[Schnitzler syndrome--report on a fourteen-year course of the disease and an overview of information on the disease]. / Schnitzleruv syndrom--popis ctrnáctiletého prubehu nemoci a prehled informací o této nemoci.

Schnitzler syndrome is a rare disease characterised by chronic urticaria and the presence of monoclonal IgM immunoglobulin, and by other symptoms. We report ourexperience with 14-year treatment of a patient. The first medical examination in our workplace was at the beginning of 1995 and the patient was diagnosed with the disease in 1996 (at the age of 52). Antihistaminics, the first medication used to relieve the symptoms of urticaria, had no subjective or objective effect. After the detection of osteolytic-osteosclerotic changes in the pelvic region, in areas with intense pain, we started treatment with pamidronate (90 mg at 28-day intervals), and the pain disappeared completely within 3 months of application of the drug. When the bisphosphonate therapy was interrupted, the pain recurred and receded completely after renewal of bisphosphonate administration. After the diagnosis, we gave the patient high doses of dexametazone (40 mg day 1-4, 10-13 and 20-23, at 28-day cycles). However, the therapy suppressed urticaria only on the days dexametasone was administered and the effect did not last when the drug was discontinued. Therefore we moved to continuous daily doses of prednisone (10-30 mg, depending on the intensity of problems), which was the only therapy with a long-term effect which was relatively well tolerated at the same time. Based on the excellent effect of 2-chlordeoxyadenosine in Waldenström's macroglobulinaemia, three cycles of this therapy were administered to the patient in 1996 (0.1 mg/kg/day, 7 days, at 28-day intervals). After the first infusion, urticarious lesions disappeared, but the positive effect on skin eruptions was limited in time and lasted only 14 days after the last infusion, i.e. the medication proved ineffective from a long-term point of view. The first improvement lasting for a longer period of time (partial remission) was achieved by regular application of interferon alpha (3 QU 3 times a week). However, adverse effects of interferon alpha prevailed after two years and the therapy was discontinued. Similarly phototherapy using the PUVA method resulted in partial regression of urticarious symptoms. Subsequently tested cyclosporine A (5 mg/kg/day) brought no benefit. Thalidomide (100 mg in the evening) administered on a continuous basis relieved pruritus and improved sleep disturbed by pruritus. However, adverse effects prevailed after 4 months and the therapy had to be discontinued, too. In 2005, we were hoping to achieve positive results with the most effective treatment for multiple myeloma of the time, a combination of bortezomib (1.3 mg/m2 i.v. on day 1, 4, 8 and 11, thalidomide 100 mg daily and dexametazon 20 mg p.o. on days 1-4 and 8-11 in 21-day cycles --VTD). A total of 4 complete cycles and 4 cycles with bortezomib reduced by 50% were applied. Urticarious eruptions were reduced by at least 50% in the course of the therapy, and also the concentration of monoclonal immunoglobin decreased temporarily by more than 50%. However, after the therapy was discontinued, the symptoms returned with their original intensity, which means that VTD regime did not provide a long-term therapeutic response. In 2007, we started the anakinra (Kineret) therapy. Skin symptoms disappeared after the first injection and a dose of 100 mg/ day has kept the patient free of skin symptoms for 12 months by now. Also the CRP value which had been constantly high returned to normal, and haemoglobin values increased to achieve physiological range. In the course of 14 years, we confirmed partial therapeutic effect of glucocorticoids administered on a continuous basis, as well as a partial therapeutic effect of interferon alpha, thalidomide and PUVA, but all the therapies had to be discontinued due to adverse effects. A major turn, i.e. the complete disappearance of skin symptoms and normalisation of CRP and haemoglobin values, only came with anakinra which has become the drug of the first choice for the above syndrome.

Cervical cancer among Vietnamese women: efforts to define the problem among Houston's population.

INTRODUCTION: Asian American/Pacific Islanders (AAPIs) in the United States (US) continue to bear a disproportionate burden of cancer. This report focuses on the interviews with local health-care delivery providers, directors, administrators, and community outreach liaisons in the Vietnamese community in Houston, Texas. METHODS: The Center for Minority Health (CMRH) at the University of Texas M.D. Cancer Center interviewed 17 leaders, as defined above, to identify factors that have a negative impact on screening practices among Vietnamese women. RESULTS: The results show that some of the barriers to cervical cancer screening include: lack of knowledge, lack of female physicians, language barriers, lack of insurance, and embarrassment. Interviews established that "prevention" is a Western concept that the Vietnamese community has not yet adopted. Vietnamese women used their churches, community physicians (licensed or not), circle of friends, and families for their health information. Many patients used herbs as alternative or complementary therapies. CONCLUSION: Cultural factors play a vital role in limiting Vietnamese women in the use of cervical screening. Further research needs to focus on identifying specific barriers and how they can be overcome.

[Detection of myeloma cells in peripheral blood using multi-parameter flow cytometry: monitoring residual disease]. / Detekce myelomových bunek v periferní krvi vícerozmernou prutokovou cytometrií: monitorování zbytkové nemoci.

BACKGROUND: The aim of this study was to evaluate significance of CD 38(+2)45-54+, CD 38(+2)45-56+ and CD 38(+2)45-138+ cells counts in peripheral blood of patients with multiple myeloma for monitoring of the minimal residual disease. METHODS AND RESULTS: A triple-color flow cytometric analysis was used for this purpose. Peripheral blood of 29 patients with multiple myeloma who underwent high dose chemotherapy and autologous stem cells transplantation was repeatedly analysed. Counts of myeloma cells in peripheral blood were compared to serum monoclonal immunoglobulin concentration, serum calcium level, serum C-reactive protein, serum beta 2 microglobulin, and number of myeloma cells in bone marrow (morphology). From 29 patients in this study, 5 patients have relapsed. Patients in relapse had significantly higher counts of CD 38(+2)45-54+, CD 38(+2)45-56+ and CD 38(+2)45-138+ cells in peripheral blood than patients in remission (geometrical average: 12.41; 6.20; 14.45 cells/microliter versus 4.08; 2.87; 2.58 cells/microliter). The number of these cells correlated well with serum monoclonal immunoglobulin level and counts of myeloma cells in bone marrow. CONCLUSION: The authors conclude that the longitudinal multi-color flow cytometric analysis of CD 38(+2)45-54+, CD 38(+2)45-56+ and CD 38(+2)45-138+ cells in peripheral blood of patients with multiple myeloma is a useful method for evaluation of the disease activity. Significance of peripheral myeloma cells count for prediction of the relapse of the multiple myeloma remains to be evaluated.

New Bet v 1 isoforms including a naturally occurring truncated form of the protein derived from Austrian birch pollen.

Bet v 1, the major pollen allergen from white birch, displays a considerable degree of heterogeneity. Until now, all molecular and immunological characterization studies of Bet v 1 isoforms have been performed with commercially available pollen of Swedish origin. In regard to clinical studies with Austrian birch pollen allergic individuals, knowledge about the isoform repertoire in Austrian birch pollen was necessary. cDNAs coding for Bet v 1 isoforms from Austrian birch pollen were cloned by PCR amplification and sequenced. Besides the Austrian variants of the Swedish isoforms Bet v 1a (62% of the clones), ALK167 (4%), and Bet v 1d/h, Bet v 1g, and Bet v 11 (24%), three sequences with a significantly lower homology to known isoforms and two Bet v 1a-homologous sequences with a 7 bp insertion coding for a truncated protein were detected. No Austrian variants of the majority of the Swedish isoforms were found. The isoforms coding for truncated proteins were expressed in Escherichia coli and tested by immunoblotting. They bound a polyclonal anti-Bet v 1 antibody but did not recognize birch pollen allergic patients' serum IgE and two Bet v 1-specific monoclonal antibodies. The similarity of the Bet v 1 isoform patterns of Swedish and Austrian birch pollen justifies the use of Bet v 1 derived from Swedish pollen for clinical studies with birch pollen allergic individuals from outside Northern Europe.

Conserved sequence motifs of CDR3 loops of TCR specific for two major epitopes of the grass pollen allergen PhI p 1.

We analyzed the cDNA sequence data of complementarity determining regions (CDR3) of epitope mapped alphabeta TCR of T cell clones (TCC). The TCC were specific for the major timothy grass (Phleum pratense) pollen allergen Phl p 1 and were derived from the peripheral blood of seven unrelated grass pollen-allergic individuals. Each TCR recognized one of two immunodominant T cell epitopes, PP73 or PP103, of Phl p 1. Although a diversity of recombined V and J segments was observed, amino acid motifs as long as five residues were conserved among CDR3 loops of TCR from TCC of different atopic individuals specific for the same peptide. The conserved sequences could comprise as much as 60% of the CDR3. All amino acid residues of the motifs of the CDR3beta and most of the CDR3alpha of all TCR used in this study were encoded by randomly added nucleotides. This indicates that they were specifically selected for by the peptide bound to the MHC class II molecule. For one selected patient, a larger number of TCC, specific for PP103, was analyzed. The TCR repertoire was limited to three different TCR. The same MHC class II molecule, DRB1*1301, was identified to present PP103 to each of the three TCR.

Sequence comparisons of the CDR3 hyper-variable loops of human T cell receptors specific for three major T cell epitopes of the birch pollen allergen Bet v 1.

We have analysed the T cell receptor (TCR) alpha and beta chain sequences of 16 human CD4+ T cell clones (TCCs) specific for three important epitopes of the major birch pollen allergen Bet v 1. The TCCs were raised from the peripheral blood of eight patients with birch pollen allergy, showing allergic rhino-conjunctivitis and allergic asthma. The TCCs from these individuals were specific for Bet v 1-derived peptides: amino acids (aa)77-92 (epitope 1), aa93-108 (epitope 2) and aa113-126 (epitope 3). The DNA sequence analysis of the TCRAV and BV regions revealed heterogeneous repertoires for recognition of the peptides. Multiple combinations of AV/AJ and BV/BJ were used. However, some inter-individual restriction was evident. A limited selection of AVS and the normally infrequently used BV1S4 was obvious in TCCs specific for epitope 1. The TCRBV13 was more frequent in TCCs recognizing epitope 3. A very narrow distribution in length could be seen in the CDR3 sequences of the beta chain of TCRs with specificity for epitopes 1 and 2. Inter-individual positional micro-restriction was observed for the aa motif LR in the tCDR3 (epitope 1), for the aa residue M in the alphaCDR3 and for the aa residue G in the betaCDR3 (epitope 3). Our results illustrate clearly that each antigenic peptide derived from a single allergen, is capable of selecting different characteristics in the responding repertoire of TCRs, thus increasing the complexity of allergen-recognition by T lymphocytes. Therefore, our findings limit the potential use of TCR targeted therapeutical strategies in Type I allergy.

[Subcutaneous chamber systems (ports) for long-term care in cancer patients]. / Podkozní aplikacní komurkový systém ("port") pri dlouhodobé lécbe onkologicky nemocných.

Maintenance of satisfactory and safe venous access in cancer patients is part of a comprehensive care of cancer patients. As the Hickman/Broviac catheter is today used an implantable port. This port is placed in a subcutaneous pocket. The catheter connected to the port leads into the central vein, usually through the subclavian vein. An application of drugs in the port is done trough the skin. In this time could be used different types of ports, one or two chambers port, low profil port, peripheral port and others. In this paper we reported our two-years experience with 33 totally implantable access systems, which has been implanted in patients with cancer in Masaryk University Hospital Brno Bohunice. In our department was most frequently used port the nonmetallic port IMPLANTOFIX (BRAUN MELSUNGEN). All ports were in place for a total of 5,582 days, in average of 169 days. A frequency of complications were 2.15 on 1,000 days. Four ports were in place for longer than 1 year. A comparison of the incidence of complications in the present study with an analysis of 23 studies reported in literature was satisfactory. Minimal maintenance care, no restriction of life activity, improving quality of life and probably less frequency of infection complications, identify it as a significant advantage for the satisfactory maintenance of venous access of oncology patients in comparison with Hickman/Broviac catheter. Both the patients and the nursing staff showed a very high degree of satisfaction with this system. In cancer patients, especially with poor venous access, and prognosis longer than 6 months can be indicated this system with the advantage for patients.