RESULTS: Significant benefit was obtained in pain control (all but 2) and function (mean improvement in range of motion 128 degrees) in the early stage so that patients could be gainfully employed. An average 100 months after THA, the overall functional results were rated excellent in 21 hips, good in 28, fair in 7, and poor in 18. Only 6 hips (8%) in our series developed clinically significant (Class III or IV) heterotopic ossification. Seventeen hips needed a 2nd operation because of loosening (11 hips), deep infection (3), malposition of acetabular component (2), and prosthetic failure (1). Another 6 hips showed definite loosening on radiographs and needed to be revised. The total incidence of failure was 31% (23 hips). The average time from the index operation to loosening was 9.5 years (range 4-13 years). Factors contributing to component loosening were young age, short stature, and decreased postoperative range of motion. CONCLUSION: Total hip arthroplasty can be very important and beneficial to patients with AS, but the patients, being young and active, and with their rigid spines, do not treat their prostheses gently and are very dependent upon their mobility. They must be kept under supervision long after THA, probably for the rest of their lives, to identify possible longterm complications.

Adult↗

PubMed1995

Nonvascularized proximal fibular autograft to treat giant-cell tumor of the distal radius.

BACKGROUND: The Distal end of the radius is the third most common site where giant-cell tumors occur. High recurrent rate was reported after curettage. To manage such aggressive tumors, wide resection is the best treatment and nonvascularized proximal fibular autograft has been widely used for reconstruction. METHODS: Eight patients were treated here with this method from April, 1988 to April, 1992. Patients' average age was 40 years, and all had grade III. Of the four males and females, six tumors were over the dominant hands and the other two, over the nondominant ones. They were treated primarily with wide resection and reconstruction with osteochondral proximal fibular autograft, from either the ipsilateral or contralateral side. Bone anastomotic site was fixed with dynamic compression plate. The soft tissue was directly repaired in six patients and reinforced with extensor carpi radialis longus tendon in two. RESULTS: Patients were regularly followed from 14-60 months, for an average of 25 months. The average grip strength of the affected hands was 77% that of the unaffected sides. There were four distal radioulnar joint diastasis, one ulnar deviation of the wrist, one volar tilt of the carpal bones and one transient peroneal palsy over the donor site. No recurrence or metastasis was found up to the last follow-up. The average total range of motion of the affected wrist (supination, pronation, dorsiflexion and palmar flexion) was 185 degrees. All Patients were satisfied with the results. CONCLUSIONS: Using proximal fibular autograft to reconstruct the bony defect and joint after wide excision for distal radial giant-cell tumor gives satisfactory results. It is a widely accepted good method, though there are many problems still unresolved.

Adult↗

PubMed1994

Identification of masked polycythemia vera from patients with idiopathic marked thrombocytosis by endogenous erythroid colony assay.

We used the methylcellulose-culture technique to determine the utility of the erythroid progenitor growth in vitro from nonadherent T-depleted bone marrow and peripheral blood cells in distinguishing polycythemia vera (PV) from essential thrombocythemia. Thirty patients with PV (group A) and 30 patients who presented with idiopathic marked thrombocytosis with platelet count greater than 1,000 x 10(9)/L and a normal or reduced hemoglobin (Hb) level (group B) were studied at initial presentation. Endogenous (erythropoietin-independent) erythroid colonies (EEC) were found in all patients in group A and 13 in group B. The numbers of EEC were comparable between patients in group A and the 13 patients with EEC in group B, 11 of whom with initial Hb levels ranging between 6.4 g/dL and 12.6 g/dL were found to have PV 2 to 45 months after initial evaluation. The number of EEC did not correlate with the time to the progression of polycythemia, whereas myelosuppression delayed the subsequent development of PV. Of the two patients with EEC in group B who did not develop PV, both received chemotherapy soon after presentation, which might preclude the evidence of polycythemia evolution. None of the other patients in group B who did not form EEC developed PV with a median follow-up of 24 months. This study indicates that the assessment of EEC in bone marrow or blood is helpful in early identification of PV or prediction of polycythemia evolution in patients with marked thrombocytosis in whom polycythemia has been initially masked or anemia is present.

Aged↗

PubMed1994

Primary small-intestinal lymphomas in Taiwan: immunoproliferative small-intestinal disease and nonimmunoproliferative small-intestinal disease.

PURPOSE: The clinicopathologic findings in 45 adult Chinese patients with primary small-intestinal lymphoma (PSIL) are described and compared with those in Western countries and in underdeveloped nations. The efficacy of combination chemotherapy is also assessed. PATIENTS AND METHOD: Six patients had immunoproliferative small-intestinal disease (IPSID) indicated by the presence of alpha-heavy chain protein (alpha-CP) in body fluids or tumor tissues. Thirty-nine patients had non-IPSID, including one with postrenal transplant lymphoma. Thirty-three non-IPSID patients received a minimum of four cycles of combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). RESULTS: All IPSID patients presented with the clinical and laboratory features of severe intestinal malabsorption, and all had diffuse lymphoplasmacytic infiltration in the mucosa of the small bowel. Lymphomas were localized mainly in the jejunum and mesenteric nodes. The histologic subtypes were diffuse large cell in two, immunoblastic in three, and diffuse mixed in one. All patients responded poorly to chemotherapy, with a median survival duration of 10.5 months. The common presenting symptoms of the 39 non-IPSID patients included abdominal pain (90%), weight loss (31%), abdominal mass (26%), obstruction (26%), and perforation (23%). Diffuse large-cell and immunoblastic lymphomas constituted 82% of cases. Four patients had stage IE, 19 stage II 1E, and 16 stage 112E disease according to the Musshoff's criteria; 22 had bulky tumors and 19 had multiple tumors. The tumors were completely resected in 14 patients. Of 33 patients treated with combination chemotherapy, 73% achieved a complete remission. With a median follow-up duration of 90 months, there have been four relapses, with only one at the primary tumor site. The overall 5-year survival and disease-free survival rates for non-IPSID patients who were treated with chemotherapy were 59% and 54%, respectively. CONCLUSION: Intensive chemotherapy produces long-term disease-free survival in locally advanced non-IPSID PSIL.

Adult↗