Diffuse large B-cell lymphoma is an aggressive type of lymphoma, potentially curable, with heterogeneous prognosis. The aim of this study was to determine prognostic significance of clinical, laboratory and immunohystochemical factors. The retrospective study was done in 50 patients with diffuse large B-cell lymphoma. The following parameters were investigated: demographic (age, sex), clinical (time to diagnosis, B symptoms, clinical stage), laboratory (erythrocyte sedimentation rate, haemoglobin, lactate dehydrogenase, albumine), standard and revised international prognostic index, and immunohystochemical parameters, cluster designation 20, B-cell-2, and Ki67 expression. There were 20 females and 30 males, their average age being 54 (22-83) years. The majority of patients had advanced disease: B symptoms in 76%, III and IV stage in 78%, increased lactate dehydrogenase in 74%, high risk standard international prognostic index in 62% of patients. B-cell leukemia/lymphoma 2 expression was found in 57%, and high Ki67 in 62% of patients.Rituximab-Cyclophosphamide, Hydroxydaunorubicin, Vincristine, Prednisolone and Rituximab-Cyclophosphamide, Hydroxydaunorubicin, Vincristine, Etoposide, Prednisolone were conducted in 72% (36), and Cyclophosphamide, Hydroxydaunorubicin, Vincristine, Prednisolone and Cyclophosphamide, Hydroxydaunorubicin, Vincristine, Prednisolone-like treatment in 28% (14) of patients. The complete remission rate was 74%, and the partial remission rate was 9%. A significant difference in survival was found between low intermediate and high intermediate S-IPI risk groups, good and bad risk R-IPI, and patients with complete remission and patients with other treatment responses. The other parameters, including Bcl-2 and Ki67 expression, and type of treatment did not show significant influence on survival. The expected five-year survival was 69 %. Our results have shown that international prognostic index, and complete remission status have prognostic significance in diffuse large B-cell lymphomas.

Introduction. ?Grey zone Lymphoma?? is associated with various entities. The last published classification of lymphoproliferative neoplasms includes mediastinal grey zone lymphoma. Precise diagnostic criteria are insufficient and establishing a diagnosis is as complex as deciding on treatment options. In this article pathologist and hematologist discuss issues on this topic through case presentation and literature review. Clinical characteristics. It presents in the younger population usually with a mediastinal mass, sometimes large, with compressive symptoms. Pathologic characteristics. Some cases can?t be classified neither as Hodgkin nor Primary mediastinal B-cell lymphoma. Morphology resembles Hodgkin, but with a positive immunophenotype for primary mediastinal or diffuse large B-cell lymphoma, and vice versa. Case report. We presented a case of a 33-year-old male with cervical lymphadenomegaly, B symptoms and clinical deterioration during the diagnostic period. After the first biopsy, differential diagnosis was Epstein-Barr virus-associated lymphoproliferative disorder or classical Hodgkin lymphoma. The second biopsy confirms Epstein-Barr virus-positive diffuse large B-cell lymphoma. The World Health Organization Classification of lymphoproliferative neoplasms - clinical perspective. The term ?grey zone lymphoma? is associated with overlapping diagnosis or uncertainty in diagnosis in more clinical settings than the ones provided in the 5th World Health Organization Classification. Discussion. For now, chemotherapeutic regimen (rituximab-cyclophos phamide, doxorubicin, vincristine, prednisone) stays the standard first line therapy for diffuse large B-cell lymphoma regardless of the Epstein- Barr virus status. Mediastinal grey zone lymphoma treatment varies: chemotherapeutic regimen (rituximab-cyclophos phamide, doxorubicin, vincristine, prednisone) was linked with better outcomes than chemotherapeutic regimen (adriablastin, bleomycin, vinblastine, dacarbazine) +/-R, but for some patients chemotherapeutic regimen (dose adjusted-etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) might be beneficial. Conclusion. Grey zone lymphoma is a rare hematologic malignancy that needs extensive sampling for correct diagnosis and is still subject to inter-observer variability.

Introduction. Niemann-Pick disease type B is an autosomal recessive disease caused by sphingomyelinase deficiency resulting in sphingomyelin accumulation in macrophages of various organs. Visceral involvement includes spleen enlargement, thrombocytopenia, dyslipidemia, sphingomyelin deposition in lung and liver, and bleeding risk. This is a rare disease and literature data about pregnancy in this setting are scarce. We present two favorable pregnancy outcomes in a patient with Niemann-Pick disease type B along with the review of the literature. Case outline. At the time of the first intended pregnancy, the patient was 34 years old. She had an extremely enlarged spleen, mild restrictive pulmonary disorder, hyperlipoproteinemia type IIb, thrombocytopenia with impaired aggregation tests. Cesarean section was indicated. She was prepared for delivery with platelet concentrates and prophylactic use of antibiotic. In the 36th week of gestation a Cesarean section without complications was performed. The newborn’s anthropometric parameters were BW 2490, BL 47 cm, HC 32 cm, and Apgar score was 7/8. Infant’s development was normal. Three years later in the second wanted pregnancy the same examinations were done. The planned Cesarean section was done without complication after the same procedures, including prophylactic use of antibiotics and platelet concentrates, and healthy female child was born. Conclusion. A multidisciplinary approach in female patients who suffer from lysosomal storage disease such as Niemann-Pick disease type B is essential and a favorable course is possible despite all risks.

Background: Chronic lymphocytic leukemia (CLL) represents the most frequent type of leukemia in the adult population. It is characterized by highly variable clinical course, predominantly initiated by different genetic alterations responsible for the regulation of cell proliferation and apoptosis. The aim of this study was to evaluate the relative expression of the BIRC3 gene in CLL patients. Methods: This cross-sectional study included 78 participants: a CLL group (n=50) and a control group of healthy subjects (n=28), both ageand sex-matched. To all participants, CBC (complete blood count), basic biochemical parameters, as well as FISH (fluorescent in situ hybridisation) for determining chromosomal aberrations and PCR analyses for determining the relative expression of the BIRC3 (Baculoviral IAP Repeat Containing 3) gene were performed. All reactions were performed on QIA quant 96 5 plex real-time PCR instrument (Qiagen, Hilden, Germany). Additionally, FISH diagnostics was performed using Abbott Vysis CLL FISH Probe Kit (Abbott Molecular diagnostics, Chicago, IL, USA). Results: Deletion of the long arm of the 13th chromosome was the most frequent chromosomal aberration in the CLL group. A statistically significant difference was observed in the relative expression of BIRC3, with higher values in the study group (p=0.019). The results of the binary logistic regression model indicated that lactate dehydrogenase and total calcium were statistically significant predictors of relative BIRC3 gene expression (p < 0.05). Conclusion: Determination of relative BIRC3 gene expression may be a valuable analysis for both the initial diagnosis and the clinical follow-up of CLL patients.

Introduction. Ecthyma gangrenosum is a rare skin disorder which commonly affects immunocompromised patients. Case Report. We report two hematological patients suffering from ecthyma gangrenosum. Pseudomonas aeruginosa was isolated from skin lesions of both patients, but bacteremia was present only in the first case. These two cases had different pathogenic mechanisms and outcomes. Conclusion. Early diagnosis and prompt combination antibiotic therapy are of utmost importance in the treatment of this condition. On the other hand, the underlying disease has a great impact on the outcome as well.