Search

Cytopenia commonly occurs in case of chronic lymphocytic leukemia. It can either precede the diagnosis of chronic lymphocytic leukemia or appear at any time during the disease. Autoimmune hemolytic anemia, immune thrombocytopenia, and partial red cell aplasia are most often found among cytopenias in chronic lymphocytic leukemia. At the same time, the development of cytopenia may be associated with the displacement of normal hematopoiesis cells by tumor lymphocytes. It is very important to accurately diagnose and identify the cause of cytopenia in chronic lymphocytic leukemia, since the prognosis and therapy differ significantly.

The aim of the study was to identify the epidemiological features of bacterial infections in patients and circulation of epidemiologically significant microorganisms in the bone marrow transplant (BMT) units. Materials and methods. The study was conducted in three BMT departments. To identify the epidemiological features of bacterial infections, a prospective observational study was conducted from 01 April 2014 to 31 August 2014 (91 patients) which included active detection of cases of infection and microbiological examination of patients. In order to detect the circulation of epidemiologically significant microorganisms, point prevalence studies (59 patients, 170 environmental samples) were conducted. To study the role of colonization, microbiological monitoring of patients on admission to the department was carried out, and repeated every week before discharge. Swab samples from skin and mucous membrane of the oral cavity and stool sampleswere included in the monitoring. In total, 639 samples from 50 patients were tested. Results. The study revealed a high incidence of infectious complications in BMT patients (100 cases of bacterial infections in 57 patients: sepsis 30.8 per 100 patients, pneumonia 26.4 per 100 patients, urinary tract infections 32.9 per 100 patients, soft tissue infection 9.9 per 100 patients, catheter-associated bloodstream infection 9.9 per 100 patients). Most infections were caused by Gram-negative bacteria, in particular K. pneumoniae. The onset of disease manifestation was preceded by colonization of the patient with these microorganisms. Fatal outcomes were observed from sepsis and pneumonia caused by K. pneumoniae and/or P. aeruginosa. Sepsis and pneumonia were often associated with cytopenia, RR 2.63 [95% CI 1.52–4.57] p = 0.004 and RR 3.97 [95% CI 2.19–7.19] p 0.001 respectively. In course of observation, the frequency of colonization of patients with microorganisms susceptible to antibiotics decreased, and colonization with resistant pathogens increased. A series of prevalence studies revealed a constant circulation of VRE in the BMT units with a contacttransmission via household objects. Multidrug-resistant gram-negative microorganisms did not have a constant circulation and appeared sporadically against the background of the constant circulation of susceptible strains of these pathogens. Conclusions. Prospective epidemiological surveillance and microbiological monitoring are required in the BMT units. Widespread contamination of household objects in the environment indicates the need to strengthen the hygienic education of patients and visitors as well as disinfection of these facilities. In addition, we recommend introduction of weekly fecal screening cultures to monitor intestinal colonization of patients with MDRO.

Background. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease of the blood system, characterized by intravascular hemolysis, cytopenia and thrombosis. Diagnostic errors with delayed diagnosis of PNH are often due to the variety of the clinical presentation and the lack of awareness of the doctors of this rare disease. Aim. The aim of the study was to characterize the options for clinical manifestation and the complexity of diagnosis of classical PNH. Materials & Methods. The research included 150 patients with PNH. The inclusion criteria were: 1) clinical and laboratory signs of intravascular hemolysis; 2) verification of the diagnosis using standard flow cytometry; 3) absence of aplastic anemia, myelodysplastic syndrome and primary myelofibrosis. The patients were of 13 to 72 years old (median age 34 years). The study population consisted of 89 (59 %) women and 61 (41 %) men. Results. The time before the diagnosis ranged from 0 to 455 months (median 33 months). The median size of the PNH clone among granulocytes and erythrocytes was 95 % and 41 %, respectively. The median of the lactic dehydrogenase was 7.2 times the upper limit of normal (ULN). Cytopenia occurred in 65 % of patients, including a combination of thrombocytopenia and neutropenia in 29 % of cases. Weakness and fatigue (99 %), hemoglobinuria (57 %), pain (52 %), icterus (46 %), dysphagia (37 %) and infection/fever (23 %) were the most common symptoms on the onset of the disease. Before the diagnosis of PNH, thrombosis or acute kidney injury was found in 22 % and 18 % of patients, respectively. Only 22 % of patients were initially diagnosed with PNH. In the remaining patients, the primary diagnosis was inadequate. Conclusion. The clinical manifestation of PNH is characterised by the presence of hemoglobinuria, cytopenia and early thrombosis in 57 %, 65 % and 22 % of patients, respectively. Errors of the primary diagnosis reach 78 % and lead to inadequate treatment. The results of the research showed the need for multidisciplinary approach and strict adherence to diagnostic algorithms of PNH in the risk groups, according to current recommendations.

Differential diagnosis of articular syndrome is one of the leading issues of primary medical sanitary care. Timely and correct diagnosis of rheumatic diseases made by an internist in an out-patient setting is a cornerstone of successful treatment and prevention of possible complications. The masks of clinical course, subtle symptoms, as well as pathomorphosis of the disease make the accurate diagnosis difficult to establish. Among rheumatic diseases more and more often isolated hematological manifestations are observed on the onset of the disease. Among them the leading place is taken by Felty’s syndrome. Being a form of seropositive rheumatoid arthritis, it is often associated with cytopenia. The aim of the article is to draw attention to the problem of timely diagnosis of Felty’s syndrome. The presented clinical case of Felty’s syndrome with agranulocytosis was based on clinical, laboratory, instrumental data obtained on examination of a patient. The features of the course, diagnostic methods and treatment methods of the pathology are discussed. The treatment efficacy of corticosteroids and cytostatics, similarities in clinical presentation and course of the syndrome with lymphoproliferative diseases and liver cirrhosis are presented. According to the obtained data a conclusion was made about the need for early diagnosis and complex examination in case of Felty’s syndrome for successful treatment of the nosology.

Aim. To characterize clinical, diagnostic and therapeutic syndromes of copper overloading in patients with hepatic lesion in combination with hemopoietic disorder. Material and methods. Treatment results and diagnostic findings are presented for patients with clinical picture of liver cirrhosis, cytopenia and copper overloading. The examination included standard clinical and specific tests, morphological investigation of the bone marrow, copper metabolism in dynamics. Results. A case of a patient is reported in whom Wilson's disease presented in debut with a picture of decompensated liver cirrhosis and immune thrombocytopania complicated by recurrent hemorrhagic syndrome. D-penicillomine treatment initiated ex juvantibus allowed verification of the diagnosis of Wilson's disease and achievement of marked clinical response. In another case laboratory signs of copper overloading were revealed in a patient with liver cirrhosis of viral etiology (HBsAg+) and deep cytopenia associated with unejfective hemopoiesis. Chelator therapy with D-penicillamine regressed cytopenic syndrome and improved functional capacity of the liver. Conclusion. Primary or secondary nature of copper overloading in patients with hepatic cirrhosis and critical cytopenia, pathogenesis of cytopenic syndrome, practical significance of copper hemochromatoses diagnosis are discussed.

Objective. To assess the synchronous intensive therapy (SIT) effect on clinical and laboratory signs in patients with systemic lupus erithematosus (SLE). Methods. SIT was performed in 30 SLE patients with unfavorable manifestation of the disease: lupus nephritis, neurolupus, cytopenia. The first stage of the treatment consisted of 3 consecutive (PA) procedures with 4-5 days intervals. Alter each PA procedure mcthylprcdnisolone 1,0 was given and after the second one — cyclophosphane (Cph) 1,0, Prednisolone dose did not exceed 40 mg/day per os. During the second stage Cph 1,0 was giveu monthly and prednisolone dose was slowly diminished till 10 mg/day to the 3 month of the treatment. Clinical efficacy was assessed after 1 and 6 month of intensive treatment and then every year. Results. In all cases SIT provided fast decrease of the disease activity after the 1st month. By the end of the 1“ stage manifestations of the lupus vasculitis, nephritis, neurolupus and cytopenia disappeared. After 6 month 93,3% of patients achieved medication-induced stabilization. Complications were absent. Conclusion. SIT is a very effective and safe method of treatment of severe SLE forms.