bluuebunny/crossref_metadata_2025_split huggingface dataset:
PB1793 REATMENT OUTCOME FOR PRIMARY CERVIX LARGE B‐CELL LYMPHOMA: A CLINICAL ANALYSIS OF 37 CASES.
Authors: Liu X. , Xia Z. , Liu Y. , Cao J. 
Published: June 2019

Background:Cervical lymphoma is a rare condition and may be difficult to diagnose and treatment. Large B‐cell lymphoma is among the most common sub‐type of Cervical Lymphoma.No standard treatments for patients with primary cervix large B‐cell lymphoma have been introduced currently. Only case reports published in the literature and indicated the R‐CHOP regimens in combination with involved field radiation therapy (RT) are effective. Shanghai Cancer Center is one of the top pathological consultant center in China.Aims:We retrospectively analyse the 37 patients with primary cervix large B‐cell lymphoma and outcome after R‐CHOP in combination with RT, explore the treatment outcome by combination chemetherapy in this very rare type of cervix entity.Methods:Thirty‐seven untreated primary cervix large B‐cell lymphoma patients received R‐CHOP like regimens. Fourteen of them underwent subsequent RT. Thirteen of them received subsequent salvage chemotherapy.Results:Of 37 patients underwent R‐CHOP chemotherapy, only 3 patients received the radical surgery. An overall response rate (ORR) was 78.3% after completion of chemotherapy.The PFS and OS rate at 5 years were 58% and 69%, respectively. For 14 patients who received RT after R‐CHOP, an estimated ORR were 89.2%. For all patients, The PFS reached the platform after 2 years follow up and the OS after 3 years according to the survival curve analysis. The international prognostic index (IPI) score were the only predictor of worse outcome.Summary/Conclusion:R‐CHOP regimens plus involved field radiation therapy led high response rate in primary cervix large B‐cell lymphoma. Patients with high IPI had a trend of less satisfied with R‐CHOP regimens plus IFRT. Future prospective and multicenter studies are needed. By the way, we are conducting a gene expression profile between DLBCL‐unspecified and primary cervix large B‐cell lymphoma by NGS in order to get the valuable information to improve the treatment outcome even by using some targeted drugs. The data will be show in the next few months.image
PB1833 PRIMARY CARDIAC LYMPHOMA
Authors: Freue J.M. , Marin Castellanos R. , Silveyra D. , Lalor N. , Scapellato J.L. , Campestri R. 
Published: June 2019

Background:Primary cardiac lymphomas (PCL) are rare, with a fatal course unless diagnosed and treated in time. Non‐neoplastic causes such as infections and thrombi account for most cardiac masses. The most common cardiac tumors are benign, such as myxomas, being sarcomas the most frequent malignancy. Symptoms are inespecific, PCL can present as heart failure, arrythmias, vena cava thrombosis, thromboembolic disease. Patients must examined thoroughly, including echocardiography, CT or MRI and bone marrow biopsy. Final diagnosis will require a biopsy of the cardiac mass. Treatment is with chemotherapies according to lymphoma subtype, usually anthracycline based regimens. If ventricular outflow tract is compromised surgery may be needed due to hemodynamic instability which is associated with a very bad prognosisAims:To describe a case of a patient with PCLMethods:A case reportResults:Case: a 59 years old woman consulted for bilateral lower extremities edema and abdominal distension. She had a previous history of HIV infection diagnosed 10 years ago treated with nevirapine, lamivudine and zidovudine. Last CD4 count was 283/mm3 and viral load undetectable.Laboratory: Hg 13,5 g/dl, WBC 9400/mm3 (Neutrophil 80%, no atypical cells or blast in blood smear), platelet 344000/mm3, coagulation test, protein electrophoresis, hepatic and renal function were normal, D‐dimer 6 mg/l (normal< 0,5), Hepatitis C and B test were negative, LDH 1200U/l(100‐210)Abdominal ultrasound showed ascites, liver and spleen were normal. Serum‐Ascites albumin gradient was > 1,1 g/dl (suggestive of transudate).AngioCT: ascites, thrombosis of iliac, right renal and inferior cava vein (IVC) thrombosis, cardiac mass in both auricles and right ventricle extending to IVC, signs of segmentary pulmonary embolism (PE).Echocardiogram: atrial mass invading interauricular septum and occupying almost 50% of atrial surface.Anticoagulation with enoxaparin 1 mg/kg BID was initiated and an angiography guided biopsy was done. Preliminary results were compatible with lymphoma so we added metilprednisolone 500 mg/day for 3 days with no clinical improvement. Chemotherapy was initiated with CHOP + methotrexate.Bone marrow biopsy showed no lymphoma infiltration and pathology results of the cardiac mass were compatible with pax5, CD10 and TDT positive b cell lymphoblastic lymphoma.On day 9, despite clinical improvement, she presented cardiac arrest and died. An echocardiogram done than day showed a 50% reduction in the mass size and fragmentation of it. An angio CT was done to rule out PE but was negative.Summary/Conclusion:PCL diagnoses is challenging and may have a dismal clinical course. A prompt diagnostic is necessary as soon as possible. We present this case due to the rarity of a solitary cardiac mass as the presentation of lymphoblastic lymphomas.image
PB1853 IMMUNOREGULATORY CYTOKINES (IL1β‐3954 AND IFNγ‐874) GENE POLYMORPHISMS IN NORTH INDIAN PATIENTS AFFECTED BY ACQUIRED APLASTIC ANEMIA.
Authors: Tripathi A. , Shukla S. 
Published: June 2019

Background:The etiopathogenesis of Acquired aplastic anemia is not clear. However immune mediated mechanisms are implicated in the genesis of this disease.Aims:The purpose of the study was to evaluate the polymorphisms in cytokine (IL1β‐3954 and IFN‐γ 874) genes in patients with acquired Aplastic Anemia (AA).Methods:In this observational case‐control study, 120 cases of AA & 120 healthy controls were taken under the standard criteria of Camitta's et al for AA. Genotyping for IL1β‐3954 and IFN‐γ 874 was performed using polymerase chain reaction and restriction fragment length polymorphism (PCR‐RFLP). Peripheral venous blood was collected in EDTA vial from all participants and DNA was extracted. Primer sequences of IL1β‐3954 and IFNγ‐ 874 genes were used under specific PCR conditions. PCR products were then digested with restriction enzymes at 37 °C for overnight. The digested products were run for gel electrophoresis and gel images were taken using the gel doc.Results:The frequency of homozygous (TT) variant genotypes of IL‐1β‐3954 was significantly associated with the higher susceptibility of developing AA when compared with the healthy controls (OR = 2.35, 95% CI = 1.10‐5.00, p = 0.0383). The mutant allele frequency (T) of IFN‐γ 874 showed a significant association with the lower susceptibility of developing AA when compared with the healthy controls (OR = 5.89, 95% CI = 3.97‐8.74, p = 0.0001).Summary/Conclusion:These findings suggest that IL‐1β‐3954 and IFN‐γ 874 gene polymorphisms may be implicated in the pathophysiology of Alastic Anemia.
PB1862 WHOLE EXOME SEQUENCING REVEALS XRCC2 MUTATION CAUSING FANCONI ANEMIA
Authors: Alhashem A. , Alabbasi B. , Alfaridi H. 
Published: June 2019

Background:The XRCC2 gene is a member of the RAD51 gene family, which encode proteins involved in homologous recombination repair of DNA damage. The XRCC2 gene acts late in the Fanconi anemia (FA)‐BRCA pathway of DNA repair. Fanconi anemia (FA) is a group of inherited disorder characterized by bone marrow failure, pancytopenia with multiple craniofacial and skeletal involvement.Aims:1‐To delinate the XRCC2 gene mutation,how it will present,and it is association with Fanocni anemia.2‐To show the importance of extensive genetic testing in a high consanguinous population, where autosomal recessive disorders are common.Methods:We are reporting here an extremely rare gene, detected by Whole exome sequence(WES) for a 3 years old Saudi girl born at Prince Sultan Medical Military City, with multiple congenital anomalies, bilateral hypoplastic thumb, cafe au lait spots, strabismus, abnormal limb morphology, dysmorphic facial features, short stature.Results:Patients had chromosomal breakage study of 100%. Her WES reveled a rare XRCC2 gene, c.643C>T p.(Arg215∗). which creates a premature stop codon.Summary/Conclusion:So far the detected variant is the second variant that was reported in the XRRC2 gene and has been associated with the Fanconi anemia of complementation group U (FANCU, OMIM: 617247). It was reported in a Saudi family on 2012. The importance of this communication is to emphasize on the open approach when dealing with a disease with wide possible genes like in FA. Moreover, to consider XRRC2 gene, especially in a Saudi population with high consanguinity and calls for further investigation of the potential contribution of XRCC2 mutations to the overall mutational load of FA.
PB1884 HIGH CXCR3 AND CXCR5 DISTINGUISH IGHVMUT FROM IGHVUNMUT CHRONIC LYMPHOCYTIC LEUKEMIA: EVIDENCE FROM CD5HIGH AND CD5LOW CLONES
Authors: Gabcova G. , Manukyan G. , Papajik T. , Mikulkova Z. , Urbanova R. , Kudelka M. , Smotkova Kraiczova V. , Zehnalova S. , Turcsanyi P. , Kriegova E. 
Published: June 2019

Background:Despite the shared pattern of surface antigens, neoplastic cells in chronic lymphocytic leukemia (CLL) demonstrate heterogeneity in their molecular and functional characteristics. Given the dynamic variability of CD5 expression and its link to the proliferative pool of neoplastic cells, more detailed characterization of CLL cell subpopulations are needed.Aims:To further characterize the chemokine receptors (CCR5, CCR7, CCR10, CXCR3, CXCR4, CXCR5) and adhesion molecules (CD54, CD62L, CD49d) on the CD5high and CD5low neoplastic clones in peripheral blood in CLL.Methods:Study cohort consisted of CLL patients (n = 60) subgrouped according to the IgHV mutational status (IgHV
mut, n = 24; IgHV
unmut, n = 36). Chemokine receptors and adhesion molecules on CD5high and CD5low cell subpopulations, determined by CD5/CD19 co‐expression, in peripheral blood were assessed by a six‐colour flow cytometry analysis on a BD FACSCanto II (Becton Dickinson). Data were analysed by basic statistics and patient similarity networks.Results:CD5high cell subpopulation expressed high levels of CXCR3 (P < 0.001), CXCR5 (P = 0.005), CCR7 (P = 0.013), CCR10 (P = 0.001), CD62L (P = 0.031) compared to CD5low cells expressing high levels of CXCR4 (P < 0.001). When comparing IgHV
mut from IgHV
unmut patients, high‐levels of CXCR3 and CXCR5 on the whole neoplastic population as well as CD5high and CD5low subpopulations were detected in the IgHV
mut patients. Multivariate patient similarity networks confirmed the significance of high CXCR3 levels predominantly on CD5low cells from IgHV
mut patients.Summary/Conclusion:Our analysis revealed that CD5high and CD5low neoplastic clones from IgHV
mut and IgHV
unmut differed mainly by the expression of CXCR3. Further characterization of neoplastic cell heterogeneity and role of CXCR3 will advance our understanding of CLL biology and its clinical relevance.Grants: MZ ČR VES16‐32339A, IGA UP_2019_014, IGA_LF_2019_001, MH CZ–DRO (FNOL, 00098892)
PB1892 REAL WORLD TREATMENT PERSISTENCE OF NEW ZEALAND IBRUTINIB CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS IN A NAMED PATIENT PROGRAM
Authors: Weinkove R. , Doocey R. , Henderson R. , Islam S. , Smith M. , Puig A. , Pateman G. , Acar M. , Amaya‐Chanaga C. , Simpson D. 
Published: June 2019

Background:Named Patient Programs (NPPs) provide access to medicines in response to unsolicited requests by physicians for specific patients before they become commercially available. Janssen New Zealand opened a NPP for ibrutinib for patients with relapsed or refractory Chronic Lymphocytic Leukemia (CLL) in September 2014. The program closed for new enrolments in April 2018, but enrolled patients have continued to receive treatment as of 7th January 2019.Aims:To analyse duration on ibrutinib treatment for patients with relapsed/refractory CLL enrolled in a New Zealand NPP.Methods:A retrospective cohort analysis was conducted using data entered by treating physicians when enrolling patients into the NPP via the Janssen Managed Access Portal (MacWeb). At each resupply patients were provided with 3 months’ supply of ibrutinib. Patient time on treatment was estimated using fulfilled resupply requests submitted by physicians. Patients were considered to be on treatment until the date of last ibrutinib resupply, plus one month. A patient was considered discontinued based on physician declaration, or if no ibrutinib had been resupplied for four months or more. All other patients were censored at the reporting date of 7th January 2019. This real‐world estimate of treatment duration was compared to time to treatment discontinuation in the ibrutinib arm of the RESONATE trial, as reported in Hillmen et al. EHA; 2016 (abstract 133196) and Tam et al. EHA; 2018 (abstract 214826). Time on treatment was evaluated using Kaplan‐Meier (KM) curves, and statistical testing was conducted using log‐rank testing.Results:112 ibrutinib‐treated patients with CLL were included in the analysis. 59% of the population were male, 37% had 3 or more prior lines of therapy before commencing therapy, and 32% had refractory disease. 73% of patients were on therapy at 12 months (95% CI: 0.64‐0.8) and median time to discontinuation has not been reached at the time of the analysis. 42% of patients were estimated to have discontinued therapy over the 49‐month period. The comparison between patient time on treatment on the New Zealand NPP and the RESONATE trial were statistically not different (HR 1.01; 95% CI: 0.71‐1.44; p = 0.96).Summary/Conclusion:This real‐world analysis estimates that patient time on NPP treatment in New Zealand closely tracks time on treatment as observed in the RESONATE trial. While there are limitations to the NPP data as it was based on unmonitored physician declarations, and duration on treatment was estimated from resupply data, these findings suggest that duration of treatment in a New Zealand non‐trial setting is similar to that in the Phase 3 clinical trial.image
PB1939 ACCURACY AND SPEED OF REPORTING MOLECULAR RESPONSE IN PATIENTS ON TYROSINE KINASE INHIBITORS THERAPY WITH GENEXPERT CARTRIDGE‐BASED BCR‐ABL1 QUANTITATION
Authors: Dreisinger M. , Stangler Herodež Š. 
Published: June 2019

Background:Monitoring of minimal residual disease (MRD) in chronic myeloid leukemia (CML) patients who are on tyrosine kinase inhibitors (TKI) is one of the best standardized molecular methods. Guidelines for treating these patients include the evaluation of molecular response (MR) based on % BCR‐ABL1 on the International Scale (IS) in defined time points. Achieving timely MR is necessary for determining the status of the disease and the use of appropriate therapy.Aims:The aim of this study was to introduce the GeneXpert cartridge‐based BCR‐ABL1 detection into routine molecular monitoring of CML Slovenian patients from north‐eastern region and compare the MR results with those obtained from a standard laboratory method.Methods:BCR‐ABL1 transcript quantification was performed in 40 peripheral blood samples from CML patients at different stages of the disease in two different laboratories. First one used automated GeneXpert BCR‐ABL Ultra Assay (Cepheid, USA) and the second one used standardized laboratory protocol. The results for both analyzes were expressed as % IS and were estimated by MR according to Laboratory recommendations for scoring deep molecular responses following treatment for chronic myeloid leukemia.Results:The analysis of the results showed that there were no statistically significant differences in the results expressed in % IS (p = 0, 272). Samples according to the estimated MR were divided into 3 groups (8 MR3 samples, 3 MR4 samples and 27 ≥ MR4.5 samples). 27/40 samples had a completely identical MR with both methods. Furthermore, 11/40 samples had higher MR obtained with GeneXpert, while 0/40 samples had lower MR compared to SP. In 2/40 cases, GeneXpert gave MR5 for sample with SP MR3 and MR4 for sample with SP MR3. None of the results obtained with GeneXpert had any effect on a different clinical decision on treatment with TKI as obtained under the SP. When the major molecular response (MMR) was analyzed, 78% agreement was achieved.Summary/Conclusion:GeneXpert BCR‐ABL Ultra Assay provides reproducible results and doesn’t show significant differences compared with the standardized laboratory protocol. From clinical aspect, GeneXpert BCR‐ABL Ultra Assay correctly classified the patients in MR and can be considered as a useful and very fast clinical tool for the molecular follow up of patients on tyrosine kinase inhibitors.
PB1954 DETECTION OF COMPLEX VARIANT T(9;22) CHROMOSOME TRANSLOCATIONS IN NEWLY DIAGNOSED CASES OF CHRONIC MYELOID LEUKAEMIA
Authors: Fareed N. , somroo N.M. , Quraishy M.S. , Rafiq S. 
Published: June 2019

Background:Chronic myeloid leukemia (CML) is a stem cell clonal disease characterized by the acquisition of a fusion protein, BCR‐ABL1 oncogene, which leads to uncontrolled proliferation of myeloid elements in all stages of differentiation. However, a small proportion of patients with CML have simple or complex variants of this translocation, involving various breakpoints in addition to 9q34 and 22q11.Aims:Detection of complex variant t(9;22) chromosome translocations in newly diagnosed cases of Chronic Myeloid Leukaemia which help us to choose therapy modalities.Methods:This study was conducted for a time period of 05 years from 2012 to 2017 in the department of Oncology/ Hematology of Civil hospital, Karachi. Between April 2012 and April 2017, 88 CML patients were diagnosed in our Department. Informed consent was obtained from the patient in accordance with the Declaration of Helsinki. Diagnosis was done on the morphological basis and BCR‐ABL mutation by PCR/FISH. Conventional cytogenetic analysis was performed on unstimulated 24‐hour culture of a bone marrow (BM) specimen. The cells were cultured and processed by conventional methods, and the chromosomes were stained with trypsin‐Giemsa banding (GTG‐banding).Results:A total of 88 patients were Philadelphia‐positive CML, out of which 53(60.2%) were males & 35(39.7%) were females. Mean age was 48 yrs ± 11.43. Out of them, the group of 9 (10.2%) patients with complex cytogenetics were consisted of 5 females and 4 males, ranging in age at diagnosis from 32 to 66 yrs. All the patients were in chronic phase at presentation. Cytogenetic analysis by G‐banding revealed the presence of 9 reciprocal three‐way variant translocations of the classical t(9;22)(q34;q11). The chromosome breakpoints involved in these complex variant translocations were the following: 1p13, 1q21, 1p22, 2q31, 3q21, 3p21, 11q13, 15q24 and Xp11.2Summary/Conclusion:In our study 09 patients had complex variants of t(9;22).The presence of these complex variants at diagnosis and during the TKI treatment may announce treatment failure and/ or transformation to advanced stage (accelerated or blast). Early identification of these abnormalities may help in choosing therapy modalities.
PB2033 IMMUNOGLOBULIN REPLACEMENT THERAPY IN ADULT HEMATOLOGICAL PATIENTS: EFFECT ON THE INCIDENCE OF INFECTIOUS COMPLICATIONS
Authors: Gerasimovich O. , Stoma I. , Iskrov I. , Milanovich N. , Lendina I. , Uss A. 
Published: June 2019

Background:The problem of infectious complications in hematology is determined by their high frequency, severity and difficulty of diagnosis. The clinical effect of immunoglobulin replacement therapy in relation to the risk of infectious complications in adult hematological patients currently needs to be assessed and revised.Aims:The aim of this prospective study was to evaluate the independent effect of immunoglobulin replacement therapy on the risk of developing infectious complications in hematological patients.Methods:The study included 34 adult patients receiving chemotherapy for hematological disease. Intervention groups (immunoglobulin replacement therapy) and the control group were matched by clinical and demographic characteristics. The primary outcome in the analysis was taken as the infection‐free period in the 180‐days survival analysis performed by Kaplan‐Meier with log‐rank test, Cox regression and Cox regression.Results:The average age of patients was 57.8 years, with sex distribution: 19 female and 15 male. Hematological diseases were distributed as follows: chronic lymphocytic leukemia ‐ 18, multiple myeloma ‐ 8, acute myeloid leukemia ‐ 2, acute lymphocytic leukemia ‐ 4, myelodysplastic syndrome ‐ 2. Among the included ‐ 15 patients had profound hypogammaglobulinemia (less than 4 g/l). Statistically significant association of immunoglobulin replacement therapy and reduction of the risk of infectious episodes was shown in a 180‐days period (log‐rank test, p = 0.037), Fig 1. The average duration of the period without infectious complications in the replacement group was 164 days (95% CI 147‐180 days), while in the control group it was 148 days (95% CI 124‐171 days). No additional confounding factors, which could cause the differences in the groups were found in the regression analysis (p = 0.035). The number needed to treat (NNT) was 3 patients (p = 0.018).Summary/Conclusion:The obtained data show that the use of immunoglobulin replacement therapy is independently associated with a longer period of infection‐free survival in adult hematological patients, what substantially improves the quality of life of such patients. The clinical effect may be assessed within 6 months from the start of immunoglobulin replacement therapy.image