Our case confirms that cyclosporine therapy is effective in treating T-celI large granular lymphocyte leukemia and suggests that indefinite treatment may not be needed to maintain the response.
Introduction. Acute myeloid leukemia is a malignant, clonal disease of a hematopoietic stem/progenitor cell, characterized by accumulation of acquired somatic genetic and epigenetic alterations. Acute myeloid leukemia is, basically, highly heterogeneous disease, so individual treatment approach is needed. With the use of high-throughput genome sequencing technologies, a full spectrum of recurrent gene mutations in acute myeloid leukemia has been discovered, which has also provided deeper insight into leukemogenesis and acute myeloid leukemia ontogeny. This review focuses on molecular markers with proven prognostic significance, which form, together with standard cytogenetics, basis for current acute myeloid leukemia risk stratification. Even in the era of molecular markers, standard prognostic factors (pre-treatment and postinduction factors) have a strong influence on the choice of postremission therapy. Conclusion. Evaluation of molecular markers and their impact on prognosis in acute myeloid leukemia should be interpreted in the context of complex gene interactions. Only comprehensive understanding of acute myeloid leukemia biology and integration of all prognostic markers enable us to timely plan risk adapted treatment.
The revised international prognostic scoring system (IPSS-R) improved cytogenetic prognostic classification in comparison with IPSS classification in patients with myelodysplastic syndromes (Greenberg P et al. Blood 2012, Schanz J et al. JCO 2011). Monosomal karyotype (MK) defined as the presence of at least two autosomal monosomies or one monosomy associated with structural abnormalities has been investigated in MDS patients with contradictory results regarding its independent prognostic significance (Patnaik M et al. Leukemia 2011, Gangat N et al. AJH 2013, Valcarcel D et al. JCO 2013). The aim of this study was to investigate the prognostic significance of MK in relation to IPSS-R and the presence of complex karyotype (CK). The study was conducted in 391 patients with primary MDS treated at three hematology departments in Serbia. The median age was 65 years, from 15 to 89 years. There was a predominance of male patients who made 60% (235) of patients. 229 (58 %) patients died. 87 patients (22 %) transformed to AML. The sixty eight % of patients were transfusion dependent. Disease modifying treatment was applied in 48 (12.3%) of patients (AML like chemotherapy in 37 patients, low dose ara-c in 11 patients, stem cell transplantation in five patients, and in one patient azacitidine). The rest of the patients received supportive treatment. Karyotypes were classified according to the International System for Cytogenetic Nomenclature Criteria. The inclusion of patients was based on criteria used in IPSS-R classification (a white blood cell count ≤12x109/l, an absolute neutrophil count ≤8x109/l, peripheral blood blasts ≤19%, and bone marrow blasts ≤30%). The distribution of patients according to FAB classification was as follows: 129 (32.99%) RA, 47 (12.02%) RARS, 143 (36.57%) RAEB, 48 (12.27%) RAEB-T, and 22 (5.6%) non proliferative CMML. The classification according to WHO 2008 classification was as follows: RCUD 29 (7.43%), RARS 39 (9.97%), RCMD 90 (23.01%), RAEB1 78 (19.95%), RAEB2 65 (16.62%), AML/RAEB-T 48 (5.27%), 5q- 11 (2.81%), MDS-u 7 (1.8%), CMML 1 15 (3.83%), CMML 2 7 (1.8%). IPSS-R distribution was: very low risk 38 (9.71%), low risk 108 (27.6%), intermediate risk 86 (21.99%), high risk 83 (21.23%), very high risk 74 (18.9%). Two patients could not be classified because of lack of all data. Median number of metaphases was 15, from 2 to 30. The abnormal karyotype was found in 166 (42.45%) patients. CK defined as the presence of at least three cytogenetic aberrancies was present in 32 patients (8.18%) with median survival of 5 months. CK shown prognostic significance regarding the overall survival (OS) (p = 0.00001) as well as time to AML transformation (p = 0.00014). MK was detected in 34 patients (8.69%). The patients with MK had significantly shorter OS in comparison with patients without MK (median survival 5 months versus 34 months, p < 0.00001, Figure 1) as well as a shorter time to AML transformation (p = 0.00006, Figure 2). If we included only the patients who have MDS according to 2008 WHO classification MK shown prognostic significance for OS (p = 0.00121) as well as for time to AML progression (p = 0.00010). The presence of MK defines the group of patients with shorter OS in high risk and very high risk IPSS-R prognostic groups (p=0.008, Figure 3). In multivariate analysis, MK shown to be independent predictor of poor survival together with age, haemoglobin concentration, platelet count and bone marrow blast cells (p<0.001, HR 2.97, 95% CI 1.91-4,66) as well as for the risk of AML transformation with bone marrow blasts and platelet count (p<0.001, HR 3.44, 95% CI 1.79-6.61). In 29 patients MK was associated with CK. However, in five patients (16% of MK + patients) with monosomy and an additional aberration who do not fulfil the criteria for CK the OS seems to be very short (median survival 3.5 months). The significance in OS between this small group of patients and other patients excluding the patients with CK was not significant (p=0.09). Yet, only in one case of MK cytogenetic aberrations were defined as high risk according to IPPS-R cytogenetic classification. In conclusion, MK has an independent prognostic significance for survival and risk of AML transformation in MDS patients particularly in high and very high risk IPSS-groups. In majority of patients with MK there is an association with complex karyotype. Yet, there are a few patients with MK who do not have CK and seems to have unfavourable prognosis. Figure 1 Figure 1. Figure 2 Figure 2. Figure 3 Figure 3. Disclosures No relevant conflicts of interest to declare.
Bacground/Aim. Groin hernias are common pathology among men population. Only curative treatment is surgical reparation with various surgical procedures for groin hernia solving. The aim of this study was to evaluate the most prevalent surgical procedures and early postoperative complications after groin hernia reparation in large series of operated patients, and to assess the morphologic characteristics of groin hernias. Methods. The retrospective study included all patients with groin hernia who underwent surgical reparation from 2009 to 2012. In all patients a demographic characteristics, including gender and age, clinical characteristics and hernia type were analyzed. The surgical procedure for hernia solving and early postoperative complications were assessed. Results. The study included 1,211 patients. The male/female ratio was 1,127/84 (p < 0.001). Inguinal hernia was found in 1,195 patients (94.5% males). Femoral hernia was found in 16 patients (25% males and 75% females). Significant difference in distribution of inguinal and femoral hernia between genders was found (p < 0.001). In males right sided inguinal hernia was present in 57.6%. In females right sided inguinal hernia was present in 7 and left sided in 5 patients. Sixsten patients had bilateral inguinal hernia, all in males. There was no significant difference in side of inguinal hernia occurrence and gender. Right sided and left sided femoral hernias were present in the same percent in males. In females a higher occurrence in femoral hernia was found on the right side then on the left one (7:5) without significant difference. There were 71.1% of patients in the age group of 51-80 and 27.2% of patients in the age group of 61-70. Surgical procedures included: Lichtenstein in 51.2% of patients, nylondarn in 29.6% of patients, Bassini in 16.2% of patients, Lothaissen in 1.7% of patients, and Halsted in 1.4% of patients. Overall, postoperative complications were present in 78 (6.4%) of patients. Wound infection was the most common complication, occurred in 2.4% of patients. Conclusion. Prevalence of inguinal hernias is higher in men population, while femoral hernias are more common in females. The most affected population is at the age between 61 and 80 years. The most commonly used open surgical procedures for groin hernia reparation are Lichtenstein and nylon-darn. Both methods have low and similar incidence rates of postoperative complications.
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