Treatment of Sickle Cell Anemia

Charache, Samuel

doi:10.1146/annurev.me.32.020181.001211

Cited by 42 publications

(3 citation statements)

References 36 publications

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“…Oxygen ( 0 2 ) therapy in hypoxic sickle cell patients in painful (infarctive, vasoocclusive) crisis is clearly useful [1][2][3][4] to prevent generalized sickling. 0 2 is also frequently used in nonhypoxic sickle cell patients in painful crisis, although the practice is controversial [1,2,4].…”

Section: Introductionmentioning

confidence: 99%

Oxygen‐induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis

1988

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The benefit of oxygen (O2) therapy in non-hypoxic sickle cell patients in painful crisis is uncertain. We report a case of a non-hypoxic sickle cell patient in painful crisis who developed marrow red cell hypoplasia requiring transfusion support after O2 therapy. The uncertain benefits of O2 use in such cases must be weighed against the serious and underrecognized risks of transfusion. In patients who develop O2-induced marrow red cell hypoplasia, cessation of O2 therapy may reverse the anemia and obviate the need for transfusion.

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Section: Introductionmentioning

confidence: 99%

Oxygen‐induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis

1988

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“…102 More recent techniques generally use 2 intravenous lines with simultaneous or sequential withdrawal of SS blood and its replacement by donor AA cells. 103,104 However, although manual procedures are effective in conducting exchange, they are tedious and time-consuming.…”

Section: Manual Methodsmentioning

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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“…It may lead to complications such as vaso-occlusive crises, acute chest syndrome, infections, strokes, renal failure, gallstones, and acute exacerbation of anemia from splenic sequestration and/or aplastic, hyperhemolytic, and megaloblastic crises. 1 , 2 …”

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confidence: 99%

Peg-interfon alpha-2a and low-dose ribavirin for treatment of hepatitis C virus infection in patients with sickle-cell anemia in Saudi Arabia

Agha

Chakik

Ali

et al. 2013

Annals of Saudi Medicine

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BACKGROUND AND OBJECTIVESData regarding the safety and efficacy of antiviral therapy with pegylated interferon (PEG-IFN) and ribavirin (RBV) in patients with sickle-cell disease (SCD) and hepatitis C virus (HCV) infection are scanty. In this study, our aim was to evaluate the safety and efficacy of antiviral therapy with PEG-IFN and low-dose RBV in patients with SCD and chronic HCV infection receiving hydroxyurea in Saudi Arabia.DESIGN AND SETTINGSThis was a prospective interventional study conducted between January 2009 and September 2012 at the outpatient departments of Haematology and Hepatology/Gastroenterology of a tertiary care hospital in Saudi Arabia.PATIENTS AND METHODSWe studied 8 treatment-naive patients (5 males, 63%) with chronic hepatitis C and SCD receiving hydroxyurea who were treated with PEG-IFN alpha-2a (180 μg weekly) and low-dose RBV (200 mg daily). Early virological response (EVR) and sustained virological response (SVR) rates were assessed in all patients.RESULTSAll patients were infected with HCV genotype 1 (n=6.8%) or 4 (n=22%). EVR was obtained in 3 patients (38%) and SVR in 6 patients (7%). During the study, there was no increase in the number of blood units transfused and emergency visits due to painful crises.CONCLUSIONin Saudi Arabian patients with SCD and chronic HCV infection on hydroxyurea, PEG-IFN and low-dose RBV treatment proved to be efficacious and safe.

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Treatment of Sickle Cell Anemia

Cited by 42 publications

References 36 publications

Oxygen‐induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis

Oxygen‐induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis

Management of sickle cell disease: challenges and risks of transfusion

Peg-interfon alpha-2a and low-dose ribavirin for treatment of hepatitis C virus infection in patients with sickle-cell anemia in Saudi Arabia

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