American journal of blood research最新文献

Methotrexate (MTX) is a commonly used anti-metabolite agent. Long-term MTX treatment can cause MTX-related lymphoproliferative disorder (MTX-LPD). T-cell LPDs comprise a small fraction of MTX-LPDs. Epstein-Barr virus (EBV)⁺ tumor cells are rarely detected in MTX-related T-cell LPDs (MTX T-LPDs). Therefore, there have been very few reports of EBV⁺ MTX T-LPD. We encountered a case of cutaneous MTX T-LPD with a unique cellular phenotype. The patient was a 71-year-old Japanese man with rheumatoid arthritis treated with MTX for 6 years. He was referred to our department with a 6-month history of red plaques and ulcerated lesions in both lower legs and a 2-week history of high fever and fatigue. Cutaneous specimens showed that medium-sized atypical lymphocytes were positive for CD3, CD4, CD30, CD56, and in situ hybridization for EBV-encoded RNA. The patient was diagnosed with cutaneous MTX T-LPD. Four months after discontinuation of MTX, the skin lesions had disappeared. This is the first report of cutaneous MTX T-LPD with CD4⁺CD30⁺CD56⁺EBV⁺ tumor cells.

In recent years the outcome of patients with multiple myeloma (MM) has significantly improved, due to new drugs. However, some agents, i.e. the alkylating drug melphalan, can be associated with an increased incidence of secondary malignancies. Myelodysplastic syndromes and acute myeloid leukemia are reported in the literature, and rarely acute lymphoblastic leukemia. Here we describe a unique case of a 56-years old female patient affected by MM since 2015 in complete remission after autologous stem cell transplant and in lenalidomide maintenance, who developed 2 years later mixed phenotype acute leukemia (MPAL). The patient, refractory to both lymphoblastic and myeloid acute leukemia regimens, achieved complete remission with bi-specific anti-CD19/anti-CD3 monoclonal antibody blinatumomab and with hypomethylating agent azacytidine plus the BCL-2 inhibitor venetoclax. She then underwent hematopoietic stem cell transplantation from HLA-identical sibling donor and she is still in complete remission after 9 months. To the best of our knowledge, there are no cases in the literature describing MPAL after autologous transplant for MM. Our patient was treated with blinatumomab and venetoclax and achieved complete remission 9 months from allogeneic transplant. The mechanism underlying the development of MPAL is not completely understood and therapies are still lacking. In this context the combination of blinatumomab, azacytidine and venetoclax successfully used in this patient may provide food for thought for further studies in this rare setting of patients.

Although isolated trisomy 9, a form of chromosome aneuploidy, is rare in acute myeloid leukemia (AML), up to 30 cases of AML involving isolated trisomy 9 have been reported to date. We report the case of a 77-year-old female with AML, in which trisomy 9 was detected as an isolated aberration. In addition, the patient's bone marrow displayed so-called sea-blue histiocytosis. The accumulation of further cases of isolated trisomy 9-harboring AML involving sea-blue histiocytosis is necessary to determine whether the coexistence of these findings is pathognomonic or a coincidence.

Relapsed/refractory diffuse large B-cell lymphoma (DLBCL) portends a poor prognosis, with an estimated overall survival of less than 6 months. In the presented case, a female patient with DLBCL refractory to multiple lines of therapy, including chimeric antigen receptor T-cells, was treated with single-agent selinexor, achieving partial response following 5 months of treatment, which allowed the patient to proceed to potentially curative allogeneic stem cell transplantion. This approach enabled the patient, who would otherwise have been considered a candidate for palliative care, to achieve the most prolonged complete response since her first lymphoma-specific treatment. This outcome implies that early identification of relapsed/refractory patients who may benefit most from this drug - either as a single agent or in drug combinations - is imperative.

Background: Adenosine 5'-triphosphate (ATP) is the most direct source of energy in organisms. Recently, it is evident that ATP plays an essential role in the immune and inflammatory systems. However, ATP is unstable when it exposed to room temperature in vitro. Therefore, our article is aim to explore the stability of ATP.

Methods and results: 28 samples of ATP were detected. Student's t test or one-way ANOVA was used to compare multiple groups. It shows that during the storage process from day 1 to day 70, the overall levels tend to decrease.

Conclusion: The level of ATP does not reduce at least in the first month when stored at -80°C. On the 70th day, there was a star drop, and the levels were lower than before.

Introduction: The inflammatory and immune cells have an important impact on Hodgkin lymphoma (HL). The derived neutrophil-lymphocyte ratio (dNLR) has been confirmed to have a similar prognostic value as the neutrophil-lymphocyte ratio (NLR) in many kinds of tumors, but it has not been explored as a prognostic marker for Hodgkin lymphoma patients.

Objective: The aim of the study is to evaluate the prognostic value of dNLR and NLR in HL.

Methods: This retrospective study included 213 newly diagnosed HL patients from 2008 to 2019. Then, the prognostic significance of dNLR and NLR in these patients was evaluated. Meanwhile, subgroup analyses based on the Ann Arbor stage and histotype were also carried out. Finally, propensity score matching was used to reduce selection bias.

Results: Patients with dNLR ≥ 2.1 showed shorter overall survival (OS) (P = 0.006). Also, patients with NLR ≥ 3.0 showed worse OS (P = 0.005) and progression-free survival (PFS) (P = 0.031). These results were also found in patients with early-stage and mixed cellularity subtype HL. Besides, high dNLR represented an independent prognostic marker for OS and high NLR remained an independent prognostic factor for OS and PFS on multivariable analysis.

Conclusion: Elevated dNLR and NLR were related to worse survival in HL patients. For the first time, the dNLR has shown the potential to be a new prognostic factor for patients with HL.

Background: There is conflicting data in the literature about the association of ABO blood type and susceptibility to COVID-19 infection. Moreover, very few studies have examined the effect of blood type on severity of COVID-19 infection.

Methods: This was a retrospective, single-center analysis of adult patients with COVID-19 infection who were hospitalized between March 8^th to July 31^st, 2020 at a regional tertiary care hospital. All patients who were hospitalized with a diagnosis of severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection and had a documented ABO blood type were enrolled in this analysis. Aims of this study were to examine the prevalence of ABO blood types in patients with COVID-19 infection and to determine the frequency of severe COVID-19 infection among ABO blood types.

Results: A total of 227 cases were identified. Our cohort had a mean age of 63.3 years and 60% were males. The most common blood type was O (49%) followed by A (36%), which was similar to the prevalence of ABO blood types in our regional population. Moreover, there was no significant difference in the frequency of severe COVID-19 infection between ABO blood types (O: 50%, A: 53%, B: 56%, AB: 57%; P=0.93), or any additional outcomes including in-hospital mortality rate (P=0.72), need for ICU admission (P=0.66), ICU free days at day 28 (P=0.51), hospital free days at day 28 (P=0.43), or need for acute renal replacement therapy (P=0.09).

Conclusion: We did not find an increased susceptibility of any blood type to COVID-19 infection, nor was there an increased risk of severe COVID-19 infection in any ABO blood types.

Healthcare workers (HCWs) due to their job profile are at utmost risk of contracting severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Serological survey is an useful tool for vulnerability mapping in an infectious disease pandemic. The aim of the current study was to assess seroprevalence of IgG against SARS-CoV-2 and its determinants among HCWs of a tertiary healthcare facility of India. It was an observational study, cross-sectional in design conducted among 919 HCWs of All India Institute of Medical Sciences, Patna, Bihar, India during September, 2020. In results, IgG seroprevalence for SARS-CoV-2 among the study subjects was 13.3% [95% confidence interval (CI): 11.2-15.6%]. In univariate logistic regression analysis; gender, occupation, place of posting, use of full personal protective equipment (PPE), prior corona virus disease (COVID)-19 infection, influenza like illness (ILI), use of steam inhalation, consumption of azithromycin, zinc and vitamin C were the significant attributes which affected the IgG seropositivity for SARS-CoV-2. In the multivariable logistic regression model; occupation, place of posting, prior COVID-19 infection and ILI were significant determinants of IgG seropositivity for SARS-CoV-2. To conclude, majority of the HCWs were found to be IgG seronegative for SARS-CoV-2. Till availability of effective vaccine all of the HCWs should abide by infection prevention and control (IPC) measures to keep themselves and their contacts protected from SARS-CoV-2.

Juvenile myelomonocytic leukemia (JMML) is a rare pediatric myelodysplastic/myeloproliferative neoplasm overlap disease. JMML is associated with mutations in the RAS pathway genes resulting in the myeloid progenitors being sensitive to granulocyte monocyte colony-stimulating factor (GM-CSF). Karyotype abnormalities and additional epigenetic alterations can also be found in JMML. Neurofibromatosis and Noonan's syndrome have a predisposition for JMML. In a few patients, the RAS genes (NRAS, KRAS, and PTPN11) are mutated at the germline and this usually results in a transient myeloproliferative disorder with a good prognosis. JMML with somatic RAS mutation behaves aggressively. JMML presents with cytopenias and leukemic infiltration into organs. The laboratory findings include hyperleukocytosis, monocytosis, increased hemoglobin-F levels, and circulating myeloid precursors. The blast cells in the peripheral blood/bone-marrow aspirate are less than 20% and the absence of the BCR-ABL translocation helps to differentiate from chronic myeloid leukemia. JMML should be differentiated from immunodeficiencies, viral infections, intrauterine infections, hemophagolymphohistiocytosis, other myeloproliferative disorders, and leukemias. Chemotherapy is employed as a bridge to HSCT, except in few with less aggressive disease, in which chemotherapy alone can result in long term remission. Azacitidine has shown promise as a single agent to stabilize the disease. The prognosis of JMML is poor with about 50% of patients surviving after an allogeneic hematopoietic stem cell transplant (HSCT). Allogeneic HSCT is the only known cure for JMML to date. Myeloablative conditioning is most commonly used with graft versus host disease (GVHD) prophylaxis tailored to the aggressiveness of the disease. Relapses are common even after HSCT and a second HSCT can salvage a third of these patients. Novel options in the treatment of JMML e.g., hypomethylating agents, MEK inhibitors, JAK inhibitors, tyrosine kinase inhibitors, etc. are being explored.

Among acute lymphoblastic leukemia (ALL), 40% of affected patients are diagnosed after the age of 20. Compared to pediatricians, adult hemato-oncologists are less familiar with complex pediatric ALL regimens and have perceived that pediatric ALL regimens are too toxic in the adult population. Meanwhile, multiple retrospective analyzes showed the superiority of pediatric regimens among the older adults and young adolescents (AYAs) group over adult regimens. A series of prospective studies have made it apparent that pediatric-inspired ALL regimens are feasible in AYAs, with manageable toxicities and potentially more encouraging results. However, the complications in the adult population are still to be explored. Although cytomegalovirus (CMV) viremia and infections are increasingly recognized in pediatric ALL cases, we generally do not experience it frequently in adult cases with conventional strategies. Herein we represent a 38-year-old man diagnosed with ALL and treated with pediatric inspired GRAALL-2003 protocol. Following a successful induction phase, he had pancytopenia, deep lymphopenia, fever and diarrhea in the 9^th month of maintenance therapy. With increased serum ferritin and triglyceride levels, he had features of macrophage activation syndrome. The bone marrow biopsy did not reveal any relapse or hemophagocytosis. We detected highly increased levels of CMV DNA (657.262 copies/mL) in blood analysis.