Which of the following is most useful in Rx of Kostmann’s Syndrome ?
Correct Answer: Filgrastim
Description: Kostmann disease was first described in 1956 as an autosomal recessive disorder characterized by severe neutropenia and onset of severe bacterial infections early in life Filgrastim/lenograstim (G-CSF) in Kostman syndrome There is an induction phase with G-CSF to evaluate the response of individuals, with an increase in absolute neutrophil count (ANC) (>1500/mL) and clinical improvement after 10-15 days. The initial daily dose is 5 mg/kg subcutaneously. If there is no response after 15 days, the daily dose is increased by 5 mg/kg. If the response is rapid or excessive (ANC >5000 /mL), the dose is halved. Once the minimal daily dose is determined, the maintenance phase can begin, with monitoring of neutrophil counts every 3-6 months. Almost two thirds of patients respond to a daily dose of 2-10 ug/kg, while 20% respond to 10-20 mg/kg. A small percentage of patients require higher doses, up to 100 mg/kg. Around 10% of patients are unresponsive to G-CSF therapy. Ref - harrison 20th edition 740 , medscape.com
Category:
Medicine
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now