PubMed Health⌕ Search

Biomedical subjects

H C Hsieh

Publications and source records attributed to H C Hsieh.

At least 55 records · Page 3Linked to original sources

Primary myelofibrosis in children: report of 4 cases.

From 1982 to 1985, four cases of primary myelofibrosis were diagnosed in our department. Three were boys and one was a girl. Their ages ranged from 7 months to 15 years. The diagnosis was made based on anemia, leukoerythroblastic change and presence of giant platelets in the peripheral blood, and a bone marrow biopsy showing myelofibrosis. Most of them had anemia, fever, and hepatosplenomegaly on admission. The anemia was severe and refractory to repeated transfusions and steroid therapy in 3 out of the 4 cases. Splenectomy was performed in 1 case, but without satisfactory results. The clinical course and blood pictures in one case resembled leukemia of megakaryocyte lineage (M7), but results of marker studies of the blast cells ruled out the possibility of M7. Three of them underwent leukemic transformation within 2 years and died soon after. The other one died of sepsis 2 weeks after diagnosis. Myelofibrosis in childhood occurs rarely, however, when it does, it always runs a rapid and fatal course.

Adolescent↗

Correlation between sonography and pathology in thyroid diseases.

Ultrasonography was performed on the thyroid glands of 47 patients with various thyroid diseases, in a fresh state, immediately following operation. The thyroid glands were then sectioned along the plane of the most significant sonographic changes. The pathologic changes of the sectioned plane were observed and compared with the sonographic changes. In nodular goiter, the sonographic changes were usually heterogeneous. The margin was either well-defined or ill-defined, and cystic changes might play a minor or major part in the thyroid nodules. In follicular adenoma, the changes were isoechoic in adenoma with embryonal type follicles and hyperechoic in adenoma with colloid type follicles. In adenoma of the oxyphilic cells, the echogenicity was somewhat greater in the hemorrhagic part compared to the area with oncocytic change only. In papillary carcinoma, the lesions usually manifested as well-limited, heterogeneous, hypoechoic nodules. Cystic degenerations were frequently noted. There were discrete particles corresponding to microcalcification in 4 out of 6 cases. In follicular carcinoma, the sonographic changes also showed well-limited nodules. However, the echogenicity was not decreased as much as in papillary carcinoma, and it could be either homogeneous and isoechoic, or hypoechoic. Relatively large particles with an acoustic sign, which corresponded to calcification, were noted in 2 out of 4 cases. In medullary carcinoma, the lesion was well-limited and hypoechoic. The particles present in sonography corresponded to calcification in the amyloid. In Graves' disease, the main sonographic change was a diffusely homogeneous, isoechoic or hypoechoic lesion. Cystic change was rarely present. Dispersed particles were rarely present and corresponded to fibrosis in the thyroid tissue.(ABSTRACT TRUNCATED AT 250 WORDS)

Goiter, Nodular↗

Direct comparisons of peripheral T-cell lymphoma with diffuse B-cell lymphoma of comparable histological grades--should peripheral T-cell lymphoma be considered separately?

Peripheral T-cell lymphoma (PTCL) forms a morphologically heterogeneous group of non-Hodgkin's lymphomas (NHL) with distinct immunophenotypes of mature T cells. Progress has been slow in defining specific clinicopathological entities to this particular group of NHL. In order to elucidate the specific characteristics of PTCL, a direct comparison of PTCL with a group of diffuse B-cell lymphomas (DBCL) was performed. Between June 1983 and December 1987, we studied 114 adults with NHL, using a battery of immunophenotyping markers. Adult T-cell leukemia/lymphoma, lymphoblastic lymphoma, mycosis fungoides/Sézary syndrome, follicular lymphoma, well-differentiated lymphocytic lymphoma, and true histiocytic lymphoma were excluded from this study since these are distinct clinicopathologic entities with well-recognized immunophenotypes. Of the remaining 75 patients, 70 who had adequate clinical information were analyzed, and of these, 34 were PTCL and 36 were DBCL. Classified according to the National Cancer Institute (NCI) Working Formulation (WF), 68% of PTCL and 31% of DBCL were high-grade lymphomas. Clinical and laboratory features were similar, except PTCL had a characteristic skin involvement and tended to present in more advanced stages with more constitutional symptoms. Induction chemotherapy was homogeneous in both groups, and complete remission rates were 62% for PTCL and 67% for DBCL. Patients with DBCL had a better overall survival than patients with PTCL, but the survival benefit disappeared after patients were stratified according to intermediate- or high-grade lymphoma. A subgroup of PTCL patients who had received less intensive induction chemotherapy was found to have a very unfavorable outcome. We conclude that (1) PTCL follows the general grading concept proposed in WF classification; (2) within a given intermediate or high grade, PTCL and DBCL respond comparably to treatment; (3) the intensity of induction chemotherapy has a crucial impact on the outcome of PTCL patients; and (4) with a few exceptions, the clinical and laboratory features of PTCL and DBCL are comparable.

Adolescent↗

Serum prolactin and growth hormone response to thyrotropin-releasing hormone in patients with pituitary adenomas.

The dynamics of growth hormone (GH) and prolactin (PRL) secretion in response to intravenous 400 micrograms thyrotropin-releasing hormone (TRH) in 13 untreated patients with pituitary adenomas (6 women, 7 men; ages 20-64 years; 8 patients with acromegaly, 3 prolactinomas and 2 non-functional adenomas) were correlated with the results of immunohistochemical studies of GH and PRL in the adenoma cells. The basal serum GH concentrations were abnormally high in 6 and normal in 2 cases of acromegaly. In 5 of 6 acromegalic patients with pure GH-containing adenomas, TRH stimulated GH release by less than 100%. One patient with acromegaly and pancreatic islet cell carcinoma had the maximum increment for GH by 303%. TRH stimulated GH release by more than 100% in 2 acromegalic patients with mixed GH/PRL-containing adenomas. In 3 patients with prolactinoma and in 2 patients with non-functional adenoma the basal GH levels were 0.5 mU/L or undetectable and there was no GH response to TRH. The peak serum PRL levels after TRH were less than 100 ng/ml in 6 acromegalic patients with pure GH-containing tumors and in 2 patients with non-functioning pituitary tumors, and were more than 230 ng/ml in 3 patients with prolactinoma and in 2 acromegalic patients with mixed GH/PRL-containing tumors. It is suggested that the TRH test may help clinically in establishing the diagnosis of prolactinoma, pure GH-secreting tumors and mixed GH/PRL-containing tumors.

Adenoma↗

Adult non-Hodgkin's lymphoma in Taiwan area: a clinicopathologic study of 123 cases based on working formulation classification.

To elucidate the clinicopathologic features of non-Hodgkin's lymphoma (NHL) in Taiwan, 123 adult patients with proven NHL were studied. They were classified according to the international working formulation as: low grade (LG), 12.2%; intermediate grade (IG), 42.3%; and high grade (HG) lymphoma, 45.5%. The most common subtypes were diffuse large cell (26.8%) and large cell immunoblastic (26.8%) lymphomas. Follicular lymphoma accounted for only 8.9% (11 cases). Complete remission rates for LG, IG and HG lymphomas were 53%, 35% and 34%, respectively. LG lymphoma had a significantly better survival than that of IG and HG lymphomas. The IG lymphoma encompassed a heterogeneous group of patients with varying prognoses but the overall survival curve was indistinguishable from that of HG lymphoma. Clinically, 66% of HG, 77% of IG and 86% of LG lymphoma presented with advanced disease. LG lymphoma had high frequencies of hepatosplenomegaly (30-50%) and bone marrow involvement (53%), whereas skin, bone and central nervous system involvement occurred exclusively in IG and HG lymphomas. Mild anemia was common and occurred in 40-50% of the patients. Hyperimmunoglobulinemia was found in 50-60% of all 3 grades of lymphoma, monoclonal gammopathy in 3 cases of IG lymphoma, and hypercalcemia in 4 cases of IG and HG lymphomas. Elevated serum lactate dehydrogenase occurred mainly in IG and HG lymphomas and was an important prognostic factor. In conclusion, the characteristic features of NHL in Taiwan include: (1) a high proportion of HG lymphoma and low proportions of LG and follicular lymphomas; (2) a heterogeneous patient composition of IG lymphoma with an unfavorable overall prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Bone marrow involvement in non-Hodgkin's lymphoma.

The frequency, pattern, extent and clinical features of bone marrow involvement in 94 cases of non-Hodgkin's lymphoma, classified according to the International Working Formulation, were reviewed. In 30% of cases there was bone marrow involvement by lymphoma at the time of diagnosis. Marrow involvement was most frequently found in the follicular small cleaved cell (57%), diffuse mixed small and large cell (56%), and lymphoblastic (56%) lymphomas. A paratrabecular pattern of marrow involvement tended to occur in low or intermediate grade lymphomas. High grade lymphomas tended to show an interstitial or diffuse pattern of marrow involvement. The results of marrow aspiration and biopsy were of equal value and complementary to each other. In 43% of cases with marrow lymphoma there was peripheral blood involvement at the time of diagnosis. Blood involvement was most frequently found in the lymphoblastic lymphoma (70%). In general, the degree of blood involvement was related to the extent of marrow involvement. Patients with marrow involvement often presented with "B" symptoms and hepatomegaly. Abnormalities in at least one of the blood counts were quite common (93%). A normal blood count made marrow involvement unlikely but not impossible. Other common laboratory findings included hyperimmunoglobulinemia (47%) and elevated serum levels of lactate dehydrogenase (56%). Patients with marrow involvement showed a significantly higher frequency of hepatomegaly and abnormal blood counts, as compared to those with negative marrows (p less than 0.05 and less than 0.001, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Characterization of the spectrum of postthymic T-cell malignancies in Taiwan. A clinicopathologic study of HTLV-1-positive and HTLV-1-negative cases.

Postthymic T-cell malignancy shows marked geographic, clinicopathologic, and prognostic diversity. The frequency and spectrum of T-cell malignancies in Taiwan were investigated. Fifty-two patients (35 male and 17 female) with a median age of 49 years, were consecutively encountered between October 1983 and April 1987; these accounted for 39% of non-Hodgkin's lymphoma cases seen in our institutions. Ten patients (19.3%) had adult T-cell leukemia/lymphoma (ATL) associated with human T-cell leukemia virus (HTLV-1). Patients with ATL had disease similar to that reported from southwestern Japan and the Caribbean. They had frequent skin lesions (60%), hypercalcemia (40%), and a rapid clinical course with a median survival of 1.3 years. The 35 HTLV-1-negative peripheral T-cell lymphomas (PTL) were similar to PTL in western countries, manifesting frequent visceral, cutaneous, and vascular tropisms. Marrow involvement was documented at presentation in 39% and Stage III/IV disease in 80% of the PTL patients. The histology of PTL usually expressed prominent reactive features which is distinct from that in ATL. Several subcategories could be defined: Hodgkin's-like PTL in nine patients, T-zone lymphoma in three, angioimmunoblastic lymphadenopathy-like lymphoma in one, Lennert's lymphoma in three, and angioinvasive lymphoma in four. Two HTLV-1-negative PTL had neoplastic cells with clover-shaped nuclei and were designated as ATL-like. Morphologic classification based on the modified Working Formulation showed prognostic correlation, with median survival of less than 6 months for large cell/immunoblastic PTL, compared with 5 years for patients with small/medium cell PTL. Both low- and high-grade PTL seem to represent an incurable disease. Classical cutaneous T-cell lymphoma (seven cases) is relatively unusual in Taiwan, compared with the frequency of PTL. Post-thymic T-cell malignancies in Taiwan include HTLV-1-positive and HTLV-1-negative diseases, both of which have a poor prognosis and resemble similar T-cell malignancies in the East and West.

Adult↗

Waldenstróm's macroglobulinemia with peripheral neuropathy--the role of IgM M-protein in autoimmune mechanism.

A total of 11 cases of Waldenstróm's Macroglobulinemia (WM) have been investigated to study the association of the disease with neuropathy. A 53-year-old female patient (Wu) had a pronounced peripheral neuropathy, in which the role of IgM M-protein could be observed. Sections of Wu's biopsied sural nerve were subjected to light and electron microscopy and direct immunofluorescence (IF). Myelin degeneration with axonal change was found, while fluorescence reaction occurred in epineurium only. Sections of a healthy dog's sciatic nerve were submitted to purified IgM M-proteins from Wu and another WM patient (Kon) without neuropathy, respectively. Indirect IF showed a deposit of fluorescence on the myelin sheath by Wu's, but not by Kon's, M-proteins. It is proposed that an autoimmune mechanism underlies the peripheral neuropathy in the WM patient.

Animals↗

Importance of exfoliatin toxin A production by Staphylococcus aureus strains isolated from clustered epidemics of neonatal pustulosis.

Clustered epidemics of pustulosis due to Staphylococcus aureus occurred in two geographically distant newborn nurseries. In nurseries A and B an attack rate of pustulosis of 0.8 and 2.0 cases per 100 live births occurred, respectively. Experimental phage type 1046/1116 belonging to phage group II dominated clustered epidemics in nursery A, while group II phage type 3A/3C/55/71 and 3A/3C/55 occurred in nursery B. Other group II strains also occasionally produced clustered epidemics. These epidemic strains were found to be making heat-stable dermal exfoliatin toxin A (ETA) which had a pI of 6.8 and a molecular weight of 32,000 and 33,000. ETA-bearing strains did not make bacteriocin. Children infected with ETA-producing strains developed extensive bullous pustulosis. Surveillance cultures of personnel revealed an ETA-bearing strain in only one person. This strain was not the same phage type as the epidemic cluster. In contrast, ETA-bearing epidemic strains were found in the inanimate environment of both nurseries. ETA protein acts as an important virulence factor in the production of neonatal pustulosis infection and appears to be linked with the ability of S. aureus organisms to stick to the inanimate environment.

Animals↗

Two coagulase-variant forms of Staphylococcus aureus isolated from blood cultures.

Two coagulase-variant forms of Staphylococcus aureus were isolated from blood cultures of a patient with infective endocarditis. The coagulase-positive isolate was hemolytic, whereas the coagulase-negative isolate was nonhemolytic. All other properties examined were identical in both strains. Since coagulase-negative S. aureus strains have been isolated from clinical specimens, laboratories should consider using a combination of other biological properties along with coagulase production for the identification of S. aureus.

Adult↗