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.

Preoperative nutritional management - compliance with current guidelines.

Introduction: Major surgery poses a significant stress to the patient. The nutritional status is one of crucial factors that have a substantial impact on the final outcome of the surgery. Preoperatively established malnutrition or an increased nutritional risk in this group of patients requires a maximum effort to minimize this negative impact as soon as the operation is scheduled.

Therapy of Castleman's disease with siltuximab - case report and review of literature.

Background: Idiopathic multicentric Castleman disease (iMCD) is characterized by constitutional symptoms, enlarged lymph nodes and laboratory test abnormalities, which are primarily related to the overproduction of interleukin-6 (IL-6). This form (iMCD) was treated earlier with cytostatics used for lymphoma, later with bio-logic therapy as rituximab, immunodulatory drugs and proteasome inhibitors, and in the last years with an anti-IL-6 antibody, siltuximab. Siltuximab is a human-mouse chimeric immunoglobulin G1k monoclonal antibody against human IL-6 approved in the European Union for the treatment of iMCD.

[Successful treatment of SAPHO syndrome (chronic nonbacterial osteomyelitis and acne) with anakinra and denosumab. Case report and review of therapy].

SAPHO is an acronym derived from capital letters of Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO). SAPHO syndrome is an umbrella term covering a constellation of bone lesions and skin manifestations. A 40-year-old male complained about his jaw and back pain, swelling of multiple joints and weight loss accompanied by physical deterioration and acne type skin lesions.

Patients benefi ts from physicians empathy and results of including of empathy development into medical training.

Background:  The extent of empathy is an individual human property, not completely dependent on cognitive intelligence. People arise with certain genetic fundament for empathy but the ability to perceive empathically develops further during the life. There has been much discussion in the medical literature about the importance of empathy and physician communication style in medical practice.

[Therapy of immunoglonuline IgG4 related disease (IgG4-RD)].

Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects.

[IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease].

Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder. Autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum, prostate and many others.

[Langerhans cell histiocytosis (LCH). Overview of symptoms of LCH, which may lead the patients to any of these medical specialists].

Langerhans cell histiocytosis (LCH) is a rare condition with incidence in adults 1-2/1 million, wherein Langerhans cells proliferate abnormally, adversely impacting organs including most frequently bones, skin, lungs, pituitary gland, lymph nodes, gums and other organs. The LCH course varies widely among patients from a self-limiting condition, to one that progresses. But LCH only very rarely culminates in death.

Slow increase of bilirubin concentration during administration of lenalidomide, bortezomib and dexamethasone for multiple myeloma (unmasking previously undiagnosed Gilbert syndrome) and disappearance of necrobiotic xanthogranuloma after complete remission of multiple myeloma.

Background: Lenalidomid ranks among immunomodulatory drugs. There are a few of the more common side effects, like a higher risk of venous trombembolism or diarrhea. Other side effects are rare.

Rosai-Dorfman-Destombes disease - histiocytic disorder with inflammatory manifestation.

Background: Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytosis characterized by accumulation of activated histiocytes within affected tissues. Although the immunophenotype of this disease was described, the pathophysiology of this disease is still not sufficiently understood. Recent studies have found NRAS, KRAS, MAP2K1, and ARAF mutations in RDD lesions, raising the possibility of a clonal origin in some forms of RDD while in other cases reactive origin or association with other malignant and autoimmune disease is supposed.

Retroperitoneal fibrosis - diagnosis and treatment.

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain.

Defensive medicine and the influence of litigation on doctors.

The term defensive medicine is used to describe the behavior of healthcare providers motivated by fear of litigation due to malpractice. It includes both avoidance behavior when the physician is unwilling to perform high risk procedures, as well as excessive ordering of extra tests and procedures. This leads to unnecessary diagnostic and therapeutic interventions which may be invasive and costly.

[Multicentric Castlemans disease. Symptoms, diagnostics and therapy].

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric (UCD) or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD).

Factors Determining Postoperative Ileus After Surgery for Colon Cancer: Comparison of Right- and Left-Sided Resections.

Introduction: The passage of flatus and stool, as well as tolerating a solid diet, represents a crucial moment in recovery after colonic resections. The present study compares functional recovery after left and right colectomies for colon cancer.

Materials And Methods: This is a retrospective analysis.

Plectin-mediated cytoskeletal crosstalk controls cell tension and cohesion in epithelial sheets.

The coordinated interplay of cytoskeletal networks critically determines tissue biomechanics and structural integrity. Here, we show that plectin, a major intermediate filament-based cytolinker protein, orchestrates cortical cytoskeletal networks in epithelial sheets to support intercellular junctions. By combining CRISPR/Cas9-based gene editing and pharmacological inhibition, we demonstrate that in an F-actin-dependent context, plectin is essential for the formation of the circumferential keratin rim, organization of radial keratin spokes, and desmosomal patterning.

Basic information for identifying psychological disorders caused by malignant disease.

Background: Improvements in cancer dia-gnosis and treatment explain a substantial increase in the number of patients chronically affected by or recovering from cancer. This is a fragile population, physically, psychologically and socially affected by the consequences of the disease and the associated treatment. The National Comprehensive Cancer Network (NCCN) reacted to this fact, creating the NCCN guidelines for survivorship.

Colonic perforation in patients with COVID-19 pneumonia - case reports.

Introduction: Gastrointestinal complications in critically ill patients during the COVID-19 pandemic pose a diagnostic and treatment dilemma.

Case Report: We present two cases of SARS-CoV-2 positive patients treated in our department for colon perforation. One patient was operated for a diastasic right colon perforation due to acute over distension of the bowel.

Social factors influence the course and prognosis of malignat diseases.

Background: A statement of the dia-gnosis of malignant disease fundamentally changes the identity of a man. A healthy person changes to a patient. A lot of studies analyzed the influence of marital status on the disease development.

[Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome].

Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome Schnitzler´s syndrome is a very rare, adult-onset, apparently acquired autoinflammatory disease. Chronic urticarial rash and symptoms of systemic inflammation including fever, arthralgia and bone pain with the presence of monoclonal immunoglobulin M (IgM), rarely IgG, are among hallmarks of the disease. We performed a retrospective study of 6 patients (5 men, 1 woman) diagnosed with Schnitzler´s syndrome fulfilling the Strasbourg criteria who had been treated at our centre in the University Hospital Brno from 2007 to 2021.

IgG4-releated disease.

Background: IgG4-related disease (IgG4-RD) is a non-malignant, chronic, immune-related disease. It was first recognized as a distinct disease in 2012 and the first classification criteria were published in 2020. This new entity can cause fibroinflammatory lesions in nearly any organ.

[Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) Part I. Pathophysiology, clinical symptoms and recommend screening for vascular malformations].

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant disorder that causes abnormal blood vessel formation. Patients with HHT may have telangiectasias and later may develop arteriovenous malformations in various organs. Pacients suffer from many complications caused by the malformations and therefore by patients with HHT must by performed screening of this arteriovenous malformations.

[Unicentric Castlemans disease. Symptoms, diagnostics and therapy].

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms - multicentric Castleman disease.

[Hereditary hemorrhagic telangiectasia (OslerWeberRendu syndrome) - Part II. Pharmacological therapy and international guidelines for the therapy 2020].

Hereditary hemorrhagic telangiectasia also known as Osler-Weber-Rendu syndrome, is an disorder that causes abnormal blood vessel formation with bleeding. Inhibition of angiogenesis amelioretes bleeding complication. Anti-angiogenic agents such as bevacizumab, aflibercept, thalidomid, lenadomid and other new anti-angiogenic thyrosinkinase inhibitors, as well as sirolimus and takrolimus have emerged as a promising systemic or local therapy in reducing bleeding complications but are not curative.

Abdominal abscess associated with salmonellosis case report.

Introduction: Although gastroenteritis is considered to be a non-surgical disease, rare complications necessitating surgical intervention may occur.

Case Report: We present a patient who underwent acute small bowel resection due to an abdominal abscess, which developed in association with Salmonella enteritis.