Hematology, transfusion and cell therapy最新文献

Background: T315I mutations of the BCR::ABL1 gene lead to resistance to tyrosine kinase inhibitors (TKIs). This study evaluated the performance of digital droplet polymerase chain reaction (ddPCR) in quantifying T315I mutations and their frequency in Philadelphia chromosome (Ph) positive hematological patients.

Methods: The course of disease and BCR::ABL1 fusion transcripts (e13a2, e14a2 and e1a2) were retrospectively reviewed in 21 patients with acute lymphoblastic leukemia (ALL) and 85 patients with chronic myeloid leukemia (CML). T315I mutation analysis was carried out using ddPCR and the limit of detection was assessed using mutant T315I DNA at varying variant allele fractions.

Results: T315I mutations were found in two ALL patients and one CML patient without remission in molecular biology and with mutation burdens of 29.20%, 40.85%, and 3.00%, respectively. The mutation burden of ALL patients was higher than that of CML patients, but there was no significant difference between the two (p-value = 0.0536). The test's limit of detection was 0.02% with a correlation coefficient greater than 0.99 between the expected and actual detection abundances.

Conclusion: T315I mutations have a high incidence in Ph-positive ALL patients even if the course of disease is short. In molecular biology, T315I mutation detection is indicated for CML patients not in remission.

Introduction: An intracellular parasite of mononuclear phagocytes, mainly distributed in the bone marrow and the spleen, causes visceral leishmaniasis. Complete blood count (CBC) reveals the poorly understood pathogenesis of anemia, leukopenia and thrombocytopenia. Our study aimed to compare the CBC with bone marrow cytomorphological features and their association with clinical outcomes to clarify this relevant issue.

Methods: The CBC and bone marrow of 118 patients were described by two hematologists and compared to check their association with each other and mortality.

Results: Peripheral cytopenias were common findings, particularly anemia, as seen in almost all patients. No relationship was found between values of hemoglobin, neutrophils and platelet count with fatal outcomes. The bone marrow was normocellular in 61.9% of the cases. Dysplasia figures were frequent and 49.1% of the samples had dysgranulopoiesis. Additionally, erythroid hyperplasia was found in 72% of the patients with severe anemia. Patients with reduced bone marrow cellularity, erythroid hypercellularity and dyserythropoiesis seem to have a riskier disease.

Conclusion: The study results suggest that the bone marrow of patients with visceral leishmaniasis manifests a reactional pattern to the inflammatory event, thereby modulating cytokines and other colony growth factors. This compensatory response may be dysplastic and ineffective and generate peripheral cytopenias of varying intensity. Further studies are needed to clarify the signaling pathways involved, which may be used as therapeutic tools in the future.

Objectives: To assess the prognostic value of C-Reactive Protein (CRP), at diagnosis and during follow-up, of patients with Hodgkin´s Lymphoma treated at the Hematology Service of the Santa Casa de São Paulo Hospital, and to correlate serum CRP levels with disease stage and treatment response.

Methods: A retrospective study involving review of 71 medical records of patients diagnosed with Hodgkin´s Lymphoma between February 2012 and January 2016 was performed. Three patients were subsequently excluded, giving a total of 68 patients for analysis. A level of CRP > 1mg/dl was considered elevated.

Results: Patients were predominantly male (61.8%) and mean age was 34 years. Fifty-three (78%) patients had advanced stage and (76.5%) had B symptoms. Elevated baseline CRP was associated with greater likelihood of B symptoms (p= 0.02) and of advanced stage (p= 0.015). Patients with Low CRP level after 5th and 6th cycles of chemotherapy was associated with complete response (p=0.04 and p=0.03, respectively). Treatment-refractory patients had greater risk of death (p=0.002).

Conclusion: CRP is clinically important for follow-up of patients with Hodgkin´s Lymphoma, where high levels were associated with advanced disease and/or presence of B symptoms. CRP level was considered a predictor of treatment response. Persistence of high CRP values during treatment was associated with refractoriness.

Introduction: Hematopoietic stem cell transplantation (HSCT) is a widely used therapy, but its success largely depends on the number and quality of stem cells collected. Current evidence shows the complexity of the hematopoietic system, which implies that, in the quality assurance of the apheresis product, the hematopoietic stem cells are adequately characterized and quantified, in which mass cytometry (MC) can provide its advantages in high-dimensional analysis.

Objective: This research aimed to characterize and enumerate CD45^dim/CD34⁺ stem cells using the MC in apheresis product yields from patients with chronic lymphoid malignant diseases undergoing autologous transplantation at the Abu Dhabi Stem Cells Center.

Methods: An analytical and cross-sectional study was performed on 31 apheresis products from 15 patients diagnosed with multiple myeloma (n = 9) and non-Hodgkin lymphomas (n = 6) eligible for HSCT. The MC was employed using the MaxPar Kit for stem cell immunophenotyping. The analysis was performed manually in the Kaluza and unsupervised by machine learning in Cytobank Premium.

Results: An excellent agreement was found between mass and flow cytometry for the relative and absolute counts of CD45^dim/CD34⁺ cells (Bland-Altman bias: -0.029 and -64, respectively), seven subpopulations were phenotyped and no lineage bias was detected for any of the methods used in the pool of collected cells. A CD34+/CD38+/CD138+ population was seen in the analyses performed on four patients with multiple myeloma.

Conclusions: The MC helps to characterize subpopulations of stem cells in apheresis products. It also allows cell quantification by double platform. Unsupervised analysis allows results completion and validation of the manual strategy. The proposed methodology can be extended to apheresis products for purposes other than HSCT.