Laboratory evaluation of the Sysmex SE-9500 automated haematology analyser.

The Sysmex SE-9c500 is a new, fully automated haematology analyser, providing a complete blood count (CBC), including a five-part differential count (DC), with flagging of morphological abnormalities. The SE-9500 was evaluated according to guidelines published by the International Committee for Standardisation in Haematology (ICSH). The results demonstrated minimal carryover (< 0.01%) and excellent linearity for WBC, RBC, HGB and platelet (PLT) (r > 0.995). Samples were stable with regard to CBC parameters after storage for up to 48 h at room temperature (RT) and 4 degrees C. Imprecision was generally acceptable for all CBC parameters (CV < 5%). Correlation between the SE-9500 and reference methods was excellent (r > 0.97) for all the major CBC parameters (WBC, RBC, HGB, PLT). There was minimal interference for WBC, RBC, HGB and PLT at high concentrations of bilirubin (BIL=224 micromol/l) or triglyceride (TG=7.78 mmol/l). SE-9500 reference values for CBC parameters are presented. Our results indicate that the SE-9500 is an excellent tool for routine haematological examination.     

Performance evaluation of BC-3200 hematology analyzer in a university hospital

The BC-3200 automated hematology analyzer was evaluated and compared with the Beckman-Coulter AcT (Ac.T diff 2) 3-part differential hematology analyzer. The BC-3200 was evaluated according to guidelines published by the International Committee for Standardization in Hematology (ICSH), Clinical and Laboratory Standards Institute (CLSI), and Department of Food and Drug Administration (FDA). The results demonstrated no background, minimal carryover (<0.5%), and excellent linearity for hemoglobin (Hb) level, white blood cell (WBC), red blood cell (RBC), and platelet (PLT) counts (>0.998). Precision was generally acceptable for all complete blood count (CBC) parameters; coefficients of variation (CVs) were within the manufacturer's claims and CVs of CBC parameters, including WBC, RBC and PLT counts, Hb and mean corpuscular volume, were <6%. Correlation between the BC-3200 and Ac.T diff 2 was excellent (r > 0.98) for all major CBC parameters (WBC, RBC, and PLT counts and Hb). We conclude that the overall performance of the BC-3200 is excellent and compares well with that of the Coulter Ac.T diff 2.     

Performance evaluation of Abbott CELL‐DYN Ruby for routine use

CELL‐DYN^® Ruby? is a new automated hematology analyzer suitable for routine use in small laboratories and as a back‐up or emergency analyzer in medium‐ to high‐volume laboratories. The analyzer was evaluated by comparing the results from the CELL‐DYN^® Ruby? with the results obtained from CELL‐DYN^® Sapphire?. Precision, linearity, and carryover between patient samples were also assessed. Precision was good at all levels for the routine cell blood count (CBC) parameters, CV% being ≤2.6, except for platelet count (PLT) at the low level with CV% of ≤6.9%. The CV% for reticulocyte percentage was highest at the low level (10.4). In a comparative study, the CELL‐DYN Ruby results showed a good correlation (R² ≥ 0.98) with CELL‐DYN Sapphire for the CBC parameters. For the absolute reticulocyte count, R² was 0.82. In the white blood cell (WBC) differentials, the between‐days precision was good for all parameters (CV%: ≤8.0), except basophil percentage and absolute basophil count (CV%: ≤31.8 and 31.3, respectively). Additionally, in the commercial control sample with the low WBC count (2.5 × 10⁹/l), the precisions in the WBC differentials were ≤16.9%. The cell types that occur in low numbers showed higher CVs, as expected. The methodological comparison of the WBC differential parameters of neutrophils, lymphocytes, and eosinophils showed excellent correlations (R² ≥ 0.97), and the correlation coefficient for absolute monocyte count and monocyte percentage were 0.91 and 0.87, respectively. For absolute basophil count and basophil percentage the correlations were weaker (R² = 0.46 and 0.34, respectively). Carryover was minimal for all the parameters studied. The linearities of WBC, red blood cell, PLTs, and hemoglobin were acceptable within the tested ranges. In conclusion, the results of the evaluation showed the performance of CELL‐DYN Ruby to be good.     

Determination of peripheral blood stem cells by the Sysmex SE‐9500

The Sysmex SE‐9500 automated haematology analyser provides an estimate of immature cells, referred to as ‘haematopoietic progenitor cells’ (HPC). The aim of this study was to evaluate the reliability and usefulness of the SE‐9500 HPC parameter as compared with the CD34 + cell count and to determine whether the HPC count was of value in predicting the optimal harvesting time for peripheral blood stem cells (PBSC). Studies were performed on 112 samples from 21 patients with haematological malignancies and 13 healthy donors undergoing progenitor cell mobilisation. Coefficients of variation for the HPC count were 30%, 23.8%, 12.4% and 8.3% respectively for samples with low (4 × 10⁶/l), medium (13 × 10⁶/l), high (250 × 10⁶/l) and very high (2413 × 10⁶/l) counts. There was good linearity for HPC measurement in both peripheral blood (PB) and purified CD34 + cell suspensions (r > 0.995), and no detectable carryover was observed. There was an acceptable correlation between HPC and CD34 + cell counts for PB samples (r=0.669) and for CD34 + cell suspensions (r=0.859). Analysis of purified CD34 + cells using the SE‐9500 HPC mode revealed that they appear both in the blast cell area and the immature granulocyte area of the analyser cell display. Quantitation of CD34 + cells and HPC during PBSC mobilisation showed good agreement between these parameters with regard to the optimal time for PBSC harvesting. These findings suggest that HPC counting with the Sysmex SE‐9500 may be clinically useful for optimising the timing of PBSC collection.     

Performance evaluation of the Z2 coulter counter for WBC and RBC counting

The Z2 Coulter Counter (Z2) is a new, single-channel semi-automated electronic counter using the Coulter principle, providing both count and concentration results of cells, as well as providing size distribution of the cell population. The Z2 was evaluated according to guidelines published by the International Committee for Standardisation in Haematology. The results demonstrated excellent linearity for WBC and RBC (r > 0.999). The Z2 showed excellent precision, with a within-run, between-run and overall coefficient of variance less than 2%. Carryover was generally acceptable (<1%). WBC and RBC were stable after blood storage for 48 h at 4 degrees C and room temperature. There was almost no interference for WBC, RBC at high concentrations of bilirubin (178.5 mumol/l) or triglyceride (14.8 mmol/l) (P > 0.05). The bias of coincidence correction in WBC and RBC counting with the Z2 was less than 1%. Correlation between the Z2 and Sysmex F-820 or SE-9500 was excellent (r > 0.996) for WBC and RBC counting. Our results indicate that the Z2 has good features of precision, accuracy, linearity and good comparison with other haematology analysers, and could be used as a calibrating instrument for WBC and RBC counting.     

Value of platelet count in the early diagnosis of nosocomial invasive fungal infections in premature infants

The aim of this study was to investigate the value of a platelet count (PLT) in the early diagnosis of nosocomial invasive fungal infections in premature infants. Based on clinical diagnosis combined with blood culture results, 72 premature infants of 5354 pediatric patients who were hospitalized in the neonatal ward of our hospital between September 2009 and February 2013 were diagnosed with nosocomial invasive fungal infections (fungal infection group). There were 58 premature infants diagnosed with bacterial infections during the same period (bacterial infection group). The control group included 74 premature infants without nosocomial infections who were hospitalized during the same period. Receiver operating characteristic (ROC) curves were used to analyze the sensitivity, specificity, and diagnostic efficacy of the PLT and white blood cell (WBC) counts and C-reactive protein (CRP) level in the diagnosis of fungal infections in premature infants. The risk factors for invasive fungal infections included birth weight < 2000 g, gestational age < 32 weeks, peripherally inserted central catheter (PICC), oxygen inhalation therapy, intravenous nutrition, and administration of antibiotics (p < 0.05). Compared with the control group, the WBC and PLT counts in the fungal infection group decreased in the early and acute stages of infection (p < 0.01), while the CRP level increased (p < 0.01). The PLT count in the bacterial infection group decreased in the early and acute stages of infection (p < 0.01) and the CRP level increased (p < 0.05). Moreover, the decrease in the PLT count in the fungal infection group was more significant than the bacterial infection group (p < 0.01) and the CRP level increased more in the fungal infection group in the early stage of infection (p < 0.01); however, there were no significant differences in the PLT count and CRP level between the fungal and bacterial infection groups in the acute stage of infection (p > 0.05). ROC curve analysis of the WBC and PLT counts and the CRP level in the early diagnosis of fungal infections showed that the area under the curve of the PLT count was 0.912 (95% confidence interval:0.863–0.961), thus indicating a high accuracy with a cutoff PLT count of 157.0 × 10⁹/L. The corresponding sensitivity and specificity were 77.8% and 94.6%, respectively. We conclude that the PLT count is a convenient, economical, and effective predictor of invasive fungal infections in premature infants and has potential in the early diagnosis of fungal infections.     

Initial performance evaluation of the UniCel® DxH 800 Coulter® cellular analysis system

The Beckman Coulter UniCel^® DxH 800 is a hematology analyzer incorporating new electronic and mechanical design with advanced algorithm technology to perform CBC, white blood cell (WBC) differential, nucleated red blood cell (NRBC), and reticulocyte analysis. Evaluation of this instrument was performed in our 800‐bed tertiary care hospital and specifically centered upon the correlation of WBC, NRBC, and platelet (PLT) enumeration when compared to a predicate analyzer, the Coulter^® LH 780, and flow cytometry (FCM) reference methods. Of particular interest were those samples with morphologically confirmed interference and extreme leukocytosis (evaluated with respect to red blood cell parameter correction). The sample set (n = 272) consisted of morphologically normal and hematologically abnormal patients. Correlation of the WBC, PLT, and NRBC showed r² values of 0.994, 0.985, and 0.910 for the DxH 800 vs. FCM, respectively. The presence of interfering particles did not affect the accuracy of the DxH 800 with respect to WBC counts. The DxH 800 showed accurate PLT and NRBC counts in the clinically significant low range when compared to FCM. Compared to the LH 780, flagging rates were significantly reduced (NRBC flag), or equivalent (WBC, PLT flag) on the DxH 800. The DxH 800 demonstrated higher sensitivity and specificity for PLTs and NRBCs and achieved a lower NRBC false negative rate compared to the LH 780. The UniCel^® DxH 800 represents a significant improvement to previous impedance analyzers in accurately detecting the presence of NRBCs at counts >1/100 WBC. Furthermore, it provides accurate PLT and WBC counts in the presence of interference and improved NRBC flagging efficiency when compared to the LH 780. Correction of red blood cell parameters is appropriate and accurate in cases of extreme leukocytosis.     

Complete blood count parameters for healthy,small‐for‐gestational‐age,full‐term newborns

No previous study has investigated the full range of complete blood count (CBC) parameters in small‐for‐gestational‐age (SGA) newborns. The main aim of this study was to compare CBC and peripheral smear parameters in term, healthy SGA neonates and appropriate‐for‐gestational‐age (AGA) neonates, and to establish CBC reference values for full‐term SGA newborns. One hundred thirty‐two healthy, term newborns (73 SGA and 59 AGA) were included. On day 1, we obtained 109 samples and on day 7 we obtained 77 samples. A CBC and peripheral smear were analyzed for each sample collected and group data were compared. We observed higher mean values for normoblast count, hemoglobin, hematocrit, and red blood cell (RBC) count in the SGA babies than in the AGA babies on day 1. The mean corpuscular hemoglobin and mean corpuscular hemoglobin concentration values for the SGA babies were decreased because of the relatively high RBC count and relatively high mean corpuscular volume we observed in this group. Of the SGA newborns, 21.9% had neutropenia and 4.7% had absolute neutrophil counts lower than 1500/μl on day 1. On both day 1 and day 7, the SGA newborns had higher mean absolute metamyelocyte counts and higher mean I : T (immature : total neutrophil ratio) values than the AGA group. The SGA babies had a lower mean absolute lymphocyte count on day 7 than the AGA group. We detected thrombocytopenia in almost one‐third of the 64 SGA newborns tested on day 1. In summary, our study clearly demonstrates that CBC parameters for healthy, full‐term, SGA newborns are different from those of healthy, term AGA newborns. This is the first study that has documented different mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, metamyelocyte counts, lymphocyte counts, and I : T in SGA babies compared with AGA babies.     

White blood cell count and mortality in patients with acute pulmonary embolism

Although associated with adverse outcomes in other cardiovascular diseases, the prognostic value of an elevated white blood cell (WBC) count, a marker of inflammation and hypercoagulability, is uncertain in patients with pulmonary embolism (PE). We therefore sought to assess the prognostic impact of the WBC in a large, state‐wide retrospective cohort of patients with PE. We evaluated 14,228 patient discharges with a primary diagnosis of PE from 186 hospitals in Pennsylvania. We used random‐intercept logistic regression to assess the independent association between WBC count levels at the time of presentation and mortality and hospital readmission within 30 days, adjusting for patient and hospital characteristics. Patients with an admission WBC count <5.0, 5.0–7.8, 7.9–9.8, 9.9–12.6, and >12.6 × 10⁹/L had a cumulative 30‐day mortality of 10.9%, 6.2%, 5.4%, 8.3%, and 16.3% (P < 0.001), and a readmission rate of 17.6%, 11.9%, 10.9%, 11.5%, and 15.0%, respectively (P < 0.001). Compared with patients with a WBC count 7.9–9.8 × 10⁹/L, adjusted odds of 30‐day mortality were significantly greater for patients with a WBC count <5.0 × 10⁹/L (odds ratio [OR] 1.52, 95% confidence interval [CI] 1.14–2.03), 9.9–12.6 × 10⁹/L (OR 1.55, 95% CI 1.26–1.91), or >12.6 × 10⁹/L (OR 2.22, 95% CI 1.83–2.69), respectively. The adjusted odds of readmission were also significantly increased for patients with a WBC count <5.0 × 10⁹/L (OR 1.34, 95% CI 1.07–1.68) or >12.6 × 10⁹/L (OR 1.29, 95% CI 1.10–1.51). In patients presenting with PE, WBC count is an independent predictor of short‐term mortality and hospital readmission. Am. J. Hematol. 88:677–681, 2013. © 2013 Wiley Periodicals, Inc.     

非酒精性脂肪性肝病胰岛素抵抗程度与全血细胞计数的相关性

目的探讨伴或不伴2型糖尿病(T2DM)的非酒精性脂肪性肝病(NAFLD)胰岛素抵抗(IR)程度与全血细胞计数各参数的相关性。方法选取糖耐量正常并除外糖尿病史的单纯NAFLD患者102例,T2DM合并NAFLD患者104例,正常对照104例为研究对象,测定空腹血糖(FPG)、空腹胰岛素(FINS)和全血细胞计数,分析胰岛素抵抗指数(HOMA-IR)与全血细胞计数各参数的相关性。结果 T2DM合并NAFLD患者IR及全血细胞计数异常程度较单纯NAFLD患者更重;相关性研究表明男性HOMA-IR与WBC、NEU、LYM、RBC、HGB、HCT呈正相关,女性HOMA-IR与WBC、NEU、LYM、MID、RBC、HGB、HCT呈正相关。结论 NAFLD时白细胞参数和红细胞参数的变化与IR密切相关,T2DM的存在加重了IR对上述血细胞参数的影响。全血细胞计数可以作为反映NAFLD患者IR程度的一种简单实用的检验指标。     

Comparison of the reticulocyte mode of the Abx Pentra 120 Retic,Coulter® General‐S™, Sysmex® SE 9500, Abbott CD 4000 and Bayer Advia® 120 haematology analysers in a simultaneous evaluation

The Abx Pentra 120 Retic, Coulter^® General‐S?, Sysmex^® SE 9500, Abbott Cell Dyn^® 4000 and Bayer Advia^® 120 were evaluated simultaneously in a general hospital laboratory. Linearity, precision, sample stability, carry‐over and comparability of the reticulocyte mode were determined following International Council for Standardization in Haematology guidelines for the evaluation of blood cell analysers. All analysers showed good results for dilution, stability and carry‐over testing. The between‐batch coefficient of variation of the General‐S? was high compared to the other analysers evaluated. Multiple correlation studies showed good agreement for all analysers in the normal and high reticulocyte range, with correlation coefficients above 0.7. Multiple correlation studies for reticulocytopenic samples (< 15.10⁹/l) were less satisfactory, with a wider range of correlation coefficients (r‐values 0.0–0.9). Overall, the General‐S?, SE 9500 and Advia^® 120 gave lower reticulocyte counts than the Pentra 120 Retic and CD 4000. Reagent costs were also evaluated. Reagent consumption was close to the manufacturers’ specifications for the SE 9500 (Search reagent), CD 4000 (CD Retic) and Advia^® 120 (Retics) but was higher than stated for the Pentra 120 Retic (Retix), General‐S? (Retic kit) and SE 9500 (Sheath reagent). Our results show that these new generation haematology analysers will meet the needs of hospital laboratories for reliable and cost‐effective reticulocyte counting.     

ALL R-87 protocol in the treatment of children with acute lymphoblastic leukaemia in early bone marrow relapse

Seventy-three children with acute lymphoblastic leukaemia (ALL) in first bone marrow (BM) relapse, occurring within 30 months from complete remission (CR), were enrolled in an Italian cooperative study (ALL R-87 protocol). This treatment programme consisted of an induction phase with intermediate-dose cytarabine (IDARA-C) plus idarubicin (IDA) and prednisone (PDN), followed by a multidrug consolidation therapy and bone marrow transplant (BMT). 55/73 children achieved CR (75.3%); 15 (20.5%) failed to respond and three (4.2%) died during induction. The response rate was significantly higher for children with a first CR duration 12 months (P = 0.0005) and for those with a white blood cell (WBC) count at relapse <20 × 10⁹/l (P=0.004). The estimated disease-free survival (DFS ± SE) at 82 months was 0.18 ± 0.05 for all responders, and 0.70 ± 0.14 for allotransplanted patients versus 0.05 ± 0.05 for those autografted (P = 0.001). The estimated probabilities of survival ± SE and event-free survival (EFS ± SE) at 83 months were 0.16 ± 0.07 and 0.13 ± 0.04, respectively, for all enrolled children. Univariate analysis showed that age <10 years at initial diagnosis and B-lineage immunophenotype favourably influenced both DFS (P = 0.001) and EFS probabilities (P = 0.0014 and P = 0.012, respectively), whereas a first CR duration 12 months and a WBC count at relapse <20 × 10⁹/l were associated only with a better EFS rate (P = 0.026 and P = 0.004, respectively). Our results show the efficacy of the IDA plus IDARA-C schedule used in the ALL R-87 protocol in high-risk relapsed ALL children. Allogeneic BMT proved effective for patients with an HLA sibling donor. In a multivariate analysis, age 10 years at initial diagnosis (P = 0.016) and WBC count at relapse 20 × 10⁹/l (P = 0.048) were independently associated with a worse disease outcome.     

Clinical utility of haematological inflammatory biomarkers in predicting 30-day mortality in hospitalised adult patients with COVID-19

Coronavirus disease 2019 (COVID-19) is a multisystem disease affecting respiratory, cardiovascular, gastrointestinal, neurological, immunological and haematological systems. The most important indices that have been studied are platelet (PLT) indices in addition to the PLT count and red blood cell distribution width (RDW). This retrospective study included 95 patients with COVID-19 and was conducted at the Hospital Isolation, Scientific and Medical Research Centre and Clinical Pathology Department at Zagazig University Hospitals, Egypt over 6 months from March to August 2021. All patients on admission had a full blood count, which included white blood cell (WBC) count, haemoglobin, RDW, PLT count and its indices in addition to PLT-to-WBC ratio (PWR) and PLT-to-lymphocyte ratio (PLR), which were calculated for all the study patients. There were significant linear correlations for higher levels of the PLR, PWR and RDW and mortality rate (p = 0.03, p < 0.001 and p < 0.001 respectively). Moreover, on multivariable analysis the RDW, PLT count and PWR levels were independent prognostic predictors for mortality with a hazard ratio [HR] of 1.25 (95% confidence interval [CI] 1.09–1.44, p = 0.002), 1.00 (95% CI 0.99–1.00, p = 0.03) and 2.3 (95% CI 1.21–4.48, p = 0.01) respectively. The RDW and PLT indices are accessible predictors that can be valuable prognostic factors for survival assessment and risk stratification of COVID-19.     

White blood cell recovery after allogeneic hematopoietic cell transplantation predicts clinical outcome

To determine whether outcome after allogeneic hematopoietic cell transplantation (HCT) could be estimated by using peripheral white blood cell count (WBC) as a metric that integrates several aspects of HCT recovery, we conducted a retrospective study of 1,109 adult patients who underwent first allogeneic HCT from 2003 through 2009. WBC at 1–3 months after HCT was categorized as low (<2), normal (2–10), and high (>10 × 10⁹ cells/L). Overall survival (OS) and progression‐free survival (PFS) were lower for patients with low or high WBC at 1–3 months after HCT (P < 0.0001). We developed a predictive three‐group risk model based on the pattern of WBC recovery early after HCT. Five‐year OS was 47, 30, and 15% (P < 0.0001) and 5‐year PFS was 39, 22, and 14% for patients in the three different risk groups (P < 0.0001). The pattern of WBC recovery early after HCT provides prognostic information for relapse, nonrelapse mortality, progression‐free survival, and overall survival. A scoring system based on the trajectory of the WBC in the first 3 months after HCT can effectively stratify patients into three groups with different PFS and OS. If validated, this system could be useful in the clinical management of patients after HCT, and to stratify patients enrolled on HCT clinical trials. Am. J. Hematol. 89:591–597, 2014. © 2014 Wiley Periodicals, Inc.     

Spurious counts and spurious results on haematology analysers: a review. Part II: white blood cells, red blood cells, haemoglobin, red cell indices and reticulocytes

Haematology analysers provide quick and accurate results in most situations. However, spurious results, related either to platelets (part I of this report) or to other parameters from the cell blood count (CBC) may be observed in several instances. Spuriously low white blood cell (WBC) counts may be observed because of agglutination in the presence of ethylenediamine tetra-acetic acid (EDTA). Cryoglobulins, lipids, insufficiently lysed red blood cells (RBC), erythroblasts and platelet aggregates are common situations increasing WBC counts. In most of these instances flagging and/or an abnormal WBC differential scattergram will alert the operator. Several situations lead to abnormal haemoglobin measurement or to abnormal RBC count, including lipids, agglutinins, cryoglobulins and elevated WBC counts. Mean (red) cell volume (MCV) may be also subject to spurious determination, because of agglutinins, excess of glucose or salts and technological considerations. In turn, abnormality related to one measured parameter will lead to abnormal calculated RBC indices: mean cell haemoglobin content (MCHC) is certainly the most important RBC indices to consider, as it is as important as flags generated by the haematology analysers (HA) in alerting the user to a spurious result. In many circumstances, several of the measured parameters from CBC may be altered, and the discovery of a spurious change on one parameter frequently means that the validity of other parameters should be considered. Sensitive flags now allow the identification of several spurious counts, but only the most sophisticated HA have optimal flagging and more simple HA, especially those without a WBC differential scattergram, do not possess the same sensitivity for detecting anomalous results. Reticulocytes are integrated now into the CBC in many HA, and several situations may lead to abnormal counts.     

A retrospective observational study of leucoreductive strategies to manage patients with acute myeloid leukaemia presenting with hyperleucocytosis

Acute myeloid leukaemia (AML) patients with hyperleucocytosis have higher early mortality, lower complete remission (CR) and overall survival (OS). Whether different pre‐induction leucoreduction strategies can improve outcome is unknown. A single centre retrospective cohort study was conducted on AML patients with a white blood cell count (WBC) >100 × 10⁹/l between 1987 and 1997, and on all AML patients between 1998 and 2006, to determine (a) the effect of four different leucoreductive strategies (leukapheresis, hydroxycarbamide, leukapheresis and hydroxycarbamide or no pre‐induction leucoreduction) on early (day 28) mortality, CR, and OS; and (b) whether a high presenting WBC remains a negative predictor of OS in patients surviving induction (first 28 d). In the 1998‐2006 cohort (n = 702), higher WBC was associated with higher early mortality and lower OS but its effects were greatly diminished in patients who survived the first 28 d (Hazard Ratio 1·094 vs. 1·002). A WBC of 34·1 × 10⁹/l had the highest sensitivity (75·6%) and specificity (67·4%) for early mortality. None of the four leucoreduction strategies differed significantly in early mortality, CR, or OS in patients with WBC>100 × 10⁹/l (n = 166). The number of leucostatic signs was a significant predictor of early mortality (P < 0·0001) and OS (P = 0·0007). The results suggest that AML patients with hyperleucocytosis should be induced, if eligible, without pre‐induction leucoreduction.     

Increased post‐induction intensification improves outcome in children and adolescents with a markedly elevated white blood cell count (≥200 × 109/l) with T cell acute lymphoblastic leukaemia but not B cell disease: a report from the Children's Oncology Group

Children and adolescents presenting with a markedly elevated white blood cell (ME WBC) count (WBC ≥200 × 10⁹/l) comprise a unique subset of high‐risk patients with acute lymphoblastic leukaemia (ALL). We evaluated the outcomes of the 251 patients (12% of the study population) with ME WBC treated on the Children's Cancer Group‐1961 protocol. Patients were evaluated for early response to treatment by bone marrow morphology; those with a rapid early response were randomized to treatment regimens testing longer and stronger post‐induction therapy. We found that ME WBC patients have a poorer outcome compared to those patients presenting with a WBC <200 × 10⁹/l (5‐year event‐free survival 62% vs. 73%, P = 0·0005). Longer duration of therapy worsened outcome for T cell ME WBC with a trend to poorer outcome in B‐ALL ME WBC patients. Augmented therapy benefits T cell ME WBC patients, similar to the entire study cohort, however, there appeared to be no impact on survival for B‐ALL ME WBC patients. ME WBC was not a prognostic factor for T cell patients. In patients with high risk features, B lineage disease in association with ME WBC has a negative impact on survival.     

Effect of combination therapy of hydroxyurea with l‐carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with β‐thalassemia intermedia

Background: l‐Carnitine and magnesium have antioxidant properties. They have the potential to stimulate production of fetal hemoglobin and stabilize the RBC membrane, respectively. Several studies have also shown the beneficial effects of hydroxyurea in thalassemic patients. We assessed the effect of combination therapy of hydroxyurea with l ‐carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with β‐thalassemia intermedia. Methods: One‐hundred‐and‐twenty patients with thalassemia intermedia (range, 4–35 yr; mean, 19 ± 6.4 yr) who had no need for blood transfusion or requirement for blood transfusion with an interval of >6 months were randomly selected. All patients had been on hydroxyurea for >6 months. They were randomly divided into four groups: group A (hydroxyurea alone); group B (hydroxyurea and l ‐carnitine); group C (hydroxyurea and magnesium chloride); and group D (hydroxyurea, l ‐carnitine and magnesium chloride). Results: In groups B, C, and D, mean Hb and hematocrit increased during 6‐month treatment (P < 0.001). Echocardiographic studies revealed a significant decrease in left ventricular end‐diastolic diameter in group B (P = 0.032), increase in pulmonary acceleration time in group C (P = 0.012), and increase in left ventricular ejection fraction in groups C and D (P < 0.000 and 0.006, respectively). Conclusion: Combination of hydroxyurea with l ‐carnitine or magnesium could be more effective in improving hematologic parameters and cardiac status in patients with β‐thalassemia intermedia than hydroxyurea alone.     

Associations and prognostic interactions between circulating levels of hepcidin,ferritin and inflammatory cytokines in primary myelofibrosis

Iron homeostasis is dysregulated in primary myelofibrosis (PMF), given the high prevalence of anemia, need for red blood cell (RBC) transfusions, and disease‐associated inflammatory state. We measured plasma hepcidin levels in 203 consecutive PMF patients at the time of first referral; hepcidin levels were significantly higher as compared to healthy controls (P < 0.0001), and were correlated with hemoglobin of <10 g/dL, RBC transfusion requirement, serum ferritin of >500 µg/L, higher dynamic international prognostic scoring system (DIPSS)‐plus risk category, the presence of circulating blasts, age of >65 years, and leukocyte count of <4 × 10⁹/L. Increased hepcidin levels predicted for inferior survival independent of six out of the eight DIPSS‐plus prognostic parameters (hazard ratio [HR] = 1.8; P = 0.02), but not when RBC transfusion requirement, hemoglobin of <10 g/dL, or increased serum ferritin were included in the Cox model. Multivariable analysis that considered the four overlapping prognostic variables revealed that increased hepcidin (HR = 1.9; P = 0.03) and increased ferritin (HR = 2.3; P = 0.04), but not hemoglobin of <10 g/dL or RBC transfusion requirement, independently retained their significance for predicting survival. Accordingly, increased levels of both hepcidin and serum ferritin (seen in 29% of patients) predicted inferior survival independent of DIPSS‐plus or increased inflammatory cytokine levels (HR = 2.4; P = 0.002), and could be considered in future prognostic models for PMF. Am. J. Hematol. 88:312–316, 2013. © 2013 Wiley Periodicals, Inc.     

Peripheral blood cell variations in cirrhotic portal hypertension patients with hypersplenism

ObjectiveTo explore peripheral blood cell variations in hepatic cirrhosis portal hypertension patients with hypersplenism.MethodsClinical data of 322 hypersplenism patients with decreased peripheral blood cells, admitted with cirrhotic portal hypertension, was retrospectively studied over the last 17 years.ResultsIn 64% (206/322) of patients, more than 2 kinds of blood cell were decreased, including 89 cases of pancytopenia (43.2%), 52 cases of WBC + PLT decrease (25.2%), 29 cases of RBC + PLT decrease (14.1%), and 36 cases of WBC + RBC decrease (17.5%); in 36% (116/322) of patients, single type blood cell decrease occurred, including 31 cases of PLT decrease (26.7%), 29 cases of WBC decrease (25%) and 56 cases of RBC decrease (48.3%). Of 227 routine bone marrow examinations, bone marrow hyperplasia was observed in 118 cases (52.0%), the remainder showed no hyperplasia. For the distinct scope and extent of peripheralblood cell decreases, preoperative blood component transfusions were carried out, then treated by surgery, after whole group splenectomy, the peripheral blood cell count was significantly higher (P<0.05).ConclusionsOf portal hypertensive patients with splenomegaly and hypersplenism, 64% have simultaneous decrease in various blood cells, 36% have decrease in single type blood cells, 52% of patients have bone marrow hyperplasia. A splenectomy can significantly increase the reduction of peripheral blood cells.