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In consultation with a sickle cell expert, transfuse people who have acute splenic sequestration and severe anemia to raise the hemoglobin to a stable level, while avoiding over-transfusion.

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Balanced against these concerns are the possible benefits of treatment which may include fewer crises, higher hemoglobin levels, decreased organ damage and extended life span.

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Oxygen may also suppress erythrocyte production, depress reticulocytosis and cause rebound sickle cell crises on discontinuation of therapy when the arterial oxygen tension is raised above the normal range.

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Pain in the bones (usually occurring symmetrically on both sides) is common because blood obstruction can directly damage bone and because bone marrow is where red blood cells are manufactured.

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No deaths were related to treatment with hydroxyurea ( Table 3 Number of Deaths, Reasons for Stopping Treatment Permanently, and Pregnancies in the Two Groups of patients as of June 30, 1994.

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It makes you feel tired, because your blood cells don't last as long as other people's do.

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In a proof-of-principle test of BCL11A as a potential therapeutic target, demonstrated that inactivation of BCL11A in sickle cell disease transgenic mice corrects the hematologic and pathologic defects associated with sickle cell disease through high-level pancellular HbF induction.

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Hydroxyurea can temporarily lower the number of white blood cells in your blood, increasing the chance of getting an infection.

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This article summarizes the role of vascular inflammation and coagulation activation, discusses potential mechanisms responsible for activation of coagulation and reviews recent data demonstrating the crosstalk between coagulation and vascular inflammation in sickle cell disease.

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A) Percentage HbF response, showing a broad distribution of percentage HbF values at hydroxyurea MTD, ranging from approximately 10% to more than 40%.

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In the MSH, patients with the greatest response in fetal hemoglobin were more likely to have 2 or more episodes of hematologic toxicity ().

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The frequency, severity, location and duration of pain crises can vary considerably, even within a specific disease subtype.

Marrow suppression and mild peripheral blood cytopenias represent predictable toxicities of daily hydroxyurea therapy and are actually desired effects during escalation to MTD.

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Some cases of acute chest syndrome may result from treatments of the crisis, including from administration of opioid pain killers (which can reduce breathing and oxygen uptake) or excessive use of intravenous fluids.

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The Orange County medical examiner found that symptoms associated with sickle-cell trait caused UCF football player Ereck Plancher to collapse and die after an off-season workout on March 18.

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