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1.
Sci Rep ; 12(1): 18374, 2022 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-36319704

RESUMEN

An evidence-based treatment for a Multiple Sclerosis (MS) relapse is an intravenous administration of 3-5 g of Methylprednisolone. In case of insufficient effect or corticosteroids intolerance, the therapeutic plasma exchange (TPE) is indicated. To assess the clinical effect of TPE in treatment of relapse in patients with relapsing-remitting MS (RRMS), we enrolled 155 patients meeting the following criteria (study period: January 2011 to February 2021): (1) age > 18, (2) RRMS according to the McDonald´s 2017 criteria, (3) MS relapse and insufficient effect of corticosteroids/corticosteroids intolerance, (4) baseline EDSS < 8. Exclusion criteria: (1) progressive form of disease, (2) history of previous TPE. Following parameters were monitored: EDSS changes (before and after corticosteroid treatment, before and after TPE; EDSS after TPE was assessed at the next clinical follow-up at the MS Center), and improvement of EDSS according to the number of procedures and baseline severity of relapse. 115 females (74%) and 40 males (26%) were included. The median age was 41 years (IQR 33-47)-131 patients underwent the pulse corticosteroids treatment and TPE, while 24 patients underwent only TPE without any previous corticosteroid treatment. Median baseline EDSS was 4.5 (IQR 3.5-5.5), median EDSS after finishing steroids was 4.5 (IQR 4.0-5.5). EDSS prior to the TPE was 4.5 (IQR 4-6), EDSS after TPE was 4.5 (IQR 3.5-5.5). We observed a significant improvement in the EDSS after TPE (p < 0.001). Sex differences were seen in TPE effectiveness, with median improvement of EDSS in females being -0.5 (IQR 1-0) and in males being 0 (IQR -0.5 to 0), p = 0.048. There was no difference in EDSS improvement by age category: 18-30 years, 31-40 years, 41-50 years, > 50 (p = 0.94), nor by total TPE count (p = 0.91). In this retrospective study of patients with an aggressive relapse and insufficient effect of intravenous corticosteroid treatment, a significant effect of TPE on EDSS improvement was observed. There was no significant difference in TPE effectivity according to the number of procedures, age, nor severity of a relapse. In this cohort, TPE was more effective in females.


Asunto(s)
Esclerosis Múltiple Recurrente-Remitente , Esclerosis Múltiple , Humanos , Femenino , Masculino , Adulto , Persona de Mediana Edad , Adolescente , Adulto Joven , Esclerosis Múltiple/tratamiento farmacológico , Intercambio Plasmático/métodos , Estudios Retrospectivos , Esclerosis Múltiple Recurrente-Remitente/tratamiento farmacológico , Recurrencia , Corticoesteroides/uso terapéutico
2.
Bratisl Lek Listy ; 122(3): 179-183, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33618525

RESUMEN

AIM: It remains unclear, why only some patients form alloantibodies against foreign RBC antigens. Transfusion of red blood cell (RBC) products and pregnancy are the most relevant causes of immunization against RBC alloantigens. Here we investigated the relationship between RBC alloantibodies, Rh phenotype, and HLA phenotype among patients with multiple RBC alloantibodiesMETHODS: In a group of 124 multi-responders ‒ including both pregnant women and transplant recipients ‒ we analysed the distribution of HLA-Class II variants in subgroups of multi-responders to RBC alloantigens according to their Rh status. RESULTS: As expected, the RhD-negative phenotype was overrepresented in our alloimmunized group (49.2 %) compared to in the general population. Importantly, HLA-DRB1*15 carriers were significantly overrepresented among D-negative multi-responders compared to D-positive multi-responders (Pc = 0.045). Furthermore, the linked HLA-DRB1*13, HLA-DQB1*06, and HLA-DQA1*01 variants were more frequent in individuals with the DCCee phenotype than in other RhD-positive phenotypes. CONCLUSION: Our present findings showed that RBC multispecific alloimmunization was associated with particular HLA-Class II variants based on Rh status (Tab. 3, Ref. 22).


Asunto(s)
Eritrocitos , Isoanticuerpos , Femenino , Cadenas HLA-DRB1/genética , Humanos , Inmunización , Fenotipo , Embarazo
3.
Klin Onkol ; 30(4): 282-288, 2017.
Artículo en Checo | MEDLINE | ID: mdl-28832174

RESUMEN

AIM: The aim of this study was to evaluate overall survival (OS) and prognostic factors in patients ineligible for chemotherapy who were treated with a hyperfractionated accelerated schedule with simultaneous integrated boost. MATERIAL AND METHODS: From May, 2008, to April, 2013, 122 patients with locally advanced nonmetastatic squamous laryngeal (14%), hypopharyngeal (30%), oropharyngeal (30%), and oral cavity (27%) cancer were treated at our institution. The median age, Karnofsky Performance Status (KPS), and gross tumor volume (GTV) of the patients were 63 years (range, 46-87 years), 80% (range, 50-100%), and 46 ml (range, 5-250 ml), resp. The median total dose of radiotherapy was 72.6 Gy (range, 62-77 Gy) at 1.4-1.5 Gy per fraction, and 55 Gy at 1.1 Gy per fraction was delivered for GTV (primary and lymphadenopathy) with a margin of 0.7 cm and regional lymphatic areas with a margin of 0.3 cm. The dose was delivered 2× a day, with a 6-8 hour interval between doses, via a 6 MeV linear accelerator. OS was estimated using the Kaplan-Meier method, and predictors of OS were analyzed using Cox proportional hazards regression. RESULTS: The median duration of the radiotherapy series was 37 days (range, 32-45 days). The incidence of grade 3 acute toxicity was 62% for mucosa (oral cavity and/or pharynx) and 0% for skin. Confluent mucositis cleared in all cases within 21 days. No grade 4 or 5 toxicities were recorded. PEG was introduced before treatment in 55 patients (45%). The 1-and 2-year OS was 65% and 32%, resp. KPS less than 80% (RR 2.4, 95% CI 1.3-4.2; p = 0.004), cancers other than oropharyngeal or laryngeal cancer (RR 2.0, 95% CI 1.1-3.5; p = 0.016), and capacity of high GTV (RR 1.006, 95% CI 1.001-1.011; p = 0.017) were found to be negative prognostic factors for OS. CONCLUSION: More than 30% of patients with poor prognosis survived for longer than 2 years. KPS before treatment was the strongest prognostic factor for better OS.Key words: head and neck cancer - radiotherapy dose fractionation - survival analysis - acceleration - hyperfractionation This work was supported by RVO-FNOs/2016 (HPV status as predictive and prognostic factor for primary and secondary head and neck cancer). The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.Submitted: 9. 3. 2017Accepted: 19. 4. 2017.


Asunto(s)
Fraccionamiento de la Dosis de Radiación , Neoplasias de Cabeza y Cuello/mortalidad , Neoplasias de Cabeza y Cuello/radioterapia , Radioterapia de Intensidad Modulada/métodos , Anciano , Anciano de 80 o más Años , Neoplasias de Cabeza y Cuello/patología , Humanos , Estimación de Kaplan-Meier , Persona de Mediana Edad , Pronóstico , Radioterapia de Intensidad Modulada/efectos adversos , Resultado del Tratamiento
4.
Klin Onkol ; 30(1): 13-19, 2017.
Artículo en Checo | MEDLINE | ID: mdl-28185460

RESUMEN

Monoclonal antibodies represent a standard part in the treatment of oncologic patients, but their efficacy in multiple myeloma used to be unsatisfactory. Daratumumab monotherapy was approved by the American FDA in 2015, after unprecedented results were obtained in a heavily pre-treated group of patients. In 2016 daratumumab was approved in combination with lenalidomide and dexamethasone, or bortezomib and dexamethasone, for the treatment of myeloma patients who have received at least one prior therapy.The toxicity of the drug is low, and is dominated by infusion-related reactions in more or less half of patients. The development as well as the management of these sometimes urgent reactions is described in depth in this review. As multiple myeloma is characterized by the presence of paraprotein (monoclonal antibody) and CD38 is a ubiquitous antigen, several unexpected complications have been reported during the administration of the drug. In this review, we aim to describe and offer some solutions for the complications that may be encountered during daratumumab treatment, such as interference with serum protein electrophoresis and immunofixation assays that may confuse the assessment of the hematological response, interference with blood compatibility testing that may cause a delay in the delivery of compatible transfusions, and difficulties that may occur in flow cytometric analysis of minimal residual disease. Because of the high activity of daratumumab and its expected widespread use, clinicians should be aware of its side effects and their management. It is also very important to inform colleagues in clinical laboratories about the initiation of daratumumab treatment in particular patient.Key words: multiple myeloma - daratumumab - infusion related reaction - flow cytometry - transfusionThis work was supported by the Czech Ministry of Education, Youth and Sports (project no. IRP- 201550) and by the Czech Ministry of Health (15-29667A).The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.Accepted: 22. 8. 2016Submitted: 12. 5. 2016.


Asunto(s)
Anticuerpos Monoclonales/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Mieloma Múltiple/tratamiento farmacológico , Anticuerpos Monoclonales/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Tipificación y Pruebas Cruzadas Sanguíneas , Electroforesis de las Proteínas Sanguíneas , Bortezomib/administración & dosificación , Dexametasona/administración & dosificación , Citometría de Flujo , Humanos , Lenalidomida , Talidomida/administración & dosificación , Talidomida/análogos & derivados
5.
Vox Sang ; 112(2): 156-162, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-28052334

RESUMEN

BACKGROUND AND OBJECTIVES: Alloimmune antibodies against red-blood-cell (RBC) antigens induced in susceptible individuals (responders) by transfusion, pregnancy or transplantation may have serious clinical consequences. The aim of this study was to investigate association of alloimmunization against selected RBC antigens with HLA-Class II. MATERIALS AND METHODS: A total of 230 responders (106 monoresponders and 124 multiresponders) were enrolled into the study. HLA-DRB1 and HLA-DQB1 variants were determined by PCR-SSO and their frequencies compared between the patients (patient subgroups) and 375 ethnically and regionally matched controls. RESULTS: Development of multiple RBC antibodies was associated with HLA-DRB1*15 and HLA-DQB1*06 allelic groups in the patients, with the relationship being particularly apparent in those with anti-C+D antibodies. Furthermore, DRB1*13 and DQB1*06 were more frequent in multiresponders with anti-E+c antibodies and DRB1*03 and DQB1*02 in those with anti-E+Cw. CONCLUSION: For the first time, we confirmed the association of HLA-DRB1*15 with RBC antibody multiresponder status and found HLA-Class II associations for three frequent RBC antibody combinations. Our data support the concept that HLA restriction plays an important role in the response to RBC alloantigens.


Asunto(s)
Eritrocitos/inmunología , Cadenas beta de HLA-DQ/inmunología , Cadenas HLA-DRB1/inmunología , Adulto , Alelos , República Checa , Femenino , Frecuencia de los Genes , Genotipo , Cadenas beta de HLA-DQ/genética , Cadenas HLA-DRB1/genética , Haplotipos , Humanos , Isoanticuerpos/sangre , Masculino , Embarazo
6.
Epidemiol Mikrobiol Imunol ; 65(2): 112-7, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27467328

RESUMEN

BACKGROUND: The purpose of the study was to evaluate new 3rd-generation test kits, EIA Borrelia recombinant IgM and IgG (TestLine, Brno, Czech Republic), in serum and cerebrospinal fluid (CSF) of children with Lyme neuroborreliosis. METHODS: Comparison of three tests was used: the whole-cell EIA from Borrelia garinii (EIA 1) was compared with the EIA using recombinant antigens (EIA 2) and immunoblot. In total, 364 samples of serum and CSF were examined. Eighty-six paired sera and CSF samples were evaluated in the first group of children with Lyme neuroborreliosis. The second group consisted of 30 children with probable Lyme neuroborreliosis. Sixty-six samples from children with neuroinfections other than borrelial etiology were used as controls. RESULTS: In the first group of children with proven LNB, EIA 2 gave significantly more positive results for IgG in serum (P = 0.006; OR = 7.5) as in CSF (P < 0.001; OR = 4.5). There was no statistically significant difference in the IgM positivity of serum (P = 0.54; OR = 0.71). EIA 2 determined significantly (P = 0.001; OR = 0.06) less positive results of IgM in CSF in the LNB patients. IgG antibody index (AI) assessed by both methods revealed similar results (P = 0.646; OR = 1.38). Both methods are comparable, but IgM AI assessed by EIA 2 showed significantly less positive results (P < 0.001; OR = 0.04).The differences in the detection of positive IgM/IgG antibodies in serum and CSF did not reach statistical significance either in the groups of children with excluded LNB or in controls. CONCLUSIONS: EIA 2 showed better results than EIA 1 and western blot for the detection of positive IgG antibodies in serum and CSF. The difference in the calculation of AI IgG by EIA 1 and EIA 2 was not noticeable in the group of LNB patients. Comparing IgG and IgM AIs calculated from both tests, the sensitivity for EIA 2 was 68% for IgG and 26% for IgM. The specificity is 100% for both tests.


Asunto(s)
Anticuerpos Antibacterianos/líquido cefalorraquídeo , Antígenos Bacterianos/inmunología , Inmunoglobulina G/líquido cefalorraquídeo , Inmunoglobulina M/líquido cefalorraquídeo , Neuroborreliosis de Lyme/diagnóstico , Técnicas Bacteriológicas , Niño , Humanos , Técnicas para Inmunoenzimas
7.
Epidemiol Infect ; 144(12): 2597-604, 2016 09.
Artículo en Inglés | MEDLINE | ID: mdl-27180603

RESUMEN

To examine evidence of positive antibodies against immunogenic proteins of Anaplasma phagocytophilum in patients with other tick-borne infections and to diagnose possible co-infections, 412 serum specimens were tested by immunoblotting using three specific Anaplasma antigens: surface proteins p44 and Asp62 and outer membrane protein A (OmpA). In total, 284 serum samples from children with Lyme borreliosis and 12 serum samples from children with tick-borne encephalitis were tested. Sera from patients with viral aseptic meningitis (n = 47) and from blood donors (n = 69) were used as controls. Among all serum specimens from patients with tick-borne infections submitted for this study, six samples (2·0%) showed positive IgM reactions and seven samples (2·4%) were IgG positive for A. phagocytophilum by immunoblot. Borderline reactivity was found in 30 samples (10·14%) for IgM and 36 samples (12·2%) for IgG. The difference between patients and blood donors was statistically significant for IgM (P = 0·006) and for IgG (P = 0·0007) antibodies. A statistically significant result was obtained for IgG (P = 0·02) but not for IgM between patients and children with aseptic meningitis. Immunoblot using three specific antigens provides novel information about the positivity of antibodies to A. phagocytophilum in children with other tick-borne infections. Taking into account clinical and laboratory findings of children despite antibody positivity, no case of human granulocytic anaplasmosis was demonstrated.


Asunto(s)
Anaplasma phagocytophilum/inmunología , Anaplasmosis/diagnóstico , Proteínas Bacterianas/inmunología , Coinfección/diagnóstico , Encefalitis Transmitida por Garrapatas/inmunología , Enfermedad de Lyme/microbiología , Adolescente , Anaplasmosis/inmunología , Anaplasmosis/microbiología , Anticuerpos Antibacterianos/sangre , Proteínas de la Membrana Bacteriana Externa/inmunología , Western Blotting , Niño , Preescolar , Coinfección/inmunología , Coinfección/microbiología , República Checa , Encefalitis Transmitida por Garrapatas/microbiología , Femenino , Humanos , Enfermedad de Lyme/inmunología , Masculino , Proteínas de la Membrana/inmunología
9.
Klin Mikrobiol Infekc Lek ; 20(1): 15-7, 2014 Mar.
Artículo en Checo | MEDLINE | ID: mdl-24960259

RESUMEN

Ocular complications are very frequent in leptospirosis and may develop in both the acute (bacteriemic) and the second (immunologic) phases of the disease. A delayed onset of these complications, even after months or years, is also possible. Keratoconjunctivitis and panuveitis belong to the most frequent signs. Targeted search for patients suspected of having leptospirosis and regular ophthalmological examinations even after cure of the acute illness may lead to early detection of ocular complications and to their specific treatment. Presented are a case report of complicated uveitis due to infection with Leptospira grippotyphosa and a literature overview.


Asunto(s)
Leptospira , Leptospirosis , Uveítis , Humanos , Leptospirosis/complicaciones , Leptospirosis/microbiología , Uveítis/etiología , Uveítis/microbiología
10.
Klin Onkol ; 27(2): 111-26, 2014.
Artículo en Checo | MEDLINE | ID: mdl-24739048

RESUMEN

BACKGROUND: Schnitzler syndrome is a very rare, acquired, autoinflammatory disease of mostly adult onset with characteristic combination of chronic recurrent urticaria and monoclonal immunoglobulin M or G gammopathy predisposing the patients to malignant lymphoproliferation. In this work, we analyzed the results of bio-logical therapy with anakinra on a national level aiming to supply data for effective pharmaco-economic estimates, lay the grounds of nationwide patient registry, raise awareness among professional public and optimize provided health care. PATIENTS AND METHODS: The retrospective study (10/ 2006- 9/ 2013) included six males with definite Schnitzler syndrome verified by the new Strasbourg criteria. All patients were pretreated with antihistamines, nonsteroidal antiinflammatory drugs and glucocorticoids. Four patients underwent two or more treatment lines including intravenous bisphosphonates, 2- chlorodeoxyadenosine (cladribine), interferonα, PUVA photochemotherapy, cyclosporine A, thalidomide, bortezomib, chlorambucil, cyclophosphamide, colchicine and methotrexate. Anakinra monotherapy was initiated in standard dosing (100 mg subcutaneously daily). RESULTS: Complete and partial remissions were achieved in five (83%) and one patients (17%), respectively. Complete remission was characterized by urticaria and pain regression (within hours), normalization of inflammatory markers (with--in days) and bone metabolism improvement assessed by the markers of osteoblastic osteoformation and osteoclastic osteoresorption in one case (within weeks). With normalized inflammatory markers (including interleukin6 and interleukin18), arthralgia and sporadic exacerbations of urticaria and fevers persist in the patient in partial remission with proven Q703K polymorphism in NLRP3 gene. The median treatment followup was 30.5 months (37.2 ± 31.2 (n = 6)). The dosing interval was prolonged in one case of complete remission to 48 hours. No serious adverse reactions occurred during anakinra application. CONCLUSION: In Schnitzler syndrome, anakinra represents an effective, verified and safe medication with potentionally longterm administration not compromising its original efficacy and subjective tolerance. Anakinra, blocking autonomous inflammatory reaction of the organism via interleukin1 pathway, is a generally accepted first line treatment that should be made available in standard dosing for all Schnitzler patients.


Asunto(s)
Antirreumáticos/uso terapéutico , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Síndrome de Schnitzler/tratamiento farmacológico , República Checa , Humanos , Inducción de Remisión , Estudios Retrospectivos , Síndrome de Schnitzler/diagnóstico , Síndrome de Schnitzler/inmunología
11.
Ceska Gynekol ; 78(2): 211-5, 2013 Apr.
Artículo en Checo | MEDLINE | ID: mdl-23710988

RESUMEN

TRALI is a major cause of serious morbidity and mortality associated with a blood transfusion. It is clinically manifested by acute respiratory distress within 6 hours of completion of transfusion. Neutrophils have the key role in the pathogenesis. They are activated mostly with leukocyte antibodies (HLA and granulocyte) that are present mainly in plasma containing blood products. TRALI is a clinical diagnosis based on hypoxemia and positive finding on lung X-ray examination. The treatment is only supportive and the mortality is about 5% to 10%. The major preventive measure is transfusing blood products from donors without leukocyte antibodies.


Asunto(s)
Lesión Pulmonar Aguda , Reacción a la Transfusión , Lesión Pulmonar Aguda/etiología , Lesión Pulmonar Aguda/inmunología , Lesión Pulmonar Aguda/mortalidad , Lesión Pulmonar Aguda/terapia , Humanos
12.
Vnitr Lek ; 59(4): 313-6, 2013 Apr.
Artículo en Checo | MEDLINE | ID: mdl-23711058

RESUMEN

Acquired haemophilia A is a rare form of acquired coagulopathy caused by autoantibodies against coagulation factor VIII. It is characterized by major bleeding complications often life- threatening. An estimated incidence is about 1- 2 cases per million inhabitants per year. The authors report case history of acquired haemophilia in 85 year old woman. Bleeding complications were well controlled by bypassing agents. Inhibitor eradication was successful after the use of second line agent rituximab.


Asunto(s)
Hemofilia A/diagnóstico , Anciano de 80 o más Años , Autoanticuerpos/sangre , Factor VIII/inmunología , Femenino , Hemofilia A/tratamiento farmacológico , Hemofilia A/inmunología , Humanos
13.
Vnitr Lek ; 59(2): 136-47, 2013 Feb.
Artículo en Checo | MEDLINE | ID: mdl-23461404

RESUMEN

UNLABELLED: Severe damage to the heart caused by AL amyloid deposits is a contraindication of high-dose chemotherapy with autologous haematopoietic stem cell transplantation. Severe heart damage caused by AL amyloid results in frequent life-threatening complications, even during the course of the classical chemotherapy treatment and it often makes keeping to the treatment schedule impossible. Scheduling heart transplantation before the treatment of AL amyloidosis will significantly improve the patients overall condition and enable them to undergo the intensive AL amyloidosis treatment with the hope that a long-term complete remission may be achieved. CASE DESCRIPTIONS: Transplantations of heart damaged by AL amyloid deposits were conducted in three patients; two men, age 48 and 54, and one woman, age 63. In the interval of 3-6 months from the heart transplantation before the scheduled AL amyloidosis treatment was initiated, an examination of bone marrow, the concentration of monoclonal immunoglobulin and free light chains was carried out. Both men had more than 10% of plasma cells in the bone marrow after the heart transplantation and the concentrations of the λ free light chains were pathologically increased. During the first-line therapy, autologous haematopoietic stem cells were harvested from peripheral blood after mobilizaton with granulocyte growth factor (filgrastim) at the dose of 5 µg/kg twice a day. During the administration of filgrastim until the end of the haematopoietic stem cell harvest, the combined immunosuppressive treatment was reduced and a corticosteroid dose was compensatory increased. The prophylactic antiviral drug valganciclovir was discontinued during the haematopoietic stem cell harvest. High-dose chemotherapy (melphalan 100 mg/m2) with autologous haematopoietic stem cell transplantation followed. In the interval from administering melphalan until the rise in neutrophil count over 2 x 109/l, antiviral prophylaxis was discontinued again, the immunosuppressive drug doses were reduced and corticoid doses were slightly increased. High-dose chemotherapy with melphalan at the of 100 mg/m2 was tolerated without major complications and without mucositis; however, in neither of the male patients did it lead to a complete haematological remission. Consequently, the second-line therapy followed using bortezomib combined with dexamethasone and also with cyclophosphamide or doxorubicin. One of these two patients reached a complete haematological remission after the bortezomib therapy; the values of free light chains were normal, immunofixation was negative, and clonal plasma cells were absent in the bone marrow. In the case of the other patient, the bortezomib therapy only induced partial remission. In this case, the third-line therapy followed, applying a combination of lenalidomide, dexamethasone and cyclophosphamide. This therapy significantly reduced the values of free light chains; however, their ratio remained pathological. To conclude, the latter response can be described as a very good partial remission. Both men currently show no signs of disease activity and are in a good clinical condition 28 and 30 months after the heart transplantation. The third heart transplantation, due to severe heart damage by AL amyloid deposits, was conducted in a woman aged 63. An examination of this woman three months after the heart transplantation showed that the original pathological values of free light chains became normal. The woman had approx. 8% of clonal plasma cells before the heart transplantation. Three months after the heart transplantation the bone marrow contained only 3% of polyclonal plasma cells. In this case, the immunosuppressive treatment with corticosteroids after the heart transplantation probably induced a complete haematologic remission. The woman is in a complete AL amyloidosis remission seven months after the heart transplantation. CONCLUSION: It was beneficial to perform the heart transplantation first and to initiate the AL amyloidosis treatment no sooner than three months after the heart transplantation in patients with severe heart damage caused by AL amyloid deposits. If the patients are in a good clinical conditions, autologous haematopoietic stem cells can be harvested after the heart transplantation and high-dose chemotherapy can be offered to the patients. If this intensive treatment does not induce remission, it is necessary to apply additional alternative treatments.


Asunto(s)
Amiloidosis/tratamiento farmacológico , Amiloidosis/cirugía , Trasplante de Corazón , Femenino , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Masculino , Persona de Mediana Edad
14.
Vnitr Lek ; 59(1): 37-58, 2013 Jan.
Artículo en Checo | MEDLINE | ID: mdl-23428001

RESUMEN

Until 2011, the gold standard of treatment for patients with AL amyloidosis was the combination of alkylating cytostatics (melphalan or cyclophosphamide) and dexamethasone. For a selected group of patients under 65 years of age with only moderate damage to their body caused by amyloid and with good cardiac function (EF> 40%), high-dose chemotherapy with autologous hematopoietic cell transplantation seems to be optimal. Patients with AL amyloidosis and low bone marrow plasma cell count generally undergo the harvest of hematopoietic cells from peripheral blood, followed by high-dose chemotherapy immediately after they are diagnosed. In contrast to multiple myeloma, high-dose chemotherapy is not preceded by several months of conventional treatment. The year 2012 witnessed a release of reports about extensive experience with new drugs that were used in Phase I and Phase II clinical trials, and in isolated cases also in Phase III, for the treatment of patients with AL amyloidosis. Based on these studies it can be concluded that among the new available drugs (bortezomib, thalidomide and lenalidomide) bortezomib is the drug with the greatest curative effect in patients with AL amyloidosis; it achieved 24-37% of complete remissions in monotherapy. The greatest number of treatment responses was reported during the treatment that combined bortezomib, alkylating cytostatics and dexamethasone. This treatment showed significantly more treatment responses during the first-line drug therapy than during therapies that followed. Clinical trials with lenalidomide combined with other drugs saw a lower number of treatment responses than the number described in treatment with bortezomib combined with other drugs. That is the reason why lenalidomide combinations are not considered the optimal first-line therapy, with the exception of AL amyloidosis with bortezomib contraindication (severe neuropathy caused by AL amyloidosis). It was confirmed that lenalidomide combined with other drugs could cause remission in patients whose disease was resistant to the initial bortezomib therapy. Lenalidomide (or alternatively also thalidomide) can therefore be used as second-line therapy if bortezomib therapy proves unsuccessful, with the possibility of achieving a complete remission. The increase in the number of complete remissions brought about by bortezomib therapies in patients with AL amyloidosis poses a question about which treatment should be used for younger patients with only moderate damage to their body, i.e. high-dose chemotherapy with autologous hematopoietic cell transplantation or combined treatment with bortezomib. Additional comparative studies are required to be able to answer that question and determine which of the aforesaid therapy modalities is optimal. A question still remains whether the increase in the number of complete remissions due to bortezomib will also bring about longer survival comparable to the results of high-dose chemotherapy treatment with autologous hematopoietic cell transplantation.


Asunto(s)
Amiloidosis/tratamiento farmacológico , Inhibidores de la Angiogénesis/uso terapéutico , Ácidos Borónicos/uso terapéutico , Bortezomib , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Inmunosupresores/uso terapéutico , Lenalidomida , Pirazinas/uso terapéutico , Talidomida/análogos & derivados , Talidomida/uso terapéutico
15.
Prague Med Rep ; 113(4): 294-8, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-23249661

RESUMEN

Transfusion-related acute lung injury (TRALI) is a severe life-threatening complication of blood transfusion, characterized by acute lung injury developing within 2-6 h of transfusion. However, TRALI is difficult to diagnose, and the initial report or suspicion of TRALI depends on close collaboration between clinical departments and transfusion centres. A total of 17 adverse post-transfusion reactions were reported to the Blood Centre of the University Hospital Ostrava as suspected TRALI between 2005 and 2010. We report two cases of serious TRALI with different pathogenetic mechanisms.


Asunto(s)
Lesión Pulmonar Aguda/etiología , Reacción a la Transfusión , Lesión Pulmonar Aguda/diagnóstico , Femenino , Humanos , Persona de Mediana Edad
16.
Vnitr Lek ; 58(11): 817-24, 2012 Nov.
Artículo en Checo | MEDLINE | ID: mdl-23256826

RESUMEN

AIM: To determine bone mineral density (BMD) in nephrology patients at the start of haemodialysis therapy and its dependence on some laboratory and clinical characteristics of the study set. METHODS: There were 73 newly haemodialyzed patients accepted in the 3 months period from the beginning of the chronic haemodialysis program. Each patient underwent measurements BMD with DXA method in the area of lumbar spine and the left hip. Ca, P and parathormone values were measured once per month during 3 months before BMD determination. 25-OH vitamin D, estradiol and blood pH were determined only once before the densitometry examination. RESULTS: BMD in the osteoporosis zone was measured most often in the area of femoral neck in the whole group (prevalence 35 %) and also in the single groups of patients (men, women, non-diabetics, diabetics). In women, BMD findings corresponding to osteoporosis values in the total hip were significantly more often (p < 0.01). In the area of femoral neck and lumbar spine the percentage of women and men in single groups (osteoporosis, osteopenia and normal values) was without any statistical differentiation. Diabetics and non-diabetics did not distinguish in the number of findings osteoporosis and osteopenia in any followed areas of skeleton. As the significant factors predicating BMD there were found: calcium level and sex for the area of the total hip, calcium level, blood pH and height for the femoral neck, and sex for the lumbar spine only. The certain degree of vitamin D deficiency was measured in nearly all patients (mean 11.5 ± 7.4 µg/l), and hypocalcaemia was demonstrated in one fifth of patients. CONCLUSION: Bone mineral density values and some laboratory parameters affecting bone metabolism are often abnormal in the patients entering the chronic haemodialysis program and must be taken into consideration.


Asunto(s)
Densidad Ósea , Fallo Renal Crónico/terapia , Diálisis Renal , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Fallo Renal Crónico/patología , Masculino , Persona de Mediana Edad , Adulto Joven
17.
Vnitr Lek ; 58(4): 266-72, 2012 Apr.
Artículo en Checo | MEDLINE | ID: mdl-22559799

RESUMEN

INTRODUCTION: The annual incidence of out-of-hospital cardiac arrest is around 90-190 cases per 100 000 inhabitants. The limiting factor for further prognosis of patients after out-of-hospital arrest is their neurological status. The S100B protein is mainly the nervous system cells product, its glial-specific and mostly expressed by astrocytes. It has been shown that after circulatory arrest its increased level correlates with the prognosis of patients. Work aims to determine the level of protein S100B in the group of patients with acute myocardial infarction without circulatory arrest, and compare it to the value in patients with acute myocardial infarction after out-of-hospital resuscitation. METHODS: 24 patients were evaluated after out-of-hospital resuscitation for the malignant arrhythmias during acute coronary syndrome (ACS). All patients were treated with mild therapeutic hypothermia. The control group consisted of 19 patients with ACS. The sample for the determination of S-100B was taken immediately on admission. Neurological status was evaluated according to the CPC scores (Cerebral Performance Categories) at discharge, patients were divided into 3 groups: CPC1 - good condition, CPC2 - moderate neurological disability, CPC3-5 - serious neurological impairment, coma or death. RESULTS: The values of protein S-100B fluctuated, in patients with no resuscitation, in range between 0.038 to 0.204 pg/ml. In patients after resuscitation without subsequent neurological disability (CPC 1) was range 0.077 to 0.817 pg/ml, in patients with moderate to severe neurological disability (CPC 2) was range 0.132-2.59 pg/ml, patients with severe neurological disabilities or deaths had S-100B levels from 0.70 to 8.53 pg/ml. According to ROC analysis we found the cut-off value for the S-100B. Cut-off value for probably a good neurological condition is < 0.23 pg/ml (specificity 93%, sensitivity 70%), and value testify for supposed severe neurological disability or death is > 1.64 pg/ml (specificity 95%, sensitivity 83%). CONCLUSION: Protein S-100B is one of the early and sensitive markers of severe brain damage in patients after cardiac arrest. Its early determination can help in prediction of patient neurological condition and help doctors to decide further action.


Asunto(s)
Reanimación Cardiopulmonar , Enfermedades del Sistema Nervioso Central/diagnóstico , Infarto del Miocardio/sangre , Factores de Crecimiento Nervioso/sangre , Proteínas S100/sangre , Síndrome Coronario Agudo/sangre , Síndrome Coronario Agudo/terapia , Adulto , Anciano , Biomarcadores/sangre , Enfermedades del Sistema Nervioso Central/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Infarto del Miocardio/terapia , Pronóstico , Subunidad beta de la Proteína de Unión al Calcio S100
18.
Vnitr Lek ; 58(4): 286-90, 2012 Apr.
Artículo en Checo | MEDLINE | ID: mdl-22559802

RESUMEN

Cystatin C is an inhibitor of lysosomal proteases and extracellular cysteine protease, it participates in the regulation of metabolism of extracellular proteins. It is fully glomerular filterable, completely absorbed and catabolised in proximal tubule cells. NGAL (neutrophil gelatinase-associated lipocalin) is an acute phase protein, participating in antibacterial immunity and his important feature is the formation of complex with metalloproteinase 9 (MMP-9), thereby increasing its activity and prevents its degradation. NGAL is freely filtered across the glomerular membrane and is reabsorbed by endocytosis in the proximal tubule. NGAL detected in urine is produced mainly in the distal nephron. The serum cystatin C and NGAL can diagnose acute renal impairment one or two days earlier in the comparison with the monitoring of renal function by serum creatinine. Moreover, compared with the information provided by creatinine or by estimated GFR, the elevated cystatin C gives, in patients with cardiovascular disease, information about worse prognosis. Increased level of NGAL was detected in patients with acute myocardial infarction, heart failure or stroke. There is a lack of data about the prognostic significance of NGAL in patients after myocardial infarction or heart failure, no data about their comparison or interaction with natriuretic peptides exists up today.


Asunto(s)
Enfermedades Cardiovasculares/sangre , Cistatina C/sangre , Lipocalinas/sangre , Proteínas Proto-Oncogénicas/sangre , Proteínas de Fase Aguda/fisiología , Biomarcadores/sangre , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/fisiopatología , Cistatina C/fisiología , Humanos , Enfermedades Renales/sangre , Enfermedades Renales/diagnóstico , Enfermedades Renales/fisiopatología , Lipocalina 2 , Lipocalinas/fisiología , Pronóstico , Proteínas Proto-Oncogénicas/fisiología
19.
Vnitr Lek ; 58(12): 896-903, 2012 Dec.
Artículo en Checo | MEDLINE | ID: mdl-23427947

RESUMEN

UNLABELLED: According to the criteria for multiple myeloma, systemic AL-amyloidosis may be divided into primary systemic AL-amyloidosis, where monoclonal gametopathy is present but the criteria for multiple myeloma are not satisfied, and systemic AL-amyloidosis with underlying multiple myeloma. There is a continuous transition between the two units. The present paper describes treatment of patients with established systemic AL-amyloidosis who satisfy the 2003 International Myeloma Working Groups criteria for symptomatic multiple myeloma (confirmed monoclonal immunoglobulin, clonal plasmocytes confirmed in the bone marrow and at least one clinical symptom of myeloma - confirmed amyloid). From 2009, a total of 10 patients with AL-amyloidosis and underlying multiple myeloma have been treated at our centre with combined bortezomib-containing regimens. The cohort includes 5 women and 5 men. Median age of these AL-amyloidosis patients at the diagnosis was 65.5 years. All 10 patients were treated with a combination of 3 drugs, bortezomib, cyclophosphamide and dexamethasone or bortezomib, doxorubicin a dexamethasone. Two of the 10 patients died during the first month of treatment. Treatment response cannot be evaluated in these patients. Haematological treatment response was evaluable in 8 patients only. Monoclonal immunoglobulin disappearance with negative urine and serum immunofixation and normalization of free light chain immunoglobulins was observed in six of the 8 patients. Treatment response according to the current IMWG was evaluated as very good partial remission (VGPR) as we did not perform bone marrow testing after the treatment to confirm complete remission according to the current criteria. One of the 8 evaluated patients died due to disease progression in the third month of treatment and there was no haematological treatment response in one who was considered to have a stable disease. Organ treatment response was evaluated in patients who were followed up for longer than 3 months of treatment only. Organ treatment response (reduced cardiac impairment) was not evaluable in a patient who had heart transplantation and then received chemotherapy. A total of 5 (83%) of the 6 evaluated patients fulfilled the criteria of organ treatment response. CONCLUSION: Our small cohort showed a high number of haematological treatment responses (VGPR in 75% of patients) as well as organ treatment response in patients with systemic AL-amyloidosis who were treated with bortezomib-containing treatment regimens.


Asunto(s)
Amiloidosis/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Mieloma Múltiple/complicaciones , Anciano , Amiloidosis/complicaciones , Ácidos Borónicos/administración & dosificación , Bortezomib , Ciclofosfamida/administración & dosificación , Dexametasona/administración & dosificación , Doxorrubicina/administración & dosificación , Femenino , Humanos , Masculino , Pirazinas/administración & dosificación
20.
Ceska Gynekol ; 76(3): 239-42, 2011 Jun.
Artículo en Checo | MEDLINE | ID: mdl-21838157

RESUMEN

OBJECTIVE: To optimize thrombocyte antibodies examination in pregnant and postpartum women. DESIGN: Retrospective analysis. SETTING: Blood Center, Faculty Hospital and Medical Faculty Ostrava University, Ostrava. METHODS: Analysis and our experience with introducing the new test for thrombocyte antibodies examination. CONCLUSION: The thrombocyte antibodies examination that was introduce in the period 2008-2009 and is the combination of basic and special testing, seems to be optimal for the neonatal thrombocytopenia detection.


Asunto(s)
Antígenos de Plaqueta Humana/inmunología , Autoanticuerpos/análisis , Periodo Posparto/inmunología , Embarazo/inmunología , Trombocitopenia Neonatal Aloinmune/diagnóstico , Femenino , Antígenos HLA/análisis , Humanos , Recién Nacido
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