Neutropenia in Patients with Thalassemia Major.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3763-3763
Author(s):  
Renzo Galanello ◽  
Raffaella Origa

Abstract Monitoring of the neutrophil count has become an important issue in the management of thalassemia patients after the introduction of the new oral chelator deferiprone, as this chelator has been associated with agranulocytosis and milder neutropenias. Although a clear relation between deferiprone and agranulocytosis has been demonstrated, it is unclear whether milder neutropenias are due to deferiprone use or to other factors such as hypersplenism, infections, autoantibodies, or other drugs. In this study we evaluated 7,708 consecutively blood counts and leukocyte differential obtained between August 2003 to June 2004 from 344 patients with thalassemia major, aged 2 to 40 years, treated with deferiprone (N=39), desferrioxamine (N=179), ICL670 (N=45), or a combination of deferiprone and desferrioxamine (N=81). Complete blood count and differential were performed with Coulter LH 750 automated haematology analyzer (Beckman Coulter, Miami, FL), which can detect nucleated red blood cells (NRBC) and correct the white blood cell (WBC) count in the presence of NRBC, platelets clumps, unlysed or fragmented RBC. The capability to correct the WBC count is particularly relevant in the monitoring of the neutrophil counts of patients with excessive NRBC, which could lead to potential errors in the differrential count. Neutropenia was defined as a neutrophils count below 1500 cells/μL, whereas agranulocytosis was defined as a neutrophil count below 500 cells/μL. No episodes of agranulocytosis were observed. Episodes of neutropenia (range 850 – 1490 neutrophils/μL) were observed in 29 (8,4%) patients. Repeated episodes of neutropenia were observed in 7 of the 29 patients. At the time of neutropenia, 23 (79%) of the patients were being chelated with desferrioxamine, 2 (7%) with deferiprone, 3 (10%) with a combination of both chelators, and 1 (3%) with ICL670. Considering the type of chelation, neutropenia was observed in 12,8% of patients being treated with desferrioxamine, 5% in patients treated with deferiprone, 3,7% in patients treated with combination therapy, and 2.2% in patients treated with ICL670. The severity of neutropenia, assessed as absolute neutrophil count, was not different on the basis of the type of chelation (p=0,26). Neutropenia was more frequent in children than in adults (31% vs 7.8%, respectively, p=0.0001). No statistically significant difference in the incidence of neutropenia was found between males and females (10.2% vs 6.5% respectively, p=0.2), anti-HCV positive and negative (7.7% vs 10.3% respectively p=0.43), or splenectomized and non-splenectomized patients (11.1% vs 8.1% respectively, p=0.5). Neutropenia appears to be a common event in patients with thalassemia major, irrespectively of the chelation therapy used. The higher incidence in children may be secondary to a greater frequency of viral infections at this age, which may produce neutropenia with several mechanisms including redistribution, sequestration and destruction of neutrophils.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4268-4268
Author(s):  
Alessia Pepe ◽  
Maria Marsella ◽  
Antonella Meloni ◽  
Valeria Caldarelli ◽  
Maria Chiara Dell'Amico ◽  
...  

Abstract Abstract 4268 Introduction. Heart disease remains the main cause of mortality in thalassemia major patients. Female patients with thalassemia major have a proved lower prevalence of cardiac complications than males and survive longer. It has been suggested that females have a better compliance than males, and therefore accumulate less iron in crucial organs like the heart (Borgna-Pignatti C et al, Haematologica 2004). The aim of our study was to verify if the decreased prevalence of cardiac disease in females could be attributed to lesser iron accumulation in their hearts as measured by multislice multiecho T2* Magnetic Resonance Imaging (MRI) technique. Methods. We performed a retrospective review of the MRI results and of clinical data about the thalassemia major patients enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) project. The MIOT is a network where MRI is performed using standardized and validated procedures and the MRI and thalassemia centers are linked by a web-based network, configured to collect patients' clinical and diagnostic data (Meloni A et al, Int J Med Inform 2009). Myocardial iron concentrations were measured by T2* multislice multiecho technique (Pepe A et al, JMRI 2006).Biventricular function parameters were quantitatively evaluated by cine images. Results. Seven hundred and seventy six thalassemia patients (370 males) were present in the MIOT database having undergone at least one MRI exam. The prevalence of cardiac disease (heart dysfunction and/or arrhythmias requiring medications) was significantly higher in males than in females (males 28% vs females 17%; P<0.0001). The analysis of different chelation treatments did not demonstrate a significant difference between patients with and without cardiac disease (P=0.59), nor between sexes (P=0.46). In addition, there was no difference in the reported compliance to chelation therapy between males and females (P=0.52). Global heart T2* values were significantly lower in both males and females with heart dysfunction (males: 20 ± 15 ms; females: 18 ± 12 ms), compared to those without dysfunction (males: 29 ± 11 ms; females: 27 ± 13 ms) (P<0.0001), but no difference was observed according to sex (Figure 1A). Global heart T2* values were not significantly lower in patients with arrhythmias compared to those without arrhythmias, nor was there a significant difference between sexes (Figure 1B). Conclusions. The confirmed higher prevalence of cardiac disease in males with thalassemia major was not correlated to a worse compliance to chelation therapy or to an higher cardiac iron burden. Increased survival of female thalassemia major patients seems to not be attributed to lower cardiac iron overload. It can be hypothesized that females tolerate iron toxicity better, possibly as an effect of reduced sensitivity to chronic oxidative stress. Disclosures: No relevant conflicts of interest to declare.


2019 ◽  
Vol 10 (4) ◽  
pp. 3356-3368
Author(s):  
Kussay M. Abbas Zwain ◽  
Samer M. Mohamed Al-Hakkak ◽  
Alaa A. Al-Wadees ◽  
Zainab Mahdi Majeed

β-thalassemia major is a chronic, inherited hematological disease that leads to chronic anemia in the affected children. One of the options of treatment in such patients was splenectomy; however, it is not without risk of many complications; one of them is the thrombotic events. A prospective study of 55 patients with β-thalassemia inscribes in this study. 14 patients (25.5%) had a normal thrombocyte count and 41 patients (74.5%) have an abnormally high thrombocytes count which was significantly associated with PVT (P. Value <0.001), Regarding the WBC count, it extended between 4000 to more than 30,000, in both genders with non-statistically significant differences between both genders, (P>0.05). Regarding the serum ferritin, the mean level was 2908.5 ± 1024.3 ng/ml. In males, the mean S. Ferritin was relatively higher than that of females, 3167.6 ± 1841.3 mg/dl, and 2573.8 ± 1150.6 ng/ml. The weight of the spleen was up to 1500 grams in 25 (80.6%) of males and 20 (83.3 %) of females while it was more than 1500 grams in the remaining and females and males, without a significant difference statistically in the spleen weight of, (P>0.05). The most frequent presenting symptom was abdominal pain. It was founded in 46 patients (83.6%), followed by fever in 76.4%, diarrhea in 58.2%, and Nausea and vomiting in 31 (56.4%). 3 patients out of the 55 (5.5%) developed portal vein thrombosis in their follow up period. Post splenectomy PVT in thalassemia the patient is relatively frequent (5.5%) complication that require a high degree of doubt for diagnosis early, especially in patients with postoperative pain of the abdomen within 2 months after surgery, Female gender, Large spleen and postoperative increase number of platelets are risk factors for PVT so one can initiate surveillance by Doppler ultrasound postoperatively and start antiplatelet prophylactic therapy immediately for such patients. 


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3827-3827
Author(s):  
Ali Taher ◽  
Wael Shamseddeen ◽  
Samir Arnaout ◽  
Walid Gharzuddin ◽  
Nabil Zeineh ◽  
...  

Abstract Introduction: Cardiac dysfunction in thalassemia remains of primary concern. The prevalence of these abnormalities varies in different phenotypic/genotypic groups of thalassemia. Objective: To describe the difference in cardiac function among Thalassemia Major ™, Thalassemia Intermedia (TI), and Sickle-Thalassemia (ST) patients. Methods: 66 TM, 41 TI and 17 ST patients were randomly selected from a population of 450 TM,100 TI and 26 ST patients respectively. The patients were interviewed and their charts reviewed and all had an echocardiography. Pulmonary Hypertension (PHT) was defined as Pulmonary Artery Systolic Pressure&gt; 30mmHg. Results: 58.8%, 50% and 41.5% of ST,TM and TI patients respectively were males. Mean age was 24.4 ± 11.6, 19.4 ± 7.6, 19.5 ± 10.9 year old for TI, TM and ST. Tricuspid Regurgitant jet was detected in 47%, 30% and 82% of TI, TM & ST patients respectively. PHT was found in 83%, 18% and 10% of TI, ST, TM patients. Deceleration Time (DT) was significantly longer in TI than in TM and ST, with no significant difference between TM and ST. End diastolic dimension (EDD) was significantly larger in TI than TM and ST (for more details about cardiac parameters refer to tables 2a and 2b). Thrombotic events occurred in 6% of TI patients and in 0 TM and ST patients. Discussion: PHT is primarily prevalent in TI patients which further supports the notion that a different pathophysiological process leads to this than in TM and ST patients. Moreover these results stress the need for better method of assessment of PHT, because of the limited number of patients in whom a TR jet could be detected. A prolonged DT is one of the earliest sign of diastolic dysfunction in thalassemics. TI patients have revealed themselves to have more impaired diastology by both prolonged DT and Isovolumic relaxation time (IVRT). Splenectomy was performed more in Lebanese TI than that reported in the literature (87% vs 65%), because most of our TI patients were managed as TM. This was before the currently recognized association of splenectomy and PHT and thrombosis. In addition chamber sizes (EDD) was significantly more in TI than both TM and ST patients. This can be attributed to the more profound chronic dilutional volume overload secondary to uncorrected anemia. Finally as is evident TI patients are developing more complications than both TM and ST. Transfusion/ chelation therapy to avoid PHT, diastolic dysfunction and thrombosis in TI patients has been proposed as a solution. If the pathophysiology of these complications is iron-mediated then prophylactic transfusion/ chelation therapy would be more advisable than waiting for them to develop. Especially in view of the morbidity associated with thrombosis, and limitation of exercise tolerance secondary to PHT. Table 1: Clinical characteristics of patients Table 2a: Significant differences between TI and ST patients Table 2b: Significant differences between TM and ST patients


Author(s):  
Hind M. Beheiry ◽  
Ibrahim A. Ali ◽  
Mazin S. Abdalla ◽  
Ahmed M. Sharif ◽  
Amal M. Saeed

Background: Pre-eclampsia is a serious disorder of pregnancy with unknown ethological factors that may occur at any stage of second or third trimester of pregnancy. The objectives of the present study were to assess changes in complete blood counts including platelets, liver enzymes and serum uric acid in pre-eclamptic cases compared to second-half normal pregnant and non-pregnant Sudanese women and their correlations to other biomarkers.Methods: This was a cross-sectional, case-control study performed from December 2008 to December 2010; in Omdurman Maternity Hospital, in concomitance with other studies in pre-eclampsia. The sample size included three groups, 72 up pre-eclamptic cases in their recent pregnancies, 96 normal pregnant in their second half of pregnancy and 63 non- pregnant (control) women; a total of 231 subjects. Questionnaire Interviews and clinical examination were done for all participants. Laboratory investigations were done including complete blood picture, liver enzymes and uric acid.  Results: The mean Hb concentration of the pre-eclamptic (11.3g/dl±1.7) was statistically significantly lower than that of the non-pregnant (12.1g/dl±0.2) (P=0.01) but not from that of the normal pregnant (11.4g/dl±0.1) (P=0.882) .There was no statistical significant difference in the mean WBC count between the pre-eclamptic (7.4x103/mm3±0.3) and non-pregnant (7.3x103/mm3±0.3) (P=0.797) and between the pre-eclamptic and normal pregnant (7.7x103/mm3±0.2) (P=0.270). There was a considerable statistical significant decrease in the mean platelets count of the pre-eclamptic (236.4/mm3±8.3) compared to the non-pregnant group (322.0/mm3±10.4) (P=0.0001) s well as to the normal pregnant (275.0/mm3±8.9) (P = 0.003). In the pre-eclamptic cases, serum ALT correlated significantly with TWCC (r=0.26, P=0.03) and serum AST (r=0.65, P=0.000). In the pre-eclamptic cases, serum AST correlated significantly with Hb (r=0.26, P=0.03), serum ALT and serum uric acid (r=0.36, P=0.01).Conclusions: There was a considerable statistical significant decrease in mean platelets count of the pre-eclamptic compared to the non-pregnant group and to the normal pregnant may be explained by hemodilution; whereas further decrease was due to pre-eclampsia. ALT and AST are strong prognostic indicators of pre-eclampsia.


2021 ◽  
Vol 99 (Supplement_1) ◽  
pp. 168-169
Author(s):  
Cynthia Jinno ◽  
Braden T Wong ◽  
Martina Kluenemann ◽  
Xunde Li ◽  
Yanhong Liu

Abstract The objective of this experiment was to investigate the effects of dietary supplementation of Bacillus amyloquefaciens on total and differential blood cell count in weaned pigs experimentally infected with a pathogenic E. coli. A total of 50 weaned pigs (7.41 ± 1.35 kg) were individually housed in disease containment rooms and randomly assigned to one of the 5 treatments: sham control (CON-), sham B. amyloquefaciens (BAM-), challenged control (CON+), challenged B. amyloquefaciens (BAM+) and challenged carbadox (CAR+). The experiment lasted 28 days with 7 days’ adaptation and 21 days after the first E. coli inoculation. The doses of F18 E. coli inoculum were 1010 CFU/3 mL oral dose daily for 3 consecutive days. Whole blood samples were collected from all pigs on d -7, and d 0, 7, 14, and 21 post infection (PI) to measure total and differential blood cell count by complete blood count (CBC) analysis. Supplementation of BAM or CAR increased (P &lt; 0.05) either the percentage or the number of lymphocytes on d 0 before E. coli inoculation. E. coli challenge increased (P &lt; 0.05) white blood cell (WBC) count on d 7 and 21 PI, while supplementation of BAM tended (P &lt; 0.10) to have low WBC on d 7 PI and had lower (P &lt; 0.05) WBC on d 21 PI compared with CON+. Pigs in BAM+ also had lower (P &lt; 0.05) neutrophil count on d 14 PI, pigs fed with CAR had lower (P &lt; 0.05) neutrophil count on d 14 and 21 PI, compared with pigs in CON+. No difference was observed in red blood cell profile among all treatments throughout the experiment. In conclusion, pigs fed with B. amyloquefaciens have similar systemic immune response to pigs in antibiotic group and have relatively lower systemic inflammation caused by E. coli compared with control group.


2019 ◽  
Vol 7 (9) ◽  
pp. 1494-1498
Author(s):  
Fatma Abdelrahman Elzaree ◽  
Manal A. Shehata ◽  
Mohamed Abdel Atti ◽  
Gihan Elzaree ◽  
Ghada M. El-Kassas

AIM: To evaluate hearing in β-thalassemia major patients on iron chelation therapy by using pure-tone audiometry (PTA) and distortion product otoacoustic emissions (DPOAE). SUBJECTS AND METHODS: This cross-sectional, descriptive study was done on (48) diagnosed, 6-18 years old, β-thalassemia major patients who had received at least 3 years iron chelating agent deferoxamine (DFO). We performed PTA, DPOAE testing, and tympanometry for all participants. SPSS was used to analyse data. P < 0.05 was accepted as statistically significant. RESULTS: No significant difference was found between PTA and DPOAE testing in their capability to detect ototoxicity. PTA and DPOAE testing for the detection of ototoxicity in BTM and BTI, kappa values (κ) were found to be 0.516 and 0.459, respectively. The whole mean serum ferritin level was 2,251.3 ng/ml. The mean level was 1,603 ± 1,380 ng/ml in the patients with SNHL and 2,405 ± 1,908 ng/ml in the patients with normal hearing with the statistically significant difference among both groups (p = 0.015). The occurrence of ototoxicity was statistically significant with increasing age (p < 0.001). There was no marked difference as regards gender (p = 0.72). CONCLUSION: Hearing loss is prevailing in patients with β-thalassemia major on iron chelating agents. Therefore, regular hearing evaluations and periodic check-ups after the initiation of chelation therapy are mandatory.


2019 ◽  
Vol 31 (5) ◽  
pp. 704-713 ◽  
Author(s):  
Francesco Dondi ◽  
Kateryna Vasylyeva ◽  
Federica Serafini ◽  
Marta Gruarin ◽  
Roberta Troìa ◽  
...  

Heinz bodies (HBs) are known to interfere with automated hematology in cats, particularly with the white blood cell (WBC) count. We evaluated the influence of feline HBs on the complete blood count (CBC) results obtained using a flow cytometry–based analyzer. We retrospectively selected cats with circulating HBs and reviewed the results of their CBCs, including red blood cell (RBC) indices, basophil/lobularity (Baso) WBC count (WBCB), peroxidase (Perox) WBC count (WBCP), and cytograms. Based on the presence or absence of HB-related artifacts in their Baso cytogram, cats were grouped into Baso-HBs and HBs groups, respectively, for comparison. The WBCB and WBCP were compared to manual counts of WBCs carried out on blood smears at 400× (MC-WBC). We included 32 cats in our study: 9 of 32 were in the Baso-HBs group, and 23 of 32 were in the HBs group. Baso-HBs cats had a significantly increased HB percentage ( p < 0.001), WBCB ( p < 0.001), difference between WBCB and WBCP ( p < 0.001), lymphocyte count ( p < 0.001), mean corpuscular hemoglobin concentration ( p < 0.001), and difference between calculated and measured erythrocyte hemoglobin concentrations ( p < 0.001) compared to HBs cats. In Baso-HBs cats, the WBCB was significantly higher than the WBCP ( p = 0.02); no significant difference was detected between the WBCP and the MC-WBC ( p = 0.88). Evaluation of automated CBC results raised the suspicion of HB-related interference when using a hematology analyzer in cats; hence, blood smear examination remains essential in routine practice.


InterConf ◽  
2021 ◽  
pp. 208-216
Author(s):  
Olena Makharynska ◽  
Ievgeniia Tokareva

The aim of the study was to evaluate “old” (WBC count) and “new” inflammatory biomarkers in hospitalized patients with non-severe community-acquired pneumonia in patients with\without Diabetes Mellitus 2 type. Materials and methods. At inclusion, clinical and laboratory parameters of pneumonia were recorded and the CRB-65 was evaluated. A total of 131 adult patients hospitalized with non-severe CAP were included in this prospective study. Levels of pro-inflammatory (IL-1β, IL-8, TNF-α), anti-inflammatory (IL-10) cytokines and markers of inflammation (CRP, complete blood count) were determined at the beginning of the disease (day O) and in 48-72 hours. Results. The level of leukocytes in CBC was significantly higher in the main group at the beginning of the disease, as well after 10 ± 2 days from day 0 the number of episodes of leukocytosis > 10 · 109 / l was higher (8.5% vs. 2%, respectively, p <0,05). There was no statistically significant difference between the studied inflammatory biomarkers levels in the both groups of patients with and without concomitant diabetes. There was a tendency to a higher number of cases of "recovery" after CAP in the group with CRP levels during hospitalization ≥ 21.8 pg / ml (22% vs. 48%, p <0.1). Conclusion. It can be assumed that such a low dynamics of inflammatory markers is associated with involutive changes in the immune system due to older age of the patients included in the study and the influence of DM on the immune system reactivity in the CAP with concomitant DM 2 type with immune response suppression can also explain absence of significant difference in inflammatory biomarkers concentration in the groups compered in this study.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3796-3796 ◽  
Author(s):  
German R. Stemmelin ◽  
Carlos A. Doti ◽  
Claudia M. Shanley ◽  
Jose M. Ceresetto ◽  
Oscar M. Rabinovich ◽  
...  

Abstract Mild chronic neutrofilia is a frequent reason for hematologic consultation and in 70% of the cases there is no identifiable cause. Objective: to determine if smoking habit could be the etiology for leucocytosis with neutrophilia in smokers with no other pulmonary associated disease. Materials and Methods: We questioned 300 consecutive blood donors from our institution, elaborating a complete record of smoking habit. Immediately before blood donation an automated complete blood count was performed. Leucocytosis was defined as a white cell count above 11 x 109/L, and neutrophilia as a neutrophil count over 7.7 x 109/L. All data is stated as mean value ± 1 SD. Results: we studied 195 (65%) men and 105 (35%) women (n=300). Mean age was 36.5 y.o. (18–69). Fifty-five percent (165/300) smoke or were previous smokers; 8.4% (14/165) of these, did not smoke at the time of this investigation, and only two have quit smoking a year prior to questioning. Mean time of duration of smoking habit was 16.4 years ± 10.6 and the average amount of cigarettes smoked through life (estimated from data referred along different phases of each subject’s history) was 1.26 x 105 ± 1.21 x 105. In the following table results from smokers (previous or actual) vs. non-smokers are compared. Leucocytosis was present in 37/165 (22.4%) of smokers and in 3/135 (2.2%) of non-smokers (p<0.001) and neutrophilia was noted in 19/165 (11.5%) of smokers and in 2/135 (1.5%) of non-smokers (p<0.001). None of the volunteers had a WBC count over 20 x 109/L. A direct association was established for the number of cigarettes smoked and the WBC count and neutrophil count. Discussion: smoking habit affects ciliar movement, inhibits alveolar macrophage function and produces hyperplasia on mucous glands within bronquial walls. These alterations result in entrapment of mucous secretions that will ultimately lead to bacterial colonization. The neutrophilia observed in smokers would be the result of a normal physiologic response to a potential infectious focus. In conclusion, is our understanding that the smoking habit should be considered as a common etiology for mild leucocytosis and neutrophilia. Smokers (n:165) Non-smokers (n:135) p (t-test) WBC x 109/L 9.6 ± 2.1 6.8 ± 1.3 < 0.001 Neutrophils x 109/L 5.7 ± 1.6 3.9 ± 1.0 < 0.001 Hct % 45.9 ± 3.7 45.2 ± 3.6 NS Platelets 207 ± 48.7 206.4 ± 45.8 NS


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5427-5427
Author(s):  
Duran Canatan ◽  
Handan Duman

Abstract Introduction: Iron overload and auto-oxidation of unpaired globin chains is the main cause of oxidative stress in thalassemia. The high levels of MDA in iron overloaded thalassemic patients is the best sample of oxidative stres. Generally decreased iron burden was associated with decreased oxidant damage. In vitro studies were shown that iron chelators such as deferoxamine and deferipron neutrolyse intraselluler free iron and inhibits oxidation. We aimed to show the antioxidant effects of capparis ovatta and deferasirox in thalassemic patients. Material-Method: A total number of 40 thalassemia major patient aged between 7–30 years, who has been taken regular transfusion (15 cc/kg/month to maintain Hb &gt;10 gr/dl) and chelation therapy (30 mg/kg/day ICL-670) are involved. They were separated into two groups as control and study group randomly. Both study and control group were followed by transfusion and chelation therapy. In study In addition study group have been taken capparis marmelade at the breakfast with a dose of a dessert-spoon (12.5 gr) younger than 10 years and a soup-spoon (25 gr) older than ten years for 6 months. Hematological and biochemical parameters, ferritin at every month and oxidative-antioxidant status (MDA, CAT, Gpx, SOD) were measured at the beginning and at the end of the study. Results: Serum ferritin and MDA levels declined significiantly in both groups (for ferritin; control group p= 0.00; study group p=0.00) during the study but a much more decrease occured at MDA levels in the cappari given group (p=0.02). There was no statistically significant difference between the groups at the initial and last SOD CAT, GPX, SOD levels. Further more in the study group a significant decrease in liver function tests was occured (AST p= 0.05, ALT p= 0.01). Conclusion: Our findings suggest that combination of capparis with deferasirox may be usefull for decreasing the effect of oxidative damage.


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