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Posted On : 22 Apr 2014

Dear Dr

1} Would you please inform, regarding
any protcol for transfusion in Sickle cell anemia
What should be the level of Hb for transfusion_?
Any relavance of retic count for transfusion should be considered_?

2} Is there any need for iron therapy in intravascular hemplysis { G6PD defeciency} because of hemosidrinuria_?

Thanks

Expert Answer :

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Answer 1

Transfusions are indicated for either episodic events triggered by an acute complication or a necessary medical intervention {e.g., neurosurgery}. In contrast to these episodic indications, some clinical problems require long-term suppression of circulating sickle cells. Chronic transfusion therapy usually achieves this goal.
EPISODIC TRANSFUSIONS are required in Acute splenic sequestration {red cell transfusions are indicated for those who become symptomatic or whose hemoglobin value falls 2 gm, dl below baseline}, Transient red cell aplasia {red cell transfusions are indicated for those who become symptomatic or whose hemoglobin value falls 2 gm, dl below baseline}, When acute chest syndrome is associated with hypoxia and a falling hemoglobin, Stroke {Chronic transfusion therapy reduces the rate of recurrent vaso-occlusive stroke and is indicated for all victims of this complication of sickle cell disease. The efficacy of transfusion in the management of acute stroke has not been well-studied, however, anecdotal reports suggest that early exchange transfusions may improve perfusion and oxygenation to brain tissue, thus limiting damage} and multiorgan failures.

Chronic transfusion therapy programs are indicated for several conditions in which the potential medical complications outweigh the risks of alloimmunization, infection and iron overload. The goal of these programs is to maintain the hemoglobin S at 30-50 percent, depending on the specific disorder. It is used in Prevention of recurrence of stroke, Pulmonary hypertension and chronic lung disease, chronic debilitating pain.

Iron supplements in hemosiderinuria may be needed if there is iron deficiency due to loss of iron in urine.

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