American journal of blood research最新文献

Introduction: Coronary artery ectasia (CAE) is defined as localized or diffuse dilatation of the epicardial coronary arteries. We aimed to elucidate the relationship between the CHA₂DS₂-VASc score and CAE.

Methods: The study population consisted of 122 patients with isolated CAE and 87 sex- and age-matched control subjects. The demographic, clinical, and laboratory profiles and CHA₂DS₂-VASc scores of patients with CAE and the control group were compared. The Markis classification was used to determine the extent of CAE. Coronary arteries in which ectasia was localized were identified. CHA₂DS₂-VASc scores were calculated for all patients. Parameters predicting the development of CAE were analyzed with multivariate logistic regression.

Results: The majority of patients with CAE were male (76, 62%) and their mean age was 58.4 ± 8.3. The CHA₂DS₂-VASc score of the CAE group was significantly higher than that of the control group (2.41 ± 1.12 vs 1.52 ± 0.73, P < 0.001). Multivariate regression analysis showed that the CHA₂DS₂-VASc score (odds ratio [OR] = 1.607, P = 0.004), left ventricular ejection fraction (OR = 0.953, P = 0.044), uric acid (OR = 1.569, P = 0.003), white blood cell count (OR = 1.001, P < 0.001), highly sensitive C-reactive protein level (OR = 1.115, P = 0.010), and smoking (OR = 2.019, P = 0.043) were independent predictors of CAE.

Conclusion: High CHA₂DS₂-VASc scores were associated with isolated CAE; therefore, the score might be a useful predictor of coronary thrombus development in patients with isolated CAE.

Introduction: Acute promyelocytic leukemia (APML), although genetically and morphologically distinct from other AML (acute myeloid leukemia) subtypes, is one of the most best responsive acute myeloid leukemia. -Conventional diagnostic methods and morphological hints often fail in the majority of the cases in the peripheral laboratories owing to resource constraints, unavailability of cytogenetic work-up, hypogranular variants, morphological mimicry by AML-monocytic and myelo-monocytic, etc. Flowcytometry (FCM), however, can be utilized as a feasible and reliable immunophenotypic diagnostic and prognostic tool for prompt identification of APML. In order to rapidly and sensitively diagnose APML we intended to suggest a cost effective, sensitive FCM panel and also to prognositicate patients.

Material and methods: In this retrospective study, flowcytometry characteristics of 123 cases of acute promyelocytic leukemia were studied including 40 hypogranular variants. The expression of markers was compared with the Mean flurescent Intensity (MFI) and percent expression of markers. A non-statistical comparison was made with cases of acute monocytic leukemia. The cases were grouped according to their immunophenotype characteristics and expression with comparison of MFI by multivariate logistic regression. The aberrant markers positive at diagnostic and remission flow test were compared with the survival outcomes, and their positive predictive values were calculated.

Results: The most common feature of side scatter property was the absence of blasts in the window and high side scatter, except hypogranular variants which had low side scatter. Immunophenotypically characterised by positivity for CD117, cMPO, and bright CD33 and CD13 positivity and lack of CD34 and HLA-DR was seen in the majority of APML including hypo-granular variant. We suggest a rapid diagnostic four-tube panel for fast and rapid diagnosis of APML, including hypogranular variants with 100% sensitivity. The study also identified six groups of immunophenotypes with significant prediction values of APML, including hypogranular variants. The study also highlights CD2, CD56, and CD9 as prognostic markers for acute promyelocytic leukemia.

Background: Anaemia is common worldwide and pregnant women are one of the most vulnerable group. Although, anaemia in the general population including pregnant women is multi-factorial in aetiology, the most frequent cause in pregnancy worldwide is iron deficiency. In Nigeria, an estimated prevalence of anaemia among pregnant women ranges from 35-75%. Anaemia in pregnancy (AIP) is associated with significant perinatal and maternal morbidity and mortality including premature birth and low birth weight.

Aim: The aim of this study was to determine the prevalence, demographic and socio-economic determinants of anaemia in pregnancy in a rural community of South-West Nigeria.

Materials and methods: One-hundred and fifty consenting pregnant women aged 18-42 years in the three trimesters were recruited from four primary health centres of Ikene Local Government of Ogun State of Nigeria after ethical approval was obtained from the Ethics Unit of the Medical officer of Health of the Local Government. Pre-tested interviewer-administered questionnaire was used to collect data on socio-demographic information and 24-hour dietary recall. Using a finger prick, the haemoglobin concentration of each respondent was determined with a haemoglobinometer (DG-300HB manufactured by DouBle, China). Data was analyzed using the Statistical Package for Social Sciences (SPSS) version 20.

Results: All the respondents belonged to low socio-economic class. The mean haemoglobin (Hb) concentration obtained in this study was 10.22±1.60 g/dL with a range of 6-14.8 g/dL. Using WHO cut-off Hb concentration of 11 g/dL, the prevalence of anaemia in this study was 67.3%. The frequency of anaemia increased with increase in age group. P=0.010. About 21.4% of those with adequate dietary iron intake were anaemic when compared with 72.1% (98 of 136) of those with inadequate dietary iron intake who were anaemic. AOR-0.090; 95% CI- 0.018-0.457; P=0.004.

Conclusion: Increasing age, low socio-economic status, poor health education and low dietary iron intake were the predominant socio-economic determinants of prenatal anaemia in the population studied. Efforts must be intensified to alleviate poverty in rural areas and give health education on iron-rich foods to girls and women of children-bearing age in the rural communities.

Background: Pediatric patients with hematological malignancy and bone marrow failure syndrome receive multiple transfusions before diagnosis and treatment. Iron overload leads to damage to vital organs like the heart, liver, thyroid, Gonads, Pancreas.

Materials and methods: A prospective study was done from June 2017-December 2019 in a tertiary care pediatric hematology oncology unit in northern India on children diagnosed with hematological malignancy and bone marrow failure syndromes receiving packed cell transfusion. After due ethical considerations and patient consent, the details were documented in predesigned proforma. All cases were planned to be investigated with Liver function test, Thyroid function test, Serum ferritin level, 2 D Echocardiography, Ultrasonography of abdomen, and MRI of the abdomen at admission and six months of enrollment.

Results: Out of 58 cases enrolled, ferritin levels were high in 65% of subjects at the start of treatment and 76% at the endpoint. Mean ferritin level was 725 ng/ml at baseline and 1268 ng/ml end of 6 month follow up period. Fifty-seven percent had a ferritin level above 1000 ng/ml, which correlated to basal ferritin level (P-value 0.005). The final ferritin level correlated strongly with the final number of packed cell transfusions (P-value 0.0002). Functional derangement of the liver was evident biochemically in 13.7% before starting treatment and 31.8% at six months follow-up period. Echocardiography detected diastolic dysfunction in 2% of patients at baseline before starting treatment and increased to 22% in 6 months follow-up period. The percentage of subclinical hypothyroidism increased from 22.8% to 48.8% during treatment.

Conclusion: Like transfusion-dependent anemias, children with hematological malignancy and bone marrow failure syndrome on chronic transfusion are at risk of transfusion-related iron overload and organ damage.

Anaemia among non-pregnant females of reproductive age remains a common public health problem globally, as well as in Sri Lanka. The objective of the study was to determine the prevalence of anaemia, asses the knowledge and the associated factors of anaemia among non-pregnant females of reproductive age in a tea estate community in Hantana, Kandy district, Sri Lanka. A descriptive cross-sectional study was conducted among 236 randomly selected non-pregnant females of reproductive age within the tea estate community belonging to two MOH (Medical Officers of Health) areas. The proportion of anaemia was determined by measuring haemoglobin (Hb) concentration using Mindray five-part automated blood analyser. The cut-off value to determine anaemia was set at 12.0 g/dL and respondents were categorized into three anaemia categories based on their Hb value. The common risk factors and knowledge regarding anaemia were assessed using a pre-tested interviewer administered questionnaire. Data was analysed with SPSS version 25. Chi-square test was used to conduct a bi-variate analysis. Prevalence of anaemia was 33.1%, among whom 53.8% had mild anaemia, 39.7% had moderate anaemia and 6.4% had severe anaemia. Anaemia was significantly associated with being employed, delivery of a baby within the past 4 years, advancing age, low income (less than 20,000 LKR) and prolonged menstrual bleeding for more than 3 days. Majority (58.5%) had poor knowledge regarding anaemia with a mean score of 5.69 (SD ± 2.42) out of 12. Since anaemia is a multifactorial condition it requires a combination of interventions such as health education and promotion activities. This study aids in assessing the prevalence of anaemia among estate workers identify the significant factors contributing to anaemia.

Background: Immune Thrombocytopenia (ITP) is characterized by low platelet counts. Splenectomy has been in practice for the treatment of ITP since the early 20th century. We aimed to analyze the data of ITP patients from our hospital who underwent splenectomy and further present the long-term outcome and safety profile in these patients.

Method: This study was a single-center, registry based study conducted at a tertiary care hospital in Northern India. Patients aged 18 years or more, who underwent splenectomy after at least one line of therapy, were included in the study. The primary outcome was the overall response rate (ORR) at one month after splenectomy. Secondary outcomes were sustained response, relapse-free survival, factors affecting the ORR, and adverse events after splenectomy.

Results: Forty-five patients of ITP were included in the study. Thirty-six patients underwent splenectomy in the first half (2001-2010), of the study period. The median age of the patients was 38 (19-56) years. The median duration from diagnosis to splenectomy was 1.76 (0.47-2.58) years. The median number of therapy received before splenectomy was 3 (1-6). The overall response rate (ORR) post-splenectomy at day 30 was 89.2% with 61.8% complete response (CR). The ORR was 88.5% at 1-year, with 48.8% CR. The relapse-free survival (RFS) at 5-years was 57.38% (95% Confidence Interval 40.59-71.02%), There was no effect of duration of disease, age, gender, and prior therapy received, on the ORR at one-month. At one year, the platelet response was significantly better in patients who had a CR at one-month than patients who had a partial response at one month. The relapse-free survival was better in patients who achieved CR after 1-month of splenectomy. During the median follow-up of 5.02 (1 month-20 years) years, there were five cases of overwhelming post-splenectomy infection (OPSI). There was no recorded incidence of perioperative mortality, deep vein thrombosis, or mesenteric thrombosis.

Discussion: Despite the variation in outcome from different studies, splenectomy gives the best possible long-term treatment-free remission amongst all the available second-line agents. It is also, one of the most financially affordable therapies. Despite advantages, the number of ITP patients undergoing splenectomy has been on the decline and largely attributable to the newer and more effective second-line therapies. There is no pre-surgery variable predicting the ORR after splenectomy.

Conclusion: Splenectomy in ITP offers a long-term sustained response at an economical cost.

Acute myeloid leukemia (AML) is a rapidly progressive hematological malignancy that is difficult to cure. The prognosis is poor and treatment options are limited in case of relapse. A comprehensive assessment of current disease burden and the clinical efficacy of non-intensive therapies in this population are lacking. We conducted two systematic literature reviews (SLRs). The first SLR (disease burden) included observational studies reporting the incidence and economic and humanistic burden of relapsed/refractory (RR) AML. The second SLR (clinical efficacy) included clinical trials (phase II or later) reporting remission rates (complete remission [CR] or CR with incomplete hematologic recovery [CRi]) and median overall survival (mOS) in patients with RR AML or patients with de novo AML who are ineligible for intensive chemotherapy. For both SLRs, MEDLINE^®/Embase^® were searched from January 1, 2008 to January 31, 2020. Clinical trial registries were also searched for the clinical efficacy SLR. After screening, two independent reviewers determined the eligibility for inclusion in the SLRs based on full-text articles. The disease burden SLR identified 130 observational studies. The median cumulative incidence of relapse was 29.4% after stem cell transplant and 46.8% after induction chemotherapy. Total per-patient-per-month costs were $28,148-$29,322; costs and health care resource use were typically higher for RR versus non-RR patients. Patients with RR AML had worse health-related quality of life (HRQoL) scores than patients with de novo AML across multiple instruments, and lower health utility values versus other AML health states (i.e. newly diagnosed, remission, consolidation, and maintenance therapy). The clinical efficacy SLR identified 50 trials (66 total trial arms). CR/CRi rates and mOS have remained relatively stable and low over the last 2 decades. Across all arms, the median rate of CR/CRi was 18.3% and mOS was 6.2 months. In conclusion, a substantial proportion of patients with AML will develop RR AML, which is associated with significant humanistic and economic burden. Existing treatments offer limited efficacy, highlighting the need for more effective non-intensive treatment options.

Coronavirus disease 2019 (COVID-19) has led to a global pandemic that has also challenged the management of various other life-threatening conditions, such as malignant disorders. In this study, we present the clinical features and treatment outcomes of twenty-seven COVID-19 positive patients with leukemia across seven different centers in Istanbul. From March 1st to December 31st 2020, 116 patients were diagnosed with acute leukemia. Thirty-two cases with acute lymphocytic leukemia (ALL), 82 cases with acute myeloid leukemia (AML), and 2 cases with mixed phenotype acute leukemia (MPAL) were identified. Of the 27 patients with the COVID-19 infection, seven patients had ALL, 19 patients had AML and one patient had MPAL. The mortality rate was 37% among the patients with AML, whereas there were no deaths in the ALL group. The mortality rate of AML patients with the COVID-19 infection was higher compared to cases without the infection (P<0.05). We could not detect any significant difference in the ALL cohort. This study, which includes one of the largest acute leukemia series in literature proved that acute myeloid leukemia patients with the COVID-19 infection have worse outcomes than patients without the infection. The high mortality among patients with acute leukemias hospitalized with COVID-19 highlight the need for aggressive infection prevention, increased surveillance and protective isolation and even modification of the therapy, in case of minimal residual disease (MRD) negativity.

Background: Relapsed or refractory (R/R) mantle-cell lymphoma (MCL) patients have a poor prognosis and their management is challenging, in absence of a golden standard as salvage treatment. Bruton's tyrosine kinase inhibitor ibrutinib represents an effective treatment for R/R MCL patients. We investigated ibrutinib efficacy and safety in daily clinical practice, together with factors that could predict disease outcome.

Patients and methods: We retrospectively analyzed 69 consecutive R/R MCL patients managed in 10 Tuscan onco-hematological centers. The treatment regimen consisted of oral, continuous, single-agent ibrutinib, maximum dosage of 560 mg once per day, until disease progression.

Results: Overall response rate was 62.3%, with a CR rate of 39.1%. After a median follow-up of 15.6 months, 40/69 patients (58%) were alive, the main cause of death was progressive disease (PD, 22/69 cases, 31.9%). Median progression-free survival (PFS) and overall survival (OS) were 17 and 34.8 months. Inferior PFS was associated with >1 prior line of therapy and B symptoms. Ibrutinib refractoriness was associated with inferior OS, median OS after ibrutinib failure was only 5 months.

Discussion and conclusion: In this real-life setting ibrutinib treatment prolonged survival in R/R MCL patients, without unexpected adverse events. Patients receiving ibrutinib as 2^nd line regimen had the most favorable outcome.

Background: Graves' disease (GD) has been associated with iron deficiency anemia (IDA). Atrophic gastritis leads to IDA and has been associated with autoimmune thyroid disease. This study prospectively determined the prevalence of atrophic gastritis markers and the relationship between these markers and markers of IDA in GD subjects.

Methods: Newly diagnosed GD patients (90) and controls (41) were studied. Of the newly diagnosed GD patients, 65 were consecutively enrolled and identified with GD irrespective of anemia, 25 had GD and IDA. Thyroid function, hematologic indices, and atrophic gastritis markers [parietal-cell antibodies (PCab), Helicobacter pylori antibodies (H. pylori ab), mean serum gastrin levels] were examined.

Results: GD patients presenting with IDA were twice as likely (64% vs. 32%, P=0.049) to harbor PCabs when compared to all other GD subjects. Unselected GD subjects (n=65) had significantly higher PCab (37% vs. 7%, P<0.001) compared to controls. Gastrin levels were significantly elevated in all GD subjects compared to controls (105 vs. 39 pg/ml, P<0.0001). This difference was magnified in PCab+ subjects (202 vs. 64 pg/ml, P=0.003). In all GD subjects, PCabs were associated with increased gastrin levels (202 vs. 75 pg/ml, P=0.0004) and lower ferritin levels (52 vs. 95, P=0.05). In GD anemic subjects, PCabs were associated with lower mean corpuscular volume (75 vs. 81, P=0.001). Gastrin levels correlated inversely with ferritin levels in all GD subjects and positively with TIBC in GD anemic subjects.

Conclusions: A significant subset of patients presenting with GD may suffer from IDA due to concurrent autoimmune atrophic gastritis.