Hematopoietic stem cell transplantation (HSCT) is an effective method of therapy which is widely used for the treatment of a number of diseases in children. “Graft-versus-host” disease (GVHD) is a severe complication that can develop after HSCT. The incidence of GVHD, according to some authors, ranges from 40 to 60%. The main target organs of acute GVHD are skin, liver, and gastrointestinal tract. Early diagnosis is critical in choosing the optimal treatment. The clinical manifestations of intestinal GVHD are non-specific. Currently, the “gold standard” of GVHD diagnosis is the histopathological assessment of biopsy samples. However, a conditioning regimen, cytomegalovirus infection, and/or treatment with immunosuppressive drugs can also induce apoptosis and lead to the same microscopic changes as the ones seen in GVHD. The main goals of an endoscopic examination are the visual assessment of the mucosa and a biopsy. Several articles describe macroscopic changes in the colonic mucosa characteristic of GVHD which can be used as a diagnostic criterion. Here, we review related literature and report a case of severe acute GVHD as well as evaluate the patient’s clinical manifestations and endoscopic findings. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.

Chronic granulomatous disease (CGD) is a primary immunodeficiency (PID), characterized by a defective production of reactive oxygen species by phagocytes. Infectious diseases are a classic manifestation of this form of PID; however, many patients present with a variety of inflammatory and immune non-infectious complications. We analyzed non-infectious complications in a group of 60 patients with CGD, who were treated in Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology (Moscow, Russia) since 2012 to February 2020. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Non-infectious manifestations were recorded in 53/60 patients, the most frequent of which were granulomatous complications (81.7% (49/60) of cases) in the following organs - the gastrointestinal tract, lungs, liver and lymph nodes. The median age of granulomas presentation was 3 years, and 45.8% of patients had a combined granulomatous lesion of several organs. Hepatomegaly (40%), splenomegaly (31.7%), dermatitis (21.7%) and chorioretinal lesions (11.3%) were other non-infectious complications. Hematological features outside acute infectious episodes included monocytosis (26.7%), eosinophilia (20%), neutrophilia (13.3%). 96.7% of patients had anemia with a decrease level of serum iron (65%), 31.7% - with signs of hemolysis. Autoimmune manifestations included arthritis, thyroiditis, immune thrombocytopenia or immune neutropenia. Non-infectious complications comprise a significant part of the CGD manifestations and often are the first symptoms of the disease. Awareness of this fact is very important for multidisciplinary approach in treatment of CGD. Delayed diagnosis and immunosuppressive therapy of non-infectious complications without prophylactic antimicrobial therapy can be life-threatening for patients with CGD.

Chronic granulomatous disease (CGD) is a primary immunodeficiency (PID), characterized by a defective production of reactive oxygen species by phagocytes. Infectious diseases are a classic manifestation of this form of PID; however, many patients present with a variety of inflammatory and immune non-infectious complications. We analyzed non-infectious complications in a group of 60 patients with CGD, who were treated in Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology (Moscow, Russia) since 2012 to February 2020. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Non-infectious manifestations were recorded in 53/60 patients, the most frequent of which were granulomatous complications (81.7% (49/60) of cases) in the following organs - the gastrointestinal tract, lungs, liver and lymph nodes. The median age of granulomas presentation was 3 years, and 45.8% of patients had a combined granulomatous lesion of several organs. Hepatomegaly (40%), splenomegaly (31.7%), dermatitis (21.7%) and chorioretinal lesions (11.3%) were other non-infectious complications. Hematological features outside acute infectious episodes included monocytosis (26.7%), eosinophilia (20%), neutrophilia (13.3%). 96.7% of patients had anemia with a decrease level of serum iron (65%), 31.7% - with signs of hemolysis. Autoimmune manifestations included arthritis, thyroiditis, immune thrombocytopenia or immune neutropenia. Non-infectious complications comprise a significant part of the CGD manifestations and often are the first symptoms of the disease. Awareness of this fact is very important for multidisciplinary approach in treatment of CGD. Delayed diagnosis and immunosuppressive therapy of non-infectious complications without prophylactic antimicrobial therapy can be life-threatening for patients with CGD.

Infectious esophagitis (IE) is commonly seen in immunocompromised patients. IE may be the first symptom of immunodeficiency state, also can be complication of immunosuppressive therapy in patients with hematological and oncological diseases. Severe complication of IE is esophageal stenosis. Patient management tactics continue to be discussed. A purpose of our publication is to demonstrate our experience in the management of patients with infectious esophageal stenosis. The etiology, features of the clinical and endoscopic picture, as well as the effectiveness of drug therapy and endoscopic methods of treatment have been analyzed. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. In each case, parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications.

Oncological, hematological and immunological diseases themselves or in the course of treatment are often accompanied by a decrease in the parameters of peripheral blood. If there are indications for endoscopy, the doctor performing the research should necessarily evaluate the safety of the procedure and the risks of bleeding in thrombocytopenia. To date, there are no single hematological criteria in the world for safe endoscopy procedure. The aim of the work is to evaluate the possibility of carrying out endoscopic studies in patients with thrombocytopenia and to determine the indications for blood transfusion therapy. The retrospective analysis included 82 patients aged 2 months to 18 years (median age – 9.6 years), who were treated at the Dmitry Rogachev National Center from January 2016 to March 2017, and who underwent diagnostic endoscopy with a platelet count ≤ 50×109/l or an endopic study with biopsy at a platelet count of ≤ 80×109/l, and 19 patients aged 1–18 years (median – 9.26 years), whom endoscopy was performed with prophylactic platelets transfusion. In the endoscopic examination, contact bleeding was observed in 17% of patients who did not receive prophylactic transfusion of platelets and in 21% of patients who underwent transfusion of platelet suspension prior to the study. When carrying out the diagnostic procedure (bronchoscopy and gastroscopy) with a platelet count of more than 20×109/l (median – 34×109/l), no spontaneous bleeding was observed, as well as minimal hemorrhagic syndrome. A safe endoscopy examination is possible to perform even in patients with thrombocytopenia, but clearly defined indications.