Oligoclonal gammopathy: An analysis of 253 cases.

BACKGROUND: Oligoclonal gammopathy (OG) is a rare disorder of the lymphoid system that is characterized by the presence of at least 2 distinct monoclonal proteins in a patient's serum or urine. The biological and clinical characteristics of this disease are as yet poorly understood. OBJECTIVES: The study aimed to assess whether there are significant differences between patients with OG regarding the developmental history (i.e., OG diagnosed at the first presentation compared to OG that has developed in patients with an original monoclonal gammopathy) and the number of monoclonal proteins (2 compared to 3). Moreover, we attempted to determine when secondary oligoclonality develops following the original diagnosis of monoclonal gammopathy. MATERIAL AND METHODS: Patients were analyzed with regard to their age at diagnosis, sex, serum monoclonal proteins, and underlying hematological disorders. Multiple myeloma (MM) patients were additionally evaluated for their Durie-Salmon stage and cytogenetic alterations. RESULTS: Patients with triclonal gammopathy (TG: n = 29) did not differ significantly from patients with biclonal gammopathy (BG: n = 223) (p = 0.81) in terms of age at diagnosis and the dominant diagnosis (MM was the most common diagnosis (65.0% and 64.7%, respectively)). In both cohorts, myeloma patients were mainly classified to the Durie-Salmon stage III. In the TG cohort, there was a higher proportion of males (69.0%) than among patients with BG (52.5%). Oligoclonality developed at various times after diagnosis (up to 80 months in the investigated cohort). However, the occurrence of new cases was higher during the initial 30-month period following the diagnosis of monoclonal gammopathy. CONCLUSIONS: There are only small differences between patients with primary compared to secondary OG, between BG and TG, and most patients have a combination of IgGκ+IgGλ. Oligoclonality could develop at any time after the diagnosis of monoclonal gammopathy, but it happens more frequently during the first 30 months, with advanced myeloma being the most prevalent underlying disorder.

Prognostic Impact of Copy Number Alterations' Profile and AID/RAG Signatures in Acute Lymphoblastic Leukemia (ALL) with BCR::ABL and without Recurrent Genetic Aberrations (NEG ALL) Treated with Intensive Chemotherapy.

Adult acute lymphoblastic leukemia (ALL) is associated with poor outcomes. ALL is initiated by primary aberrations, but secondary genetic lesions are necessary for overt ALL. In this study, we reassessed the value of primary and secondary aberrations in intensively treated ALL patients in relation to mutator enzyme expression. RT-PCR, genomic PCR, and sequencing were applied to evaluate primary aberrations, while qPCR was used to measure the expression of RAG and AID mutator enzymes in 166 adult ALL patients. Secondary copy number alterations (CNA) were studied in 94 cases by MLPA assay. Primary aberrations alone stratified 30% of the patients (27% high-risk, 3% low-risk cases). The remaining 70% intermediate-risk patients included BCR::ABL1pos subgroup and ALL lacking identified genetic markers (NEG ALL). We identified three CNA profiles: high-risk bad-CNA (CNAhigh/IKZF1pos), low-risk good-CNA (all other CNAs), and intermediate-risk CNAneg. Furthermore, based on RAG/AID expression, we report possible mechanisms underlying the CNA profiles associated with poor outcome: AID stratified outcome in CNAneg, which accompanied most likely a particular profile of single nucleotide variations, while RAG in CNApos increased the odds for CNAhigh/IKZF1pos development. Finally, we integrated primary genetic aberrations with CNA to propose a revised risk stratification code, which allowed us to stratify 75% of BCR::ABL1pos and NEG patients.

Epidemiology of multiple myeloma in Poland in the years 2008-2017.

Introduction: Multiple myeloma is the third most common blood cancer in Europe and accounts for approx. 10-15% of these cancers. The objective of this study was to determine the incidence, prevalence, mortality and survival in multiple myeloma (ICD code: C90.0) patients in Poland in the years 2008-2017. Material and methods: The analysis used the data on healthcare services provided to patients with multiple myeloma defined with the ICD-10 (International Statistical Classification of Diseases and Related Health Problems) code C90.0 and reported by healthcare entities to the National Health Fund (NFZ). Results: In 2009, the C90.0 incidence per 100,000 inhabitants was 6.4, while in 2017 it was 8.3. The prevalence in the same period increased by 76%, from 13.6/100,000 to 23.9/100,000. The mortality to prevalence ratio gradually decreased from 78% in 2008 to 22.8% in 2017. The 1-year, 3-year and 5-year survival rates in patients with this diagnosis made in the years 2009 and 2013 were 70.5%, 51.5% and 40.2% versus 78.4%, 60.3% and 48.3%, respectively. Conclusions: The incidence and prevalence of multiple myeloma and survival rates in Poland were continuously increasing in the studied period. These trends may result from the aging of Polish society, better recognisability of multiple myeloma and/or improved access to increasingly more effective therapies in Poland. The impact of these factors on the epidemiology of multiple myeloma requires further studies.

Effectiveness of tinnitus therapy using a mobile application.

BACKGROUND: The World Health Organization reports that the number of tinnitus sufferers is increasing year on year. Given the common use of mobile devices and the availability of applications designed to support patients in tinnitus therapy and reduce tinnitus severity, patients seeking help are likely to try this form of support. The aim of this study was to evaluate the effectiveness of a mobile application in tinnitus sound therapy, in this case ReSound Tinnitus Relief™. METHODS: The study involved 52 patients hospitalized for tinnitus. All participants used the free ReSound Tinnitus Relief application for 6 months. The application is based on sound therapy. Patients were advised to use the application for at least 30 min per day, the sounds should not completely mask the tinnitus, and they should be listened to via a loudspeaker. The effects of the therapy were evaluated by means of standardized questionnaires for tinnitus severity: the Tinnitus Handicap Inventory and the Tinnitus Functional Index. RESULTS: The study showed a reduction in tinnitus severity as measured by both questionnaires. The general severity decreased after the first 3 months and again in the following 3 months of using the application. In both questionnaires the biggest changes were observed in the subscales of emotions. CONCLUSIONS: Results obtained here from standardized questionnaires indicate that the tested application may contribute to tinnitus reduction. However, it is advisable to conduct further research on the applicability of such technology in medical practice.

Prevalence of hypogammaglobulinemia and its management with subcutaneous immunoglobulin supplementation in patients after allogeneic hematopoietic stem cell transplantation-a single-center analysis.

Secondary immunodeficiencies are frequently observed after allo-HSCT. The efficacy of subcutaneous IgG preparations in this population is unknown. A retrospective single-institution study involved 126 adult patients transplanted in 2012-2019 for hematological malignancies. Patients were tested every 2-3 weeks for plasma IgG concentration during the 1st year after transplantation and supplemented with facilitated subcutaneous immunoglobulin when they either had IgG concentration < 500 mg/dl or between 500 and 700 mg/dl and recurrent infection. The IgG concentration < 500 mg/dL was diagnosed in 41 patients, while 500-700 mg/dL in 25 and altogether 53 patients received IgG supplementation. The median number of IgG administrations was 2. The median time to the first IgG administration after allo-HSCT was 4.1 months, while to the next administration (if more than one was required) 53 days (prophylactic group) and 32 days (group with infections). We did not observe any significant toxicity. Two situations were associated with increased probability of meeting criteria for IgG supplementation: diagnosis of either acute lymphoblastic leukemia (ALL) or chronic lymphocytic leukemia (CLL) (83.8% versus 39.3% for other diagnosis, p = 0.000) and the systemic use of corticosteroids (64.2% versus 31.5% for patients without systemic corticosteroids, p = 0.005). Over 40% of the adult recipients may require at least incidental immunoglobulin supplementation during the first year after allo-HSCT. Low IgG concentrations are associated with inferior outcomes. The subcutaneous route of IgG administration appeared to be safe and may allow for long persistence.

IDH2 mutations in patients with normal karyotype AML predict favorable responses to daunorubicin, cytarabine and cladribine regimen.

Mutations in isocitrate dehydrogenase 1 and 2 (IDH1/2) genes occur in about 20% patients with acute myeloid leukemia (AML), leading to DNA hypermethylation and epigenetic deregulation. We assessed the prognostic significance of IDH1/2 mutations (IDH1/2+) in 398 AML patients with normal karyotype (NK-AML), treated with daunorubicine + cytarabine (DA), DA + cladribine (DAC), or DA + fludarabine. IDH2 mutation was an independent favorable prognostic factor for 4-year overall survival (OS) in total NK-AML population (p = 0.03, censoring at allotransplant). We next evaluated the effect of addition of cladribine to induction regimen on the patients' outcome according to IDH1/2 mutation status. In DAC group, 4-year OS was increased in IDH2+ patients, compared to IDH-wild type group (54% vs 33%; p = 0.0087, censoring at allotransplant), while no difference was observed for DA-treated subjects. In multivariate analysis, DAC independently improved the survival of IDH2+ patients (HR = 0.6 [0.37-0.93]; p = 0.024; censored at transplant), indicating that this group specifically benefits from cladribine-containing therapy. In AML cells with R140Q or R172K IDH2 mutations, cladribine restrained mutations-related DNA hypermethylation. Altogether, DAC regimen produces better outcomes in IDH2+ NK-AML patients than DA, and this likely results from the hypomethylating activity of cladribine. Our observations warrant further investigations of induction protocols combining cladribine with IDH1/2 inhibitors in IDH2-mutant.

Pseudoneutropenia as a factor-limiting access to chemotherapy for cancer patients: the effect of a simple meal.

BACKGROUND: Absolute neutrophil count (ANC) below 1.5 G/l or 1 G/l is commonly used as a factor to determine the decision to administer antineoplastic treatment including chemotherapy and novel agents to cancer patients. This practice is based on observations that below this ANC, there is an increased risk of bacterial and fungal infection. This is further based on the assumption that this parameter always correctly reflects the true shortage of these germ-fighting cells in patients. In reality, the circulating pool of neutrophils is only one of four reservoirs (bone marrow, circulating, marginal and tissue pools) and its size is influenced not only by shortage but also by transient shift of cells between these reservoirs. The aim of this study was to evaluate whether repeated blood collection affects ANC in the patient. METHODS: We retrospectively analysed the medical records of cancer patients with 0.8 G/l ≤ ANC < 1.5 G/l in whom CBC was repeated based on the physician's decision, which was done on the same day roughly 2 h after the first one. RESULTS: The patients at the time of repeating CBC had consumed breakfast. In 31 out of 32 patients, ANC exceeded 1 G/l or 1.5 G/l and antineoplastic treatment was administered as originally planned. There were no infectious complications observed. CONCLUSION: Cancer patients should not be fasting prior to blood collection, with the exception of special and rare situations. To achieve the maximum clinical benefit, delays and/or reductions of antineoplastic treatment should be avoided wherever possible. Pseudoneutropenia is an unnecessary reason for postponing chemotherapy.

Electrophysiological correlates of focused attention on low- and high-distressed tinnitus.

OBJECTIVES: The study aimed at determining the EEG correlates of concentration on either low or high-distressed tinnitus. METHODS: Sixty-seven patients (36 women, mean age = 50.34 ± 12.94 years) with chronic tinnitus were assigned to either a high (HD) or low (LD) tinnitus-related distress group based on THI results. All participants took part in the EEG study comprising two 3-4 min blocks of focusing on either tinnitus (Tinnitus Focus Condition, TFC) or the sensations from one's own body (Body Focus Condition, BFC). The absolute power and current density of 8 frequency bands in 7 clusters were compared between conditions and groups. RESULTS: The most pronounced differences were found in the HD patients in the TFC, relative to the BFC, i.e. reduced power of frontally distributed low alpha (8-10 Hz) and posterior high alpha (10-12 Hz) as well as lower current density of 8-10 Hz rhythm over the right frontal/anterior cingulate cortex and higher middle beta (15-18 Hz) density in the precuneus. The HD, relative to LD patients, in both conditions, exhibited increased low beta (12-15 Hz) power over the left middle area and greater higher beta (15-25 Hz) power in the left posterior region. CONCLUSIONS: The present study contrasted bioelectrical activity, acquired when concentrating on tinnitus with EEG data collected whilst patients focused on their body. Decreased alpha power and current density in the frontal/cingulate cortex when listening to bothersome tinnitus might reflect greater cortical arousal whereas increased beta power and density in the precuneus/posterior cingulate activity in this condition could be indicative for elevated tension or augmented cognitive/emotional processing of tinnitus sound. Enhanced beta rhythm in patients with high versus low tinnitus distress, observed independently of the study condition, may be due to greater self-focused attention or more active processing of sensations derived from the own body.

Infectious Complications in Patients With Multiple Myeloma After High-Dose Chemotherapy Followed by Autologous Stem Cell Transplant: Nationwide Study of the Infectious Complications Study Group of the Polish Adult Leukemia Group.

BACKGROUND: Multiple myeloma (MM) has become a chronic disease in majority of patients, and remission consolidation with autologous hematopoietic stem cell transplant (ASCT) remains the backbone of treatment in transplant-eligible patients. OBJECTIVE: The aim of this multicenter cross-sectional nationwide retrospective study was to evaluate the epidemiology, etiology, and outcome of infections in patients with MM undergoing ASCT in 13 Polish transplant centers, carried out on behalf of the Infectious Complications Study Group of the Polish Adult Leukemia Group. METHODS: A total number of consecutive 1374 patients with MM treated in Polish adult transplant centers from 2012 to 2014 were followed for infectious complications up to day +100 after ASCT in nationwide study. RESULTS: Altogether 490 infection episodes in 336 patients (49% male, aged 21-72 years) were reported, including 145 episodes of neutropenic fever (103 patients) and 34 episodes of clinically documented infections (CDIs) (27 patients). Among microbiologically confirmed infections there were 251 episodes of bacterial infections (180 patients), 42 episodes of fungal infections (38 patients), and 18 episodes of viral infections (17 patients). The overall incidence of infections reached 13.1% for bacterial, 3.6% for fungal, and 1.3% for viral infections. There were 16 cases of infection-related deaths after ASCT (1.2%). The mortality risk factors included multidrug-resistant bacteria etiology (odds ratio [OR], 3.5; P = .033), coexistence of bacterial and fungal infection (OR, 6.3; P = .002), and CDI (OR, 5.5; P = .007). CONCLUSION: ASCT in patients with MM was connected with low risk of life-threatening infections. However, multidrug-resistant bacteria bacterial etiology, mixed etiology, and CDI increased the risk of fatal outcome.

Outcomes of Jehovah's Witnesses with hematological malignancies treated without transfusions - single center experience.

Malignancies of the hematopoietic system frequently are associated with severe cytopenias requiring transfusions of blood components. Refusal of blood components by Jehovah's Witnesses (JW) produces challenges to treatment. In this report we describe the outcome of hematological malignancies of JW patients treated without transfusions. Altogether, eight JW, diagnosed 1994-2015, 6 (75%) females, the median age at diagnosis 40 years (range, 20-78), were included into the analysis. The diagnoses were: acute lymphoblastic leukemia (2, 25%), acute myeloid leukemia (2, 25%), non-Hodgkin's lymphomas (4, 50%). One patient died without treatment while the remaining 7 patients received treatment, including imatinib in 1 patient with BCR-ABL1+ acute lymphoblastic leukemia. Five (62.5%) patients received erythropoiesis stimulating agents. Median hemoglobin concentration at diagnosis was 8.7 g/dL (range, 6.3-13.1), and it decreased to 3.2 g/dL (range, 2.6-9.3) during first-line treatment. Median platelet count at diagnosis was 52 × 109/L (range, 15-392). All patients became thrombocytopenic upon treatment reaching median platelet count 8 × 109/L (range, 2-85). Five patients developed respiratory failure. Anemia contributed substantially to the death of 3 out of 6 patients (50%). One patient (17%) developed central nervous system bleeding in the course of thrombocytopenia. Objective response rate was 43%, with 29% complete remissions after first-line treatment. Despite the median overall survival of 15.3 months (95% CI, 0.2-52.2), all but one acute leukemia patients succumbed shortly after the diagnosis. To conclude, the outcome of JW treated because of hematological malignancies without blood transfusions is very dismal, nevertheless, selected patients can obtain complete remissions. Anemia contributes significantly to the death of JW.

Prior blood donations do not affect efficacy of G-CSF mobilization nor outcomes of haematopoietic stem cell collection in healthy donors.

BACKGROUND AND OBJECTIVES: Many consider volunteer blood donors as ideal candidates for unrelated haematopoietic progenitor cell (HPC) donation. However, frequent blood donations could influence the results of HPC mobilization. To our best knowledge, there are no data on the possible impact of repeated blood donation on efficiency of subsequent HPC mobilization by granulocyte colony-stimulating factor (G-CSF). MATERIALS AND METHODS: We compared outcomes of HPC mobilization in unrelated donors with and without a history of blood donation. We conducted a prospective study on 287 consecutive donors admitted to the Department of Hematology since January 2016. The final analysis included 153 donors who agreed to take part in the study and had undergone stem cell mobilization with G-CSF. RESULTS: History of blood donations prior to haematopoietic stem cell mobilization with G-CSF does not have a significant impact on the number of collected CD34+ cells in the first leucocytapheresis (516.2 x 106 (170-1148) in blood donors vs 490.5 x 106 (101-1154) in non-donors) (P = 0.32). In all donors, in this study mobilization of HPC was successful: 87.5% of blood donors and 85.6% of non-donors collected the required cell number in a single apheresis. In blood donors, a higher number of blood donations within 2 and 5 years prior to HPC mobilization correlated significantly with successful donation within one leucocytapheresis (P = 0.014 and P = 0.024, respectively). CONCLUSION: Multiple blood donations do not significantly influence the outcome of HPC collection in unrelated donors. Blood donors and non-donors have similar results of HPC collection, so there is no reason to favour either group.

Bone marrow harvest in donors with anaemia.

BACKGROUND: Bone marrow harvest (BMH) for haematopoietic stem cell transplantation is a well-established procedure. The guidelines of World Marrow Donor Association provide information on donor selection. However, some of the guidelines regarding donors with anaemia prior to harvest lack in supporting data from clinical studies. With this study, we aimed to provide such data. MATERIAL AND METHODS: In this retrospective, single-centre study, we analysed the interplay between haemoglobin levels and BMH and BMH impact on haemoglobin levels in a cohort of 149 unrelated BM donors, including 13 subjects with mild anaemia. RESULTS: The BMH led to significantly lower decrease in haemoglobin levels in donors with anaemia than in control group (1·79 g/dl vs. 2·56 g/dl, P < 0·0001). The following parameters: BMH volume (ml), BMH volume/donor body weight (ml/kg), total nucleated cells (TNC) in product (×108 ) and TNC/kg recipient body weight in product (×108 /kg) did not differ significantly between those two analysed groups (P > 0·05). Median BM volume harvested from anaemic donors was 16·34 ml/kg; none of them required blood transfusion after BMH. CONCLUSION: Mild anaemia prior to BMH does not significantly impact the collection results. The BMH is safe and feasible in donors with mild anaemia.

Lack of persistent remission following initial recovery in patients with type 1 diabetes treated with autologous peripheral blood stem cell transplantation.

AIMS: To assess metabolic control in patients with newly diagnosed type 1 diabetes mellitus who underwent immunoablation followed by autologous peripheral blood stem cell transplantation (APBSCT) as a treatment of diabetes. METHODS: APBSCT was performed in 23 patients. Control group comprised 8 non-APBSCT patients in whom after diagnosis insulin therapy was initiated. Fasting plasma glucose, glycated hemoglobin, fasting and postprandial C-peptide were assessed in all subjects and continuous glucose monitoring was performed at 6th, 12th, 24th, 36th, 48th month after transplantation. The APBSCT group was observed for 72 months. RESULTS: Six months after the procedure, 22 of 23 transplant patients remained insulin-free, but after 6 years, there was only one APBSCT insulin-free patient. Good glycemic control was observed in all patients throughout the observation period, although fasting plasma glucose in control group was significantly higher in comparison with the both transplanted groups up to the 36th month. HbA1c values were significantly lower in the insulin-free group only at the 24th and 36th month. Fasting and postprandial C-peptide concentrations were higher in APBSCT group as compared with control group. The most serious adverse event was a fatal case of Pseudomonas aeruginosa sepsis. CONCLUSIONS: The effectiveness of APBSCT as a treatment for newly diagnosed DM1 seems to be limited in time. The metabolic control of APBSCT patients is similar to conventionally treated patients. The lower fasting plasma glucose and higher C-peptide achieved with APBSCT seem to not exceed the risks associated with the procedure.

Stem cell mobilization in patients with dialysis-dependent multiple myeloma: Report of the Polish Myeloma Study Group.

INTRODUCTION: High-dose chemotherapy with autologous hematopoietic stem cell transplantation (auto-HSCT) improves the outcome of patients with multiple myeloma (MM). It seems that auto-HSCT is also a feasible therapeutic option in MM dialysis-dependent (MMDD) patients. However, to perform transplantation, a sufficient number of stem cells must be collected. MATERIALS AND METHODS: Given that data on mobilization of auto-HSC efficacy and safety in dialysis-dependent patients are limited, we report data from all Polish Centers belonging to the Polish Myeloma Study Group. Twenty-eight dialysis-dependent MM-patients were enrolled into this retrospective analysis. The study population comprised patients diagnosed between 2004 and 2015 in whom an attempt to collect auto-HSC was made (68%: women, median age: 56). Patients received granulocyte-colony stimulating factor (G-CSF) alone or in combination with chemotherapy and autologous peripheral blood stem cells (auto-PBSCs) were collected by leukapheresis. RESULTS AND CONCLUSIONS: The success rate in terms of obtaining sufficient number of CD34(+) cells/kg for an auto-HSCT (≥2 × 106 cells/kg body weight) during the first mobilization attempt was 92% (26/28 patients), and for 2 auto-HSCTs (≥4 × 106 cells/kg) - was 75% (21/28 patients). After the second mobilization attempt (undertaken in 8 patients), a sufficient number of CD34(+)/kg cells for an auto-HSCT was obtained for all patients and the number of CD34(+)/kg collected cells was sufficient for 2 auto-HSCT in 6 additional patients. Hematologic toxicity and infections were the most frequent complications. Higher doses of cytarabine (>1.6 g/m2 ) and cyclophosphamide (> 2 g/m2 ) should be avoided in MMDD patients due to toxicity. Further studies are needed to establish mobilization regimens, confirm their safety, and dosing in MMDD patients.

Experience with Saccharomyces boulardii Probiotic in Oncohaematological Patients.

Very few reports have been published to date on the bloodstream infections caused by Saccharomyces spp. in oncohaematological patients, and there are no guidelines on the use of this probiotic microorganism in this population. We describe the use of probiotic preparation containing Saccharomyces boulardii in a large group of oncohaematological patients. We retrospectively analysed the data from 32,000 patient hospitalisations at the haematological centre during 2011-2013 (including 196 haematopoietic stem cell transplant recipients) in a tertiary care university-affiliated hospital. During the study period, 2270 doses of Saccharomyces boulardii probiotic were administered to the oncohaematological patients. In total, 2816 mycological cultures were performed, out of which 772 (27.4%) were positive, with 52 indicating digestive tract colonisation by Saccharomyces spp., mainly in patients with acute myeloid leukaemia (AML), myelodysplastic syndrome (MDS) or multiple myeloma (MM). While colonised, they were hospitalised for 1683 days and 416 microbiological cultures of their clinical samples were performed. In the studied group of patients, there were six blood cultures positive for fungi; however, they comprised Candida species: two C. glabrata, one C. albicans, one C. krusei, one C. tropicalis and one C. parapsilosis. There was no blood culture positive for Saccharomyces spp. Our study indicates that despite colonisation of many oncohaematological patients with Saccharomyces spp., there were no cases of fungal sepsis caused by this species.

Impaired right ventricular function as a predictor of early mortality in patients with light­ chain cardiac amyloidosis assessed in a cardiology department.

INTRODUCTION    Light­chain (AL) amyloidosis is the most common cardiac amyloidosis. Despite progress in treatment, early mortality remains a substantial problem in these patients. OBJECTIVES    The aim of this study was to determine a clinical profile of patients diagnosed with AL amyloidosis in a cardiology department, as well as to define the cut­off point for early mortality and identify predictors of early mortality in this population. PATIENTS AND METHODS    The study included 30 patients (14 women; median age, 61.5 years) with AL amyloidosis confirmed by echocardiography and biopsy of 2 organs. RESULTS    Six patients were diagnosed with stage II amyloidosis according to the Mayo 2004 classification, and 24 patients-with stage III. Early mortality was defined as death during 102 days after diagnosis and was observed in 14 patients. Patients who died earlier were younger and more frequently reported a weight loss of more than 10 kg and orthostatic hypotension than patients who died later. Moreover, they had higher concentrations of high­sensitivity troponin T and N­terminal pro­B­type natriuretic peptide (NT­proBNP) and worse left and right ventricular (RV) contractility. In the Cox models, the age of less than 64 years, NT­proBNP levels exceeding 4968 pg/ml, RV end­diastolic diameter of less than 34 mm, and tricuspid annular plane systolic excursion lower than 13 mm were significant predictors of mortality within 102 days after diagnosis. CONCLUSIONS    We presented the results of the first Polish prospective noninterventional study on AL amyloidosis diagnosed in the cardiology department. We found that patients have advanced disease at the time of diagnosis. Younger age, impaired RV function, and higher concentrations of cardiac markers are predictors of worse prognosis.

Mild chronic graft-versus-host disease may alleviate poor prognosis associated with FLT3 internal tandem duplication for adult acute myeloid leukemia following allogeneic stem cell transplantation with myeloablative conditioning in first complete remission: a retrospective study.

Internal tandem duplication (ITD) of the FLT3 gene (Fms-like tyrosine kinase 3) is the most commonly found mutation in acute myeloid leukemia (AML). The significance of FLT3-ITD at diagnosis was retrospectively estimated for allo-HSCT (allogeneic hematopoietic stem cell transplantation) outcomes in 140 patients, median age of 38, undergoing allo-HSCT after myeloablative conditioning in first complete remission of AML. FLT3-ITD was detected at AML diagnosis in 42/140 (30%) of included into this study patients. At 3 years, relapse incidence (RI) following allo-HSCT in AML patients with intermediate or normal karyotype was significantly higher in those FLT3-ITD positive than FLT3-ITD negative [52.9 vs. 20.4%, P = 0.002]. Additionally, patients with mild chronic graft-versus-host disease (cGvHD) had significantly lower RI compared to patients with moderate or severe grade cGvHD or those not experiencing cGvHD, respectively, 4.8 vs. 36.0 vs. 27.8%, P = 0.032. FLT3-ITD was harboring a poor prognosis in AML with intermediate or normal karyotype and significantly increased risk of relapse following allo-HSCT. It appears that allo-HSCT does not cure patients with FLT3-ITD, unless they develop symptoms of mild cGvHD and graft versus leukemia, which may decrease RI.

Epstein-Barr Virus-Positive Diffuse Large B cell Lymphoma in the Experience of a Tertiary Medical Center in Poland.

The role of Epstein-Barr virus (EBV) in the biology and clinical characteristics of diffuse large B cell lymphoma (DLBCL) is still poorly defined. A new provisional entity EBV-positive DLBCL of the elderly has been described in Asian population. Its incidence and prognosis remains unknown in middle European patients. Clinical data and tissue samples were collected from 74 Caucasian patients with DLBCL, aged between 23 and 86 years, treated at a single institution. Lymphoma morphology was reassessed, laboratory procedures included in situ hybridization specific for EBV-encoded small RNAs (EBER), immunohistochemical staining for latent membrane protein and serological testing for EBV-specific antibodies. EBER staining revealed 12.2 % of EBV-positive cases, whereas 9.5 % were diagnosed as EBV-positive DLBCL of the elderly. Serologic EBV markers did not correlate with the presence of EBV in tissue samples (P > 0.10). Elderly EBV-positive cases had lower BCL-6 (P = 0.038) and higher CD30 (P = 0.049) expression and were characterized by higher progression risk (median time-to-progression 12.5 months vs not reached; P = 0.029) and a trend towards worse overall survival (median overall survival 24.5 months vs not reached; P = 0.059). EBV-positive DLBCL of the elderly occurs relatively frequently in Polish population and may be associated with inferior prognosis in comparison with DLBCL, not otherwise specified.