摘要:

Serum soluble interleukin-6 receptor (sIL·6R) concentrations were measured in 50 patients with plasma cell dyscrasias using a commercially available immunoenzymatic assay kit. There were 40 patients with multiple myeloma (MM), 5 patients with monoclonal gammopathy of undetermined significance (MGUS), 3 patients with solitary plasmacytoma (SPC), 1 patient with chronic myelogenous leukaemia and multiple myeloma (CML + MM), and 1 patient with plasma cell leukaemia (PCL). We found that serum sIL-6R concentrations were higher in MM patients (62.53 ± 38.85 ng/ml) than in 20 normal volunteers studied (36.75 ± 13.79 ng/ml) (p < 0.01). The cut-off value of 65 ng/ml seen in 2 of our controls was arbitrarily taken as the upper limit of the control range for serum sIL-6R; according to this criterion, 14 patients with MM (35%), 1 patient with SPC, the unique patient with CML + MM, and the unique patient with PCL had elevated concentrations of the receptor. Patients with MGUS had normal sIL-6R values. In MM patients, serum sIL-6R levels correlated with the clinical phase of the disease: they were elevated in patients with early or late active disease and ranged within normal limits in patients with plateau-phase disease (p < 0.001). Thirteen of 27 patients with active MM had elevated serum sIL-6R values, i.e. 48.1%, but only 1 out of 13 patients with disease in the plateau phase, i.e. 7.7% (p < 0.05). Furthermore, in the entire group of MM patients, serum sIL-6R levels correlated with the concentrations of serum β2-microglobulin (p < 0.02), CRP (p < 0.01), ferritin (p < 0.01) and LDH (p < 0.01), while they did not correlate with disease stage, haemoglobin levels, proportion of marrow myeloma cells, the values of serum IL-6, the levels of serum albumin, or the grade of bone lesions. We conclude that elevated serum sIL-6R levels should be related to the growth of myeloma cells and suggest that serum sIL-6R concentrations may be used as an indicator of disease activ

ISSN:0001-5792    

DOI:10.1159/000203682

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

In Taiwan, as in other areas of Asia, the incidence of chronic lymphocytic leukemia (CLL) is low. A retrospective analysis was conducted to elucidate the clinicopathologic features of CLL patients in Taiwan. Of the 47 cases of CLL enrolled in this study, 45 were immunophenotyped as B-CLL; the other 2 were T-CLL. It was found that the lower the Binet and Rai stages of the B-CLL, the longer patients survive (p = 0.0131 and 0.0142, respectively). Univariate analysis showed that fatigue, splenomegaly, hepatomegaly and anemia are associated with poor survival with p values of 0.0203, 0.0184, 0.0001 and 0.0171, respectively. By multivariate analysis with Cox’s proportional hazard model, hepatomegaly and decrease in body weight were the two most significant predictors of survival. However, molecular parameters of κ or λ immunoglobulin (Ig) gene rearrangement or double allele rearrangement of Ig gene did not significantly increase the predictability of the progno

ISSN:0001-5792    

DOI:10.1159/000203683

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

We have determined the relative quantities of γ- and β-mRNAs and the α/β-mRNA ratios in 37 patients with β-thalassemia major with specific genotypes, namely 8 with a homozygosity for codon (CD) 39 (C→T), 7 with a homozygosity for IVS-I-110 (G→A), 5 with a homozygosity for IVS-I-6 (T→C), for 15 patients with compound heterozygosities for 2 of these 3 mutations, and for 2 patients with the IVS-I-110 (G→A)/-8 7 (C→G) mutations. None had an α-thalassemia. Twelve patients had thalassemia intermedia and the remainder, transfusion-dependent severe conditions. Differences in phenotype were observed for compound heterozygotes involving the IVS-I-6 (T→C) mutation in combination with either the IVS-I-110 (G→A) or the CD 39 (C→T) mutations: patients with thalassemia intermedia had a lower α/ β-mRNA ratio, about half of that of the patients with severe β-thalassemia major. This might suggest a higher β-mRNA synthesis in some patients than in others with the same genotype; mutations in promoter, enhancer, and/or locus control region sequences may be responsible for these differences. In vitro chain synthesis data were too incomplete to be helpful in this study. The RT-PCR procedure allowed the separation of abnormal (extended) mRNA from normal β-RNA in subjects carrying the IVS-I-110 (G→T) mutation. The relative quantities of this βTh-mRNA (% of βA+ βTh) were determined by scanning of the appropriate autoradiograms; they averaged 25% for homozygotes and about 4% for heterozygotes, indicating a consider

ISSN:0001-5792    

DOI:10.1159/000203684

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

The bactericidal activity of neutrophils depends primarily on free oxygen radicals released by the activation of NADPH oxidase when neutrophils are stimulated by microorganisms. Severe glucose-6-phosphate dehydrogenase (G6PD) deficiency is associated with decreased NADPH production. Increased susceptibility to recurrent bacterial infections in children with severe neutrophil G6PD deficiency as a consequence of decreased NADPH production has been reported earlier. In this study, the in vitro activity of neutrophils from normal and G6PD-deficient individuals was assessed by measuring the [14C]CO2 released via the hexose monophosphate shunt from radiolabeled [1-14C]-glucose and the nitroblue tetrazolium (NBT) dye reduction test. Our results show that the G6PD activity of neutrophils from 48 individuals, identified as severely erythrocyte (RBC) G6PD deficient ( < 2 U/1012 RBC) was 23% of the enzyme activity of neutrophils from 53 individuals with normal RBC G6PD levels (98.8 U/1012 RBC). However, the results of functional assays of neutrophils as measured by hexose monophosphate shunt and the NBT test were comparable in G6PD-deficient and normal individuals, suggesting that a reduced activity of G6PD to as low as 23% of normal does not affect neutrophil function.

ISSN:0001-5792    

DOI:10.1159/000203685

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Loss of a chromosome 7 is associated with a poor prognosis in acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS). Some studies have shown higher frequencies of monosomy 7 (-7) in dividing than nondividing myeloid cells, which might indicate that -7 confers a proliferative advantage on the host cell. As other groups have not been able to confirm this, we compared the -7 frequencies in bone marrow metaphases as studied with conventional cytogenetics and in interphase cells using primed in situ (PRINS) labeling. We found significantly higher -7 frequencies in metaphase than in interphase cells irrespective of diagnosis and presence or absence of additional chromosome aberrations. Further, we found a significant correlation between the -7 percentages in resting and dividing cells. Finally, as our material showed a clear male preponderance, Mitelman’s Catalog of Chromosome Aberrations in Cancer was searched for -7. Of 815 cases with AML or MDS, 491 (60.3%) were found to be men. To our knowledge, this is the first observation of a clear deviation from the 1:1 sex ratio in -7 patient

ISSN:0001-5792    

DOI:10.1159/000203686

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

A 55-year-old man with myelofibrosis was treated with natural α-interferon with a good hematologic response. Initially, he had anemia, leukocytosis, thrombocytosis and hepatosplenomegaly. A bone marrow biopsy showed replacement with fibrosis with an increase in megakaryocytes. Natural α-interferon (α-IFN) was started at a dose of 3 × 106 units/day. The leukocyte and platelet counts gradually normalized, and the liver and spleen decreased in size. However, the patient complained of a dry cough and dyspnea on the 61st treatment day, when the accumulated dose of α-IFN treatment had reached 1.8 × 108 units. He subsequently developed acute respiratory failure (PaO2 < 60 mm Hg) with bilateral lung infiltrations, suggesting the occurrence of interstitial pneumonitis associated with α-interferon therapy. Immediately, the α-interferon was discontinued and high-dose methylprednisolone (1.5g/day) was administered for 3 days. This treatment was followed by oral prednisone therapy. Steroid therapy brought about gradual improvement as suggested by a repeat radiograph. Since high levels of fibrogenic cytokines, such as PDGF and TGF-β, have been reported in patients with myelofibrosis, it is necessary to pay attention to interstitial pneumonia as a complication in α-IFN therapy for myel

ISSN:0001-5792    

DOI:10.1159/000203687

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Follicular cell carcinoma (FCC) of the thyroid is rarely found during childhood. We report a 12½-year-old girl with acute lymphoblastic leukemia, who developed rapidly growing FCC of the thyroid, 3 years after bone marrow transplantation. The role of chemotherapy in the induction of such secondary tumors after transplantation is discussed, and a proposal for the approach to these patients is suggested

ISSN:0001-5792    

DOI:10.1159/000203688

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

A case of acute renal failure, due to occlusion of renal vessels in a patient with acute promyelocytic leukemia (APL) treated with all-trans-retinoic acid (ATRA) and tranexamic acid has been described recently. We report a case of acute renal failure in an APL patient treated with ATRA alone. This case further supports the concern about thromboembolic complications associated with ATRA therapy in APL patients. The patient, a 43-year-old man, presented all the signs and symptoms of APL and was included in a treatment protocol with ATRA. After 10 days of treatment, he developed acute renal failure that was completely reversible after complete remission of APL was achieved and therapy discontinued. We conclude that ATRA is a valid therapeutic choice for patients with APL, although the procoagulant tendency is not completely corrected. Thrombotic events, however, could be avoided by using low-dose heparin.

ISSN:0001-5792    

DOI:10.1159/000203689

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

Primary renal non-Hodgkin’s lymphoma (NHL) with acute renal failure is a very rare condition, which frequently occurs in bilateral renal involvement. We report a 26-year-old male with primary bilateral renal NHL presenting with acute renal failure. A CT scan of the abdomen showed markedly enlarged kidneys with multinodularity and para-aortic lymphadenopathy. A percutaneous renal biopsy demonstrated ‘follicular center lymphoma, diffuse, small cell’. Thirteen cycles of systemic chemotherapy with cyclophosphamide-doxorubicin-vincristine-prednisolone (CHOP) and ifosfamide-methotrexate-etoposide-bleomycin (IMVP-Bleo) regimens were administered, which resulted in normalization of renal function with improvement of renal lymphoma. Since there was no further change of renal lesions after initial partial remission, a follow-up renal biopsy was performed 10 months after diagnosis, and no residual lymphoma was

ISSN:0001-5792    

DOI:10.1159/000203690

出版商:S. Karger AG    

年代:1997

数据来源: Karger

摘要:

The present report concerns a case of hepatocellular carcinoma (HCC) with marked hyperfibrinogenemia. The plasma fibrinogen level reached as high as 1,704 mg/dl. Since treatment against HCC resulted in reduction of plasma fibrinogen and PIVKA-II, an HCC marker, the hyperfibrinogenemia appears to be related to HCC. Immunohistochemically, the HCC specimen from this patient reacted strongly with antiserum to human fibrinogen, suggesting that the elevated fibrinogen was due to synthesis of this protein by the carcinoma cells, not to decreased fibrinolytic activity.

ISSN:0001-5792    

DOI:10.1159/000203691

出版商:S. Karger AG    

年代:1997

数据来源: Karger