1.Microcytosis in children and adolescents with the sickle cell trait in Basra, Iraq
Rawshan Zuhair JABER ; Meàad Kadhum HASSAN ; Sadeq Khalaf AL-SALAIT
Blood Research 2019;54(1):38-44
BACKGROUND: Microcytic anemia, the most common form of anemia in children and adolescents, is a heterogeneous group of diseases that is acquired or inherited. We assessed the frequency and causes of microcytosis in children and adolescents with the sickle cell trait (SCT). METHODS: This descriptive study included 95 subjects (49 males and 46 females) with SCT who attended Basra Center for Hereditary Blood Diseases for evaluation. Investigations included complete blood count, high performance liquid chromatography, capillary electrophoresis, and measurement of serum ferritin and transferrin levels. RESULTS: SCT subjects had a low hemoglobin (Hb) concentration (9.79±1.75 g/dL), low mean corpuscular volume (MCV, 67.43±9.22), low mean corpuscular Hb (21.15±3.64), and a normal red cell distribution width (RDW, 14.00±2.30). Among 95 SCT subjects, 81 (85.26%) had microcytosis, 12 (12.63%) had normal MCV, and 2 (2.11%) exhibited macrocytosis. Sixty-three (77.78%) SCT subjects with microcytosis were iron deficient, and 18 (22.22%) had normal iron levels. The mean serum ferritin and HbA2 levels were significantly lower, while the RDW, sickle Hb, and serum transferrin levels were significantly higher in patients with microcytosis and iron deficiency compared to non-iron deficient subjects (P<0.05). Correlation coefficients did not reveal a significant association between the MCV and iron status of SCT subjects (P>0.05). CONCLUSION: Despite the frequent occurrence of iron deficiency in SCT subjects, co-inheritance of alpha-thalassemia seemed to be the cause of low MCV in non-iron deficient individuals with microcytosis. Genetic analysis is required to understand the genetic basis of this phenomenon.
Adolescent
;
alpha-Thalassemia
;
Anemia
;
Blood Cell Count
;
Child
;
Chromatography, Liquid
;
Electrophoresis, Capillary
;
Erythrocyte Indices
;
Ferritins
;
Hematologic Diseases
;
Humans
;
Iraq
;
Iron
;
Male
;
Sickle Cell Trait
;
Transferrin
2.Effective sickle hemoglobin reduction by automated red cell exchange using Spectra Optia in three Emirati patients with sickle cell disease before allogeneic hematopoietic stem cell transplantation.
Seung Jun CHOI ; Hanwool CHO ; Ki Seong EOM ; Jong Wook LEE ; Yonggoo KIM ; Jihyang LIM
Blood Research 2018;53(4):325-329
No abstract available.
Anemia, Sickle Cell*
;
Hematopoietic Stem Cell Transplantation*
;
Hematopoietic Stem Cells*
;
Hemoglobin, Sickle*
;
Humans
4.Beating the odds: A case report on the successful management of a non-immune hydrops fetalis due to hemoglobin Bart's disease.
Javier Maria Jane Ellise S. ; Cheng Maria Rosario C. ; Abat Marinella Agnes G.
Philippine Journal of Obstetrics and Gynecology 2016;40(3):23-28
Hemoglobin Bart's hydrops fetalis, characterized by a deletion of all four a-globin genes is the most severe and lethal form of Thalassemia disease. Mortality rate usually ranges from 60-100% of cases. Given the poor overall prognosis, most countries resort to pregnancy termination or expectant management as the only options to offer affected pregnancies.
This paper presents a case of the successful management of a primigravid, diagnosed with hydrops fetalis at 29 4/7 weeks age of gestation. She delivered successfully to a live, preterm, baby boy who was later found out to have hydrops fetalis due to Hemoglobin Bart's disease, and currently, continues to thrive past eight months of age.
This report aims to improve the clinicians' knowledge regarding the work up and management of pregnant patients diagnosed with hydrops fetalis, and increase the clinician's awareness on the epidemiology, importance of targeted screening, and diagnosis of Alpha-Thalassemia in Filipino patients.
Human ; Female ; Adult ; Alpha-thalassemia ; Hydrops Fetalis ; Hemoglobin Bart's ; Hemoglobin, Sickle ; Anemia, Sickle Cell ; Prognosis
5.Salmonella Typhi Osteomyelitis in a Non-sickle Cell Patient: Three Cases Report
Kyoo Ho SHIN ; Kyung Hwan YOUN ; Jun Seop JAHNG
The Journal of the Korean Orthopaedic Association 1996;31(5):1228-1233
Salmonella osteomyelitis is uncommon and it often develops in patients with sickle cell anemia and other hemoglobinopathy. Especially, osteomyelitis caused Salmonella typhi in a nonsickle cell patients is a very rare event. We report three cases with review of literature.
Anemia, Sickle Cell
;
Hemoglobinopathies
;
Humans
;
Osteomyelitis
;
Salmonella typhi
;
Salmonella
6.A family study of HbS in a Malay family by molecular analysis.
Hafiza, Alauddin ; Noor, Hamidah Hussin ; Noor, Farisah A Razak ; Azlin, Ithnin ; Ainoon, Othman
The Malaysian Journal of Pathology 2010;32(2):137-41
Sickle cell disease (SCD) is an inherited red cell disorder, characterized by the tendency of haemoglobin S or sickle haemoglobin to polymerize and assume a characteristic sickle shape. Molecular analysis has been the mainstay of detection method when confirmation is required. Previously a polymerase chain reaction (PCR)-based restriction enzyme analysis was used for this purpose. A simple bidirectional allele-specific amplification, recently described by Waterfall in 2001 was used to detect the GAG --> GTG mutation on codon 6 of the beta globin gene. Two sets of primers for the mutant and the wild type alleles were used in a single PCR reaction to amplify the regions of interest. The resultant PCR products will produce two fragments at 517 and 267 base pair (bp) respectively. This report highlights the investigations for SCD in the family of a 16-year old girl with recurrent painful crisis affecting the lower limbs whereby the family members are asymptomatic for the disease. Her haemoglobin electrophoresis at an alkaline pH showed dense bands at the HbS and HbF regions, while her father and two sisters had bands at HbS, HbF and HbA. The PCR analysis showed that she was homozygous for the mutation by the presence of only one band at 267 bp fragment, while the father and her sisters were heterozygotes, with the presence of two bands at 267 as well as 517 bp fragments. DNA sequencing of the sample confirmed the mutation. In conclusion, this case report highlighted the simple and cheap yet practical method for molecular confirmation of the presence of HbS gene in subjects with homozygous or heterozygous state of the condition.
Anemia, Sickle Cell/*diagnosis
;
Anemia, Sickle Cell/*genetics
;
Base Sequence
;
Fathers
;
Hemoglobin, Sickle/*genetics
;
Heterozygote
;
Homozygote
;
Malaysia
;
Mutation
;
Nucleic Acid Amplification Techniques
;
Pedigree
;
Polymerase Chain Reaction
;
Siblings
7.Translocation Renal Cell Carcinoma t(6;11)(p21;q12) and Sickle Cell Anemia: First Report and Review of the Literature.
Damien CHASTE ; Emmanuel VIAN ; Gregory VERHOEST ; Pascal BLANCHET
Korean Journal of Urology 2014;55(2):145-147
Translocation renal cell carcinoma (RCC) is a family of rare tumors recently identified in the pediatric and young adult population. We report the first case of a young woman from French West Indies with sickle cell anemia who developed a translocation RCC t(6;11)(p21;q12). Usually people with the sickle cell condition are known to develop renal medullary carcinoma (RMC). To our knowledge, this is the first case described in the literature of a translocation RCC associated with sickle cell disease. Here we discuss the relation between translocation RCC, RMC, and sickle cell disease.
Anemia, Sickle Cell*
;
Carcinoma, Medullary
;
Carcinoma, Renal Cell*
;
Female
;
Hemoglobin, Sickle
;
Humans
;
Kidney Neoplasms
;
West Indies
;
Young Adult
8.A Case of Sickle Cell Anemia with a Lack of High Frequency Red Blood Cell Antigen.
Hyun Ji LEE ; Kyung Hwa SHIN ; Hyerim KIM ; Seri JEONG ; Seom Gim KONG ; Hyung Hoi KIM
Korean Journal of Blood Transfusion 2018;29(2):188-193
Patients with sickle cell anemia are chronically transfused. Therefore, it is important to prevent the alloimmunization of RBC antigens. The authors identified a high frequency antigen-negative blood group in patients with sickle cell anemia. As the number of foreigners residing in Korea is increasing, it is necessary to know what to consider when transfusing blood to sickle cell anemia patients. Patients with sickle cell anemia should be informed of the exact blood group type using extended RBC typing to confirm the ABO, Rh, Kell, and Duffy blood types at diagnosis or before the first blood transfusion. Extended matched blood transfusion can reduce the risk of alloimmunization of RBC antigens.
Anemia
;
Anemia, Sickle Cell*
;
Blood Transfusion
;
Diagnosis
;
Duffy Blood-Group System
;
Emigrants and Immigrants
;
Erythrocytes*
;
Humans
;
Korea
9.Spinal versus general anesthesia for Cesarean section in patients with sickle cell anemia.
Mohamed H BAKRI ; Eman A ISMAIL ; Gamal GHANEM ; Mahmoud SHOKRY
Korean Journal of Anesthesiology 2015;68(5):469-475
BACKGROUND: Sickle cell anemia (SCA) increases the rate of maternal and fetal complications. This pilot study was designed to compare the maternal and fetal outcomes of spinal versus general anesthesia (GA) for parturients with SCA undergoing cesarean delivery. METHODS: Forty parturients with known SCA scheduled for elective Cesarean delivery were randomized into spinal anesthesia (n = 20) and GA groups (n = 20). Perioperative hemodynamic parameters were recorded. Postpartum complications were followed up. Opioid consumption was calculated. Blood loss during surgery and the number of patients who received intraoperative or postpartum blood transfusion were recorded. Patient satisfaction with the type of anesthesia was assessed. The Apgar score at 1 and 5 min, neonatal admission to the intensive care unit, and mortality were also recorded. RESULTS: Blood loss was significantly higher in the GA than spinal group (P = 0.01). However, the number of patients who received an intraoperative or postpartum blood transfusion was statistically insignificant. Significantly more patients developed intraoperative hypotension and bradycardia in the spinal than GA group. Opioid use during the first 24 h was significantly higher in the GA than spinal group (P < 0.0001). More patients had vaso-occlusive crisis in the GA than spinal group without statistical significance (P = 0.4). There was one case of acute chest syndrome in the GA group. No significant differences were observed in postoperative nausea and/or vomiting, patient satisfaction, or hospital length of stay. Neonatal Apgar scores were significantly better in the spinal than GA group at 1 and 5 min (P = 0.006 and P = 0.009, respectively). Neonatal intensive care admission was not significantly different between the two groups, and there was no neonatal mortality. CONCLUSIONS: Spinal anesthesia may have advantages over GA in parturients with SCA undergoing Cesarean delivery.
Acute Chest Syndrome
;
Anemia, Sickle Cell*
;
Anesthesia
;
Anesthesia, General*
;
Anesthesia, Spinal
;
Apgar Score
;
Blood Transfusion
;
Bradycardia
;
Cesarean Section*
;
Female
;
Hemodynamics
;
Humans
;
Hypotension
;
Infant
;
Infant Mortality
;
Infant, Newborn
;
Intensive Care Units
;
Intensive Care, Neonatal
;
Length of Stay
;
Mortality
;
Patient Satisfaction
;
Pilot Projects
;
Postoperative Nausea and Vomiting
;
Postpartum Period
;
Pregnancy
;
Vomiting
10.Recurrent Priapism Associated with Splenectomy.
Hyung Il LEE ; Dae Gon KIM ; Young Jin SEO ; Dal Bong HA ; Kyung Seop LEE
Korean Journal of Urology 2002;43(8):720-722
Priapism is a prolonged erection of the corpus cavernosum that is unaccompanied by sexual desire. It has been classified into 2 main groups: primary (idiopathic) or secondary. The common conditions associated with a secondary priapism include sickle cell anemia, trauma, leukemia, a cancerous invasion of the penis, drugs, alcohol ingestion, various venous thromboembolic diseases, intravenous fat for parenteral nutrition, and other less common disorders.1 A splenectomy has not been previously identified as a predisposing factor except for a report of 3 cases of priapism who underwent a splenectomy.2 We experienced a case of recurrent priapism following a splenectomy that developed in 30 years old man.
Adult
;
Anemia, Sickle Cell
;
Causality
;
Eating
;
Humans
;
Leukemia
;
Male
;
Parenteral Nutrition
;
Penis
;
Priapism*
;
Splenectomy*