Platelet and Red Blood Cell Indices in Harris Platelet Syndrome.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3988-3988 ◽  
Author(s):  
Harris V.K. Naina ◽  
Samar Harris

Abstract Inherited giant platelet disorders are a group of rare disorders characterized by thrombocytopenia, giant platelets and variable bleeding symptoms. Naina et al., described a new giant platelet disorder called Harris Platelet Syndrome (HPS), the most common inherited giant platelet disorder occurring in up to one third of blood donors from north eastern part of Indian subcontinent. HPS is characterized by an autosomal dominant mode of inheritance with normal to severe thrombocytopenia (less than 50x109/L), giant platelets (mean platelet volume more than 10fL) and absent bleeding symptoms with normal platelet aggregation studies. Occasionally abnormalities in red blood cell morphology have been associated with certain giant platelet disorders such as stomatocytosis in Mediterranean Macrothrmboctopenia, dyserythropoiesis in GATA 1 associated macrothrombocytopenia and thalassemia, in X Linked Thrombocytopenia Thalassemia (XLTT). This study was undertaken to analyze the platelet and red blood cell indices in blood donors with Harris Platelet syndrome. A total of 203 blood donors were included in this study, 101 blood donors from northeaster part of India with MPV more than 12fl (normal 7–10fl) and 102 blood donors from southern part of India. Before blood donation, all donors were questioned about a history of bleeding conditions, in either themselves or their relatives. Blood samples were collected in ethylenediaminetetraacetic acid (EDTA). Automated platelet counts were performed using a Coulter STKS (Coulter, Hialeah, Florida) within 2 hours of collection. Peripheral blood smears were examined to confirm thrombocytopenia, giant platelets and red blood cell morphology. There was a significant difference between platelet count (Mean ±SD) 136± 40 Vs 262 ± 53 in southern India (p<0.000). Thirty three donors with HPS had a normal platelet count with MPV more than 12fL. MPV was 13.6±0.13 (range 12 to21.9fL) in donors with HPS and 7.3 ±0.6 (range 6–9.2fl) in southern Indian blood donors. The platelet distribution width (PDW) was 17.4±0.8 in donors with HPS and was 16.38±0.5 in southern India blood donors(p<0.000). Though there was a significant difference between hemoglobin, 13.8 ± 1.0 vs and 14.7± 1.1 (P<0.00), there was no significant difference between RDW, MCV, MCH, MCHC. Peripheral blood smear did not show any obvious red blood cell abnormality, but showed giant platelets and thrombocytopenia. Harris platelet syndrome is associated with normal to severe thrombocytopenia, giant platelets and significant platelet anisocytosis. There was no associated red blood cell abnormalities observed with HPS.

Author(s):  
Giuseppe Lippi ◽  
Gian Luca Salvagno ◽  
Martina Montagnana ◽  
Elisa Danese ◽  
Gian Cesare Guidi

AbstractRecent evidence suggests that red blood cell distribution width (RDW), a simple measure of anisocytosis, may predict the risk of adverse clinical outcomes in both the general population and in patients with severe pathologies. Since it was also shown that the birth season influences the lifetime disease risk, this study was aimed to investigate whether an association may exist between adult RDW values and birth season.The study population consisted in healthy Caucasian blood donors aged 18 or older, undergoing routine laboratory testing before regular blood donation.Overall, 6122 healthy blood donors were included in this study (median age 41 years; 1807 women and 4315 men). Age, sex, mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) but not hemoglobin and hematocrit were found to be independent predictors of RDW. When the study population was classified according to birth season, a significant difference was found for RDW values, but not for age, sex, hemoglobin, hematocrit, MCV and MCH. Subjects born in spring exhibited RDW values generally higher compared to those born in other seasons, reaching statistical significance when compared to those born in summer and winter. In particular, subjects born in spring had a 33% (p=0.014) higher probability of displaying increased RDW values in adulthood compared to those with summer birth.Despite additional studies that are needed to confirm these original findings, the evidence that a significant link exists between birth season and adult anisocytosis provides a plausible explanation for the association between birth season and lifetime disease risk.


Author(s):  
Majid Vafaei ◽  
Bijan Keikhaei-dehdazi

Background: Red blood cell (RBC) transfusions are frequently used in patients with sickle cell disease (SCD) to treat and prevent of their disease complications. However repeated blood transfusions are often complicated by RBC alloimmunization. Race and antigenic pattern differences are the common risk factor to develop alloimmunization. This study was performed to determine the frequency of RBC alloimmunzation in sickle cell patients and healthy blood donors.Methods: This is a cross sectional study that has been done on 140 patients with SCD and 140 healthy blood donors from April 2015 to April 2016. The RBC phenotype of all patients and donors investigated by Tub method and all panel test phases were done at immunohematology laboratory of Iranian Blood Transfusion Organization of Ahwaz.Results: Of all SCD patients 61 (43.6%) were male and 79 (56.4%) were female. 68 (48.5%) were HbSS and 72 (51.4%) were S/B thalassemia. The mean age of patients was 19.69 (range: 2-60) years. Of all patients, 114 (81.25%) had received transfusion. The RBC alloimmunization rate among SCD patients was 7.1% and 50% of the RBC alloimmunization had anti-Kell and 30% had anti-Rh. The comparison of the RBC phenotypes between the group of patients with SCD and the group of blood donors (non-Arab) revealed a statistically significant difference in the frequency of S (54% vs. 67%, p=0.024), M (82.7% vs. 90.7%, p=0.049), and FYb (73.4% vs. 55.7%, p=0.002).Conclusions: Althoguh alloimmunization rate in SCD patients in this study was lower than reported by other studies but cross matchining at least for the Rh and Kell systems from the time of initial transfusion may decrease the incidence of alloimmunization. 


2012 ◽  
Vol 1 ◽  
pp. 13
Author(s):  
Ning Zhang

<p><strong>Objective: </strong>The changes of urinary red blood cell morphology and the average volume of red blood cell were examined by MCV. <strong>Method:</strong> In 100 cases of patients with urinary sediment microscopy of hematuria, and used blood cell analyzer for determination for MCV in urinary RBC. <strong>Results</strong><strong>: </strong>Renal hematuria MCV and the outer peripheral blood MCV has significant difference (<em>p</em> &lt; 0.01) and non-renal hematuria and peripheral blood MCV had no significant difference, glomerular hematuria and non-glomerular hematuria erythrocyte deformity was significantly different (<em>p</em> &lt; 0.01), renal hematuria MCV is less than or equal to 73 fL, abnormal red blood cells with 2 or more, deformity rate is more than or equal to 76%. <strong>Conclusion:</strong> RBC phase combined with MCV detection of urine RBC is the practical value in diagnosis of glomerular hematuria.</p>


2021 ◽  
pp. 107815522110120
Author(s):  
Victoire Béchet ◽  
Hubert Benoist ◽  
Frédéric Beau ◽  
Fabienne Divanon ◽  
Stéphanie Lagadu ◽  
...  

Background Caregivers in healthcare settings are exposed to a risk of antineoplastic drug contamination which can lead to adverse health effects. Biological monitoring is necessary to estimate the actual level of exposure of these workers. This study was conducted with the aim of assessing blood contamination levels by irinotecan and its metabolites of pharmaceutical staff operating inside and outside a compounding unit. Methods The study took place within the pharmaceutical unit of a French comprehensive cancer centre. Blood samples were collected from the pharmacy workers operating inside and outside the compounding unit, and analysed by UHPLC-MS/MS. Plasma and red blood cell irinotecan and its metabolites (SN-38; APC) were determined with a validated analytical method detection test. Results A total of 17/78 (21.8%) plasma and red blood cell-based assays were found to be contaminated among staff. Overall, the total number of positive assays was significantly higher for staff members working outside the compounding unit than for workers working inside it ( P = 0.022), with respectively 5/42 (11.9%) and 12/36 (33.3%) positive assays. For plasma dosages, the “outside” group had a significantly higher number of positive assays ( P = 0.014). For red blood cell-based assays, no significant difference was found ( P = 0.309). Conclusions This study reveals that pharmaceutical staff serving in health care settings are exposed to a risk of antineoplastic drug contamination, not only inside the compounding room but also in adjacent rooms. The results would help to raise awareness and potentially establish protective measures for caregivers working in areas close to the compounding room as well.


Blood ◽  
2013 ◽  
Vol 121 (1) ◽  
pp. 9-9 ◽  
Author(s):  
Etheresia Pretorius ◽  
Boguslaw Lipinski

Transfusion ◽  
2021 ◽  
Author(s):  
Kelsey Hazegh ◽  
Bradley D. Anawalt ◽  
Larry J. Dumont ◽  
Tamir Kanias

2021 ◽  
Vol 12 ◽  
Author(s):  
Chongjun Wu ◽  
Ting Xiong ◽  
Zhongjin Xu ◽  
Chunlei Zhan ◽  
Feng Chen ◽  
...  

ObjectiveTo investigate the clinical and genetic characteristics of hereditary spherocythemia (HS) in Chinese children, and to analyze the potential genotypic/phenotypic associations.MethodsThe clinical data and gene test results of children with HS were collected. All patients were diagnosed by gene test results, and the laboratory results were obtained before splenectomy. The data of red blood cell (RBC), hemoglobin (HB), mean red blood cell volume (MCV), mean red blood cell hemoglobin (MCH), mean red blood cell hemoglobin concentration (MCHC), and hematocrit (HCT) were statistically analyzed according to different mutation genes. Statistical methods for comparison between groups Mann–Whitney test analysis, two-terminal p &lt; 0.05 was considered significant difference.ResultsA total of 15 children were enrolled in our hospital, and 14 variants were found (nine variants have not been reported before), including 10 ANK1 mutations (seven ANK1 truncated mutations) and five SPTB mutations. Patients with ANK1 mutations had more severe anemia than those with SPTB mutations (significantly lower RBC, HB, MCHC, and HCT).ConclusionThis is one of the few studies on the genetic and clinical characteristics of children with HS in China. This study identified the unique genetic and clinical characteristics of Chinese children with HS and analyzed the pathogenic genotype–phenotypic association. The results confirmed that the anemia degree of HS patients caused by ANK1 was more serious than that of patients with SPTB deficiency. However, further study of the correlation between genotype and phenotype requires a larger sample size.


2019 ◽  
Vol 34 (6) ◽  
pp. 496-503
Author(s):  
Arwa Z. Al-Riyami ◽  
Ali Al-Marhoobi ◽  
Saif Al-Hosni ◽  
Sabah Al Mahrooqi ◽  
Michael Schmidt ◽  
...  

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