Akutní lymfoblastická leukemie dospělých s pozitivitou filadelfského chromozomu a fúzního genu BCR/ABL je agresivní hematoonkologické onemocnění, typické pro pacienty vyššího věku. Léčba spočívá v podávání chemoterapie a inhibitoru tyrozinkinázy. Maximálního efektu lze dosáhnout pomocí alogenní transplantace krvetvorných buněk, tu je však schopna podstoupit jen malá část pacientů. V případě relapsu je prognóza onemocnění extrémně nepříznivá., Philadelphia chromosome positive adult acute lymphoblastic leukemia with BCR/ABL fusion gene is an aggressive hematological malignancy most common in the elderly. Both chemotherapy and a tyrosine kinase inhibitor are engaged in the treatment. The best outcome can eventually be achieved using allogeneic hematopoietic stem cell transplantation. However, only a minority of patients can undergo such intensive therapy. Disease relapse is associated with an extremely dismal prognosis., František Folber, Eva Létalová, Michael Doubek, and Lit.: 23
Only a few cases of pneumocystis pneumonia (PCP) in Cushing’s syndrome have been published in the literature so far. In the majority of these patients, the pneumonia occurred after reduction of the hypercortisolism with medicamentous treatment. We report two cases of PCP during conservative treatment of hypercortisolism. We describe clinical, imaging and laboratory findings in two patients and review published cases of pneumocystits pneumonia in Cushing’s syndrome. A 60-year-old woman and 20-year-old man with Cushing’s syndrome due to ectopic ACTH syndrome were treated at our department. Both developed pneumocystis pneumonia early after treatment with ketoconazole and ethomidate bromide had been introduced and the levels of cortisol rapidly decreased. PCP prophylaxis in patients with high cortisolemia should be started before treatment of hypercortisolism in current practice. Gradual lowering of plasma cortisol should also reduce the risk of infection by Pneumocystis jiroveci., Filip Gabalec, Alžběta Zavřelová, Eduard Havel, Jaroslav Cerman, jr., Jakub Radocha, Ioannis Svilias, Jan Čáp, and Literatura 10
According to our research, Uzbek individuals with Parkinson’s disease have high prevalence of 0/0 polymorphisms of GSTT1 and GSTM1 genes, as well as combinations of GSTT1 (0/0)/GSTM1/(0/0) genotypes. These mutations are associated with an earlier debut of the disease, its mixed form, and rapid rate of progression. Determination of mutations in GSTT1 and GSTM1 genes of xenobiotic detoxification in patients with Parkinson’s disease confirms multifactorial nature of this pathology and the role of the influence of various external factors in the modification of clinical signs of disease and its prognosis., Khanifa Khalimova, Malika Raimova, Rustam Matmurodov, Khurshidakhon Rasulova, Amina Burnasheva, Yekaterina Jmirko, Rustam Mukhamedov, and Literatura
Úvod: Jedná se o retrospektivní analýzu souboru nemocných s mnohočetným myelomem ošetřených ve FN Hradec Králové v období od 1/2007 do 6/2010 pomocí perkutánní vertebroplastiky. Materiál a metodika: U 13 nemocných (3 muži a 10 žen), medián věku 65 let, bylo celkem ošetřeno 18 obratlových těl v oblasti thorakolumbální páteře. Sledovali jsme bolesti pomocí VAS (visual analoque scale), četnost komplikací, funkční výstup nemocných (stejný, zlepšený, zhoršený) a spotřebu analgetik (zvýšená, stejná, snížená). Nemocní byli ošetření na angiografickém kompletu transpedikulárním či posterolaterálním přístupem. Výsledky: Provedení perkutánní vertebroplastiky mělo statisticky významný vliv na pokles bolesti po výkonu (p = 0,002). Přitom jsme neprokázali žádnou symptomatickou komplikaci, k asymptomatickému úniku kostního cementu mimo obratlové tělo došlo u 11 % nemocných. Zlepšení úrovně života udávalo 100 % nemocných. Snížení spotřeby analgetik jsme prokázali u 92 % nemocných a u zbývajících 8 % zůstala spotřeba analgetik stejná. Závěr: Perkutánní vertebroplastika je miniinvazivní zákrok s minimem komplikací a rychlým nástupem analgetického efektu u nemocného., Introduction: We present retrospective analysis of a group of patients with multiple myeloma treated in FN Hradec Králové from 1/2007 to 6/2010 by percutaneous vertebroplasty. Material and methods: A total of 18 vertebral bodies were treated in thoracolumbar spine region in 13 patients (3 men and 10 women), median age of 65 years. We watched the pain using the VAS (visual analoque scale), frequency of complications, functional outcome of patients (same, improved, worsened) and consumption of analgetics (increased, same, decreased). Patiens were treated at angiographic suit using transpedicular or posterolateral access. Results: Vertebroplasty has a statistically significant effect on the pain relief after procedure (p = 0,002). We didn´t find symptomatic complications, asymptomatic leakage of bone cement outside the vertebral body was observed in 11 % of patients. Improving the standard of living was reported by 100 % of patients. Reducing consumption of analgetics has been confirmed in 92 % and 8 % of patients remained the same consumption of analgetics. Conclusion: Percutaneous vertebroplasty is a minimally invasive procedure with minimum of complications and rapid onset of analgetic effect in patients., Pavel Ryška, Vladimír Maisnar, Václav Málek, Tomáš Kvasnička, Jiří Jandura, Eva Štěpánová, Jana Hrubešová, Svatopluk Řehák, and Lit.: 39
Purpose: To investigate the protective action of Ceruloplasmin (Cp) on the lysis of erythrocytes in men with prostate tumors. Material and Methods: The blood erythrocytes of the patients with benign hyperplasia of prostate (BHP) and prostate adenocarcinoma (CaP) were studied. Patients at the age 60-75 with early stages of a cancer have been investigated. The control group was consisted of apparently healthy males with the compatible age. n=15 for each group. The clinical stage of the disease was diagnosed by means of rectal, histological and echographic examination of the prostate gland. Photometric methods were applied to register lysis dynamics in order to test out the protective action of Cp. Results: The nonspecific protective function of Cp preparation on BHP and CaP erythrocytes as well as on the control group erythrocytes was revealed. CaP erythrocytes have shown more sensitivity to the lysis provocative factor than BHP and the control group erythrocytes, that was presumably attributed to the structural and functional changes of the erythrocytes developed in the presence of malignant tumor., N. Kotrikadze, M. Zibzibadze, L. Ramishvili, M. Alibegashvili., T. Chigogidze, L. Managadze, and Literatura
Cíl. Pseudomyxom peritonea je velmi vzácné semimaligní onemocnění. Záměrem našeho sdělení je pojednání o typickém nálezu, možnostech léčby a komplikacích. Metoda. Nedávné případy z našeho pracoviště ilustrují patognomické znaky nemoci v CT obrazu, obvyklý klinický nález, průběh onemocnění i svízelnost jeho léčby. Ve dvou našich kazuistikách je příčinou pseudomyxomu peritonea mucinózní cystadenom appendixu, v jednom appendikální low grade cystadenokarcinom, v posledním případě mucinózní cystadenokarcinom neznámého původu. U prvního pacienta se navíc objevila raritní komplikace - metastatická ložiska intrathorakálně. Závěr. Pseudomyxom peritonea je klinický syndrom, který je způsoben excesivní produkcí gelatinózního ascitu buňkami mucinózního cystadenomu či cystadenokarcinomu různého původu, nejčastěji se však jedná o tumory appendixu. Typický morfologický nález v zobrazovacích metodách určuje jejich významné místo v diagnostice, stagingu a monitorování výsledků léčby pseudomyxomu., Aim. Pseudomyxoma peritonei is an uncommon semimalignous disease. The aim of our statement is the discussion about the typical finding, the possibilities of treatment and complications. Method. The recent findings from our workplace illustrate pathological signs of the disease on CT-scan, usual clinical finding, the progression of the disease and the arduous of its treatment. In two of our cases is the cause of pseudomyxoma peritonei mucinous cystadenoma of the appendix, in one low grade mucinous cystadenocarcinoma and in the last case the mucinuos cystadenoma of unknown origin. There has appeared by the first patient a rare complication - intrathoracic implants lesions. Conclusion. Pseudomyxoma peritonei is a clinical syndrome that is caused by the excessive production of gelatinous ascites cells mucinous cystadenoma or cystadeno-karcinoma of different origin, but mostly these are the cases of appendix tumors. The typical morphological found in the imaginig methods determines their important place in the diagnostics, staging and the monitoring of the results of the treatment of pseudomyxoma., Žáčik M, Kacvinská T, Pernická B, Pospíšková M, Palíšek J., and Literatura 12
Pacienti s metastatickým kolorektálním karcinomem (mKRK) by měli v průběhu své léčby obdržet všech pět látek účinných u tohoto onemocnění. Tyto látky se skládají ze tří chemoterapeutik: 5-fluorouracil/leucovorin (5-FU/LV), irinotecan, oxaliplatina a dvou monoklonálních protilátek: bevacizumab namířený proti vaskulárnímu endoteliálnímu růstovému faktoru a inhibitor epidermálního růstového faktoru cetuximab nebo panitumumab. Léčebné možnosti třetí linie závisí na tom, jaké látky byly použity v předchozí terapii. U pacientů předléčených 5-FU, oxaliplatinou a irinotecanem představuje léčba kombinací cetuximab a irinotecan atraktivní možnost., Patients with metastatic colorectal cancer (mCRC) should receive all five drugs active in this disease during the overall course of their treatment. These drugs consist of three chemotherapeutic agents: 5-fluorouracil/leucovorin (5-FU/LV), irinotecan and oxaliplatin and two monoclonal antibody: bevacizumab directed against the vascular endothelial growth factor and the epidermal growth factor receptor inhibitor cetuximab or panitumumab. Treatment options for third-line therapy depend on what agents have been used in previous therapy. In patients with prior treatment with 5-FU, oxaliplatin, and irinotecan, treatment with the combination of cetuximab and irinotecan is an attractive option., Ilona Kocáková, and Lit.: 6
Česká republika patří celosvětově mezi země s nejvyšším výskytem kolorektálního karcinomu (KRK), jehož incidence však mezi českými kraji není distribuována rovnoměrně. Ve všech epidemiologických ukazatelích (incidence, mortalita i prevalence) tohoto maligního onemocnění dominuje Plzeňský kraj. Vzhledem k prodlužování průměrné délky života vzrostl v posledním desetiletí podíl KRK jako druhého metachronního primárního maligního onemocnění. V roce 2000 byl zahájen Národní program screeningu KRK, který t. č. nabízí asymptomatickým jedincům starším 50 let možnost výběru ze dvou screeningových metod (testy na okultní krvácení a primární screeningová kolonoskopie). Od roku 2006 bylo v rámci screeningu kolonoskopicky vyšetřeno 57 255 osob, u 17 760 (31,0 %) z nich byly diagnostikovány a odstraněny adenomové polypy a u 2 665 (4,7 %) byl nalezen karcinom., The Czech Republic belongs to the countries with highest colorectal cancer (CRC) incidence worldwide which differs in regions of this country. The Pilsner region stands out in all epidemiologic features (incidence, mortality, prevalence). The proportion of CRC as the second metachronous primary malignant disease has increased in past ten years, due to the elongation of life expectancy. The National colorectal cancer screening programme was launched in year 2000. Nowadays two screening methods (fecal occult glood tests and primary screening colonoscopy) are offered to asymptomatic individuals aged over 57 255 people have been examined by colonoscopy since year 2006. Adenomas were removed in 17 760 of them (31.0 %) and cancer was diagnosed in 2 665 (4.7 %) individuals., Miroslav Zavoral, Štěpán Suchánek, Gabriela Vepřeková, Ondřej Májek, Jakub Gregor, Ladislav Dušek, and Lit.: 13
Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS., Edvard Ehler, Jan Latta, Petra Mandysová, Jana Havlasová, Milan Mrklovský, and Literatura 22
Mediterranean spotted fever (MSF) in Bulgaria shows increasing severity, especially in patients over sixty years. As T lymphocytes are the primary effector cells in MSF and are altered by the ageing process we aimed to specify the changes in their numbers in elderly patients. We performed a clinicoepidemiological study, haematological and biochemical analysis of 132 patients of 60+ and 30 patients between 19 and 57 years with MSF. We investigated the cell immunity of 20 patients of 60+ using immunofluocytometry. The control group consisted of 10 younger patients and 10 healthy individuals of 60+. MSF showed more unfavourable course in the elderly.We found T-lymphocytes depletion in all patients with MSF. Activated T lymphocytes were increased in elderly patients with MSF. We found significant differences between the number of activated T lymphocytes in elderly patients and healthy elderly persons. The observed T-lymphocytes depletion in MSF corresponded to the disease severity. The increase in the activated T lymphocytes in patients over 60 years contributes to an adequate immune response., Margarita Gospodinova, and Literatura