Overview of histiocytic and dendritic disorders by the 5th version of WHO Classifi cation of Hematolymphoid Tumours from 2022.

BACKGROUND: Histiocytoses are rare disorders characterized by the accumulation of macrophages, dendritic cells, or monocyte-derived cells in various tissues and organs of children and adults, with a wide range of clinical manifestations, presentations, and histology. The histiocytoses are classified according to the WHO Classification, the last version of which was published in 2022, or according to the Histiocyte Society Classification, with the last version published in 2016. PURPOSE: This text provides an overview of histiocytoses as described in the WHO Classification 2022.

[Rare case of a giant parathyroid adenoma]. / Raritní prípad obrovského adenomu prístítného telíska.

Currently, primary hyperparathyroidism is the third most common endocrine disorder worldwide. In the last years, though, the numbers of patients with this diagnosis have been clearly increasing. Females are affected more often, and the age of patients is usually over 50 years. The diagnosis is guided by clinical symptoms of the patient and by biochemical and imaging methods results. [1] When parathyroid adenoma is identified as the source of primary hyperparathyroidism, the necessary extent of resection is performed. The present case involved a rapid diagnostic process necessary to stabilize the patient, together with localization of the adenoma for the indicated surgery. Ultrasound-guided exploration found a surprisingly large tumour of the parathyroid gland causing the acute clinical condition with symptoms suggestive of primary hyperparathyroidism with discrete mechanical compression of the upper mediastinum.Key words: critical diagnosis of hyperparathyroidism acute-active parathyroid adenoma extirpation of a huge parathyroid tumour - normalization of the clinical condition.

[Laparoscopic adrenalectomy--indications and selection criteria]. / Laparoskopická adrenalektomie--indikace a selekcní kritéria.

INTRODUCTION: Laparoscopic adrenalectomy has become a standard surgical procedure for the most of adrenal gland disorders. Hormonal active adenomas, feochromocytomas even some malignant tumors are the most frequent indications. The number of operations for accidentally diagnosed foci has grown rapidly. It has been suggested to revise recommendations specifying criteria, based on which incidentaloma is indicated for adrenalectomy. The aim of this work is to compare the results of adrenalectomies for hormonal active lesions and incidentalomas. MATERIAL AND METHODS: An analysis of 65 patients who underwent adrenalectomy in the Department of Surgery University Hospital Brno Bohunice from 2005 to 2010. Correlation between preoperative examination outcomes and postoperative histology findings was performed. Furthermore, findings in patients indicated for surgery for hormonally active versus for hormonally inactive suprarenal tumors were compared. RESULTS: Thirty-eight patients underwent laparoscopic surgery for hormonally active adrenal tumors, one for bilateral metastasses of bronchogenic carcinoma. In 26 cases adrenalectomy was indicated for incidentaloma. Adrenal hyperplasia was the commonest histological finding in the group with hormonally inactive tumors. No carcinoma was detected in this group. In 5 of 19 patients operated for suspective feochromocytoma, the procedure did not result in blood pressure adjustment and feochromocytoma was histologically confirmed in 11 out of the 19 subjects. The size of the tumors was significantly higher in incidentalomas, compared to hormonally active pathologies. No incidentaloma and hypertension subjects experienced alteration in their clinical condition after the procedure. CONCLUSION: Laparoscopic adrenalectomy is a standard procedure in the majority of hormonally active focal suprarenal conditions. Patients with accidentally detected suprarenal tumors should be carefully indicated, taking into consideration internal comorbidities and any surgical procedures in a patient's history. The benefit of adrenalectomy for the clinical condition alteration is arguable in incidentalomas. The National Institutes of Health U.S.A. (NIH) consensus guidlines should be strictly followed during the decision making proces. Indication for adrenalectomy in tumors of less than 6 cm and with benign appearance on CT or MRI is not considered rational.

[The value of intraoperative ultrasonography in parathyroid surgery]. / Výteznost peroperacní ultrasonografi v chirurgii prístítných telísek.

INTRODUCTION: Ultrasound of the neck is a sovereign complementary imaging method suitable for determining morphological changes in thyroidology which uses the so-called B-mode two-dimensional images for the neck area that are complemented by duplex Doppler imaging. Diagnostic verification is performed by sonographists from among radiologists and endocrinologists providing regular outpatient care; the surgeon uses ultrasound for navigation to attain the focus to be operated on. Sonography will provide information on the structure of the thyroid, of the parathyroids, their relationship to the surrounding organs (location, size, relationship to breathing and swallowing pathways). We search for the presence of any pathological abnormalities such as the affection of other organs, tumour invasion into surrounding tissue; it is also important to assess the size and nature of the cervical lymph nodes pretracheally, prelaryngeally, paratracheally, along the vascular plexus and supraclavicularly, where thyroid tumours may form metastases. USG assessment of the regional lymph nodes should not be forgotten before surgical management of thyroid nodules or the parathyroids. Thanks to the introduction of ultrasound as an additional screening method in parathyroid surgery we have gained the opportunity of more precise and successful localisation of pathological parathyroid lesions, which is beneficial for patients with hyperparathyroidism. MATERIAL AND METHODS: We worked with a group of patients from 2002 to 2010 when we selected 370 prospectively followed patients operated on for endocrine disease of the thyroid and parathyroid glands. The group consisted of 77 men and 293 women. This group was further selected according to the required criteria specified in the text. Since we are focusing on peroperative ultrasound and on the detection and specification of the position of the parathyroid glands for the surgeon, we are concentrating chiefly on pathological parathyroid glands. These are imaged as circumscribed hypoechoic lesions with hyperechoic margin. Ultrasonography is considered both a sovereign standard diagnostic method and a very valuable navigational method used to localise pathological glands. We use a linear as well as a special "hockey stick" probe which has a higher resolution. RESULTS: For comparison, both detection methods - gamma navigation and ultrasound - are dealt with. We are fully aware of the small number of patients in the selected groups. The results of the statistical analysis show that the use of ultrasound and gamma navigation shortens the overall operation time. More interesting, however, is the indicator of the range of variation, i.e. the difference between the maximum and minimum length of operation. When using one or the other detection method, the variation range is significantly lower and it is apparent that using the gamma-ray or ultrasound navigation system, the detection of the parathyroid gland, particularly when unusually located, is much simpler and thus faster. This finding is also supported by an additional parameter, variance, which expresses the variability of the distribution of values - around the average length of operation. CONCLUSION: Preoperative and intraoperative detection of hyperactive parathyroid glands is useful for minimizing the extent of surgical intervention, reducing operation time and decreasing the risk of perioperative complications. It is not necessary to use a radionuclide and therefore the method can be used even where there is no nuclear medicine station. It is also possible to detect glands which do not accumulate radionuclides and thus cannot be imaged with MIBI scintigraphy/SPECT CT. Still, an experienced surgeon remains irreplaceable. We dare say that if such a surgeon can make use of the above mentioned auxiliary methods in addition, the rate of success in this field of surgery increases substantially.

[Schnitzler syndrome: case report, the experience with glucocorticoid and anakinra (Kineret) therapies and monitoring of systemic cytokine response]. / Schnitzler-syndrom: popis prípadu, zkusenosti s lécbou glukokortikoidy a preparátem anakinra (Kineret) a sledování cytokinové odpovedi organizmu.

Schnitzler syndrome is a rare idiopathic disease characterized by chronic urtica, presence of monoclonal IgM immunoglobuline and further, less common symptoms. This case report describes another case of this disease affecting a male adult born in 1963. The first symptoms, eruptions of non-pruritic urticarial rash, appeared in this patient at the age of 43. In addition, bone pains (mainly tibias) and joint pains (mainly knees) were present. Later on however, severe attacks of fever, chills and shaking together with bone and joint pains were added to during which new urticarial eruptions appeared. Primarily, the man was followed up without any substantial therapeutic results at a department of dermatovenerology, subsequently, due to a finding of monoclonal IgM kappa immunoglobulin (serum concentration 1.9 g/l) he was referred to our department for the reason of gammopathy being a differential diagnosis. On a CT scan hyperostosis in claviculae and pelvic bones was identified. Also on the CT, an increase in cortical thickness was described in the long bones of the lower extremities, where areas of technetium pyrophosphate accumulation were identified on a bone scintigraphy. These areas were found in the chest and sacral regions as well. From the blood exams, the proinflammatory status of the organism was apparent (CRP 35.9 mg/l, erythrocyte sedimentation rate 92 mm/h, leukocytes 12.4 x 10(9)/l). After excluding other differential diagnoses, the patient was diagnosed with Schnitzler syndrome. As regards therapy, we made initial use of the effect of corticoids which abated the symptoms, however, these were causing serious adverse reactions in the form of iatrogenous Cushing's syndrome. The therapy took a turn only after biologic therapy with anakinra (interleukin-1 receptor antagonist) had started, which minimized the Schnitzler symptoms with very good drug tolerance. In the work we measured serum levels of interleukins for disease activity monitoring. The most sensitive were interleukins IL-6 and especially IL-18 the levels of which were the highest at the time of clinical exacerbation of the disease, whereas the levels of IL-1beta and TNF-alpha (tumour necrosis factor) were during all measurements below the limit of detection. Concerning the growing numbers of the reports on successful biological therapy with anakinra and our positive experience, we propose that the therapeutic response to anakinra should be included within the diagnostic criteria of Schnitzler syndrome, which is significant above all in differential diagnosis thereof.

[Higher incidence of thyropathy in patients with oesophageal achalasia. Genetic, autoimmune, regional or just a random association?]. / Vyssí výskyt tyreopatií u achalázie jícnu. Genetická, autoimunitní, regionální ci jen náhodná souvislost?

INTRODUCTION: The etiology of esophageal achalasia is still largely unknown. Inflammatory response to an initial stimulus on the level of genetic and/or immune predisposition may be the underlying cause of the disease. The final result is progressive disappearance of ganglion cells in the myenteric plexus and motility disorder. Autoimmune thyropathy (AIT) is a typical disease involving genetic background and immune response disorder. PATIENTS AND METHODS: 44 patients (of which 30 women and 14 men) with diagnosed esophageal achalasia and a control group of patients with esophageal reflux of corresponding age and sex were screened for thyroid disease. RESULTS: Thyroid disease was diagnosed in 15 out of 44 patients with achalasia (34%). Thyropathy was detected in 11 women (37%) and 4 men (28%). AIT was detected in 10 patients, in 4 of whom with hypfunction, nontoxic cystic or nodular goitre was detected in 4 patients, 1 patient was after strumectomy for benign node. Positive antithyroid antibody was newly detected in 4 patients with achalasia; subclinical hypothyreosis was found in one of them. There were two cases ofAIT with subclinical hypofunction and 1 case of nontoxic goitre in the control group (7%). The difference was statistically significant (p < 0.01). CONCLUSION: The incidence of thyroid disease proved higher in patients with achalasia than in the controls. The rate of occurrence of thyroid disease exceeded significantly the occurrence in the population. The association of achalasia with prevailingly autoimmune thyropathy may corroborate the importance of autoimmunity in the etiopathogenesis of the disease.

[Pitfalls in the diagnosis of carcinoids in patients treated with aminosalicylates]. / Uskalí v diagnostice karcinoidu u pacientu lécených aminosalicyláty.

INTRODUCTION: Carcinoid is one of the most common endocrine active tumours of the gastrointestinal tract. 90% of all carcinoids originate from enterochromaffine cells in the GIT. In the literature the relationship of carcinoid of the bowel and IBD is mentioned, in particular Crohn's disease. The screening test used under our conditions is assessment of the excretion of the metabolite serotonin, 5-hydroxyindole acetic acid (HIAA) in urine. The authors wish to draw attention to falsely positive results of 5-HIAA in urine by the HPLC method in patients with CD treated with aminosalicylates (ASA). METHODS: In order to rule out carcinoid in chronically active CD the authors assessed after discontinuing known interfering drugs the excretion of HIAA by the HPLC method in 14 patients. The results were confirmed in laboratories of the Czech Academy of Sciences using mass spectrometry by desorption and ionization with a laser in the presence of matrix (MALDITOF MS), analytical procedures during processing of the specimens were modified according to Coward. In two patients urinary HIAA excretion was assessed on five consecutive days after discontinuation of ASA. RESULTS: The mean values of HIAA excretion by the HPLC method was highly suspicious of interference. Using the MALDI-TOF MS the authors did not detect 5-HIAA in the fraction of the interfering peak. After discontinuation of 5-ASA the interference disappeared after 4 days. By adjustment of the pH of the mobile buffer phase according to Coward the interfering peak was separated from the 5-HIAA peak. HIAA excretion assessed by the HPLC method was not significantly higher in patients after discontinuation of 5-ASA. CONCLUSION: The authors wish to draw attention to the possible development of carcinoid on the background of chronically active CD. Using assessment of urinary HIAA excretion by the HPLC method as a screening test it is essential to discontinue 5-ASA for at least 4 days before collection of urine or modify the analytical procedure when processing the specimen.

[Protective effect of essential phospholipids on liver injury due to total parenteral nutrition]. / Protektivní vliv esenciálních fosfolipidu na poskození jater úplnou parenterální výzivou.

OBJECTIVE: It is known that total parenteral nutrition (TPN) causes liver damage by various mechanisms and leads to an increase of transaminases and obstructional enzymes. From this aspect TPN can be considered an external factor which causes liver damage. In our investigation we wanted to find out whether parenteral administration of essential phospholipids (EPL) can have a protective effect on this damage. PATIENTS AND METHODS: Our investigation comprised 20 patients where TPN was indicated, usually on account of severe acute exacerbation of a non-specific inflammation of the gut. The patients were divided into two groups. Ten patients were treated by intravenous administration of essential phospholipids (Essentiale, Aventis), 50 mg every 6 hours for a period of two weeks. The control group comprised ten patients without hepatoprotection. The bilirubin, ALT, AST, GMT, ALP values were assessed before the initiation of the study, on the seventh and fourteenth day. The results were statistically processed by the paired and non-paired t-test. RESULTS: The baseline results of the mentioned tests did not differ significantly between groups. Bilirubin and AST did not change significantly during the investigation. In the control group we found, as compared with baseline values, a significantly increase of ALT on the seventh and fourteenth day, a significantly increase of GMT on the seventh and fourteenth day and a slight non-significant rise of ALP on the fourteenth day. In the ELP treated group, as compared with baseline values, a significant rise of ALT occurred on the fourteenth day. We did not observe a significant rise of GMT and ALP. Between the ELP treated and control group a significant increase of GMT and ALP occurred in the control group, the other values did not differ between groups. CONCLUSION: Parenteral EPL administration can have a favourable effect on liver damage caused by TPN, associated with cholestasis and biliary sludge. This conclusion can be hypothetically explained by improved bile fluidity and protection of the bile pole of the hepatocyte by essential phospholipids. Therefore their administration during TPN can be recommended.

Incidence of chronic pancreatitis in the Czech Republic.

There have been only a few studies dealing with the incidence of chronic pancreatitis published. Over the last 80 years, original studies describing the incidence of chronic pancreatitis were undertaken, including studies in Scandinavia, Switzerland, Hungary and Poland. Incidence varied geographically, from 1.6 new cases per year among 100,000 inhabitants in Switzerland to 23 new cases among 100,000 inhabitants in Finland. The aetiology of 70% of all cases was alcoholic pancreatitis. The incidence of chronic pancreatitis in the Czech Republic is 7.9 per 100,000 inhabitants per year, similar to that of Denmark and Germany, but higher than that of Poland or Switzerland. In our study, the main aetiological factor was alcohol (65.4% of all cases, with consumption of alcohol of > 75.0 g/day).