Open Journal of Blood Diseases
ISSN / EISSN : 21643180 / 21643199
Current Publisher: Scientific Research Publishing, Inc. (10.4236)
Total articles ≅ 113
Latest articles in this journal
Published: 22 January 2020
Open Journal of Blood Diseases, Volume 10, pp 1-11; doi:10.4236/ojbd.2020.101001
Multicolor flow cytometry (MFC) is essential to the diagnosis of non-Hodgkin lymphoma (NHL). In our institution, MFC specimens are submitted by pathologists or an ordering provider. As endoscopy has revolutionized the ability to biopsy the gastrointestinal (GI) tract, our lab increasingly receives provider-submitted, endoscopically-acquired GI biopsies (PEGIB) for MFC analysis. This study evaluates the clinical utility of MFC performed on PEGIB and proposes a new testing algorithm to enhance the pathology team’s role in MFC test utilization. Fifty-five archival PEGIB MFC cases were identified and histories were reviewed. MFC was non-contributory to the overall diagnosis in 85% of PEGIB. Retroactively implementing an algorithm that used PEGIB permanent section screening to triage the 55 archival cases resulted in the appropriate identification of 100% of specimens whose diagnosis would have benefitted from MFC analysis, and the optimization of test utilization by decreasing unnecessary MFC studies.
Published: 22 January 2020
Open Journal of Blood Diseases, Volume 10, pp 12-21; doi:10.4236/ojbd.2020.101002
EmoTypeSC™ test is a new cheap, faster, and appropriate screening method for neonatal diagnosis of sickle cell disease. The literature reports a few cases of its applicability. This study extends the cases study and reviews the available literature. The sample consisted of 99 subjects, including 87 newborns (36 girls and 51 boys; 1.9 - 4.9 kg BW) sampled among 566 babies bone at six hospitals in Kisangani city (Democratic Republic of Congo) during March-April 2019; height infant-adolescents (<18 years); and four adults. Duplicate blood samples of 75 newborns, spotted on filter paper, were transferred to Liège in Belgium for LC-MS test confirmation. Of 99 subjects, 74.74% tested HbAA, 24.26% HbAS and 1% HbSS. The prevalence of HbAS compared to the HbAA phenotype was 15/60 (20%) by HemoTypeSC™ and 14/61 (18.7%) by LC-MS. The concordance between the two methods was 98.3% or a discordance of 1.7%. The findings support the validity of the HemoTypeSC™ test as a sensitive, specific point of care test, cheap and reliable for poor African populations.
Open Journal of Blood Diseases, Volume 10, pp 55-60; doi:10.4236/ojbd.2020.102007
Open Journal of Blood Diseases, Volume 10, pp 37-40; doi:10.4236/ojbd.2020.102004
Objective: In Japan, red blood cell (RBC) solution is usually transported by car from a medical institution to the patient’s house for home transfusion. However, there are no regulations for transporting blood by car in the medical setting. Therefore, we assessed and compared the methods (containers) used for transporting the RBC solution by car. Materials and Methods: Irradiated RBC solution samples (280 mL) supplied by the Japan Red Cross Society were each divided into two bags. The quality of blood transported by car (1 - 2 hours) in an active transport refrigerator (ATR) (control group) was compared with that transported in a cooler, or styrofoam box (study group). We tested the hemolytic effects of transportation by car, storage, and filtration through a transfusion set on the lactate dehydrogenase (LD) levels in the RBC solutions. Results: Post-filtered LD levels were significantly higher in the RBC solutions transported in a cooler-box with inadequate temperature control when compared to those transported in an ATR with optimal temperature control. However, under conditions of optimal temperature control, the post-filtered LD levels were comparable in the control and study (both cooler and styrofoam boxes) group RBC solutions. Conclusion: Temperature management is critical for the maintenance of the quality of the RBC solution transported by car.
Open Journal of Blood Diseases, Volume 10, pp 48-54; doi:10.4236/ojbd.2020.102006
Hemophagocytic lymphohistiocytosis (HLH), which was first described in 1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease. HLH is characterized as cytokine release syndrome which is caused by excessive but non-malignant activation of macrophages and/or histiocytes in bone marrow and other reticuloendothelial systems. EBV-HLH is the most common type of infection-associated HLH, has a high mortality rate without prompt and effective treatment. A previous study showed that the one-year mortality rate of EBV-HLH patients is 75%. Here we report a case of EBV-associated hemophagocytic syndrome in adult, and the lessons from the treatment process. Through this case, we think that for EBV-related HLH, EBV-DNA should also be monitored in addition to hemophagocytosis-related indicators during treatment. In addition, DEP regimen may not be suitable for patients who have received at least partial response, because impaired immunological functioning may lead to EBV and hemophagocytic re-activity.
Open Journal of Blood Diseases, Volume 10, pp 41-47; doi:10.4236/ojbd.2020.102005
Background: There are no consensual diagnosis criteria to make diagnosis of sickle cell intrahepatic cholestasis and identify its 2 variants. Here we delineate the frequency of the complication in its severe form and describe its clinical features. Methods: We included in the study all the patients with sickle cell disease who were presenting fever, jaundice, pain in the upper right quadrant and filling up the biological criteria of the severe sickle cell intrahepatic cholestasis (serum direct bilirubin ≥ 78.5 mg/dL). Patients with evidence of viral hepatitis or gallstones were not included in the study. Results: Sixty-two patients with an average age of 21.5 years (range 18 and 25 years) meet the inclusion criteria, minimum serum direct bilirubin: 82 versus 528 for the maximum. The liver cytolysis was major and ranged from 310 to 1550 UI/L. Forty-nine patients (83.9%) did not develop any organ failure. The symptoms and biological abnormalities resolved with supportive treatment within 7 days. The global lethality was 16.1% associated with liver failure. Conclusion: Severe sickle cell intrahepatic cholestasis is more frequent than we thought and was mainly represented by borderline clinical feature. Early intervention may reduce the progression to organ failure stage and ultimate death.
Open Journal of Blood Diseases, Volume 10, pp 23-36; doi:10.4236/ojbd.2020.102003
In the Democratic Republic of the Congo, the sickle cell trait carriage is estimated at 25%. Routine neonatal screening is not a common practice, leading to a very late diagnosis. In this study, the screening of pregnant women was assessed as well as their attitudes. This is an analytical cross-sectional study conducted in 245 pregnant women, sampled by convenience in four hospitals in Kisangani city (Democratic Republic of Congo) and screened using the sickle SCAN® test, from February 1 to July 31, 2019. The sensitivity and specificity of the latter were determined using liquid chromatography coupled with mass spectrometry as the gold standard. The attitudes of 240 pregnant women without previous screening history were assessed upon the announcement of the results. The sensitivity of screening for hemoglobin (Hb) AA and Hb AS was 96.69% and 98.39%, respectively; while the specificities were 99.43% and 96.32%, respectively. The Kappa coefficient (κ) was excellent. Concerning attitudes, Hb SS pregnant women and 55.17% of AS pregnant women worried when the results relating to their hemoglobin status were announced. The sickle SCAN® test was found reliable for sickle cell disease screening in Kisangani. The announcement of the results, mainly positive, raises worry among pregnant woman. Therefore, we recommend the involvement of a clinician psychologist for pre-screening counselling and for results announcement, as well as early newborns and unmarried teenage girls screening.
Open Journal of Blood Diseases, Volume 9, pp 41-46; doi:10.4236/ojbd.2019.92005
Introduction: Angioimmunoblastic T Cell Lymphoma (AITL) is a well-recognized subtype of peripheral T cell lymphoma. It occurs predominantly in the lymph nodes with presence of systemic symptoms and carries a dismal prognosis. AITL accounts for about 1% - 2% of all cases of non-Hodgkin lymphoma. Case presentation: A 59-year-old gentleman of Chinese ethnicity with no prior medical illness presented to Tengku Ampuan Afzan Hospital with a two-month history of fever, anorexia, unintentional weight loss and generalized lymphadenopathies. Physical examination revealed diffuse lymphadenopathies involving the cervical, axillary and inguinal regions bilaterally. He had hepatosplenomegaly. An excisional biopsy of the cervical and inguinal lymph nodes was compatible with AITL. The bone marrow biopsy demonstrated disease infiltration. He was treated with 6 cycles of Etoposide-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) induction chemotherapy followed by consolidation high dose therapy-autologous stem cell transplant (HDT-ASCT). He achieved complete remission on 18-Fluoro- deoxyglucose Positron Emission Tomography (18-FDG-PET) imaging. His bone marrow biopsy showed disease clearance. Conclusion: The diagnosis of AITL remains challenging and often poses a dilemma to clinicians and lymphoma pathologists. The natural history of AITL remains very variable with many of them relapsing subsequently despite achieving prior complete remission.
Open Journal of Blood Diseases, Volume 9, pp 60-76; doi:10.4236/ojbd.2019.93007
Sickle Cell Disease (SCD) is one of the most common genetic diseases in the world. It is associated with oxidative stress which occurs as a result of HbS unstable character causing a rise in the formation of free radicals. The aim of this study was to determine some antioxidant enzymes activities among patients with SCD. We investigated the superoxide dismutase (SOD), and glutathione peroxidases (GPx) levels among 60 children aged 1 - 14 years with SCD. Twenty-two age-matched non-SCD children served as control. The study subjects were divided into two groups; steady state A (n = 30) and vaso- occlusive crisis (VOC) B (n = 30). The SOD, and GPx levels were significantly lower among the SCD subjects compared to controls (p = 0.000). There were no statistically significant differences in the SOD and GPX levels between sickle cell disease patient in steady state (A) and those in crisis (B) (p = 0.998 and 0.555) respectively. There was a statistically significant difference between the SOD and GPX levels between sickle cell disease patient in steady state (A) and non-sickle cell controls (p = 0.005 and 0.000) respectively as well as between sickle cell disease patient in VOC (B) and non-sickle cell controls (p = 0.000). There were no statistically significant differences in the SOD and GPX levels of sickle disease subjects based on age, gender, maternal level of educational attainment, occupational group and income (p = 0.629 and 0.476; p = 0.382 and 0.417; p = 0.450 and 0.314 and p = 0.397 and 0.762 and p = 0.553 and 0.929) respectively. There were no statistically significant differences in the SOD and GPX levels of sickle disease subjects of Hausa/Fulani extraction versus Yoruba (p = 0.714 and 0.856), between Hausa/Fulani extraction versus Igbo (0.917 and 0.486) and between Yoruba extraction versus Igbo (p = 0.740 and 0.965) respectively. This study confirms that SCD children have lower values of antioxidant enzymes compared to controls. SOD and GPX levels in sickle cell disease patient in steady state and vaso-occlusive crisis are significantly lower compared that of non-sickle cell controls. Patients with SCD may benefit from substances with antioxidant properties which can potentially reduce the complications associated with the disease.
Open Journal of Blood Diseases, Volume 9, pp 47-59; doi:10.4236/ojbd.2019.93006
Background: Mature red blood cells lack protein synthesis and are unable to restore inactivated enzymes, damaged cytoskeleton and membrane proteins. An oxidation breakdown of band 3 is probably part of the mechanism leading to the generation of a senescent cell antigen. This specific signal serves for the clearance of RBCs by inducing the binding of autologous IgG and C3, leading to phagocytosis. In addition, phosphatidilserin molecules appear in the outer membrane and the CD47 expression diminishes. Methods: Erythrocytes of different ages from whole blood were studied by flow cytometry analysing light scatter proprieties, binding of autologous IgG, C3 complement deposits, externalization of phosphatidylserine and CD47 expression. Dot-plot analysis based on forward scatter versus side scatter parameters showed two RBCs populations of different sizes and density. RBCs were further incubated with Alexa 488 IgG, APC-anti-C3, PE-annexin-V and PE-CD47. The comparison of the values obtained for the different variables studied in SeRBC and YRBC populations was carried out by the Student t-test for matched samples or by the Wilcoxon test (after verification of the normality assumption). Results: The percentage of IgG and C3 positive cells was significantly higher in senescent red blood cells population. The fraction of annexin-V positive RBCs was also larger in SeRBCs while the CD47 expression was lower in this population. Conclusions: These results indicate that flow cytometry allow differenciation of erythrocytes populations of different ages, turning this tool into an useful alternative option to study erythrocyte aging process. These findings will contribute to a better understanding of the process and mechanisms involved in erythrocyte senescence process.