摘要:

Multiple myeloma (MM) is a disease of terminally differentiated B lineage cells and thus alterations in circulating B cells may be anticipated. We studied peripheral blood B cells by flow cytometry in 45 untreated MM patients and compared the results to 25 age-matched controls. The total lymphocyte count and the absolute number and percentage of CD20+ cells were significantly decreased in MM patients. Analysis of the relative amounts of CD20+ cells expressing surface immunoglobulin κ or λ light chain isotype did not show either clonal B cell excess or light chain isotype suppression. The percentages of CD10+ and CD20+10+ cells were low both in MM patients and in controls. We consider that the CD20+ cells analysed in this study mainly consisted of normal polyclonal B cells. However, the percentage of the CD20+ cells in the peripheral blood of MM patients was a prognostic factor for survival, both as a continuous and as a dichotomized variabl

ISSN:0001-5792    

DOI:10.1159/000204253

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Three patients with paroxysmal nocturnal hemoglobinuria (PNH) and severe anemia were treated with high-dose recombinant human erythropoietin (rHEpo) and low-dose corticosteroids. During therapy their hemoglobin levels gradually improved and no blood transfusions were required. Neither rHEpo nor corticosteroids caused any side effects. This study shows that high rHEpo and low corticosteroid doses may improve the anemia of PNH patients.

ISSN:0001-5792    

DOI:10.1159/000204254

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

The family investigated showed the presence of multiple genetic disorders among their members. The presumable defective genes were related to coagulation factor X, familial hypercholesterolemia, mitral valve prolapse, and hypertrophic cardiomyopathy. The parents were offspring of two siblings, and their children comprised a nonidentical twin. While the proband demonstrated factor X deficiency, obstructive hypertrophic cardiomyopathy, and primary hypercholesterolemia, her parents and her elder sister were considered heterozygous for factor X. In addition, her mother had elevated plasma cholesterol levels, and her father primary hypercholesterolemia as well as mitral valve prolapse. The twin sister, heterozygous for factor X deficiency, displayed mitral valve prolapse. The clustering of defective genes in this family was considered to represent ‘coincidental’ occurrences rather than being linked to each ot

ISSN:0001-5792    

DOI:10.1159/000204255

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Primary lymphomas of the spermatic cord (LSC) are rare and have only been described in 10 cases in the literature. The present study is a review of the clinicopathological features of LSC described in the cases reported in the literature and presents a new case. LSC is a tumour affecting middle-aged men and is frequently misdiagnosed as an inguino-scrotal hernia. It is often of high malignancy grade. Stage I LSC has a poor prognosis when treated only locally.

ISSN:0001-5792    

DOI:10.1159/000204256

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 57-year-old woman affected with Sjögren’s syndrome without bleeding history developed spontaneous hematomas at the arms, the left foot and the thigh, cutaneous hemorrhages and hematuria. Routine coagulation tests showed a prolongation of activated partial thromboplastin time associated with a marked reduction of factor VIII activity (VIII :C 5%). Other deficiencies of blood coagulation factors, especially von Willebrand factor, were excluded. Measurement of factor VIII inhibitor revealed an activity of 26.4 Bethesda units/ml. These findings were consistent with the diagnosis of acquired hemophilia A due to the presence of a factor VIII inhibitor. The patient was treated with a combination of prednisone and azathioprine. The therapy led, in a few months, to a significant reduction of factor VIII: C inhibitor and she did not require replacement therapy. Furthermore, there was a complete remission of the bleeding tendency. Long-term therapy for about 3 years induced the complete disappearance of the inhibitor and a full normalization of coagulation tes

ISSN:0001-5792    

DOI:10.1159/000204257

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Primary lymphoma of the scrotum is rare. We report a case of primary immunoblastic lymphoma of the scrotum in a homosexual who was negative for antibodies to human immunodeficiency virus. Immunoperoxidase stains of paraffin-embedded tissue were consistent with a T-cell phenotype. Although complete remission was initially induced with surgical extirpation of the tumor and combination chemotherapy, isolated skin relapse developed which again responded to chemotherapy.

ISSN:0001-5792    

DOI:10.1159/000204258

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We report a case with refractory anaemia terminating in an acute leukaemia, which showed from the very beginning an intense eosinophilia that lasted for the whole disease, and in which the eosinophilic metaphases, as documented by the ‘Morphology, Antibody, Chromosome’ technique, were normal. An unusual karyotypic anomaly in the setting of a myelodysplastic syndrome could only be detected in the neutrophilic series. A general approach to detect structural aberrations of specific human chromosomes in metaphase cells by chromosomal in situ suppression hybridization of DNA libraries from sorted human chromosomes has been applied for chromosomes 11,3 and 2, in order to identify an extra copy of chromosom

ISSN:0001-5792    

DOI:10.1159/000204259

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Essential thrombocythemia (ET), one of the chronic myeloproliferative disorders, is a clonal disorder of multipotent stem cells. Although most patients with ET have a prolonged benign course, a minority of patients may develop a blastic crisis similar to chronic myelogenous leukemia (CML). A case of ET terminating in blastic crisis 8 years after the initial diagnosis is presented. The blast cells were cytochemically and immunophenotypically consistent with the acute myelogenous leukemia with minimal myeloid differentiation subtype of the FAB classification. From the review of the literature on blastic transformation of ET, acute leukemia with an M4 or M7 phenotype occurred more frequently. In addition, three valuable factors to predict the leukemic transformation of ET appear to be karyotypic abnormalities, such as involvement of chromosome 21, previous therapies with a mutagenic potential, and the capability of bone marrow cells to form in vitro spontaneous colonies as in CML.

ISSN:0001-5792    

DOI:10.1159/000204260

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Transverse grooves of the nails, designated as Beau’s lines, were observed in a patient with malignant lymphoma given chemotherapy. Beau’s lines disappeared in the treatment-free intervals. This observation supports the concept that these lines are the result of the suppressed growth of the nail matrix caused by antimitotic dr

ISSN:0001-5792    

DOI:10.1159/000204261

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 57-year-old man with IgG multiple myeloma developed pulmonary infiltration caused by pulmonary amyloidosis, for which continuous transdermal dimethyl sulfoxide (DMSO) treatment was given. After 8 weeks from the start of DMSO treatment, a dramatic reduction of pulmonary infiltration as determined by chest roentgenogram was observed, and arterial blood gas levels were improved. No serious side effect of DMSO was encountered. We conclude that a therapeutic trial with transdermal DMSO administration brought about a marked regression of the pulmonary infiltrate.

ISSN:0001-5792    

DOI:10.1159/000204262

出版商:S. Karger AG    

年代:1994

数据来源: Karger