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Aims:Lactate dehydrogenase (LDH) and platelet count are routinely used to evaluate response to treatment and discontinuation of treatment in thrombotic thrombocytopenic purpura (TTP). This study aimed to evaluate the mean platelet volume (MPV) and platelet distribution width (PDW) in immune TTP (iTTP) as markers of treatment response.Methods:This retrospective, multicenter study included patients diagnosed with iTTP with severe ADAMTS13 deficiency. We studied the correlations of MPV and PDW values with platelet count, LDH, total bilirubin hematocrit and mean corpuscular volume, which are used to evaluate the response to total plasma exchange (TPE) or relapse in iTTP. The study variables were recorded at the time of diagnosis, 1st week of TPE treatment, and the time of the last TPE. The correlation analyses were performed between the values before the initial TPE, and after the first week and last TPE.Results:The study included 28 patients, 20 females with iTTP [median age (minimum-maximum): 45 (23-74) years]. MPV correlated positively with LDH (r=0.533, p=0.002) and negatively with hematocrit (r=-0.445, p=0.002) and platelet count (r=-0.560, p=0.002). PDW also correlated positively with LDH (r=0.339, p=0.008) and negatively with hematocrit (r=-0.244, p=0.032) and platelet count (r=-0.285, p=0.022).Conclusions:The results showed that MPV and PDW correlated with LDH and platelet count, which are currently used to evaluate the response to treatment in iTTP. Changes in MPV and PDW may serve as a surrogate of treatment response in these patients as an indicator of response to plasmapheresis.

Abstract Background Neoadjuvant treatment is the standard treatment in locally advanced ESCC. However, the optimal chemotherapy regimen is not known. Method This is a retrospective observational cohort study conducted with propensity score matching. Patients with resectable ESCC from 13 tertiary centers from Türkiye were screened between January 2011 and December 2021. We compared the efficacy and safety of neoadjuvant chemoradiotherapy with the CF and the CROSS regimens in patients with ESCC. Results Three hundred and sixty‐two patients were screened. Patients who received induction chemotherapy (n = 72) and CROSS‐ineligible (n = 31) were excluded. Two hundred and fifty nine patients received neoadjuvant chemoradiotherapy. After propensity score matching (n = 97 in both groups), the mPFS was 18.4 months (95% CI, 9.3–27.4) and 25.7 months (95% CI, 15.6–35.7; p = 0.974), and the mOS was 35.2 months (95% CI, 18.9–51.5) and 39.6 months (95% CI 20.1–59.2; p = 0.534), in the CF and the CROSS groups, respectively. There was no difference between subgroups regarding PFS and OS. Compared with the CF group, the CROSS group had a higher incidence of neutropenia (34.0% vs. 62.9%, p

Abstract Introduction Resistance to immune checkpoint inhibitors (ICI) is a significant issue in metastatic renal cell carcinoma (mRCC), as it is in the majority of cancer types. An important deficiency in immunooncology today is the lack of a predictive factor to identify this patient group. Myeloid-derived suppressor cells (MDSC) are a type of cell that contributes to immunotherapy resistance by inhibiting T cell activity. While it accumulates in the tumor microenvironment and blood, it can also accumulate in lymphoid organs such as the spleen and cause splenomegaly. Therefore we aimed to evaluate the effect of increase in splenic volume, which can be considered as an indirect indicator of increased MDSC cells, on survival outcomes in mRCC patients. Methods We analyzed 45 patients with mRCC who received nivolumab as a second-line or subsequent therapy. Splenic volume was analyzed from baseline imaging before starting nivolumab and from control imaging performed within the first 6 months of treatment initiation. Additionally, we analyzed how patients’ body mass index (BMI), IMDC risk score, ECOG performance status, nephrectomy status, neutrophil-lymphocyte ratio (NLR), Platelet-to-lymphocyte ratio (PLR) and sites of metastasis. Results Median splenic volume change was 10% (ranging from − 22% to + 117%) during follow-up. Change in splenic volume was found to be associated with overall survival (OS) and progression-free survival (PFS) (p = 0.025, 0.04). The median PFS in patients with increased splenic volume was 5 months, while it was 17 months in patients without increased splenic volume. (HR 2.1, 95% CI (1–4), p = 0.04). The median OS in patients with increased splenic volume was 9 months, while it was 35 months in patients without increased splenic volume (HR 2.7, 95% CI (1.1–6.2), p = 0.025). In four patients with decreased splenic volume, neither PFS nor OS could reach the median value. Log-rank p value in respectively (0.015, 0.035), The group in which an increase in volume was accompanied by a high NLR had the shortest survival rate. Basal splenic volume was analyzed separately. However, neither PFS nor OS differed significantly. Conclusion Our findings suggest that the change in splenic volume throughout immunotherapy regimens may be utilized to predict PFS and OS in mRCC patients undergoing treatment.

Objective: Achieving an early molecular response (EMR) is crucial for improving the prognosis of patients with chronic myeloid leukemia (CML). The halving time (HT) and reduction ratio (RR) of BCR: : ABL1 transcript levels have recently emerged as additional prognostic indexes besides the BCR: : ABL1 International Scale (IS). We aimed to investigate the prognostic role of BCR: : ABL1 transcript levels, HT, and RR on molecular response kinetics at 3 months in patients with newly diagnosed chronic-phase (CP)-CML. Materials and Methods: Forty patients with CP-CML who received first-line imatinib treatment were included in this study. BCR: : ABL1 transcript levels and molecular responses at baseline and at 3, 6, 12, and 24 months of treatment were evaluated retrospectively. Major molecular response (MMR) at 12 months and event-free survival (EFS) were determined as primary endpoints and the effects of treatment kinetics on these parameters were examined. Results: Of the 40 patients, BCR: : ABL1 IS was ≤10% at 3 months in 72.5%, representing EMR. The rate of event occurrence was 45.5% in patients with BCR: : ABL1 IS of >10%, whereas it was 6.9% in those with BCR: : ABL1 IS of ≤10% (p=0.004). MMR was detected in 62.1% of the patients with EMR and in 9.1% of those without EMR (p=0.003). The cut-off value for achieving MMR was 24 days for HT and 0.04 for RR. Deep molecular response (DMR) at 24 months was associated with HT of ≤24 days and RR of ≤0.04. EFS was found to be significantly better in the group with BCR: : ABL1 IS of ≤10% and HT of ≤24 days (p=0.001) and in the group with BCR: : ABL1 IS of ≤10% and RR of ≤0.04 (p=0.007) compared to others. Conclusion: Our findings revealed that MMR could be predicted via EMR as well as by HT and RR. Additionally, HT of ≤24 days and RR of ≤0.04 were more important than BCR: : ABL1 IS of ≤10% in achieving DMR at 24 months, and the combination of BCR: : ABL1 IS of ≤10% with both HT of ≤24 days and RR of ≤0.04 has the best predictive value for EFS.

Background: The transmembrane receptor tyrosine kinase-like orphan receptor 1 (ROR1) has acted on the causation and sustentation of mature B cell lymphomagenesis for chronic lymphocytic leukemia (CLL) cells. The aim of this study was to show whether there is a relationship between the level of ROR1 surface expression in CLL cells and disease findings. Materials and Methods: The level of ROR1 cell surface expression was determined in accordance with the flow cytometric analysis of CLL patients at the first diagnosis time. 2 groups were formed according to the high and low ROR1 levels. The cut-off point to the ROR1 level was calculated for advanced-stage disease by using receiver operating characteristic (ROC) curves. A two-sided p-value

Objective: This study aimed to retrospectively evaluate the efficacy, safety, and survival outcome of single-agent ibrutinib therapy in chronic lymphocytic leukemia patients. Materials and Methods: A total of 136 patients (mean age +- standard deviation: 64.6+-10.3 years, 66.9% males) who had received at least one dose of ibrutinib were included in this retrospective multicenter, noninterventional hospital-registry study conducted at 33 centers across Turkey. Data on patient demographics, baseline characteristics, laboratory findings, and leukemia-cell cytogenetics were retrieved. Treatment response, survival outcome including overall survival (OS) and progression-free survival (PFS), and safety data were analyzed. Results: Overall, 36.7% of patients were categorized as Eastern Cooperative Oncology Group (ECOG) class 2-3, while 44.9% were in Rai stage 4. Fluorescence in situ hybridization revealed the presence of del(17p) in 39.8% of the patients. Patients received a median of 2.0 (range: 0-7) lines of pre-ibrutinib therapy. Median duration of therapy was 8.8 months (range: 0.4-58.0 months). The 1-year PFS and OS rates were 82.2% and 84.6%, respectively, while median PFS time was 30.0 (standard error, 95% confidence interval: 5.1, 20.0-40.0) months and median OS time was 37.9 (3.2, 31.5-44.2) months. Treatment response (complete or partial response), PFS time, and OS time were better with 0-2 lines versus 3-7 lines of prior therapy (p

Recent advances in hematology analyzers have enabled to improve the reliability in the results and also provided additional hematological parameters. In the present study, we aimed to determine the reference ranges for automated erythrocyte and reticulocyte parameters in healthy individuals on Sysmex XN 1000 hematology analyzer.

This study aimed to assess the efficacy and safety of FOLFIRI and paclitaxel in patients with advanced gastric cancer (AGC) who were previously treated with first-line modified docetaxel, cisplatin, 5-fluorouracil (mDCF), or 5-fluorouracil, oxaliplatin, docetaxel (FLOT). Patients who received a triplet regimen in the first line setting and were treated with FOLFIRI or paclitaxel in the second-line treatment were included. The study included 198 patients, with 115 receiving FOLFIRI and 83 receiving paclitaxel. The median age was 58 (range = 24–69). The median progression-free survival (mPFS) was 5.2 [95% confidence interval (CI) = 4.4–5.5] months in the FOLFIRI arm, and 4.1 (95% CI = 3.3–4.6) months in the paclitaxel arm (p =.007). The median overall survival (mOS) was 9.4 (95% CI = 7.4–10.5) months in the FOLFIRI arm and 7.2 (95% CI = 5.6–8.3) months in the paclitaxel arm (p =.008). Grade 3–4 neuropathy was higher in patients receiving paclitaxel compared to those receiving FOLFIRI (p =.04). Grade 3–4 diarrhea was 8% in the FOLFIRI arm and 2.4% in the paclitaxel arm (p =.02). Beyond progression with docetaxel-based triplet chemotherapy, FOLFIRI may be preferred as a second-line treatment over paclitaxel due to its longer mPFS and mOS. [ABSTRACT FROM AUTHOR]

Introduction: The current study aims to evaluate the OX40, TIM-3, LAG-3, and PD-L1 targeted pathways in the regulation of T-cell activity in sarcoma patients to determine their relationship with overall survival (OS). Method: This study included one hundred and eleven patients with bone and soft tissue sarcoma diagnosed in two centers between 2010 and 2020. OX40, LAG-3, TIM-3 and PD-L1 expression levels were evaluated immunohistochemically from pathology preparations. Results: PD-L1 staining was detected in tumor cells, OX40, LAG-3, TIM-3 staining was detected in inflammatory cells in tumor tissue. In univariate analysis, no significant relationship was found between OX40, TIM-3, LAG-3, and PD-L1 staining and overall survival (respectively: p = 0.12, p = 0.49, p = 0.31, p = 0.95). When grade and stage at diagnosis, which were found to be significant in univariate analysis, along with OX-40, TIM-3, LAG-3, and PD-L1, were evaluated in multivariate analysis, a positive effect of OX-40 staining on overall survival was determined (p = 0.009). Considering the correlation between PDL-1 and OX40, TIM-3, and LAG-3 staining, a significant positive correlation was found between PDL-1 and TIM-3 and LAG-3 staining (respectively; p = 0.002, p = 0.001). Conclusions: There was no significant relationship between the PDL-1 staining percentage of tumor cells and OX40, TIM-3, and LAG-3 staining in inflammatory cells with the OS of sarcoma patients. However, detecting a significant positive correlation between PDL-1 staining and TIM-3 and LAG-3 staining also holds promise for finding effective targetable combination therapies that can prolong survival in sarcoma patients in the future. [ABSTRACT FROM AUTHOR]

Objective: Autologous hematopoetic stem cell transplantation (aHSCT) after high dose chemotherapy is a standard treatment for multiple myeloma (MM) patients. The successful aHSCT depends on collection of sufficient numbers of hematopoietic progenitor stem cells and sustained engraftment following infusion. The aim of the present study is to determine the the impact of bone marrow fibrosis (BMF) on the clinical outcomes of MM patients who underwent aHSCT. Methodology: Retrospectively, bone marrow trephine biopsy analyzed in 73 MM patients who were treated with hematopoietic stem cell transplantation (aHSCT) following bortezomib based induction regimen. The BM biopsy samples of all patients were re-evaluated by a single pathologists The patients divided into 4 groups according to fibrosis degree and the correlations in initial characteristic features, therapeutic response, survival, mobilization and engraftment outcomes were reviewed between the groups. Results: Comparative analyses revealed that the median apheresis number was found statistically different according to groups (p=0.04). No significance was detected between the fibrozis grade and the number of peripheral blood CD34+ cell collection results and recovery time of neutrophils and platelets. Overall survival and progression free survival were found similar in groups, however relapse of disease was statistically different in patients with fibrosis (p=0.01). Conclusion: After induction treatment, a regression was observed in fibrosis grade of patients who had fibrosis at the time of diagnosis. Therefore we suggest to evaluate fibrosis status in all MM patients during each histopathological examination. Difficulties may be experienced during stem cell collection in transplant eligible MM patients with fibrosis at diagnosis. Therefore, we recommend that clinicians should be more careful in these patients during the induction treatment and stem cell mobilization.

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Classical serum sickness is a type III immune complex-mediated hypersensitivity disease caused by immunization of the host by non-human serum proteins. Anti-thymocyte globulin is one of the agents mostly responsible. Prophylactic steroids are used in the treatment protocol of aplastic anemia in addition to anti-thymocyte globulin. A 33-year-old female patient diagnosed with aplastic anemia developed severe serum sickness with anti-thymocyte globulin, despite the administration of a prophylactic dose of methylprednisolone. The patient responded dramatically to a single dose of pulse steroid therapy. There are reports that high-dose steroids (1-2 mg/kg/day to 500-1000 mg/day methylprednisolone) and/or therapeutic plasma exchange are beneficial treatment options. Our report shows the benefit of a single pulse dose of steroid and gradual tapering of the dose in this case. [ABSTRACT FROM AUTHOR]

Objective: To examine granulocytic and non-granulocytic cells in children with severe congenital neutropenia (SCN) and their nonneutropenic parents. Materials and Methods: Fifteen patients with SCN and 21 nonneutropenic parents were evaluated for a) CD95, CD95 ligand, annexin V, propidium iodide, cell cycle, and lymphocyte subsets by flow cytometry; b) rapid cell senescence (of leukocytes) by senescenceassociated β-galactosidase stain; c) aggregation tests by aggregometer; d) in vitro bleeding time by PFA-100 instrument; e) mepacrine-labeled dense granule number of thrombocytes by fluorescence microscope; and f) hematomorphology by light and electron microscope. HAX1, ELANE, G6PC3, CSF3R, and JAGN1 mutations associated with SCN were studied in patients and several parents. Results: Significant increase in apoptosis and secondary necrosis in monocytes, lymphocytes, and granulocytes of the patients and parents was detected, irrespective of the mutation type. CD95 and CD95 ligand results implied that apoptosis was non-CD95- mediated. Leukocytes of 25%, 12.5%, and 0% of patients, parents, and controls showed rapid cell senescence. The cell cycle analysis testable in four cases showed G1 arrest and apoptosis in lymphocytes of three. The patients had HAX1 (n=6), ELANE (n=2), G6PC3 (n=2), and unidentified (n=5) mutations. The CD3, CD4, and NK lymphocytes were below normal levels in 16.6%, 8.3%, and 36.4% of the patients and in 0%, 0%, and 15.4% of the parents (controls: 0%, 0%, 5.6%). The thrombocytes aggregated at low rates, dense granule number/ thrombocyte ratio was low, and in vitro bleeding time was prolonged in 37.5%-66.6% of patients and 33.3%-63.2% of parents (vs. 0% in controls). Under electron and/or light microscope, the neutrophils, monocytes, lymphocytes, and thrombocytes in the peripheral blood of both patients and parents were dysplastic and the bone marrow of patients revealed increased phagocytic activity, dysmegakaryopoiesis, and necrotic and apoptotic cells. Ultrastructurally, thrombocyte adhesion, aggregation, and release were inadequate. Conclusion: In cases of SCN, patients' pluripotent hematopoietic stem cells and their non-neutropenic parents are both affected irrespective of the genetic defect.

Objective: Atypical chronic lymphocytic leukemia (CLL) is most frequently confused with mantle cell lymphoma (MCL). Several markers may contribute to the diagnosis of CLL. However, there is no consensus on which markers are needed to be used in flow cytometry for the diagnosis of CLL. The aim of the present study was to investigate the role of CD43 and CD200 markers in the differential diagnosis between CLL and MCL. Materials and Methods: To address this issue, 339 consecutive patients with CLL and MCL were included in the flow cytometry lymphoproliferative disease panel for evaluation of CD43 and CD200 expressions, but not in the Matutes scoring system. Results: CD200 was expressed in 97.3% of atypical CLL cases, whereas it was dimly expressed in only 6.1% of MCL cases. CD43 expression was 95.7% in atypical CLL cases. In the MCL cases, its expression rate was 39.4%. Conclusion: CD43 and CD200 were found to be more valuable markers than CD22, CD79b, and FMC7. CD43 and CD200 could also be considered as definitive markers in atypical CLL patients, for whom the Matutes scoring system remains ineffective.

Byline: Hayriye. Sahinli, Nalan. Akyürek, Mukaddes. Yilmaz, Olcay. Kandemir, Ayse. Duran, Sezer. Kulaçoglu, Gökhan. Uçar, Elif. Acar, Ahmet. Özet, Mahmut. Gümüs, Ö. Ç. Öksüzoglu, Nuriye. Özdemir Background: Programmed death-ligand 1 [...]

Background This study aimed to evaluate the efficacy and side effects of bortezomib, cyclophosphamide and dexamethasone (VCD) treatment, which is frequently preferred in primary care in patients with multiple myeloma in our country, with two applications per week and one application per week. Methods A total of 141 patients who received VCD in the induction treatment of newly diagnosed multiple myeloma were retrospectively reviewed and analysed. Both treatment groups were evaluated in terms of efficacy and side effects. Results A total of 141 patients with newly diagnosed multiple myeloma who received VCD in induction therapy were included in the study. The median age was 62 years. Among 141 patients included in the study, 57 patients received treatment two days a week and 84 patients received treatment one day a week. Sixty-one (43.3%) patients were female and 80 (56.7%) were male. There was no significant difference between the two groups in terms of post-treatment response rates after 2nd cycle VCD regimen (p=0.378) and 4th cycle VCD regimen (p=0.965). Patients receiving weekly VCD regimen had a significantly higher rate of receiving other regimens, and additional VCD regimen of autologous stem cell transplant (ASCT) was significantly higher in patients who received a VCD regimen twice a week compared to the other group (p<0.001). ASCT was performed in 73% of the patients (n: 103). In 54 patients with ASCT at the end of 4th cycle VCD, there was no significant difference between very good partial response/complete response rates and partial response/sub responses between the two groups according to the 3rd month post-transplant responses (p=0.612). Neuropathy was observed in seven (12.3%) patients receiving twice-weekly VCD regimens, while neuropathy was observed in 16 (19.3%) and neutropenia in two (2.4%) patients receiving weekly VCD regimens. The two groups had no significant difference regarding side effects (p=0.387). Conclusion Our study found no significant difference in the treatment response rates of patients receiving weekly VCD and twice-weekly VCD. The low rates of ASCT in the weekly VCD group were thought to be related to the fact that the patients receiving the weekly regimen were older than the other group and were unsuitable for ASCT due to age. No difference was observed between the two groups regarding the frequency of side effects. [ABSTRACT FROM AUTHOR]