Which medications in the drug class Colony-stimulating factors are used in the treatment of Shwachman-Diamond Syndrome?

Updated: Nov 17, 2020
  • Author: Antoinette C Spoto-Cannons, MD, FAAP; Chief Editor: Hassan M Yaish, MD  more...
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Answer

Colony-stimulating factors

These agents provide red cell line stimulation.

Epoetin alfa (Epogen, Procrit)

FDA approved for treatment of chronic anemia. Stimulates division and differentiation of committed erythroid progenitor cells; induces release of reticulocytes from bone marrow into bloodstream. SC route provides sustained serum levels compared to IV route. Reduce dose when hematocrit reaches target range of 30-36% or hematocrit increases >4 points over any 2-wk period. Hold dose if hematocrit >36%.

Filgrastim (Neupogen)

FDA approved for severe chronic neutropenia.

GCSF activates and stimulates production, maturation, migration, and cytotoxicity of neutrophils. If IV route is used and GCSF final concentration is < 15 mcg/mL, add 2 mg of albumin/mL to prevent drug adsorption to administration set.


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