scholarly journals ALLOIMMUNIZATION IN PATIENTS WITH SICKLE CELL DISEASE AND THALASSAEMIA: EXPERIENCE OF SINGLE CENTRE FROM OMAN

2017 ◽  
Vol 9 (1) ◽  
pp. e2017013 ◽  
Author(s):  
Anil Pathare ◽  
Salam Alkindi
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Supportive Care ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Significant Risk ◽  
Red Cell ◽  
Cell Disease ◽  
Single Centre ◽  
Screening And Identification

Background: Blood transfusion is an integral part of the supportive care for patients with sickle cell anemia and thalassaemia. The hazard of red cell alloimmunization, however, is one of the main complications of this therapy. Objectives: The aim of this study was to evaluate the incidence of red cell alloimmunization in Omani patients with sickle cell anemia and thalassaemia. Methods: This study included 262 patients whose historical transfusion records were available. One hundred and twenty-nine patients with thalassaemia who were attending the day care unit for regular transfusions, and 133 sickle cell anemia patients admitted at our hospital were included in this study. The Diamed gel system was used for the screening and identification of atypical antibodies. Results: The rate of alloimmunization in sickle cell anemia patients was 31% (n=41), whereas in thalassaemia patients it was 20% (n=26). Antibodies to E, e, C, c, D, K, S, Fyª, Kpª, Jkª and Cw were observed. Among the two groups, 8 developed nonspecific antibodies, and 12 developed more than one antibody; however, 85% of patients were also immunized to Rh and Kell antigens. Conclusions: Red cell transfusions are associated with a significant risk of alloimmunization. It is, therefore, imperative to perform an initial extended red cell phenotyping for both donors and recipients, and carefully select ABO, Rh and Kell matched donors.

Prevalence of Sickle Cell Anemia In Eastern India

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4822-4822
Author(s):  
Abhijit Chakraborty ◽  
Jayasri Basak ◽  
Deboshree Majumdar ◽  
Soma Mukhopadhyay ◽  
Sagnik Chakraborty ◽  
...  
Keyword(s):  
Risk Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
West Bengal ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Counts

Abstract Abstract 4822 Background: Sickle cell disease is an inherited disorder of hemoglobin synthesis. This is due to replacement of Valine for Glutamic Acid in position six of the Beta globin chain of hemoglobin. This genetic alteration yields unstable RBC which lasts for 10–20 days. In stressful conditions the cells become sickle shaped and get lysed. There are about 20 million people with sickle cell disease in India. During January 2009- May2010 camps were held in various parts of West Bengal, Jharkhand, Chattisgarh. Along with various mutations of thalassemia, we also observed sickle cell anemia among them. This triggered our interest to study the spectrum of the sickle mutation co-inheritant with different mutations such as Homozygous Sickle Cell, Sickle Cell-Beta0 Thalassemia, Sickle Cell-Beta+ Thalassemia, Severe β+ thalassemia genes, Moderate β+ thalassemia genes, Mild β+ thalassemia genes Sickle cell-HbE Thalassaemia, Sickle cell-HPFH Thalassaemia, in said part of India. Since Indian patients with SS disease had higher hemoglobin, red cell counts and higher HbF levels and lower HbA2, MCHC, MCV, and reticulocyte counts, hence a high hemoglobin is a risk factor for painful crises and may also be a risk factor for avascular necrosis of the femoral head, proliferative sickle retinopathy, and acute chest syndrome. Methods: We have screened 332 individuals in eastern part of India during the period January 2009- May 2010. 3ml of peripheral blood was collected in EDTA vial from each individual. NESTROFT (Naked Eye Single Tube Red Cell Osmotic Fragility Test) was performed on spot. Then Complete Blood Count was done within 24 hours of collection. HPLC (High Performance Liquid Chromatography) was performed to identify the samples for confirmation. In our observation in case of sickle cell anaemia HbF (Fetal haemoglobin), Hb (haemoglobin), MCV (mean corpuscular volume) values ranges between 0–10 %, ≤7-10g/dl, 65–90fl respectively. ARMS (Amplification Refractory Mutation System) PCR (polymerase chain reaction) was done to confirm the mutation. Result: Conclusion: Of the total samples collected in the camps held at various places of Jharkand, Chattisgarh & West Bengal 87 of them was carriers of sickle cell anemia. There was 7 homozygous (SS), 14 sickle beta, 12 double heterozygous for HPFH (High Persistance of Fetal Hemoglobin) & sickle cell anemia. In conclusion, the manifestations of sickle cell disease are influenced by a variety of other genetic and environmental factors. The occurrence of the disease against different genetic and environmental backgrounds provides experimental models that contribute to understanding the variability in clinical and hematological expression of the disease. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Central nervous system complications and management in sickle cell disease

Blood ◽  
2016 ◽  
Vol 127 (7) ◽  
pp. 829-838 ◽  
Author(s):  
Michael R. DeBaun ◽  
Fenella J. Kirkham
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Prevention ◽  
High Income ◽  
Cell Disease ◽  
Cerebral Infarcts

Abstract With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.

scholarly journals Hyperhaemolysis in Sickle Cell Disease Is Not Necessarily a Transfusion Reaction

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4595-4595 ◽  
Author(s):  
Elizabeth A Jones ◽  
Louise Smith ◽  
Russell D Keenan
Keyword(s):  
Intensive Care ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Organ Failure ◽  
Sickle Cell ◽  
Red Cell ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
Life Threatening ◽  
Red Cell Transfusion

Abstract Hyperhaemolysis is a rare life threatening complication in sickle cell disease with rapidly dropping haemoglobin, intravascular haemolysis and haemoglobinuria leading to multi organ failure and death. The literature reports that hyperhaemolysis in sickle cell disease is a complication of red cell transfusion (Aragona et al., 2014 J. Pediatr. Hematol. Oncol.) and suggests management based on with holding further transfusion to avoid aggravating the haemolysis and using immunosuppression (Win 2009 Expert Rev. Hematol.). In the literature, all cases of hyperhaemolysis in addition to a recent blood transfusion, were in or had had a recent sickle cell crisis. We report a case of life threatening Hyperhaemolysis in a 5 year old child following a sickle cell crisis who had never previously been transfused. We suggest that, at least in this case, the hyperhaemolysis cannot be transfusion related. The theoretical case for management of withholding transfusion may not be sound and potentially dangerous. A female child with known sickle cell disease presented with temperature and chest pains, she had a Hb 72g/L (stable over a few years). She initially improved with oxygen, fluids and antibiotics. 36 hours after admission she acutely deteriorated with increasing pallor and dropping oxygen saturations. She started passing frank red urine which initially was considered to be haematuria but on investigation was haemoglobinuria. Her Hb dropped to 47g/L with no evidence of blood loss. Within hours of developing haemoglobinuria she required intensive care for respiratory support. She rapidly developed multi-organ failure requiring oscillatory ventilation, inotropes, and haemofiltration for renal support. She was managed with emergency red cell transfusion (her first ever) and within 12 hours of haemoglobinuria received a full red cell exchange transfusion. There were ongoing antibiotics for clinical respiratory infection and she was later confirmed to have influenza B. No steroids or other immune suppression were given. There was no evidence of acute bleeding to explain a drop in haemoglobin at any point. With maximum intensive care support including further transfusions she gradually improved and has made a full recovery. No deterioration was observed following transfusion. She has remained well since. She is now 13 years old and following such a dramatic episode she has remained on a transfusion programme with successful oral iron chelation. She has not experienced any further episodes of hyperhaemolysis and no red cell antibody has been detected at any time. This case demonstrates that hyperhaemolysis in sickle cell disease does not require a previous transfusion. We suggest that it is possible the previous reported cases are also not due to blood transfusion but are an acute form of haemolysis seen on the background of a chronic haemolytic disease. An increase in the rate of haemolysis may be related to other acute complications of sickle cell disease. We propose that the optimum management of hyperhaemolysis should include full supportive care including maintaining haemoglobin by transfusion. Immunosuppression in this case could have led to a worse outcome as influenza pneumonia was the likely initial trigger of the episode. Disclosures No relevant conflicts of interest to declare.

scholarly journals Is there a role for selective vasodilation in the management of sickle cell disease?

Blood ◽  
1988 ◽  
Vol 71 (3) ◽  
pp. 597-602 ◽  
Author(s):  
GP Rodgers ◽  
MS Roy ◽  
CT Noguchi ◽  
AN Schechter
Keyword(s):  
Blood Flow ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Microvascular Obstruction ◽  
Controlled Study ◽  
Control Group ◽  
Cell Disease ◽  
Retinal Arteriolar

Abstract To test the hypothesis that microvascular obstruction to blood flow at the level of the arteriole may be significant in individuals with sickle cell anemia, the ophthalmologic effects of orally administered nifedipine were monitored in 11 steady-state patients. Three patients with evidence of acute peripheral retinal arteriolar occlusion displayed a prompt reperfusion of the involved segment. Two other patients showed fading of retroequatorial red retinal lesions. Color vision performance was improved in six of the nine patients tested. The majority of patients also demonstrated a significant decrease in the amount of blanching of the conjunctiva which reflects improved blood flow to this frequently involved area. Such improvements were not observable in a control group of untreated stable sickle cell subjects. These findings support the hypothesis that inappropriate vasoconstriction or frank vasospasm may be a significant factor in the pathogenesis of the microvascular lesions of sickle cell disease and, further, that selective microvascular entrapment inhibition may offer an additional strategy to the management of this disorder. We believe a larger, placebo-controlled study with nifedipine and similar agents is warranted.

Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
J. Michael Taylor ◽  
Paul Horn ◽  
Heidi Sucharew ◽  
Todd A Abruzzo ◽  
Jane Khoury
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Care Utilization ◽  
Cell Disease ◽  
Common Procedure ◽  
Transfusion Therapy ◽  
Stroke Population ◽  
Clinical Classification Software ◽  
Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

Pathogenesis of Hyposthenuria in Sickle Cell Anemia

PEDIATRICS ◽  
1960 ◽  
Vol 26 (6) ◽  
pp. 1051-1051
Author(s):  
Clarence L. Morgan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Genetic Defect ◽  
Urine Osmolality ◽  
Normal Kidney ◽  
Cell Disease

Schlitt and Keitel report (Pediatrics, 26: 249, 1960) complete reversibility of hyposthenuria in a 6-month-old subject with sickle cell disease following transfusion over a 4-day period of 290 ml of blood with a rise in maximal urine osmolality from 700 to 1100 mosmol/l. They cite this as evidence against an independent genetic defect being causal in the etiology of hyposthenuria in sickle cell disease. It is well known that the concentrating capacity of the normal kidney increases as the ratio of urea to other solutes in the urine approaches 0.35, and the approximate range of improvement may be from 650 to 1100 mosmol/l.1

PATHOGENESIS OF HYPOSTHENURIA IN PERSONS WITH SICKLE CELL ANEMIA OR THE SICKLE CELL TRAIT

PEDIATRICS ◽  
1960 ◽  
Vol 26 (2) ◽  
pp. 249-254
Author(s):  
L. Schlitt ◽  
H. G. Keitel
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Renal Damage ◽  
Sickle Cell Trait ◽  
Urinary Concentration ◽  
Cell Disease ◽  
Renal Vessels

Hyposthenuria was investigated in subjects with sickle cell trait and in patients with sickle cell anemia. The following were observed: 1) in subjects with sickle cell trait both normal and reduced maxima of urinary concentration are found, whereas all untreated patients with sickle cell anemia over 6 months of age have hyposthenuria; 2) hyposthenuria becomes increasingly more severe with advancing age in both sickle cell anemia and sickle cell trait; 3) in a 6-month-old patient with sickle cell anemia and hyposthenuria, the maxima of urinary concentration returned to normal after two transfusions of normal erythrocytes. Reasons are presented for favoring the hypothesis that hyposthenuria in sickle cell disease is due to renal damage, possibly from intravascular sickling of erythrocytes in renal vessels or from the presence of "free" circulating S-hemoglobin.

Retinal microvascular analysis using Fundus Fluorescein Angiography in Egyptian sickle cell disease patients

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Nayera H El Sherif ◽  
Mahmoud A Kenny ◽  
Waheed S Elhalfawy
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Fluorescein Angiography ◽  
Sickle Cell ◽  
Large Sample Size ◽  
Cell Disease ◽  
Fundus Fluorescein Angiography ◽  
Transfusion Therapy ◽  
Hydroxyurea Treatment ◽  
Trans Cranial Doppler

Abstract Background Sickle cell disease can affect retina of eye via vaso-occulsive changes that occur in micro-vessels of retina which could be analysed by using Fundus Fluorescein Angiography. Aim To analyze macular microvascular alternation in patients with SCD by Fundus Fluorescein Angiography (FFA) and to assess the role of potentially contributory Clinico-pathological factors including Trans-Cranial Doppler, genotypes, hydroxyurea, transfusion therapy and finally iron overload state on the development of macular alterations. Method This was across-sectional study which included 30 Sickle cell disease patients randomly recruited from the Paediatric Haematology clinic, children Hospital, Ain Shams University, Cairo, Egypt. Complete blood count (CBC), Trans-Cranial Doppler (TCD) and Fundus Fluorescein Angiography. Results In our study, there were 30 patients with mean age (14.1± 4.02), 5 patients had abnormal/conditional Trans-Cranial, 15 patients had Vaso-occlusive crises, 11 patients were on regular simple blood transfusion; all 30 studied sickle cell disease patients had normal Fundus Fluorescein Angiography and eye examination and only one patient hadabnormal visual acuity;A 29 years oldgirl who had five attacks of cerebral strokes last year, on regular simple blood transfusion and Hydroxyurea treatment with abnormal TCD and recurrent Vaso-occlusive crises in last two years, Although her vision is hand movement yet Fundus Fluorescein Angiography was normal. Conclusion we didn’t find any Retinal microvascular alternation in our studied SCD patients using Fundus Fluorescein Angiography, we related our results to the fact that our studied SCD patients were young and all our studied patients were on hydroxyurea therapy with fair compliance, further studies using large sample size are warranted in order to illustrate the utility of Fundus Fluorescein Angiography (FFA) as a tool for better detection of sickle retinopathy.

Sign in / Sign up

Export Citation Format

Share Document