Update on acute promyelocytic leukemia in the pediatric population

Acute promyelocytic leukemia (APL) has shown differences in incidence around the world, it has a characteristic molecular, pathogenic and clinical development mechanism. Despite being a low incidence acute myeloid leukemia (AML) compared to other AML and having a good response to therapy, it continues to be of interest due to the toxicities of its current treatment, to mutations that have shown resistance to those drugs, and to the few possibilities of alternative treatment with all-trans retinoic acid (ATRA) and anthracyclines in the pediatric population. Furthermore, the description of new mutations that originate APL produced by LPA generates new interest in studies that may shed light on their effects on the disease’s incidence, prognoses, and response to treatment. This article aims to review different studies that describe findings of APL in terms of incidences, treatments, side effects, survival, especially in the pediatric population. Different studies propose the use of ATRA - arsenic trioxide (ATO) as the first line of treatment, however, few have been carried out in the pediatric population and the use of ATO is not recommended in children under 5 years of age. On the other hand, the differentiation syndrome (DS) has been d

Dubin Johnson Syndrome associated with defective ristocetin – induced platelet agglutination

Background: This is the first reported case of platelet aggregation defect in association with Dubin-Johnson Syndrome. Case presentation: We reported a 14 months old female infant with recurrent attacks of mild to moderate ear and nose bleeding and moderate amount of subarachnoid hemorrhage with ristocetin induced platelet agglutination other than Von Willebrand Factor disease and Bernard–Soulier syndrome. The patient also had fluctuating bilirubin level (Maximum of 15 mg/dl) and remaining liver function tests were normal; the patient diagnosed as Dubin Johnson Syndrome by liver biopsy. Conclusions: Dubin-Johnson Syndrome may be associated with platelet aggregation defect.

Evaluation of serum Hepcidin level and Iron profile among Sudanese patients with anemia of end stage kidney disease

ackground: Anemia of chronic disease is anemia found in certain chronic disease state, is typically marked by the disturbance of iron homeostasis or hypoferremia. This condition leads to shortage of iron for hemoglobin synthesis but the iron storage in bone morrow is left undisturbed. Patients with chronic kidney disease are usually anemic because of defective erythropoeisis and inflammation. Materials and methods: Some of red blood cell profile (Hb, PCV, RBCs count and RBCs indices) were determined by the automated Hematology Analyzer and Cobas e 411 was used to determine the levels of serum iron, ferritin, TIBC, and transferrin saturation percentage. Enzyme – Linked immunoassay (ELISA) was used to determine the level of hepcidin. Results: The results show the mean of the RBCS profile (RBCs count, Hb, PCV) (3.353±88cell/l, 10.62±2.4g/dl, 32.59±6.82%) in patients with ACKD Vs (4.048±0.47cell/l, 12.52±1.57g/dl, 37.92±4.79%) in control groups P.value (0.000, 0.000, and 0.000) respectively. Serum hepcidin levels higher in patients with ACKD compared with healthy controls mean (161.55±29.8ng/ml Vs 82.05±13.4ng/ml. P. value (0.000). The mean value of the iron profile, S. iron, S. ferritin and TS % (61.353±29,8ug/dl, 195.3.62±19.4ng/ml, 21.59±12.82%) in patients with ACKD Vs (82.048±0.47ug/dl, 80.52±1.57ng/ml, 28.92±4.79%) in control groups P.value (0.000, 0.000, and 0.000) respectively. Conclusion: In the present study there is significant association between CKD and RBCS profile (RBCs count, Hb, PCV). The hepcidin levels were significantly higher in patients with ACKD compared with healthy controls. The Statistical significant differences showed in the comparison between the study variables (RBCs profile, Iron profile, hepcidin level) and the end stage of CKD (dialysis dependent), in the RBCs count, Hb, PCV, S. iron, S.ferritin, TIBC. TS %, hepcidin level.

Diagnostic dilemma and challenges of granulocytic sarcoma: a review

The maleficent tumour granulocytic sarcoma is an unusual cancer indite of progressive myeloid precursor cells. It is a Chloroma basically present at an extramedullary location spread through immature granulocytic cells. It can affect any part of the body but the primary site is soft tissues, outside bone marrow, lymph nodes. The root cause may be many factors i.e. genetic, environmental, carcinogens etc. This proliferative type of cancer is sometimes misdiagnosis in contradictory to other acute/chronic tumours. For a histopathologist, it is a challenge for an accurate diagnosis. The tissue biopsy is the known diagnostic technique used since days in cancer diagnosis. To address this review, our main focus is to investigate, analyse the diagnostic limitations or research gaps in granulocytic sarcoma recognition at the clinical level and what can be the possibilities that have developed recently in tumour detection at an early stage. Next, we will try to develop a hypothesis from pinpoint methods used in it as molecular diagnosis, immune-histochemical biomarkers, flow cytometry (FACS) for confirmation of sarcoma so that specificity and sensitivity can be well examined. The prognosis/diagnosis in myeloid tumour detection depends on key factor innovation and medical devices and that should be filled in research and so a gold standard diagnostic kit should be developed.

Seroprevalence of markers of transfusiontransmissible diseases in positive blood donors detected in routine screening in the Blood Bank of Hospital Carlos Luis Valverde Vega, Costa Rica. Period 2015 – 2019

Blood is one of the fundamental tissues of our body. Blood donation allows blood banks to meet hospital needs for blood components. Blood transfusion is an activity that carries a risk, originated by a series of causal actions derived from the individual characteristics of donors and patients. Blood components are biological products and as such, they can never be exempt from the risk of transmitting infectious diseases, such as hepatitis B, HIV, syphilis, among others. Thanks to the investigation of the donor database of the Blood Bank of Hospital Dr. Carlos Luis Valverde Vega and the annual statistics of the blood bank, the statistics referring to donations were obtained, according to the type, grade of acceptance and as well as the prevalence of mandatory screening infectious diseases in the blood bank, between the years 2015 and 2019, with the respective authorization of the corresponding scientific ethics committee. This study reported a prevalence for Anti HB Core of 0.99%, 0.14% for HBsAg, 0.11% for syphilis, 0.05% for hepatitis C, 0.02% for HIV and 0% for the disease de Chagas and HTLV I / II. By using the bases of this work, future analyzes with a similar premise may be developed in different blood banks of the different health centers in our country.

β-globin variants present in Western Sudan

Objective: A variety of observations of the hemoglobin D (Hgb D) phenotype has occurred in association with family studies of patients with sickle cell disease. Very little is known about the occurrence and prevalence of the Hgb D variant and its impact on blood profiles among Sudanese. This study was aimed at determining the percentage of Hb D in North Darfur State, West Sudan, whose population has been shown to have hemoglobin-S (Hb S) disease. Methods: From December 2017 to August 2018, this descriptive community-based investigation was conducted. Six hundred and sixty-six (666) people were randomly selected to participate in this study. With each participant's there was verbal consent. A questionnaire was designed to collect personal details. 5 mL of venous blood was gathered in EDTA containers. The Hb D variant was checked using Sebia Minicap Automated Capillary Electrophoresis System- USA and frequency was calculated using version 21.0 of the software package for social science (SPSS). Result: The prevalence of Hb variants was as follows: AD=0.6%, SS=2%, AS=10.5 %, and AA=86.9%. Conclusion: The prevalence of Hb D variant was 4 (0.6 %) in 666 participants from four western Sudanese tribes, beside reported of Hb SS and Hb AS.

Evaluation of hemolysis during storage of red blood cell concentrates processed by centrifugation and settling method by simple gravity in Burkina Faso

Background: Hemolysis is one of the red blood cell concentrates (RCCs) storage concerns. In Burkina Faso, hemolysis rate was not routinely assessed in RCCs. Our study aimed at assessing the degree of hemolysis in RCCs processed by centrifugation (centrifuged RCCs) and whole blood settling by simple gravity (Sedimented RCCs) in Burkina Faso. Methods: We conducted a cross-sectional comparative study on 46 prepared by centrifugation and 46 prepared by sedimentation, matched on their collection date and initial volume of whole blood. The hemolysis percent was measured on Days 0, 7, 14, 21, 28 and 32. Results: In the centrifuged RCCs, the hemolysis percent on D0 was 0.232% versus 0.199% for the sedimented RCCs (p = 0.046). At D32, the average hemolysis percent was 835% for the sedimented RCCs and 0.779% for the centrifuged RCCs (p = 0.042). The degree of hemolysis increased gradually between D0 and D32 with an average increase of 0.120% for centrifuged RCCs and 0.116% for sedimented RCCs. Conclusion: The degree of hemolysis at D32 in both centrifuged RCCs and sedimented RCCs falls below standards. Therefore, it necessary to revisit blood components processing procedures, focusing on the centrifugation parameters and the handling conditions.

Hypothesis: disodium EDTA and vitamin C combination therapy for COVID -19

COVID.19 Pandemic represent major problem facing research community worldwide. Based on information mentioned in book entitled; COVID-19: Man-made pandemic, Lead AND Cadmium mutate influenza virus produce: SARS COV-2 we suggest Disodium EDTA Vitamin C combination therapy successful protocol to overcome this lethal health condition. This protocol need pre-clinical experiments to a prove it.

How’s going on the T-Cell Brazil project

Lymphomas are a large, heterogeneous and infrequent group of neoplasms. Peripheral t-cell lymphomas (PTCL) is still rarer, and it makes up about 10-15% of lymphoid malignancies. Its prognosis is poor and remains a challenge. In April 2017, the first Brazilian collaborative effort was created to collect data from T-cell Lymphoma patients diagnosed in the five distinct macro-regions of Brazil. To better understand PTCL, according to the revised WHO-2017 classification, excluding Mycosis fungoides, Sézary syndrome, and chronic Lymphoproliferative disorders, all histological subtypes diagnosed from January 2015 to December 2022 will be enrolled. Tissue biopsied, immunophenotypic markers from consecutive patients at each site will be reviewed by panels of expert hematopathologists every year. So far, 381 cases were enrolled by 32 Brazilian centers, most of them are from the Southeast and South region. Twenty-two cases were not evaluable due to lack of complete data; hence, 359 were analyzed. The overall survival at 24-month was 52% (95% CI: 46-58%) and progression-free survival 37% (95% CI: 31-43%) with a median time of follow-up of 10 months (0.2-64). T-cell Brazil Project has many challenges to overcome, mainly due to its vast territory and its disparity. However, we already have another sixteen centers waiting for approval in their Ethics Committees. It was created a network around the country, mainly among the pathologists, with educational goals and exchange of experiences. It is crucial to obtain knowledge of local epidemiology to optimize resources, design clinical trials, and identify minority entities, contributing to increasing the quality o

Actionable assessment of iron stores in blood donors

Before blood donation, blood banks screen potential donors for low hemoglobin or hematocrit, usually in blood drops obtained through finger prick. Although different technologies exist for this, including a couple of non-invasive systems, all detect anemia and prevent that anemic people give blood. However, a reasonable number of people bear sideropenia without anemia, and should not donate blood either. Nowadays, they do. To understand this issue better, to identify these invisible unfit blood donors, and to design cost-effective ways to detect and protect them, we evaluated erythrocyte zinc protoporphyrin and serum ferritin in a cohort of 315 blood donors. In addition, we evaluated the number of blood donors bearing iron overload, invisible as well among donors, and discuss the importance of the extension of blood banks as epidemiological surveillance centers for iron metabolism disorders.