Irena Djunic

Severe form of haemophilia in women is an extremely rare condition. Owing to the rarity of the disease there are no precise recommendations concerning the optimal management of pregnancy and delivery in these patients. We are reporting the clinical course and management of a 30-year-old woman with a severe form of haemophilia A (factor VIII <1 IU/dl) during her first pregnancy and delivery. Antepartum, she was treated on demand by FVIII concentrate and she delivered at 37 weeks of gestation by cesarean section. In postpartal period an excellent control of bleeding was obtained by regularly administering FVIII concentrate for several days as well by concomitant use of tranexamic acid and oral contraceptive pills in the next 6 weeks.

The aim of this 10-year retrospective study was to investigate prognostic clinical and laboratory factors significant for the outcome of patients with mucosa associated lymphoid tissue (MALT) lymphoma. The study involved 87 patients diagnosed with MALT lymphoma: 37 (42.5%) with gastrointestinal (GI) and 50 (57.5%) with non-GI localization. The following pretreatment laboratory parameters were analyzed: hemoglobin, serum albumin and lactate dehydrogenase (LDH) level, beta2-microglobulin (bgr;2-M) and bacteriological (H.pylori) status. Estimated clinical features were: stage of disease, ECOG performance status (PS), tumor mass, number of extranodal localizations, presence of B symptomatology, splenomegaly and enlarged lymph nodes. Diagnosis of MALT lymphoma was based on histopathological analysis of tissue samples, obtained by endoscopy or surgery. The median disease-free survival (DFS) was 36 months and the 5-year overall survival (OS) was 64%. OS rate of patients with non-GI localization was higher compared with patients with GI localization (p=0.001). Multivariate analysis showed hypoalbuminemia to be the most significant parameter associated with poor OS (p<0.001) for both patient groups. The most significant prognostic factor for poor OS in patients with GI localization was LDH level (p=0.031), while hypoalbuminemia was the most significant prognostic factor for poor OS in the group with non-GI disease localization (p=0.001). Proper therapeutic approach for MALT lymphoma patients could be planned taking into consideration poor prognostic parameters, i.e. hypoalbuminemia and elevated LDH for GI patients and hypoalbuminemia for non- GI lymphoma patients.

Background Some patients with paraproteinemia have platelet aggregation disorders and the aim of this study was to examine disturbance of platelet aggregation in healthy blood donors by isolated paraprotein in vitro. Methods Using Rivanol, paraprotein was separated from the serum of ten patients with paraproteinemia, who had decreased platelet aggregation with several inducers. Platelet aggregation in ten healthy donors was measured with and without addition of the isolated induced paraprotein. The test was repeated with added human immunoglobulins for intravenous use. Results Average of maximal levels of platelet aggregation has been significantly decreased in plasma rich in platelets (PRP) of healthy donors after addition of paraprotein when inducers are used: adenosine diphosphate (ADP) (P = 0.007), collagen (COL) (P = 0.008), ristocetin (RIS) (P = 0.001), and epinephrine (EPI) (P = 0.002). Average of latent time of platelet aggregation was significantly prolonged in healthy donors after addition of paraprotein with inducers: COL (P = 0.008), RIS (P = 0.008) and EPI (P = 0.006) while addition of human immunoglobulins caused no change in platelet aggregation. In comparison, when human immunoglobulins were added, maximal platelet aggregation and latent time did not change significantly. Paraprotein isolated from patients with paraproteinamia, who had decrease platelet aggregation, had significantly decreased platelet aggregation when added to PRP of healthy donors, in vitro. Conclusion Platelet aggregation was not significantly changed was confirmed with addition of human immunoglobulins.

The objective of this single-center study was to determine the pretreatment risk factors and influence of comorbidity on outcome in patients with acute myeloid leukemia (AML). The research involved 145 patients with AML during a 58-month follow-up period. The results suggest that the most significant predictor of poor overall survival (OS) is an adverse karyotype (P = 0.007), while for poor rate of complete remission (CR) it is age ≥55 years, and for early death the most significant predictor is comorbidity, as scored by the Hematopoetic Cell Transplantation Comorbidity Index (HCT-CI), P = 0.001. When we divided the patients into two groups: aged ≥55 years and aged <55 years, these predictors differed. In the group aged ≥55 years the most significant predictor of OS (P = 0.013) and for early death (P = 0.003) was HCT-CI (P = 0.013), while in the younger group it was karyotype (P < 0.001). The most significant predictor of CR in the elderly was increased serum lactate dehydrogenase (LDH) level (P = 0.045). In the younger patients, the most significant predictor of CR was leukocytosis (P = 0.001) and for early death it was infection as the comorbidity (P = 0.007). We point out the importance of comorbidity for OS and early death, as well as the impact of infection in patients with AML.

The aims of this study were to investigate the frequency and prognostic relevance of CD56 expression in patients with acute myeloid leukemia (AML) and to compare the importance of CD56 expression with standard prognostic factors, such as age, leukocytosis, cytogenetic abnormalities and performance status. We analyzed the data of 184 newly diagnosed patients with non-promyelocytic AML and a follow-up of 36 months. The median patient age was 58 years, with a range of 18–79. CD56+ antigen was recorded in 40 patients (21.7%). CD56 + was the most significant risk factor for OS: P = 0.05. The most significant factor for a poor rate of CR was age ≥ 55 years (P = 0.001). CD56 positivity had no significant influence on CR rate, but it was the most significant risk factor for disease-free survival (P = 0.005). The CD56 antigen is an independent prognostic risk factor, and its presence should be measured regularly for a better prognostic assessment of patients with AML.

Acquired von Willebrand syndrome (AvWS) is an uncommon complication of multiple myeloma (MM), and it is believed to be connected with paraprotein. The aim of this study was to determine the incidence of AvWS in patients with MM, and estimate the role of paraprotein in its occurrence. The study included 40 patients with MM. The plasma level of paraprotein, platelet adhesion on glass pearls, plasma von Willebrand factor antigen concentration, and ristocetin-induced platelet aggregation (RIPA) were measured initially. Absence of RIPA was found in six patients with MM (15%); however, all six of them had normal levels of von Willebrand factor antigen. Paraprotein was isolated from the serum of these patients. Platelet aggregation was measured in six healthy donors before and after addition of the isolated paraprotein. RIPA was significantly decreased in healthy donors in the presence of paraprotein (P<0·001). The same test was repeated with added human immunoglobulins for intravenous use without any change in RIPA. A significant negative correlation between plasma paraprotein level and RIPA was found (P<0·001). These investigations have shown that paraprotein is associated with AvWS in patients with MM.

The degradation product of collagen type I carboxy terminal telopeptide (ICTP) represents a new biochemical parameter that reflects the changes in the resorption properties of skeletal system. Affection of the skeleton is one of the most important characteristics of multiple myeloma (MM). We estimated significance of ICTP as osteolysis predictor and overall survival in comparison with standard prognostic parameters β₂-microglobulin and C-reactive protein (CRP), in patients with MM. With our results, we have shown significant difference in serum level of ICTP (P = 0.009) between patients with and without osteolysis on conventional radiography. It was proved that ICTP is the most significant predictor of osteolysis (P = 0.09), while CRP is the most significant risk factor for overall survival (P < 0.01). Being highly significant predictor of osteolysis, ICTP can be used for identification of patients with MM who had increased risk for developing osteolytic lesions.