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Biomedical subjects

C K Lin

Publications and source records attributed to C K Lin.

At least 145 records · Page 8Linked to original sources

Iron overload in Chinese patients with hemoglobin H disease.

We examined the iron status of 23 adult patients with hemoglobin H (Hb H) disease. None of them had received multiple blood transfusions or prolonged iron therapy. Studies included serum iron and ferritin concentrations, transferrin saturation, a desferrioxamine test, computed tomography (CT) scan of the liver, and liver biopsy. Iron overload was found in 17 patients (73.9%), especially in males and in patients with splenomegaly (92.9% and 100%, respectively). Four patients with excessive alcohol consumption had clinical manifestations of severe iron overload. Idiopathic hemochromatosis associated HLA antigens, i.e., HLA-A3, -B7, or -B14, were not found in any of the 15 patients tested. These findings indicate that iron overload is common in adult patients with Hb H disease; such patients should abstain from alcohol and be considered for treatment with an iron chelating agent before irreversible organ damage occurs.

Adult↗

A patient with large granular lymphocytosis of unusual phenotype and polymorphic T-cell receptor beta-chain gene rearrangement.

The authors describe a patient with large granular lymphocytosis who presented with fever of unknown origin and jaundice. Immunophenotyping showed that most of the large granular lymphocytes (LGLs) were CD3-, CD16-, and NKH-1 (Leu-19)+ lymphocytes. Lymphocytosis of this subset of LGLs has not been reported. Analysis of T-cell receptor gene showed polymorphic T-cell receptor beta-chain (TCR beta) gene configuration. Functional studies showed reduced natural killer cell function. The clinical course was very aggressive and resistant to chemotherapy. These features again raise the controversial issues of the ontogeny and heterogeneity of LGLs and their relationship to natural killer cells and T-cells.

Adult↗

Efficacy of a modified improved technique for detecting red cell haemoglobin H inclusions.

Alpha-thalassaemia is a common disease in Taiwan. A feature useful in diagnosis is the excess of of beta-chains that result from impaired alpha-chain production. These excess chains assemble into beta 4 tetramers (i.e. Hb H) which can be detected by its rapid anodal migration on alkaline electrophoretic media as seen in Hb H disease. However, this technique cannot, and conventional Hb H inclusion staining rarely can visualize alpha-thalassaemic traits due to small quantities of Hb H formed in these patients. The staining for Hb H inclusion bodies uses brilliant cresyl blue (BCB) or methylene blue (MB) as an oxidant to denature Hb H as intracellular inclusions. We have improved the technique of Jones (who modified the original method in order to obtain enriched young red cells) by prolonging the incubation time from 30 min to 3 h. The sensitivity of this modified improved method was 91% for detecting obligatory alpha-thalassaemic traits as shown in table. No false positive results were seen in beta-thalassaemia or in others. We believe this can be used as a confirmatory test in heterozygous alpha 1-thalassaemia and homozygous alpha 2-thalassaemia.

Anemia, Hypochromic↗

The lack of antiplatelet effect of crude extracts from ganoderma lucidum on HIV-positive hemophiliacs.

Effects of the extracts from Ganoderm lucidum (GL-P) to influence immune status of the hemophiliacs with positive HIV antibody and reversed helper/suppressor T-lymphocyte ratio were studied. Since the extracts from G. lucidum have been reported to contain high levels of adenosine, the untoward antiplatelet effect of the extracts on hemophiliacs were highly concerned. Five patients of hemophilia A voluntarily received the extracts which has been analyzed to contain 150 mg of adenosine in 100 gm of the extracts. Patients were estimated to take 1.35 mg of the adenosine daily. Platelet aggregation tests before and after the trial of the extracts showed no significant change. Our crude extracts of the Ganoderma lucidum was considered not to have untoward antiplatelet effect in vivo despite the high contents of adenosine.

Adult↗

Myelodysplastic syndrome presenting with generalized cutaneous granulocytic sarcomas.

A patient with myelodysplastic syndrome (refractory anemia with excess of blasts in transformation, RAEB-T) presented with generalized granulocytic sarcomas involving the skin. The diagnosis was confirmed by skin and bone marrow biopsies. Partial myeloperoxidase deficiency was noted in the circulating polymorphonuclear leukocytes and the more differentiated tumor cells in the granulocytic sarcoma. This observation suggests that such leukocytes may be derived from the abnormal 'leukemic' clone.

Aged↗

Iron overload in untransfused patients with hemoglobin H disease.

Two Chinese patients with hemoglobin (Hb) H disease without a history of blood transfusion developed iron overload at the age of 45 and 53 years, respectively. Human leukocyte antigen (HLA) system types of these 2 patients were A19, A11, B13, B62 and A2, A24, BW55, respectively, which are not related to the common haplotypes for idiopathic hemochromatosis. Since severe iron loading is a rare clinical manifestation in untransfused patients with Hb H disease, the iron overload in both patients may be due to environmental or underlying genetic factors.

Humans↗

Hemoglobin H disease complicated iron overload and HLA expression in Chinese.

HLA-A,B antigens were typed in 15 patients with Hemoglobin H disease; 10 of them had iron overloading. No significant HLA antigen specificity was identified to be at risk as compared with 170 controls. However, HLA-A11 was suggested to have significant negative risk, and may have a protective effect. (RR = 0.1, p = 0.0015). It seems that increased iron absorption in Hemoglobin H disease is not genetically associated with idiopathic hemochromatosis and certain HLA antigen expressions.

Adult↗

Hemoglobin H disease--ten years' experience.

Eighty-eight patients with Hemoglobin (Hb) H diagnosed in our hospital in the past decade were reviewed. Among them, 37 were males and 51 were females, and their age ranged from 10 to 77 years. In physical examination, 43.3% of them showed to have jaundice, 47% had splenomegaly, 14% had hepatomegaly and 19.3% had gall stone. Hb electrophoresis revealed the presence of Hb H (1.4-40%), Hb F (23/88, range: 0.5-7.5%) and decreased Hb A2 (mean = 1.23 +/- 1.2%). The study of iron status showed increased serum ferritin concentration (mean = 421.4 +/- 343.7 ug/dl) and transferrin saturation ratio (53.9% +/- 20.5%). Hemosiderosis was found in three out of four patients received liver biopsy. Although most patients did not receive blood transfusion frequently, iron overload was not uncommon in the patients with Hb H disease. Further study would be needed to explore the true relationship between iron overload and Hb H disease.

Adolescent↗

Chronic neutrophilic leukemia--report of a case and review of the literature.

A male patient, aged 78, was found with chronic neutrophilic leukemia (CNL). The patient showed sustained mature neutrophilic leukocytosis, splenomegaly, a high leukocyte alkaline phosphatase score, elevated serum vitamin B12 and uric acid, myeloid hyperplasia and absence of ph' chromosome in the bone marrow, with no evidence suggesting this condition to be a leukemoid reaction to an underlying disease. In addition to the above mentioned features, some functional characteristics of CNL cells were compared with normal cells. CNL is a very rare disease; some thought it as a variant of chronic myelogenous leukemia (CML). In this report a review of the literature is also included.

Aged↗

Hypophysectomy decreases and growth hormone increases the turnover and mass of rat liver glutamine synthetase.

Hypophysectomy diminishes rat liver glutamine synthetase (GS) activity and growth hormone (GH) administration restores this activity to normal levels; brain GS is unaffected. We have now investigated the effects of long-term hypophysectomy (45-day) and GH treatment on the GS mass (amount of enzyme) and turnover in rat liver and brain. Labeled GS was isolated by immunoprecipitation at intervals between one and six days after pulse administration of [U-14C] leucine and the GS half-life (t1/2) was determined. The GS mass was obtained by immunoassay and by calculation using the specific activity of purified GS. GS turnover was calculated by multiplying the GS mass by the first-order rate constant of degradation (kd). During the time course of each experiment, the GS mass did not change, indicating that in each of the three hormonal states studied, a steady state existed. Hypophysectomy increased the t1/2 of hepatic GS from 3.8 to 8.8 days and decreased GS turnover from 0.38 to 0.1 microgram/100 g body wt/day; the GH regimen used restored the turnover to above normal levels, 0.6 microgram/100 g body wt/day. The GS mass decreased from 2.0 to 1.2 micrograms/100 g body wt and GH restored the GS mass to normal levels. The brain enzyme was not affected by hypophysectomy or GH.

Animals↗

The reappraisal of dilute tissue thromboplastin inhibition test in the diagnosis of lupus anticoagulant.

The dilute tissue thromboplastin inhibition (DTTI) test (Schleider et al, 1976) is a sensitive but non-specific test for lupus anticoagulant (LA). False positive results are seen in patients with clotting factor deficiency involving the extrinsic pathway and also in some patients with specific factor inhibitors (Triplett et al, 1983; Rosove et al, 1986). Since the effect of LA is phospholipid dependent but those of factor deficiency and specific inhibitors are not, we analyse the test results by comparing the degree of inhibition using different dilutions of tissue thromboplastin and express it as the DTTI index. This is defined as the clotting time ratio with 0.2% tissue thromboplastin divided by the clotting time ratio with 2% tissue thromboplastin. We also perform a dilute tissue thromboplastin time with platelet substitution to see if this could neutralize the inhibition caused by LA. Both of these modifications can reliably distinguish LA from other conditions associated with prolonged APTT better than the original DTTI test.

Adolescent↗

Arrangements of alpha-globin gene cluster in Taiwan.

In a gene mapping study on 217 newborn babies in Taiwan with alpha- and zeta-globin probes, we have observed 4 cases (1.84%) of alpha-thalassemia-2 heterozygotes (zeta zeta-alpha/zeta zeta alpha alpha) without increased levels of hemoglobin (Hb) Bart's in the cord blood. Eleven subjects (5.07%) were found to have the South East Asian alpha-thalassemia-1 haplotype (zeta zeta--SEA/zeta zeta alpha alpha) with increased Hb Bart's levels ranging from 2.2 to 9%. One case, with Hb Bart's level of 14% in the cord blood, was found to have the genotype of zeta zeta--SEA/zeta zeta alpha alpha T (0.46%). Four heterozygotes (1.84%) were found with the triple alpha gene anti-rightward arrangement (zeta zeta alpha alpha alpha 3.7/zeta zeta alpha alpha). Twenty-one heterozygotes (9.68%) were found to have the triple zeta-globin gene arrangement (zeta zeta zeta alpha alpha/zeta zeta alpha alpha). A new triple zeta-globin gene variant with a BamHI polymorphism was also observed in this study.

Asian People↗

[X-linked dyskeratosis congenita with aplastic anemia--genetic and hematologic studies].

Two brothers in a Chinese family were affected with dyskeratosis congenita (DC). The proband's stepbrother died of pancytopenia and rectal adenocarcinoma. The proband with progressive pancytopenia was studied in genetics and hematology. Family investigations suggest that the disorder was inherited as an X-linked recessive transmission. No specific chromosome aberrations were detected in both peripheral blood and bone marrow cells. Bone marrow biopsy revealed markedly hypocellular marrow without prominent reticulin fibers. Hematopoiesis was assessed by assay of granulomonocytic (CFU-GM) colonies and clusters in vitro. Numbers of CFU-GM were profoundly reduced in marrow cultures from our patient. Although a defect in the stromal microenvironment and lymphocyte mediated suppression could not be excluded. It is possible that the defect is intrinsic to the hematopoietic stem cells. The familial disease described by this paper favors that the dyskeratosis congenita is X-linked with constitutional aplastic anemia. It is believed that the pancytopenia with hypocellular marrow in DC is an inherited rather than an acquired condition.

Adolescent↗

Combined cold- and warm-antibody autoimmune hemolytic anemia--review of the literature and a case report.

This report described the second case in the literature of Waldenstrom's macroglobulinemia with combined cold- and warm- antibody autoimmune hemolytic anemia (CCW-AIHA). Both IgG and IgM red cell auto-antibodies (auto-Abs) were present in the patient's serum. The IgM auto-Abs had a high thermal amplitude and anti-I blood group specificity. The IgG auto-Abs did not reveal blood group specificity. This patient depended on steroids to control hemolysis, but responded well to chemotherapy and plasmapheresis. CCW-AIHA has rarely been reported. In this study, the literature is reviewed and the characteristic serological and clinical features of this rare category of autoimmune hemolytic anemia (AIHA) are discussed.

Adolescent↗

Fine needle aspiration biopsy cytology of superficial lymph nodes: comparative with histopathology.

Fine needle aspiration biopsy cytology was done in 215 lymph nodes involving cervical, submandibular, supraclavicular, axillary and inguinal regions. One hundred and forty of these were diagnosed by histopathology after excisional biopsy or surgery, including 46 cases of metastatic tumor, 57 of lymphoma (46 were non-Hodgkin's lymphoma and 11 were Hodgkin's disease), and 37 of benign lymphadenopathy. Diagnosis of metastatic malignancy was easily made by aspiration biopsy cytology with a total sensitivity of 95.7% (44/46). The diagnosis of lymphoma was less satisfactory with a total sensitivity of 82.5% (47/57). The specificity for diagnosis of benign lymphadenopathy was 97.3% (36/37) with one false positive. The concordance of cell type in aspiration biopsy cytology and histopathology was 75% (33/44) in metastatic malignancy, and 73.5% (28/38) in lymphoma. Typical Reed-Sternberg giant cells were detected in 4 of 11 cases of Hodgkin's disease by aspiration cytology. Out of the 37 cases of benign lymphadenopathy diagnosed by aspiration cytology, 7 were cases of tuberculous lymphadenitis, of which typical Langhans' giant cells were found in 2, and acid fast stain bacilli in 3 cases. There was no complication or needle tract spreading throughout the procedure. We concluded that aspiration biopsy cytology is a simple, safe, reliable, and quick diagnostic method.

Adolescent↗

Release of colony-stimulating activity by resting and activated monocytes, T cells and B cells.

Purified populations of monocyte, T lymphocyte, and B lymphocyte from normal human peripheral blood were used for the investigation of elaboration/release of granulocyte-macrophage colony-stimulating activity (GM-CSA). Cell separation was performed by a series of techniques including density-cut centrifugation, adhering incubation, carbonyl iron ingestion and E rosette formation. The purity of the isolated cell population was over 95% with a mean viability of 98%. After collection, the cells were resuspended at a concentration of 1 x 10(6)/ml in RPMI-1640 medium containing 5% fetal calf serum and incubated for 7 days at 37 degrees C to prepare conditioned media (CM) for assay of GM-CSA. The results showed that the monocytes could constitutively secrete a considerable amount of GM-CSA, whereas no CSA was produced by T cells or B cells under normal conditions. All the three cell populations released GM-CSA when activated by mitogen stimulation. Monocyte-derived GM-CSA production was greatly enhanced by zymosan (Zym), lipopolysaccharide (LPS) and concanavalin A (Con A), resulting in an augmentation approaching 3 to 4 times the untreated control. For T lymphocytes, the most contributive stimulants to induce GM-CSA release were phytohemagglutinin (PHA) and Con A, while Zym and LPS were not effective. B lymphocytes, after treatment with pokeweed mitogen (PWM) or PHA, were also capable of releasing large amounts of GM-CSA with a peak level near to PHA-stimulated T lymphocytes. The finding suggested that, when activated, B cells may participate in the inflammatory response and in the regulation of granulopoiesis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

The effect of lipopolysaccharide on the production of GM-EA, GM-CSA, and PGE2 by human monocyte-derived lipid-containing macrophages.

Monocyte-derived lipid-containing macrophage (MDLM) was the major source of granulomonopoietic enhancing activity (GM-EA) but these well-differentiated cells were unable to synthesize constitutively the granulocyte-macrophage colony-stimulating activity (GM-CSA) that was contributed mostly by the younger monocytoid cells. The presence of various concentrations (0.5-10 micrograms/ml) of lipopolysaccharide (LPS) potentiated the production of GM-EA by MDLM. Enhancement of GM-EA production peaked at about 0.5 micrograms/ml of LPS, but at higher doses (10-40 micrograms/ml) LPS became suppressive. In parallel, LPS-induced production of prostaglandin E2 (PGE2) was observable only at higher doses (10-40 micrograms/ml), suggesting a correlation between PGE2 production and LPS-mediated suppression of GM-EA synthesis. At optimal concentration (0.5 micrograms/ml), LPS could effectively override the inhibitory effect of interferon-gamma on the production of GM-EA. In addition, GM-CSA production by MDLM can be partly restored by stimulation with high doses of LPS (10-40 micrograms/ml). These results suggest that MDLMs have functional potentials similar to the younger macrophages and may play an important role in the regulation of myelopoiesis through the release of GM-EA and related regulators.

Colony-Stimulating Factors↗