[Case report of a patient with advanced and disseminated gastric carcinoma treated by s-1]. / Kazuistika pacienta se znacne pokrocilým a diseminovaným karcinomem zaludku léceným preparátem S-1.

BACKGROUND: Prognosis of patients with metastatic gastric cancer is abysmal, usually just a few months. S-1 is a peroral fluoropyrimidine antitumor drug. It is a fixed combination of three effective drugs - tegafur, gimeracil and oteracil potassium. CASE: This is a case report of a 71-year-old man treated for local advanced and metastatic gastric carcinoma treated with combination of S-1 and cisplatin as a first line of therapy. Treatment response reached partial remission and lasted for six months. Treatment was very well tolerated, with no grade 3 and 4 toxicity. After progression, the patient was treated with further lines of therapy. CONCLUSION: In the Czech Republic, experience with S-1 drug is very limited. Our case report showed a good treatment response and minimal toxicity of this treatment, in concordance with results of the study FLAGS.

Utility of a rescue endovascular therapy for the treatment of major strokes refractory to full-dose intravenous thrombolysis.

OBJECTIVE: Large artery occlusion (LAO) in patients with major stroke predicts poor revascularization by intravenous thrombolysis (IVT) and more likely results in a poor outcome. We focused on the effects of intra-arterial thrombolysis (IAT) and endovascular mechanical recanalization (EMR) as rescue therapies in major strokes refractory to IVT. METHODS: A retrospective analysis of 87 patients (National Institutes of Health Stroke Scale >20), who did not respond to full-dose IVT due to LAO, was performed based on their endovascular therapy status. IAT was performed as an intraclot infusion of alteplase, and EMR was provided by the Solitaire device™ (Covidien, Dubin, Ireland). The recanalization and 3-month outcome rates after IAT/EMR were correlated with a group of patients who were scheduled to receive endovascular treatment but who underwent only IVT. RESULTS: We achieved successful recanalization by IAT and EMR in 68.7% and 76.1% of patients, respectively. Despite no significant differences in mortality between IAT and EMR, a trend towards better outcomes after IAT and a statistically significant increase for outcome-modified Rankin scale (mRS) 0-3 (45.7%) and mRS 0-2 (34.9%) after EMR was noted when compared with IVT. The degree of recanalization did not correlate with the functional results except for the good-moderate outcome after successful recanalization by EMR. CONCLUSION: EMR by the Solitaire device is a safe and beneficial method for the rescue treatment of patients with major stroke whose neurological status does not improve and who fail to recanalize the LAO after a 1-h full dose of IVT. ADVANCES IN KNOWLEDGE: The article verifies efficiency of the Solitaire device in major strokes.

[Interventional radiology options to address certain complications of the orthotopic liver transplant]. / Moznosti intervencní radiologie pri resení ne-kte-rých komplikací ortotopické transplantace jater.

At the clinic of imaging method St. Annas University Hospital solved complications arising after ortotopic liver transplantation. For more than 15 years of cooperation with CKTCH Brno intervention was performed on both the arterial and venous system, but most on the biliary tract. The order was a unit patients, which correlates with other comparable work. In the years 1998- 2013 we conducted one intervention on arterial bed, 3× intervention in hepatic venous system and we solved biliary complications in 7 transplant. .

[Comparison of MRCP a ERCP in diagnosis of choledocholithiasis]. / Srovnání MRCP a ERCP v diagnostice choledocholitiázy.

INTRODUCTION: Choledocholithiasis is the most common cause of biliary obstruction. Each of the testing methods used in its diagnosis has its advantages and disadvantages. OBJECTIVE OF THE STUDY: The objective of this prospective study is to compare endoscopic retrograde cholangiopancreatography with magnetic resonance cholangiopancreatography in the diagnosis of choledocholithiasis on the basis of own experience and literature data. Set of patients and methodology: The set was studied from the beginning of 2007 to the end of 2012 (i.e. six years). The study assessed prospectively 45 patients (age range 28- 72 years) with symptoms of biliary obstruction, who first underwent magnetic resonance cholangiopancreatography and subsequently endoscopic retrograde cholangiopancreatography. RESULTS: The sensitivity, specificity and diagnostic accuracy of magnetic resonance cholangiopancreatography was lower, both in our set of patients and according to the literature data, compared to the endoscopic retrograde cholangiopancreatography (92%, 91% or 93 %). CONCLUSION: Considering the frequency of complications (in some cases serious ones) following endoscopic retrograde cholangiopancreatography, the magnetic resonance cholangiopancreatography is, in spite of its lower sensitivity, the method of choice in the diagnosis of choledocholithiasis by means of noninvasive methods, on the basis of which it is possible to refer the patients subsequently for therapeutic endoscopic retrograde cholangiopancreatography.

Extensive Al amyloidosis presenting with recurrent liver hemorrhage and hemoperitoneum: case report and literature review.

BACKGROUND: Spontaneous hepatic bleeding is a rare but potentially life-threatening complication of primary systemic amyloidosis. Although the liver is a common site of amyloid deposition, clinical presentation is usually mild or absent. CASE: We report a case of a female patient, who had been repeatedly surgically revised because of liver rupture and hemoperitoneum. Initially, the computed tomography finding was interpreted as liver hemangioma. However, based on liver biopsy, the diagnosis had to be changed to primary systemic amyloidosis, and the patient was referred to our hematooncology department. Due to a considerably advanced disease, the patient was eligible only for palliative chemotherapy with cyclophosphamide and dexamethasone, which could not deflect the course of rapidly progressing liver destruction. CONCLUSION: The cause behind ruptured and bleeding liver does not always need to be hemangioma but rather amyloidosis. In cases of advanced disease and in patients with contraindications for aggressive treatment, the outlook for complete hematological and organ treatment response is very limited. An early diagnosis is of utmost importance. Although liver biopsy brings the definite results, screening for monoclonal protein in serum or urine, leading to a search for AL amyloidosis, may be sufficient for diagnosis. The presence of some of the warning signs (B-symptoms such as fevers or subfebrile temperatures, fatigue, weight loss; and paraneoplastic laboratory findings such as elevated C-reactive protein and erythrocyte sedimentation rate) should raise suspicion of a lymphoproliferative disease.

Benefits of individual imaging methods for diagnosis and monitoring of activity of multiple myeloma.

BACKGROUND: Multiple myeloma pathogenesis, pathology, symptoms and imaging techniques used in clinical diagnostic algorithm, the indications and the differences between currently available imaging methods. DESIGN: The article describes advantages and disadvantages of basic X-ray imaging and recommended skeleton screening, as the method of first choice, followed by description of the most frequently affected areas and Mirels score. The present golden standard magnetic resonance (MR) imaging, its potential and also recommended MR indications. Concerning computer tomography (CT) imaging, mainly comparison between CT and MR and X-ray imaging its indications and benefits as the interventional instrument are mentioned. The arcticle also focuses on the role of skeleton scintigraphy with Tc-pyrophosphate, which is not recommended today, and the role of positron emission tomography with fluorodeoxyglucose (FDG-PET) in the assessment of the therapy effectiveness and prognosis for patients, its future and present limitations. The next commonly used radioisotope imaging with 99Tc-sestamibi (MIBI) and its comparison to other methods, especially to the FDG-PET and recommended indications for both techniques. Last aim is description of specification of bone tissue density with Dual Energy X-ray Absorption scanning method (DEXA). CONCLUSION: These imaging methods are commonly used as additional diagnostic tests in initial diagnostic -work-up and in follow-up due to frequent relapses of multiple myeloma.

[Eyelids with yellow granulomas and cough - periocular xanthogranuloma associated with adult-onset asthma. A case study and an overview of clinical forms of juvenile xanthogranuloma and its therapy]. / Ocní vícka se zlutými granulomy a kasel - periokulární xantogranulom dospelých spojený s astmatem. Popis prípadu a prehled klinických forem juvenilního xantogranulomu a terapie.

Histiocytic diseases caused by proliferation and accumulation of phagocytosing macrophages (foamy macrophages) have many clinical forms. These are classified under "juvenile xanthogranuloma" within the WHO classification of blood disorders. Localized forms with benign course include normolipaemic xanthomatosis, xanthogranuloma and necrobiotic xanthogranuloma. Disseminated forms in children take a form of so called "disseminated juvenile xanthogranuloma" or Erdheim-Chester disease in adults. We describe a case of a patient who, at 53 years of age, first noticed yellow granulomas on her eyelids. The disease progressed gradually and, at 59, affects the eyelids as well as their closest surroundings. According to MR and PET-CT, the disease gradually infiltrated the inside of the orbit, orbital fat as well as extraocular muscles and started to cause exoftalmus of one of the eyes. Propagation of the xanthogranuloma into the orbit and infiltration of extraocular muscles might impair eye function. Over the last year, the patient complained of cough. Pulmonary function evaluation confirmed recent asthma bronchiale. These findings correspond to periocular xanthogranuloma associated with adult-onset asthma. No other abnormities have been shown in this patient. Exoftalmus was observed in 2011 after 6 years of monitoring with very slow progression of eyelid and extraocular infiltration. Therefore, prednisone was initiated in 2011, leading to cessation of exoftalmus. It is not known at present whether this is a permanent improvement with a suppression of histiocytary proliferation or whether this was a temporary improvement due to suppression of inflammatory changes in the xanthogranuloma with no effect on histiocytary proliferation. Progression during therapy with corticosteroids would warrant cytostatic treatment. The discussion section provides an overview of diseases caused by foamy histiocytes with illustrations and an overview of experiences with their treatment.

[Six-year follow-up of a patient with multiple angiomatosis involving skeleton, thoracic and abdominal cavities and the gut wall]. / Sestileté sledování pacienta s mnohocetnou angiomatózou postihující skelet, brisní i hrudní dutinu a stenu trávicí trubice.

BACKGROUNDS: Multiple angiomatosis is a rare disease causing angiomatous lesions in multiple organs and tissues with a risk of life-threatening haemorrhage. OBSERVATION: A young man was diagnosed with multiple angiomatosis at the age of 28 after two years of back and abdominal pain. Laparotomy revealed multiple spongy lesions mostly within the retroperitoneal space. Also, an involvement of the gut wall, bones and mediastinum was evident. After 6 years of treatment, the disease has been stabilized. Bone pain ceased with a significant contribution of zoledronate. Using CT and MR imaging, the effectiveness of antiangiogenic drugs was evaluated. Furthermore, treatment response was evaluated using laboratory values for coagulation and blood count, as angiomatous proliferation is known to be associated with disseminated intravascular coagulation and anaemia. RESULTS: Baseline laboratory examination revealed elevated D-dimer (more than 20 µg/mL), low fibrinogen (1.4 g/L), and the presence of fibrin monomers. After treatment with 6 mil. IU of interferon-alpha thrice weekly, there was only partial improvement in D-dimer (17.2 µg/mL) and fibrinogen (1.5 g/L) concentrations but fibrin monomers remained positive. After thalidomide (100 mg daily), D-dimer decreased to 6.1 µg/mL and fibrinogen levels increased to 1.9 g/L with the disappearance of fibrin monomers. CT scanning showed significant regression of angiomatous lesions. Progressive neuropathy was the reason to lower the dose of thalidomide by half and this caused D-dimer to rise again. Switching to lenalidomide 10 mg daily led to an increase in D-dimer to 10.8 µg/mL and decrease in haemoglobin concentration to 124 g/L. Fibrin monomers became positive again. Combined therapy with thalidomide (50 mg/day) and lenalidomide (10 mg days 1-21 in 28-day cycles) has led to stabilisation of the disease. Median concentration of haemoglobin increased to 131 (84-141) g/l. The median of D-dimer decreased to 9.3 (8.0-17) µg/mL. CONCLUSION: Thalidomide in the dose of 100 mg daily led to better stabilisation of the disease than interferon-alpha. However, lowering the dose because of adverse effects failed to be effective sufficiently. Lenalidomide 10 mg daily was well-tolerated but insufficient to improve D-dimer and haemoglobin concentrations. Therefore, for further treatment we have decided to use the combination of lenalidomide and thalidomide in doses of 10 mg and 50 mg, respectively because both drugs have desirable antiangiogenic activities with different adverse effect profiles. On this therapy, the patients disease has been stable for 9 months.

[MR-documented remission of pituitary stalk infiltration in patients with Langerhans cell histiocytosis following treatment with 2-chlorodeoxyadenosine]. / MR dokumentované vymizení infiltrate infundibula hypofýzy u pacientu s histiocytózou z Langerhansových bunek po lécbe 2-chlorodeoxyadenosinem.

In adult patients, Langerhans cell histiocytosis (LCH) manifests most frequently with one or more osteolytic lesions or, alternatively, with pulmonary involvement with nodules and cysts or with skin lesions. Infiltration ofthe central nervous system is a rather rare sign of LCH. The LCH cells have an unexplained affinity to hypothalamus and to pituitary stalk and, consequently, central diabetes insipidus is the most frequent clinical sign of brain involvement in LCH. We describe treatment of 2 adult patients with LCH in whom central diabetes insipidus was the first sign of LCH and MR confirmed pituitary stalk infiltration. The first man was diagnosed with diabetes insipidus and pituitary stalk infiltration at 33 years of age. LCH was confirmed 2 years later by histology of verrucous lesions on the skin of perianal area. The disease affected the skin and CNS. The patient was treated with 2-chlorodeoxyadenosine (5 mg/m2 s.c. for 5 consecutive days of a 28-day cycle). No pituitary infiltration was evident on an MR image after the 4th cycle. Residual perianal infiltration was irradiated. The patient has been in complete remission for 44 months following treatment completion, although vasopressin and testosterone substitution is required. The second man was also diagnosed with diabetes insipidus and pituitary stalk infiltration at 33 years of age. Pulmonary involvement was identified with high resolution CT(HRCT) and high CD1a and S-100 positive elements with bronchoalveolar lavage. This patient further had external auditory canal infiltrations causing chronic discharge from the ears. The patient was treated with 2-chlorodeoxyadenosine as above. A follow up MR after the 4th cycle showed reduction in the infiltration diameter from 5.5 to 3.0 mm. Therefore, 2-chlorodeoxyadenosine 5 mg/m2 s.c. was combined with dexamethasone 20 mg p.o. during the 5th and 6th cycle. The MR image after treatment completion showed remission of the pituitary stalk infiltrate. External auditory canal infiltration diminished as did the nodules in pulmonary parenchyma. Nevertheless, vasopressin substitution is still required. The patient has been in complete remission for 8 months from the completion of the treatment. Pituitary stalk infiltration disappeared after the treatment with 2-chlorodeoxyadenosine in 2 patients; after 4 cycles in the first and after 6 cycles (with an addition of dexamethasone during the last 2 cycles) in the second.

[The value of various imaging techniques in diagnosing and monitoring the disease activity of multiple myeloma]. / Prínos jednotlivých zobrazovacích metod pro diagnostiku a sledování aktivity mnohocetného myelomu.

Imaging techniques such as RTG, CT, MR and PET are key in diagnosing multiple myeloma. Their selection, combinations and sequence of their application are important for early and correct diagnosis. It is the clinical experience with this condition complemented by suitable imaging diagnostics that leads effective treatment.

[Central diabetes insipidus in adult patients--the first sign of Langerhans cell histiocytosis and Erdheim-Chester disease. Three case studies and literature review]. / Centrální diabetes insipidus u dospelých osob--první príznak histiocytózy z Langerhansových bunek a Erdheimovy-Chesterovy choroby. Popis trí prípadu a prehled literatury.

Central diabetes insipidus with an onset in adulthood is very rare. Unlike in children, central diabetes insipidus in adults is more frequently caused by inflammatory processes and neoplastic infiltrations that do not originate from the neuronal tissue than primary neuronal tissue tumours. Rare histiocytic neoplasias (Langerhans cell histiocytosis, xanthogranulomatosis and Erdheim-Chester disease) have a specific affinity to hypothalamus and the pituitary stalk not only in paediatric patients but also when occurring in adults. We describe 3 cases of central diabetes insipidus with an onset in adulthood. Diabetes insipidus was the first sign of Langerhans cell histiocytosis in 2 patients, and it was the first sign of Erdheim-Chester disease in one patient. MR imaging showed pathological infiltration and dilated pituitary stalks in all 3 patients. PET-CT proved useful in differential diagnosis, showing further extracranial pathological changes either on the basis of significant glucose accumulation or on the basis of CT imaging. The Langerhans cell histiocytosis in the first patient has also manifested itself as an infiltration of the perianal area with intensive accumulation of fluorodeoxyglucose (FDG) - SUV 8.6 and gingival inflammation indistinguishable from parodontosis. Histology of the perianal infiltrate confirmed Langerhans cell histiocytosis. Infiltration of the pituitary stalk disappeared from the MR image after 4 cycles of 2-chlordeoxyadenosin (5 mg/m2 5 consecutive days). The PET-CT of the 2nd patient showed only borderline accumulation of FDG in the ENT area, while simultaneously performed CT imaging showed cystic restructuring of the pulmonary parenchyma and nodulations consistent with pulmonary Langerhans cell histiocytosis. Bronchoalveolar lavage identified higher number of CD1 and S100 positive elements, consistent, once again, with pulmonary LCH also affecting pituitary stalk and ear canal. The PET-CT of the third patient showed increased activity in the long bones and ilium near the sacroiliac joint. Biopsy of the focus in the ilium confirmed foam histiocyte infiltration immunochemically corresponding to Erdheim-Chester disease. Additional imaging assessments revealed the presence of further signs of the disease. Pituitary infiltrate biopsy in this patient did not elucidate the diagnosis but resulted in complete panhypopituarism. Central diabetes insipidus in adulthood might be the first sign of so far undiagnosed extracranial disease, in our case of histiocytic neoplasias, and PET-CT has an excellent potential to detect extracranial symptoms of these conditions. Therefore, the high-risk pituitary stalk infiltrate biopsy should always be preceded by comprehensive examination aimed at identification of extracranial manifestations of the pituitary gland diseases.

[Evaluation of neoadjuvant chemo-radiotherapy with locally advanced rectal cancer by comparing tumour volume before and after treatment]. / Hodnocení neoadjuvantní chemoradioterapie u lokálne pokrocilého karcinomu rekta srovnáním objemu tumoru pred lécbou a po lécbe.

BACKGROUND: Neoadjuvant chemotherapy is a standard preoperative therapeutical procedure with locally advanced rectal adenocarcinoma. The aim of the study was to compare the tumour volume reduction before and after the oncological therapy in relation to the change in the CEA value and to the outcome of the histopathological evaluation of response to the treatment. PATIENTS AND METHODS: In the years 2004-2008, 274 rectal cancer patients were evaluated, of which 64 underwent neoadjuvant CRT with subsequent surgery and had also completed other inclusion criteria. The tumour volume before and after the CRT, percentage reduction in the tumour volume and the relation to the change in the CAE value and the histopathological evaluation were evaluated. RESULTS: The distance between the anus and the tumour was from 3 to 15 centimetres, the average value being 8.1 centimetres. In 5 cases the tumour was not histologically found in the resected specimen. Average value of the CEA value before the CRT was 18.12 ng/ml, range 0.7-98.1 ng/ml, after the CRT the average value was 7.00 ng/ml, range 0.5-18.7 ng/ml. The average tumour volume before CRT was 32.48, range 10.3-88.5, after the CRT the average volume was 20.13, range 4.7-55.1. CONCLUSION: A relation between the change in the T value and the volume reduction before and after the CRT of statistical significance has been proven in this group of patients. This relation however has not been proved in the N value change. Only in one-third of the evaluated patients was there a positive change in both T and N classification. No relation between the CEA value and the tumour volume change has been proven.

[Interdigitating dendritic cell sarcoma of lower extremities resistant to high dose chemotherapy BEAM with peripheral blood stem cell transplantation]. / Sarkom z interdigitujících dendritických bunek dolní koncetiny rezistentní k vysokodávkované chemoterapii BEAM s autologní transplantací kmenových krvetvorných bunek--popis prípadu a prehled literatury.

Interdigitating dendritic cell sarcoma is a rare neoplasm forming part of the group of malignancies derived from histocytic cell line. This nosological unit can be detected only by special immunohistochemical exams. A young man aged 25 found a tumorous swelling in the proximal part of his left crus. The pathological process affected proximal tibial epiphysis and adjacent soft tissues. The first FDG-PET examination performed in the process of determining the clinical stage of the disease showed a high activity in the site of primary tumour (SUV 7.71) and in the site of regional inguinal node (SUV 4.25). Histological examination of a diagnostic excision specimen of the tumour in the tibia and the extirpated enlarged regional nodes in the left groin led to the diagnosis of interdigitating dendritic cell sarcoma. The diagnosis was confirmed pathologically by another two centres in the Czech Republic and, due to the unusual nature of the diagnosis, also in Regensburg, Germany. Treatment started with chemotherapy, applied to patients with aggressive lymphomas in the framework of clinical studies, i.e. a combination of MegaCHOP. After 4 cycles, however, there was no visible response on the site of primary tumour. MegaCHOP therapy was therefore discontinued after the 4 cycles. Subsequently, we referred the patient for a high-dose chemotherapy with autologous bone marrow transplantation, similarly to aggressive lymphomas. The collection of blood producing stem cells from peripheral blood was successfully performed after ESHAP chemotherapy. A verificatoin FDG-PET examination was performed before high-dose chemotherapy. Increased activity was detected only in left proximal crus, with an SUV of 4.6. One month after ESHAP chemotherapy, BEAM high-dose chemotherapy with autologous transplantation of blood forming tissue was performed. High-dose chemotherapy was followed up by radiotherapy targeted on the primary tumour in the crus (70 Gy). The third verification FDG-PET examination was performed 3 months after radiotherapy. The examination showed a continuing higher activity in the region of the primary tumour (SUV 2.69) and a new centre of activity was detected in the left inguinal nodes region (SUV4.09). The activity corresponded to the presence of viable tumour tissue in the primary nidus and new metastases in inguinal nodes, without proofs of further proliferation at the time. Nodes of the left groin were removed. Histological examination showed affection of the node by the same type of tumour, i.e. a continuing activity of the disease despite chemotherapy. Due to suspected continuation of viable tumour in the crus judging by the intensity of accumulation of FDG-PET and the proof of a new affection of regional nodes, surgical treatment was preferred after the failure of chemotherapy. After the removal of inguinal nodes, left knee joint exarticulation was performed. This was followed by regional inguinal node region radiotherapy (56 Gy). The last fourth PET-CT examination carried out 4 months after the radiation therapy of the inguinal region showed massive dissemination into the region ofileac and paraaortic nodes (lymphadenopathy up to 6 cm in diameter) with an activity of 5.9 to 6.73 SUV units. Currently, we test the sensitiveness of the disease to 2-chlordeoxyadenosin and look for additional therapeutic options. To our knowledge, the above description is the first documented case of interdigitating dendritic cell sarcoma located in the tibia and crus soft tissue. We have not found any description of high-dose therapy supported by autologous transplantation of blood-forming tissue for this type of tumour in relevant literature. In this case, we record chemoresistance to high-dose chemotherapy and certain radiosensitivty of the tumour at the same time.

[Diabetes insipidus followed, after 4 years, with dysarthria and mild right-sided hemiparesis--the first clinical signs of Erdheim-Chester disease. Description and depiction of a case with a review of information on the disease]. / Diabetes insipidus, následovaný po 4 letech dysartrií a lehkou pravostrannou hemiparézou--první klinické príznaky Erdheimovy-Chesterovy nemoci. Popis a zobrazení prípadu s prehledem informací o této nemoci.

In 2004, diabetes insipidus was the first clinical sign of Erdheim-Chester disease in our patient. Following introduction of substitution therapy with adiuretin, the patient had no further health complaints for four years until 2008 when he gradually developed dysarthria and, consequently, movement disorder in the form of mild right hemiparesis. The first CNS CT scan (2004) did not reveal any pathology. The first pathological MRI of the brain in 2006 - thickening of pituitary stalk by pathological infiltration to 4-5 mm. During the following year, further infiltrates were detected in the CNS. The number and size of CNS infiltrates increased gradually on MRIs performed repeatedly up to 2008. Erdheim-Chester disease has become suspected based on PET-CT examination at the end of 2008. CT showed irregular structure of the skeleton with noticeable sclerotic foci in otherwise osteoporotic bone structure; changes were the most evident in the long bones of lower limbs, in the pelvic bones, skull and arms, while only one vertebra was affected from within the entire spine. Finding ofthickened aortic wall (up to 8 mm) as another pathological circumstance was consistent with the Erdheim-Chester disease-associated changes described as coated aorta. CT scan revealed clear fibrotic changes in the area of retroperitoneum. Applied fluorodeoxyglucose has accumulated in the bone foci described on CTscans as well as in the thickened wall ofthe thoracic and abdominal aorta (SUV 3.6). Tc-pyrophosphonate skeleton scintigraphy showed the same bone foci as PET-CT. Full body MRI showed pathological signal from the bone marrow of the above mentioned locations, particularly during STIR imagining, where there was clear abnormal signal corresponding to accumulated histiocytes, the higher signal of which was well-differentiated from the normal bone marrow. Measurement of bone mineral density with DEXA confirmed reduced density in lumbar vertebrae to the average value of - 2.7 SD (the lowest value was -3.1SD). The disease is associated with elevated inflammatory parameters: leucocytosis, thrombocytosis, elevated CRP and fibrinogen levels. Diagnosis was verified following histological assessment ofiliac bone marrow, where focal infiltrations with foamy histiocytes of typical immunophenotype (CD68+, CD1a-, S100-) were confirmed. Treatment was initiated with chemotherapy consisting of 2g/m2 of cyclophosphamide on day 1 and 200 mg/m2 of etoposide IV infusion on days 1-3, and followed by administration of 5 microg/kg of G-CSF and collection of haematopoietic peripheral blood stem cells (PBSC). PBSC collection was followed by 5-day administration of 5 mg/m2/day of 2-chlorodeoxyadenosine (Litac) administered to the patient at monthly intervals.

[Langerhans cell histiocytosis in adult patients--a disease with many faces. Experience of a centre and an overview of the disease symptoms]. / Histiocytóza z Langerhansových bunek u osob dospelého veku--nemoc s mnoha tváremi. Zkusenosti jednoho pracoviste a prehled projevu nemoci.

Over a period of 18 years, 17 patients with proven Langerhans cell histiocytosis (LCH) were treated at the Haematological Clinic in Brno. In 13 of them, the disease was diagnosed at adult age, and 4 patients were referred to the centre with LCH diagnosed at early child age. One of these 4 patients suffered from repeated recurrences of the disease at adult age and was diagnosed with progressive neurodegenerative damage of the CNS at the age of 25 which in its terminal phase resulted in the patient's immobility, loss of sphincter control, incapacity to communicate and death at the age of 32. LCH was diagnosed at adult age in 13 patients. The form with primary bone involvement was detected in 8 out of 13 patients (62%). Only 2 of 13 patients (15%) had multiple bone lesions upon diagnosis, the remaining 6 patients (46%) had only one lesion at the time of diagnosis. Repeated recurrence of bone involvement was only recorded in 3 out of 13 patients (23%). The combination of recurrent bone involvement and the development of lung affection (dyspnoea, irritating cough, nodularities and cysts in HRCT images) were documented in 2 out of 13 patients (15%). One of the patients diagnosed with LCH at the age of37 had repeated recurrence of bone involvement, which was also treated by 2 cycles of high-dose chemotherapy and autologous transplantation. He died of bronchopneumonia due to the affection of the lungs by LCH at 48 years of age. Primary extraoseal (extamedular) involvement was diagnosed in 5 out of 13 patients (38%) (mandibular gum infiltration, single cervical node infiltration, hand skin infiltration, infiltration of the perineal region and infiltration of the hypophysial infundibular and primary lung form of LCH). In the 1st case, excision was the solution applied to the infiltration of the lingual side ofthe gums, without further recurrence. In the 2nd case, the infiltrated region of skin over the metacarpophalangeal joint was irradiated and the infiltration disappeared. In the 3rd case, the first sign ofthe disease was diabetes insipidus in a 34-year-old man, and an infiltrate in the anal region similar to condylomata acuminata. The diagnosis was confirmed 2 years after the development of diabetes insipidus from perianal infiltrates. After treatment with leustatin in 4 cycles (10 mg a day for 5 consecutive days), control MR showed that the infiltration in the hypophysial infundibular had disappeared, while the finding in the perianal region only regressed by 50% after therapy with leustatin, the reason for subsequent application of radiotherapy (20 Gy). The finding in the perianal region is normal one year after therapy, but substitution therapy with adiuretin is still necessary. The 4th patient was a case of LCH with primary pulmonary involvement diagnosed on the basis of HRCT and lavage with an immunohistochemical proof (expression of CD1 and of protein S-100) of a high number of Langerhans cells. The occurrence of LCH at adult age is rare and the disease may affect the skeleton as well as other organs. Therefore each new osteolytic lesion should be submitted for histological exam, as well as each pathologic formation, because diagnosing the disease without a microscopic and immunohistochemical exams is not possible. In the case of occurrence of diabetes insipidus at adult age, LCH should be considered as one of the possible underlying diseases. LCH pulmonary involvement should be considered in patients with an interstitial pulmonary process and the examinations should be focused accordingly (thoracoscopy with sampling for histological exams or bronchoalveolar lavage) plus the indispensable immunohistochemical examination.

[Treatment of multiple angiomatosis involving the skeleton and the abdominal and thoracic cavities with interferon alpha, thalidomide and zoledronate]. / Lécba mnohocetné angiomatózy postihující skelet, brisní i hrudní dutinu interferonem alpha, thalidomidem a zoledronátem.

Multiple angiomatosis is a very rare disease formed by histologically benign angiomas spreading beyond single organ or tissue. In the case reported herein, hemangiomas affected several vertebrae of a young man and spread through his peritoneal cavity projecting to his stomach and causing recurrent hematemesis. Also affected was the mediastinum. The patient suffered from bone pain and digestive problems. Initial treatment involved 2 drugs with antiangiogenic effect: interferon alpha (initial dose of6 million units 3 times a week, later reduced to 3 million units 3 times a week due to adverse effects) and zoledronate (4 mg i.v. every 28 days). Even though the therapy eliminated bone pain after 2 months, CT check at a later stage showed but little regression of the mass of the angiomas in the abdominal cavity and the mediastinum. Substantial reduction in the mass of the angiomas to merely residual quantity, i.e. partial remission of the disease, was achieved only after the addition of 100 mg/day thalidomide (Myrin) to the above mentioned doses of interferon and zoledronate administered on a regular basis. However, the disease recurred after the therapy was interrupted, and the above triple combination therapy has had to be restored. Maintenance therapy will succeed to repeated achievement of remission of angiomas. A very good therapeutic effect was recorded for combined interferon alpha, thalidomide and zoledronate in this specific case of multiple angiomatosis.

[Systemic and paraneoplastic manifestations of malignant diseases]. / Systémové a paraneoplastické projevy maligních onemocnení.

Timely diagnosis of malignant diseases largely depends on attention being given to early symptoms and on timely start of an extensive diagnostic process. Only this way can a tumour be diagnosed in its initial stage, and better effect of therapy can be achieved. The following overview provides a list of systemic (paraneoplastic - distant) manifestations of a tumour, and of symptoms related to local tumour expansion. The objective of the overview is to draw attention to all early symptoms of malignant diseases in patients, and to contribute to timely diagnosis and treatment.

[Recommendations for early identification of damage to the skeleton by malignant processes, and for early diagnosis of multiple myeloma]. / Doporucení pro casné rozpoznání postizení skeletu maligním procesem a pro casnou diagnostiku mnohocetného myelomu.

The number of newly diagnosed cases of multiple myeloma in the Czech Republic is about 3-4 per 100 000 persons per year. In the higher age groups, the incidence increases. Multiple myeloma is an illness that reacts well to treatment which can result in periods of remission lasting for years. Some of the patients are even able to return to work. A pre-requisite for successful treatment is early diagnosis and this is usually in the hands of first line physicians. This is the reason why the Czech Myeloma Group, in conjunction with neurologists, orthopedicians and radio diagnosticians has issued the following recommendations for first line physicians containing a more detailed description of the symptoms and the diagnostic pitfalls of the disease. This disease reminds a chameleon for the variety of its symptoms. For the sake of clarification, we shall divide multiple myeloma symptoms into five points, each of which is reason enough to warrant an examination to confirm or rule out a malignant cause of health problems (a negative result does not automatically mean exclusion). If any of the recommended examinations results positive, the diagnostic process must be continued, in which case a general practitioner refers the patient to a specialist health centre. Observing these recommendations should minimize the number of cases of late diagnosis. 1. Bone destruction symptoms. - Unexplained backache for more than one month in any part of spine even without nerve root irritability or without pain in other part of skeleton (ribs, hips, or long bones). - Pain at the beginning of myeloma disease is very similar to benigne common discopathy, however the intensity of backache is decreasing within one months in benigne disease. In the case of malignant process the intensity of bone pain is steadily increasing. - Immediate imaging and laboratory investigation are indicated by resting and night pain in spinal column or in any part of skeleton. - Backache with the sign of spinal cord or nerve compression should be sent for immediate X Ray, and focussed CT/MRI followed by acute surgery if needed. - Osteoporosis especially in men and premenopausal women. 2. Features of changed immunity or bone marrow function. Persistent and recurrent infection, typical is normochromic anaemia, with leucopenia and trombocytopenia. 3. Raised erythrocyte sedimentation rate even increase concentration of total plasma protein. 4. Impaired renal function. Increased level of creatinin or proteinuria, nephrotic syndrome with bilateral legs oedema. 5. Hypercalcemia with typical clinical symptoms (polyuria with dehydratation, constipation, nausea, low level conscience, coma). Every one from these points has to be reason for general medical doctor to start battery of tests: -X-ray of bones focused to painful area (mandatory before physiotherapy, local anaesthesia or other empiric therapy). If plain X-ray does not elucidate pain and symptoms are lasting more than one month, please consider all circumstances and results from laboratory investigation. This patient needs referral to the centre with MRI/CT facilities (CT or MRI is necessary investigation in case of nerve root or spine compression). -Investigation of erythrocyte sedimantion rate (high level of sedimentation of erythrocyte can indicate multiple myeloma). -Full blood count. -Basic biochemical investigation serum and urine: serum urea, creatinin, ionts including calcium, total protein, and albumin CRP (high concentration of total protein indicates myeloma, low level of albumin indicates general pathological process, similary increased concentration of fibrinogen, impaired renal function indicates myeloma kidney, however hypercalcemia is typical for highly aggressive myeloma). -Quantitative screening for IgG, IgM and IgA in serum (isolated raised level one of immunoglobulin with decreased level of the others indicates myeloma). -Common electrophoresis of serum is able to detect monoclonal immunoglobulin level at few gramm concentration. If all the laboratory investigation are in normal level the possibility that the current problems are multiple myeloma origine is smaller, but it does not exclude one of rare variant--non secretory myeloma (undifferentiated plasmocyt lost characteristic feature to produce monoclonal immunoglobulin). If any of tests indicate the possibility of myeloma, patient require urgent specialist referral to department with possibility to make diagnosis of malignant myeloma.