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Biomedical subjects

J H Wu

Publications and source records attributed to J H Wu.

At least 37 records · Page 2Linked to original sources

A cDNA from human bone marrow encoding a protein exhibiting homology to the ATP1gamma1/PLM/MAT8 family of transmembrane proteins.

A cDNA clone, IWU-1, was cloned from human bone marrow. Its putative open reading frame encoded a protein of 115 amino acids with a calculated molecular mass of 12.9 kDa. The deduced amino acid sequence exhibited high homology (>68%) to members of the ATP1gamma1/PLM/MAT8 family of single transmembrane proteins, primarily in the region containing the putative transmembrane domain. The sequence at the amino-terminal side exhibited high homology (>61%) to the cytoplasmic region of the angiotensin II type 1 receptors.

Amino Acid Sequence↗

Quantitative assessment of phagocytic activity of hemocytes in the prawn, Penaeus merguiensis, by flow cytometric analysis.

BACKGROUND: The blood cells of crustaceans are involved in phagocytosis of invading microorganisms, contributing to their defense mechanisms. In this study, phagocytic activity of hemocytes of the prawn, Penaeus merguiensis, was quantitated by means of flow cytometric analysis. METHOD: This study was done in vitro. Hemolymph, which was extracted from prawns, was mixed with an equal volume of anticoagulant. Heat-killed Escherichia coli prestained with propidium iodide (PI) was then added. Hemocytes were fixed at various time intervals for flow cytometric analysis. This study was supplemented with electron micrographs using transmission electron microscopy (TEM), which showed three populations of hemocytes. RESULTS: It was observed that those hemocytes that were more active engulfed and digested bacteria readily, thus having higher red fluorescence intensity. The phagocytic activity was expressed as fluorescence unit or engulfed E. coli number per hemocyte. CONCLUSIONS: With this approach, the phagocytic and cellular activity of individual hemocyte populations could be studied quantitatively.

Animals↗

Localization of androgen receptor expression in human bone marrow.

Androgens have been shown to modulate the haematopoietic and immune systems and have been used clinically for stimulating haematopoiesis in bone marrow failure conditions. To identify the bone marrow cell types as potential targets of androgens, an androgen receptor (AR)-specific antibody was used to localize the AR in normal human bone marrow biopsies. The results show that AR was ubiquitously expressed in the bone marrow of both males and females. Furthermore, the AR expression pattern did not change with age. Stromal cells, macrophages, endothelial cells, myeloblasts, myelocytes, neutrophils, and megakaryocytes expressed AR. In contrast, AR was not detected in the lymphoid and erythroid cells, or in eosinophils. These results indicate that androgens may exert direct modulating effects on a wide spectrum of bone marrow cell types via AR-mediated responses.

Adolescent↗

Influence of CETP gene variation on plasma lipid levels and coronary heart disease: a survey in Taiwan.

Cholesteryl ester transfer protein (CETP) transfers cholesteryl ester from high-density lipoprotein (HDL) to very low-density lipoprotein (VLDL), low-density lipoprotein (LDL) and chylomicron in exchange for triglycerides. Two CETP genetic variation and four polymorphisms are investigated by polymerase chain reaction (PCR) and restriction enzyme digestion in a population of Taiwan. The results show that a very rare variation frequency is found for CETP intron 14 splice site G-->A change. The population shows a predominant 405Ile allele (61%), 442Asp (97.7%), intron 1Taq1B(+) G allele (52%), intron 8 Msp1(-) A allele (89%) and intron 9 EcoN1(-) T allele (59.2%) in the control group. Patients with coronary heart disease (CHD) have more CETP EcoN1(+/+) GG genotype (25.3%) than the controls (13.6%) (P=0.049). The intron 1 Taq1B(-) A allele is associated with a high HDL cholesterol and apoA1 level, the EcoN1(+) G allele with a low apoA1 level and the 442Gly with both high total cholesterol and LDL cholesterol levels. Paradoxically, the 442Gly is also present with a higher frequency (5.2%) in HDL cholesterol > or =65 mg/dl group than that in the general population (2.3%) (P=0.04).

Adult↗

Clonality analysis using X-chromosome inactivation patterns by HUMARA-PCR assay in female controls and patients with idiopathic thrombocytosis in Taiwan.

OBJECTIVE: Analysis of X-chromosome inactivation patterns (XCIPs) is a useful tool in the diagnosis of clonal disorders. The human androgen receptor (HUMARA) locus is especially useful for clonality study. The present study was conducted 1) to determine the heterozygosity rate for HUMARA locus in Taiwanese women, 2) to determine the frequency of excessive skewing in different cell types, and 3) to determine the utility of XCIPs in the differential diagnosis of thrombocytosis. PATIENTS AND METHODS: XCIPs by HUMARA-PCR assay were performed on purified granulocytes and T cells from 73 female patients presenting with idiopathic persistent thrombocytosis (IT), 10 patients with reactive thrombocytosis (RT), and 46 bone marrow samples from female controls. XCIPs of buccal mucosa cells were also compared with those of T cells in 57 patients with IT. The percentage of clonal granulocytes was calculated after correcting for the degree of Lyonization in T cells. RESULTS: The heterozygosity rate for the HUMARA gene was 89.1% in Taiwanese females. The median age of informative IT patients and controls was 59 (18-92) and 58 (19-89), respectively. Excessive skewing (allele ratio <0.33) was more frequent in granulocytes than in T cells in both controls (12/43 vs 9/43, p = 0.080) and IT patients (56/64 vs 25/64, p < 0.001). XCIPs were the same for both buccal mucosa and T cells in 43 patients but were different in 14 patients. Of the 43 informative controls, 31 had a polyclonal pattern; an ambiguous pattern was found in nine; and the remaining three, aged 71, 73, and 80, respectively, had a clonal pattern. A clonal pattern was found in 42 IT patients, a polyclonal pattern in 12, and an ambiguous pattern in 10 of the 64 IT patients. The frequency of clonal, polyclonal, and ambiguous patterns in the 40 IT patients with age < or = 65 was 55.0%, 30.0%, and 15.0%, respectively. None of the IT patients aged >65 had a polyclonal disease. IT patients aged >65 had a significantly higher frequency of clonal pattern (p = 0.030) and a significantly lower frequency of polyclonal pattern (p = 0.002) than those with age <65. Of the eight heterozygous patients with RT, one aged 80 exhibited a clonal pattern, and the remaining seven had a polyclonal pattern. CONCLUSIONS: The present study on Taiwanese females showed a heterozygosity rate of 89.1% for the HUMARA gene. Our results confirmed that IT is a heterogeneous disorder in terms of clonality. Twenty-three percent of IT patients exhibited a greater than 20% difference in allele expression for buccal mucosa and T cells. Presence of a clonal XCIP in young patients with IT can serve as a positive marker for the diagnosis of clonal thrombocytosis, and elderly patients with polyclonal XCIPs are unlikely to have essential thrombocythemia.

Adolescent↗

Antioxidant activity of extracts from Acacia confusa bark and heartwood.

The antioxidant activity of extracts from bark and heartwood of Acacia confusa was evaluated by various antioxidant assays, including free radical and superoxide radical scavenging assays and lipid peroxidation assay as well as hydroxyl radical-induced DNA strand scission assay. In addition, an ex vivo antioxidant assay using a flow cytometric technique was also employed in this study. The results indicate that both bark and heartwood extracts clearly have strong antioxidant effects. Similar inhibitory activities for each test sample were found for both 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical generation and lipid peroxidation. As for the superoxide radical scavenging activity, the heartwood extract was more effective than the bark extract. Furthermore, the heartwood extract protected PhiX174 supercoiled DNA against strand scission induced by ultraviolet photolysis of H2O2, and it reduced the amounts of intracellular hydrogen peroxide, a reactive oxygen species, when it was co-incubated with human promyelocytic leukemia (HL-60) cells under oxidative stress.

Acacia↗

In situ identification of polyphosphate- and polyhydroxyalkanoate-accumulating traits for microbial populations in a biological phosphorus removal process.

Polyphosphate- and polyhydroxyalkanoate (PHA)-accumulating traits of predominant microorganisms in an efficient enhanced biological phosphorus removal (EBPR) process were investigated systematically using a suite of non-culture-dependent methods. Results of 16S rDNA clone library and fluorescence in situ hybridization (FISH) with rRNA-targeted, group-specific oligonucleotide probes indicated that the microbial community consisted mostly of the alpha- (9.5% of total cells), beta- (41.3%) and gamma- (6.8%) subclasses of the class Proteobacteria, Flexibacter-Cytophaga (4.5%) and the Gram-positive high G+C (HGC) group (17.9%). With individual phylogenetic groups or subgroups, members of Candidatus Accumulibacter phosphatis in the beta-2 subclass, a novel HGC group closely related to Tetrasphaera spp., and a novel gamma-proteobacterial group were the predominant populations. Furthermore, electron microscopy with energy-dispersive X-ray analysis was used to validate the staining specificity of 4,6-diamino-2-phenylindole (DAPI) for intracellular polyphosphate and revealed the composition of polyphosphate granules accumulated in predominant bacteria as mostly P, Ca and Na. As a result, DAPI and PHA staining procedures could be combined with FISH to identify directly the polyphosphate- and PHA-accumulating traits of different phylogenetic groups. Members of Accumulibacter phosphatis and the novel gamma-proteobacterial group were observed to accumulate both polyphosphate and PHA. In addition, one novel rod-shaped group, closely related to coccus-shaped Tetrasphaera, and one filamentous group resembling Candidatus Nostocoidia limicola in the HGC group were found to accumulate polyphosphate but not PHA. No cellular inclusions were detected in most members of the alpha-Proteobacteria and the Cytophaga-Flavobacterium group. The diversified functional traits observed suggested that different substrate metabolisms were used by predominant phylogenetic groups in EBPR processes.

Acetates↗

Further characterization of apolipoprotein B genetic variations in Taiwanese.

Apolipoprotein B (apoB, protein; APOB, gene) is the main protein component of low-density lipoprotein (LDL) and plays an important role in blood lipid metabolism. Previously, we have reported four APOB coding regions, 5' signal peptide, and 3' repeat sequence polymorphisms in our population. In this report, we further characterize other APOB genetic variations. The results illustrate that the mutation frequencies for Arg3500Gln (1/846 alleles), Arg4019Trp (2/786 alleles), -265 C/T promoter region (0/264 alleles), and intron 2 A/G (0/450 alleles) are very low. Our population showed a frequency of 68.9% for the B4311 Ser allele. The B4311 Asn allele was associated with a higher apoB level than the Ser group (p < 0.05) in normal controls. In the normal controls, a higher B4311 Asn/Asn genotype frequency was found in the group with total cholesterol (TC) > 200 mg/dL and apoB concentration > 85 mg/dL than in the group with a TC < 200 mg/dL and apoB < 85 mg/dL (p = 0.03 for TC comparison).

Analysis of Variance↗

Expression of mPer1 and mPer2, two mammalian clock genes, in murine bone marrow.

Although circadian variations in hematopoiesis have been well documented, the molecular mechanism of the circadian rhythms remains elusive. To determine if a clock system exists in bone marrow to mediate the circadian rhythms, we analyzed the expression of mPer1 and mPer2, both mouse homologues of the Drosophila period gene and known regulators of the clock system, in murine bone marrow by relative quantitative reverse transcriptase-polymerase chain reaction (RT-PCR). We demonstrated that both genes were expressed in bone marrow. Furthermore, the expression patterns of mPer1 and mPer2 in total bone marrow cells exhibited two peaks over a 24-h period. In contrast, the expression patterns of these two genes in the Gr-1-positive cells isolated from bone marrow mainly contributed to one of the two peaks. These results indicate that a clock system exists in bone marrow and suggest that the circadian rhythms in bone marrow are lineage- and/or differentiation stage-dependent.

Analysis of Variance↗

Secondary structure and calcium-induced folding of the Clostridium thermocellum dockerin domain determined by NMR spectroscopy.

Assembly of the cellulosome, a large, extracellular cellulase complex, depends upon docking of a myriad of enzymatic subunits to homologous receptors, or cohesin domains, arranged in tandem along a noncatalytic scaffolding protein. Docking to the cohesin domains is mediated by a highly conserved domain, dockerin (DS), borne by each enzymatic subunit. DS consists of two 22-amino-acid duplicated sequences, each bearing homology to the EF-hand calcium-binding loop. To compare the DS structure with that of the EF-hand helix-loop-helix motif, we analyzed the solution secondary structure of the DS from the cellobiohydrolase CelS subunit of the Clostridium thermocellum cellulosome using multidimensional heteronuclear NMR spectroscopy. The effect of Ca(2+)-binding on the DS structure was first investigated by using 2D (15)N-(1)H HSQC NMR spectroscopy. Changes in the spectra during Ca(2+) titration revealed that Ca(2+) induces folding of DS into its tertiary structure. This Ca(2+)-induced protein folding distinguishes DS from typical EF-hand-containing proteins. Sequential backbone assignments were determined for 63 of 69 residues. Analysis of the NOE connectivities and H(alpha) chemical shifts revealed that each half of the dockerin contains just one alpha-helix, comparable to the F-helix of the EF-hand motif. Thus, the structure of the DS Ca(2+)-binding subdomain deviates from that of the canonical EF-hand motif.

Amino Acid Sequence↗

Carbohydrate specificity of an agglutinin isolated from the root of Trichosanthes kirilowii.

The root of Trichosanthes kirilowii, which has been used as Chinese folk medicine for more than two thousand years, contains a Gal specific lectin (TKA). In order to elucidate its binding roles, the carbohydrate specificities of TKA were studied by enzyme linked lectinosorbent assay (ELLSA) and by inhibition of lectin-glycoform binding. Among glycoproteins (gp) tested, TKA reacted strongly with complex carbohydrates with Galbeta1-->4GlcNAc clusters as internal or core structures (human blood group ABH active glycoproteins from human ovarian cyst fluids, hog gastric mucin, and fetuin), porcine salivary glycoprotein and its asialo product, but it was inactive with heparin and mannan (negative control). Of the sugar inhibitors tested for inhibition of binding, Neu5Ac alpha2-->3/6Galbeta1-->4Glc was the best and about 4, 14.6 and 27.7 times more active than Galbeta1-->4GlcNAc(II), Galbeta1-->3GalNAc(T) and Gal, respectively. From these results, it is suggested that this agglutinin is specific for terminal or internal polyvalent Galbeta1-->4GlcNAcbeta1-->, terminal Neu5Ac alpha2-->3/6Galbeta1-->4Glc and cluster forms of Galbeta1-->3GalNAc alpha residues. The unusual affinity of TKA for terminal and internal Galbeta1-->glycotopes may be used to explain the possible attachment roles of this agglutinin in this folk medicine to target cells.

Carbohydrate Metabolism↗

Cyclophosphamides as hypoxia-activated diffusible cytotoxins: a theoretical study.

Cyclophosphamides have been in clinical use as anti-cancer drugs for a long time and much research has been directed towards reducing their side effects. Here we have performed a theoretical investigation into the possibility of designing bioreductive analogues of cyclophosphamides. Our calculations have employed semiempirical molecular orbital AM1-SM2 and PM3-SM3 calculations, as implemented in MOPAC 93, which include a modified Born method for the treatment of solvation. We have investigated the effect of bioreductive activation on the beta-elimination reaction that is central to the activation of cyclophosphamides. The approach was tested on two known bioreductive agents, including CB1954, and gave results in agreement with experiment. Non-local density functional calculations on CB1954 and its metabolites, including the radical anion, were in agreement with the semiempirical calculations. The calculations have identified a number of potentially novel bioreductive cyclophosphamides. In particular, our calculations identified compounds in which the initial one-electron reduction was not activating. Such compounds are likely to be more effective bioreductive agents, as the beta-elimination will not compete under oxic conditions with the important re-oxidation required for the protection of oxic tissue.

Animals↗

Characterization of Porphyromonas gingivalis-induced degradation of epithelial cell junctional complexes.

Porphyromonas gingivalis is considered among the etiological agents of human adult periodontitis. Although in vitro studies have shown that P. gingivalis has the ability to invade epithelial cell lines, its effect on the epithelial barrier junctions is not known. Immunofluorescence analysis of human gingival epithelial cells confirmed the presence of tight-junction (occludin), adherens junction (E-cadherin), and cell-extracellular matrix junction (beta1-integrin) transmembrane proteins. These transmembrane proteins are expressed in Madin-Darby canine kidney (MDCK) cells. In addition, MDCK cells polarize and therefore serve as a useful in vitro model for studies on the epithelial cell barrier. Using the MDCK cell system, we examined the effect of P. gingivalis on epithelial barrier function. Exposure of the basolateral surfaces of MDCK cells to P. gingivalis (>10(9) bacteria/ml) resulted in a decrease in transepithelial resistance. Immunofluorescence microscopy demonstrated decreases in the amounts of immunoreactive occludin, E-cadherin, and beta1-integrin at specific times which were related to a disruption of cell-cell junctions in MDCK cells exposed to basolateral P. gingivalis. Disruption of cell-cell junctions was also observed upon apical exposure to bacteria; however, the effects took longer than those seen upon basolateral exposure. Cell viability was not affected by either basolateral or apical exposure to P. gingivalis. Western blot analysis demonstrated hydrolysis of occludin, E-cadherin, and beta1-integrin in lysates derived from MDCK cells exposed to P. gingivalis. Immunoprecipitated occludin and E-cadherin molecules from MDCK cell lysates were also degraded by P. gingivalis, suggesting a bacterial protease(s) capable of cleaving these epithelial junction transmembrane proteins. Collectively, these data suggest that P. gingivalis is able to invade the deeper structures of connective tissues via a paracellular pathway by degrading epithelial cell-cell junction complexes, thus allowing the spread of the bacterium. These results also indicate the importance of a critical threshold concentration of P. gingivalis to initiate epithelial barrier destruction.

Animals↗

Immunobiologic, cytogenetic and drug response features of a newly established cell line (SCRC-1) from renal small cell carcinoma.

PURPOSE: We describe the establishment and preliminary characterization of a cell line designated SCRC-1, which was derived from a primary renal small cell carcinoma. MATERIALS AND METHODS: Continuous cultures of a primary stage IVa renal small cell carcinoma and a xenograft in nude mice derived therefrom were characterized by immunohistology, electron microscopy, immunofluorescence/flow cytometry, cytogenetic analysis, and an in vitro drug resistance assay. RESULTS: SCRC-1 cells were reactive with antibodies to NSE, chromogranin-A, bombesin, Bcl-2, CD44s, CD44v6, CD44v7 to 8, vimentin and S100 protein (predominantly beta-subunit), and were unreactive with antibodies to EMA, CD54, EGFR(R1), URO-5, URO-7, URO-8 and URO-10. A similar immunoprofile was also found in both the primary tumor and the xenograft. Cytogenetic analysis revealed the following common clonal aberrations in all 50 metaphases analyzed: 45, XX, t (X;10;18) (p11;p11;q11), -der(18)t(X;10;18), indicating the clonal nature of this neoplasm. SCRC-1 cells showed low drug resistance to cyclophosphamide, doxorubicin, gemcitabine and fluorouracil, intermediate resistance to carmustine and mitomycin-C, and extreme resistance to cisplatin. CONCLUSION: We have documented the initial characterization of SCRC-1, which may be the first cell line reported to be derived from a primary small cell carcinoma of the kidney. This cell line can be used for further studies uncovering the biology and histogenesis of this rare cancer and delineating differences among small cell carcinomas of the kidney and other histological types.

Adult↗

Cytogenetic pattern of childhood leukemia in Taiwan.

BACKGROUND: Cytogenetic analyses of leukemic cells can be used to define specific subgroups of leukemia with different prognoses and, thereby, indicate appropriate treatment for individual cases. In this study, we investigated the cytogenetic pattern of childhood leukemia in Taiwanese patients. METHODS: A modified trypsin method of Seabright was used for G-banding of metaphase cells. RESULTS: From October 1996 to January 1999, 111 children with a diagnosis of leukemia were enrolled in the study. Of these, 73 patients had a diagnosis of acute lymphoblastic leukemia (ALL) and 63 of these patients had successful karyotyping of their leukemic cells. Among them, 20 (30.3%) had a normal karyotype, five had hypodiploidy (all had 45 chromosomes), five had low hyperdiploidy (47-50 chromosomes), 16 (24.2%) had high hyperdiploidy (> 50 chromosomes), and 20 had pseudodiploidy. Chromosomal translocation was identified in 24 (36.4%) of the ALL patients, 17 of whom had recurrent translocations including 10 with CD10+ B-precursor ALL [4 with t(9;22), 5 with t(1;19), and 1 infant with t(8;14)(q24;q11)], one neonate with CD10- early pre-B ALL with t(4;11), three B-cell cases with t(8;14), and three T-cell cases [2 with t(11;19)(q23;p13), and 1 (11;14)(p13;q11)]. One B-precursor patient had dic (9;12). Karyotypes of the 30 patients with acute myeloid leukemia (AML) included eight with t(8;21); seven with the French-American-British-M2 subtype (FAB-M2) and one with M1. All four of the patients with M3 had t(15;17), one patient with M4 had inv(16) and 7q-, one with M4Eo (M4 with eosinophilia) had t(7;16)(q21;q22), one with M0 had t(4;11)(q21;q23), and the remaining 11 had a normal karyotype. Three of the five adult-type chronic myeloid leukemia patients had standard Philadelphia chromosome, and the other two had a variant-form of Philadelphia chromosome. Both of the patients with juvenile myelomonocytic leukemia and one patient with myelodysplastic syndrome had a normal karyotype. CONCLUSIONS: Most findings were similar to previous reports. Although the high proportion of FAB-M2 patients (7/8) with t(8;21) and the consequently higher frequency (26.7%) of this translocation in the 30 AML cases in this study might have significance, a larger series of cases is needed to establish this finding.

Adolescent↗

DNA polymorphisms at the apolipoprotein A1-CIII loci in Taiwanese: correlation of plasma APOCIII with triglyceride level and body mass index.

BACKGROUND AND PURPOSE: Apolipoprotein (APO) A1-CIII genes are linked within a 2.6-kb region on human chromosome 11. ApoA1 is the main component of high-density lipoprotein (HDL), and apoCIII inhibits lipoprotein lipase activity. Genetic variations in APOA1-CIII may affect the function of apoA1/apoCIII and plasma lipid/lipoprotein levels, and thus, the risk of developing atherosclerosis. This study compared the frequency distributions of genetic variations in APOA1-CIII genes and their influence on plasma lipid concentrations in Taiwanese patients with coronary artery disease (CAD) and in healthy controls. METHODS: Six restriction site variations (RSVs) of the APOA1-CIII gene complex were investigated by DNA amplification using polymerase chain reaction and restriction enzyme digestion in 229 control subjects and 131 CAD patients during the period from 1992 through 1996. The blood lipid profiles of these subjects were also determined. RESULTS: Thirty-seven distinct six-RSV genotypes were observed. Separate comparisons of the frequency distributions of the six genetic variations showed no significant differences between CAD patients and controls subjects, but the combined six-RSV-genotypes showed different frequency distributions between these two groups. Nine of the 37 six-RSV genotypes were found only in the CAD patients and higher frequencies of two of these types were observed in the CAD patients than in healthy controls. The effects of these genetic variations were on high-density lipoprotein cholesterol in women (for MspIB, PstI, SstI and PvuII RSV) and total cholesterol (for PvuII RSV), low-density lipoprotein cholesterol (for XmnI RSV), and apolipoprotein B (for MspI and SstI RSV) levels in men in the control group. Elevated plasma apoCIII concentration was significantly associated with an increased plasma triglyceride level and body mass index in the control group (P < 0.0001). CONCLUSIONS: Analysis of the frequency distribution of six RSVs of the APOA1-CIII gene complex in Taiwanese CAD patients and control subjects showed that the effect of genotype on plasma lipid levels was gender-specific and that the apoCIII level was closely associated with plasma triglyceride level and body mass index.

Alleles↗

A rational design of bioactive taxanes with side chains situated elsewhere than on C-13.

The structure of alphabeta-tubulin was refined and used in the docking study for taxuspine D, paclitaxel and their analogues. The conformational space in the binding site was explored by molecular dynamics. The interaction energy was calculated by minimization in the active site of beta-tubulin. The C-5 cinnamoyl side chain in taxuspine D is found to mimic the C-13 side chain of paclitaxel. A virtual taxane with a new C-5 side chain is predicted to be more active than taxuspine D. The C-13 side chain could be replaced with a novel C-5 side chain if the conformation of the core skeleton is modified.

Antineoplastic Agents↗

Immunoglobulin D multiple myeloma.

BACKGROUND: Immunoglobulin D (IgD) multiple myeloma (MM) is rare, accounting for less than 2% of all patients with MM in Western countries. In Taiwan, the frequency and clinicopathologic features of IgD MM have not yet been reported. METHODS: The clinicopathologic features and treatment outcome of patients with IgD MM diagnosed between January 1, 1982, and December 31, 1998, in Chang Gung Memorial Hospital were retrospectively reviewed. Nineteen patients with IgD MM were diagnosed. Of those patients, the medical records of 16 were available for review. RESULTS: Most of the patients were male (11/16) with a median age of 59 years. The most common presenting features included bone pain (56%), gastrointestinal discomfort (38%), general malaise (38%), and body weight loss (25%). The majority of patients had cytopenia (88%), renal function impairment (75%), hypercalcemia (63%), hyperuricemia (69%), elevated beta 2-microglobulin levels (86%), and Bence Jones proteinuria (92%). Serum protein electrophoresis showed an M-peak in 9 cases (9/12), whereas immunoelectrophoresis or immunofixation identified an IgD monoclonal gammopathy in all 16 patients. All of the M-proteins were of a lambda-light chain type. The IgD level ranged between 584 and 129,000 IU/ml (normal, < 100 IU/ml). All the patients had stage III disease except one (stage I). Four patients were still alive at the time of this analysis. The median survival time was 12 months. Infection was the leading cause of death (50%). CONCLUSION: The present series showed that IgD MM had aggressive clinical features, male predominance, a high frequency of renal function impairment, high incidence of M-protein undetected by serum protein electrophoresis, a predilection for lambda-light chains, and a short period of survival.

Adult↗