Here, we report the outcome of 226 myeloma patients presenting with extramedullary plasmacytoma or paraosseous involvement in a retrospective study conducted in 19 centers from 11 countries. ...Extramedullary disease was detected at diagnosis or relapse between January 2010 and November 2017. Extramedullary plasmacytoma and paraosseous involvement were observed in 130 patients at diagnosis (92 of 38) and in 96 at relapse (84 of 12). The median time from multiple myeloma diagnosis to the development of extramedullary disease was 25.1 months (range 3.1-106.3 months) in the relapse group (median follow up: 15 months). Imaging approach for extramedullary disease was computed tomography (n=133), positron emission tomography combined with computed tomography (n=50), or magnetic resonance imaging (n=35). The entire group received a median two lines of treatment and autologous stem cell transplantation (44%) following the diagnosis of extramedullary disease. Complete response was higher for paraosseous involvement
extramedullary plasmacytoma at diagnosis (34.2%
19.3%;
=NS.) and relapse (54.5%
9%;
=0.001). Also paraosseous involvement patients had a better progression-free survival (PFS) when recognized at initial diagnosis of myeloma than at relapse (51.7
38.9 months). In addition, overall survival was better for paraosseous involvement compared to extramedullary plasmacytoma at diagnosis (not reached
46.5 months). Extramedullary plasmacytoma at relapse had the worst prognosis with a PFS of 13.6 months and overall survival of 11.4 months. In the multivariate analysis, paraosseous involvement, extramedullary disease at diagnosis, International Staging System (ISS-I), and undergoing autologous stem cell transplantation improved overall survival independently. This cohort demonstrated that extramedullary disease benefits from front-line autologous stem cell transplantation and extramedullary plasmacytoma differs from paraosseous involvement in terms of rate and duration of response, with even worse outcomes when detected at relapse, constituting an unmet clinical need.
Myelofibrosis is reported in around 40% of newly diagnosed chronic myeloid leukemia (CML) patients and have an important role in the pathobiology and prognosis of CML. This retrospective study aimed ...to evaluate the effects of bone marrow (BM) fibrosis on disease prognosis and the effects of specific tyrosine-kinase inhibitors (TKIs) on BM fibrosis in CML patients.
The study included 96 patients (>18 years) diagnosed with chronic phase (CP) CML. The clinical and demographic information were collected from the medical files. Post-treatment BM aspirate and core biopsy samples were analyzed for the presence of fibrosis and dysplasia.
The mean age of the study patients was 52.69 years; 47.9% of the patients were female. At the onset, 53 (63.1%) patients had BM fibrosis. The difference in the overall survival of the patients with respect to BM fibrosis grades was significant (p = .001). Within the BM fibrosis grade groups, there were significant differences between grade 0 vs. grade 2, grade 0 vs. grade 3, and grade 1 vs. grade 3 (p = .005, p = .002, and p = .003 respectively) There was no significant association between the presence of BM fibrosis at the onset and not responding to first-line therapy (p = .724). Moreover, no significant association was found between the presence of BM fibrosis at the onset and molecular (p = .623) or cytogenetic response (p = .535) to first-line therapy. Additionally, the association between the type of second-line and third-line therapy and molecular response (p = .773 and p = .424, respectively) or cytogenetic response (p = .298 and p = .641) was not significant.
Although BM fibrosis seems to be a crucial complication of CML with a poor prognosis, it can be reversed via TKI treatment which may result in improved survival. It might be considered to check the BM for this complication on a regular basis during therapies to test its prognostic influence in CML patients in prospective controlled trials. Further studies focused on this issue are required to utilize BM fibrosis as a candidate prognostic factor.
BM fibrosis seems to be a crucial complication of CML with a poor prognosis if it could not be reversed during treatment. It might be considered to check the BM for this complication on a regular basis during therapies to test its prognostic influence in CML patients in prospective controlled trials. Further studies focused on this issue are required to utilize BM fibrosis as a candidate prognosticfactor.
Drug-drug interactions (DDIs) occur when one drug interferes with the pharmacological activity of another and can lead to increased side effects. The purpose of this study was to examine potential ...interactions between antimicrobials and other drugs in patients with hematological malignancies (HMs).
The medications used by 233 patients with HMs before and during hospitalization in Ankara City Hospital Hematology Clinic services between January 2021 and July 2021 were examined. Potential DDIs (pDDIs) were identified through UptoDate, Drugs.com, and MedScape databases. The effects of major antimicrobial-related pDDIs on patients were examined. Agreement between the two interaction systems was judged based on the kappa test. SPSS R Version 4.0.2 was used in the statistical analysis of the data,
<.05 was considered significant.
The prevalence of polypharmacy before hospitalization was determined as 22.7%. Diagnosed with acute leukemia and multiple myeloma, more antimicrobial-related pDDIs were detected during hospitalization (
<.001). A total of 758 antimicrobial-related pDDIs, which were in the major category in at least one of the three databases, were detected in 72.5% (169/233) of the participants. It was determined that the total hospitalization period of patients with major antimicrobial-related pDDIs was longer (
<.001). There was negligible agreement between UptoDate and Dugs.com and between Drugs.com and MedScape (kappa: 0.008 for both). There was no compatibility between UptoDate and MedScape (kappa<0).
Interactions between antimicrobials and other drugs are undesirable problems. Further studies are required to evaluate the clinical and economic effects of the interactions on patients with HMs.
Next-generation sequencing (NGS)-based technologies are novel methodologies for the diagnosis, prognostic assessment and decision of individualized treatment strategy in hematological neoplasia. NGS ...led to a more comprehensive understanding of the mutational landscape, especially in the myeloid neoplasms. Herein, we present the results of the patients who underwent NGS with the suspicion of myeloid neoplasia.
Retrospective data from a total of 13 patients were analyzed who were diagnosed between 01.10.2018 and 01.06.2021. There were four myeloid panels in the NGS. Panel 1 consists of ASXL1, CALR, CBL, CEBPA, CSF3R, and DNMT3A mutations. Panel 2 consists of EZH2, FLT3, IDH1, IDH2, JAK2, KIT, KRAS, and MPL mutations. Panel 3 consists of NPM1, NRAS, RUNX1, SETBP1, and SF3B1 mutations. Panel 4 consists of SH2B3/LNK, SRSF2, TET2, TP53, U2AF1, ZRSR2 mutations.
Median age was 48. Diagnoses were AML (n=7), AA (n=1), MDS (n=2), DLBCL (n=1), MM (n=1), and Evans syndrome (n=1). Seven cases with malignant diagnoses were eligible for intensive therapy. There were no mutations detected by NGS in MM, AA, DLBCL, and Evans syndrome cases. Biallelic CEBPA mutation accompanied FLT3 mutation in 1 case. IDH1 and NPM mutation were detected in 1 APL case. MPL, SRSF2, ASXL1, CBL, U2AF1, SF2B1, and TET2 were mutations detected in cases with dysplasia.
In our cohort, NGS did not add any significant information in the lymphoid malignancies and benign hematological cases. NGS helped to define the allelic ratio of FLT3+ mutations and helped to accurately define the ELN risk of AML. Mutations that were detected in the cases with dysplastic bone marrow findings were concordant that were reported in the literature. Larger case series are needed in order to define the therapeutic and prognostic implications.
Objective: Down syndrome (DS) is a genetic disorder caused by the presence of a third copy of chromosome 21.It is usually associated with physical growth delays, mild to moderate intellectual ...disability, and characteristic facial features .Children with DS are at an elevated risk of leukemia, especially myeloid leukemia. On the other hand, children with DS are at a 20-fold increased risk for acute lymphoblastic leukemia (ALL).In our case, we presented a patient with DS who was diagnosed with ALL. Case report: 19-year-old male was admitted to the emergency department due to abdominal pain.On his physical examination,splenomegaly was detected.In laboratory examinations; kidney and liver function tests were normal,lactate dehydrogenase:372 U/L,uric acid: 5.4 mg/dl,white blood cell:25000 × 106/L,lymphocyte: 15780 × 106/L, neutrophil:1140 × 106/L,hemoglobin:10 gr/dl, thrombocyte:12000 × 106/L,coagulation tests were normal and in peripheral blood smear evaluation,90% blast cells were detected. Methodology: Peripheral blood flow cytometry evaluation was compatible with B-ALL(TdT,CD19,CD10,CD34,cCD79a,CD58,CD9,CD38,CD123,CD20,CD81,CD22 positivity in atypical cells).Bone marrow biopsy was hypercellular.There was diffuse blastic cell infiltration,which stained extensively with TDT,CD79a.Chromosomal analysis is 47XY,+21 and t(12,21) (p13.2;q22.12) (ETV6/RUNX1) (FISH) and 14q32.33 (IGH) FISH were positive,t(9;22) P190 -p210,t(4;11), t(1;19),11q23 were negative.The risk classification was standard risk. Results: AUGMENTED BFM induction chemotherapy protocol was started.Pancreatitis was developed after peg-asparaginase and chemotherapy-related hepatotoxicity(grade 1) was developed.Central nervous system prophylaxis(intrathecal methotrexate) was applied.The control bone marrow biopsy performed after induction was normocellular,the blast rate was <5%.BFM standard risk first consolidation chemotherapy protocol was started.He died of septic shock on the eighteenth day of the first consolidation treatment. Conclusion: Cases of DS-ALL cases are at greater risk for serious side effects from chemotherapeutics,mortality and recurrence than non DS-ALL.Because children with DS have a higher incidence of treatment-related toxicity,survival rates are lower than non-DS children.During ALL induction chemotherapy life-threatening side effects are tumor lysis syndrome,thrombosis,bleeding and infection.In the UKALL 2003 study,DS associated with a significantly increased risk of death from sepsis during chemotherapy.
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by accumulation of ultra-large von Willebrand factor (vWF) due to the significantly reduced activity ...ADAMTS13. Limited studies have been published examining the blood group as an epidemiological factor that can contribute to development of TTP. It has been suggested that due to low vWF levels, the distribution of the “O” blood group among TTP patients may be lower than anticipated compared to the blood group distribution rates in the normal population. The aim of this study was to explore the relationship between blood groups and the clinical outcome of immune TTP (iTTP). Methods: Thirty patients with iTTP with severe ADAMTS13 deficiency were enrolled. Data collection commenced in January 2011 and was completed by June 2020. It was analyzed whether there was a difference between the blood groups in terms of frequency of iTTP, response to treatment, frequency of relapse, and clinical and laboratory results. Results and Conclusions: The distribution of group “A” among patients with iTTP was higher than expected. Although not statistically significant, patients with blood group “O” required more TPE for the treatment and relapse rate was statistically higher than other blood groups. Mortality rate in all patients was 6.7%. Although blood group “A” is a risk factor for iTTP, the frequency of relapse is higher in the blood group “O.”
The scoring system used for chronic lymphocytic leukemia (CLL) cannot make an accurate diagnosis in some cases. Novel markers are available for the differential diagnosis of CLL, especially from MCL. ...However, these markers are still not incorporated into diagnostic algorithms. We investigated the role of CD43, CD81, CD200, and ROR1 in the differential diagnosis of CLL and their expression in non-CLL cases.
We investigated the role of CD43, CD81, CD20, and ROR1 in the differential diagnosis of CLL by incorporating them into the diagnostic panel after studying peripheral blood or bone marrow samples of 165 patients with 8-color flow cytometry.
CD43 positivity was a sensitive marker but had a lower specificity for CLL. CD43 had high diagnostic value for CLL (sensitivity 100%, specificity 88.5%, AUC 98.0%). CD200 was a specific marker for CLL (sensitivity 98%, specificity 90%, AUC: 96%). CD81 expression was highest in the MCL cases, with a median expression rate of 68.5% (range: 54 - 82.5%). It was negative in all the CLL cases. For CLL, CD81 negativity had a sensitivity of 95%, a specificity of 82% and an AUC of 92%. ROR1 was positive in all CLL and MCL cases. CD79b, on the other hand, was a fairly sensitive and specific marker for MCL.
CD43, CD81, CD200, and ROR1 should be incorporated into diagnostic algorithms for the differential diagnosis of CLL, especially from MCL.
Abstract
Objectives
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.
Methods
One hundred and thirty-three subjects with normal physical examination and complete blood count results within the reference ranges were included in the study. Venous blood samples collected in tubes containing K
2
-EDTA were analyzed on Sysmex XN-1000. The references intervals for IRF, RBC He, Ret He, LFR, MFR, HFR, Delta He, Micro R, Macro R, Hypo He and Hyper He were determined according to CLSI EP28-A3c.
Results
The reference ranges of the parameters were estimated with 90% confidence intervals. The reference ranges were 3.4–17% for IRF, 26.9–32.8 pg for Ret-He, 25.2–30.5 pg for RBC-He and 0.5–3.7 pg for delta-He. Gender specific reference ranges were calculated for of Ret-He (male (M): 26.8–32.9 pg, female (F): 23.9–33.6 pg), RBC-He (M: 26.3–30.8 pg, F: 25.3–30.5 pg) and delta-He (M: 0.5–3.7 pg, F: 0.3–3.7 pg).
Conclusions
The new reticulocyte and erythrocyte parameters may be conveniently used in clinical diagnosis and follow-up of patients, as they offer reliable, automated and cheap results. Each laboratory is recommended to determine its own reference intervals considering the differences like the instrument used and population studied.
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