Transfusion in Sickle Cell Disease: Experience from a Gujarat Centre

Mehta, Vishal; Mistry, Abhishek; Raicha, Bhavesh; Italia, Yazdi; Serjeant, G. R.

doi:10.1007/s12098-013-1163-z

Cited by 8 publications

(10 citation statements)

References 9 publications

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“…However, a study by V. Mehta et al in 2013 reported frequency of transfusions was 5.2%. 10 In this study, frequency of HbSS genotype patients reported 74.57%. Similar study reported by Hassan M et al in 2003 frequency of HbSS 88% 1.…”

Section: Aim and Objectivesmentioning

confidence: 54%

Assessment of Pattern of Blood Transfusion in Patients With Sickle Cell Anemia From a Tertiary Healthcare Centre in Chattisgarh

Kujur¹,

Joshi²

2015

jebmh

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Sickle cell Anemia is an autosomal recessive genetic haematologic disorder. Although, Red cell transfusion is currently the most accepted therapy for most acute and many chronic complications of Sickle cell Anemia patients. AIM: To assess total number of transfusions, age at the start of transfusion, time interval between transfusions, indications and Transfusion Transmitted Infections (TTI). MATERIAL AND METHODS: This is a prospective study in a blood bank in a tertiary care hospital in Raipur, over a period of 1 year. A total of 350 Sickle cell Anemia patients who were already diagnosed were included in study. RESULTS: Out of 13208 blood units, 849(6.42%) were transfused, 79.15% transfusion was done between ages 1-5 years. 36% patients had pretransfusion haemoglobin level below 6.0 g/dl presented with anemia and other complications. Seropositivity was found for viral markers i.e. HIV 1 & 2 and HBV, 0.57% and 0.28% respectively. CONCLUSION: 87.5 % transfusions observed between 0-1 month interval. Hydroxyurea is a drug that is used to help complications of Sickle cell Anemia patient and reduces demand of transfusion.

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Section: Aim and Objectivesmentioning

confidence: 54%

Assessment of Pattern of Blood Transfusion in Patients With Sickle Cell Anemia From a Tertiary Healthcare Centre in Chattisgarh

Kujur¹,

Joshi²

2015

jebmh

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“…The African forms of sickle cell disease have more severe features than those with the Asian haplotype where frequent alpha thalassemia and persistence of HbF may protect against some of the serious early pathologies of the disease. More information is needed on the Asian haplotype, 117 before the role of transfusion can be determined in that population, and transfusion in the steady state 118 requires justification. As the Asian haplotype of SS disease becomes better documented and understood, the role of transfusion therapy in its management will be more readily defined.…”

Section: Resultsmentioning

confidence: 99%

Management of sickle cell disease: challenges and risks of transfusion

Serjeant¹

2016

IJCTM

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Homozygous sickle cell (SS) disease is associated with rapid red cell destruction and a tendency to block flow in blood vessels. The bone marrow expansion needed to compensate for the rapid red cell destruction increases metabolic demands and folate requirements but also renders the bone marrow prone to suppression by renal impairment and infections especially those with human parvovirus B19. The abnormal red cells also tend to block blood vessels impairing flow in the bone marrow (dactylitis, bone pain crisis, hip necrosis), the spleen (acute splenic sequestration, chronic hypersplenism, loss of the normal filtering system rendering patients prone to overwhelming septicemia), the lungs (pulmonary embolism, acute chest syndrome), and the brain (ischemic stroke, hemorrhage). Transfusion plays a role in addressing all of these pathologies. During the acute lowering of hemoglobin due to acute splenic sequestration and aplastic crisis and the persistent lowering of hemoglobin due to chronic hypersplenism and chronic renal failure, top-up transfusions may help in maintaining oxygen delivery and symptomatic relief. Addressing vaso-occlusion is more complex but best documented in preventing recurrent stroke and primary stroke following detection of cerebral vessel stenosis by transcranial Doppler. Although top-up transfusions have minimal side effects, potentially serious complications arise from chronic transfusion, and there are many unanswered questions on the duration of such therapy and the natural history of the underlying complications. These issues are addressed with the knowledge currently available.

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“…The development of models of care appropriate to societies with high frequencies of variant haemoglobins and limited resources has made the Jamaican experience of value to other societies. As a result, the Jamaican experience has been sought by colleagues in Brazil, Greece (119), Turkey, Saudi Arabia (120-122), Bahrain, Uganda (123,124) and India (125)(126)(127)(128)(129), and has also contributed to the development of sickle cell care in more developed countries such as the USA and the UK. These collaborations have also provided research opportunities for learning about the variability of sickle cell disease and the determinants of this variability.…”

Section: Geographic Comparisonsmentioning

confidence: 99%

Jamaica and Research in Sickle Cell Disease

Gr¹,

Serjeant²

2017

West Indian Med J

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Many developments have occurred in sickle cell disease and care over the last 50 years in Jamaica. The clinic population grew from 50-60 in the mid-1960s to 5500 in late 1999. During this period, the number of staff serving sickle cell patients increased from 2 to 28, comprising physicians, paediatricians, nurses, laboratory technologists, social workers, computer staff and statisticians. The physical facilities have improved greatly, and data management has evolved from the typewritten long narrow paper strips in the late 1960s to sophisticated electronic patient management systems. The many physical resources and the superb opportunities of an 'island laboratory' have provided a unique basis for clinical research into the disease.

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Transfusion in Sickle Cell Disease: Experience from a Gujarat Centre

Cited by 8 publications

References 9 publications

Assessment of Pattern of Blood Transfusion in Patients With Sickle Cell Anemia From a Tertiary Healthcare Centre in Chattisgarh

Assessment of Pattern of Blood Transfusion in Patients With Sickle Cell Anemia From a Tertiary Healthcare Centre in Chattisgarh

Management of sickle cell disease: challenges and risks of transfusion

Jamaica and Research in Sickle Cell Disease

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