Granulocyte Stimulating Factor (G-Csf)

Granulocyte Colony Stimulating Factor(G-CSF)

Q. 1

Drug of choice for Neutropenia due to cancer chemotherapy is

 A Vitamin B-12 
 B

IL 11

 C Filgrastim 
 D

Erythropoietin

Q. 1

Drug of choice for Neutropenia due to cancer chemotherapy is

 A Vitamin B-12 
 B

IL 11

 C Filgrastim 
 D

Erythropoietin

Ans. C

Explanation:

Filgrastim REF: KDT 6TH edition, page 833, internet resources

See APPENDIX 37 “ANTICANCER DRUGS TOXIC AMELIORATION”


Q. 2 Relative to filgrastim (G-CSF), sargramostim (GM-CSF)
 A Has greater  oral bioavailability
 B Is more  likely  to cause   thrombocytopenia 
 C Is more  likely  to elicit  an allergic reaction
 D Stimulates production of a wider  variety  of hematopoietic stem  cells
Q. 2 Relative to filgrastim (G-CSF), sargramostim (GM-CSF)
 A Has greater  oral bioavailability
 B Is more  likely  to cause   thrombocytopenia 
 C Is more  likely  to elicit  an allergic reaction
 D Stimulates production of a wider  variety  of hematopoietic stem  cells
Ans. D

Explanation:

Stimulates production of a wider variety of hematopoietic stem cells

GM-CSF  has  wider  biologic  activity  than  G-CSF; it stimulates  early myeloid stem cells in addition to cells destined become neutrophils.


Q. 3

Kostmann’s syndrome-treatment is

 A

Anti-thymocyte globulin + cyclosporin

 B

Anti-thymocyte globulin + cyclosporin + GM­CSF

 C

G-CSF

 D

GM-CSF

Q. 3

Kostmann’s syndrome-treatment is

 A

Anti-thymocyte globulin + cyclosporin

 B

Anti-thymocyte globulin + cyclosporin + GM­CSF

 C

G-CSF

 D

GM-CSF

Ans. C

Explanation:

G-CSF [Ref : Nelson Ighle p. 913]

  • Kostmann’s syndrome is an inherited disorder of the bone marrow.
  • It is also known as severe congenital neutropenia.
  • It is inherited in an autosomal recessive manner.
  • Congenital neutropenia is usually very severe and neutrophils are often completely absent in the blood of these patients at the time of diagnosis.
  • These patients usually show arrest of maturation of neutrophils at the promyelocyte stage.

– This means that their neutrophils rarely. fully mature into the cells that are capable of fighting infections. – As a result these patients usually suffer from severe infections ?

– Omphalitis (infection of the navel)

– Pneumonia

– Skin abscesses

– Otitis media during their first few years of life.

Pathogenesis

  • Kostmann’s syndrome or severe congenital neutropenia is believed to he caused by defect in a gene on chromosome (in p35-p34.3) that codes for granulocyte colony stimulating factor (GCSF).
  • This disease is believed to be caused due to defect in receptor in granulocyte colony stimulator factor.

– This receptor is located on granulocytes or neutrophils. The purpose of this receptor is the binding of the granulocyte to the cytokine (GCSF) in order to give signal to the cell to mature, to multiply, and enhance .function.

GCSF is a natural cytokine produced by the human body.

– Patients with congenital neutropenia also produce GCSF hut because of the defect in GCSF receptor the response of their neutrophils to the normal amounts of GCSF in the blood is reduced.

– These patients will respond to higher dose of GCSF (This is the basis of tit of disease).

  • In some patients the GCSF receptor develops changes that could also indicate progression towards leukemia. Treatment
  • Patients with congenital neutropenia respond to administration of GCSF.

– As soon as congenital neutropenia is diagnosed, patients should start treatment with GCSF. – This treatment usually stabilizes the neutrophil count of the patient.

– The response of these patients to GCSF treatment is different. There is a big variation in the dose of GCSF that different people receive.

– A small group of patient does not respond to even very high doses of GCSF.

  • In patients who do not respond to GCSF doses of 100 mcg/kg within fourteen days, a search for bone marrow donor should be started immediately and bone marrow transplantation should be performed as soon as matching donor is identified.


Quiz In Between


Q. 4 Drug of choice for Neutropenia due to cancer chemotherapy:
 A Vitamin B-12
 B IL 11
 C Filgrastim
 D Erythopoie tin
Q. 4 Drug of choice for Neutropenia due to cancer chemotherapy:
 A Vitamin B-12
 B IL 11
 C Filgrastim
 D Erythopoie tin
Ans. C

Explanation:

Filgrastim


Q. 5

Filgrastim is used in treatment of:

 A

Anemia

 B

Neutropenia

 C

Malaria

 D

Filarial

Q. 5

Filgrastim is used in treatment of:

 A

Anemia

 B

Neutropenia

 C

Malaria

 D

Filarial

Ans. B

Explanation:

Filgrastim is a recombinant human granulocyte colony stimulating factor (G-CSF) which is a 175 – aminoacid glyco-protein.

It differs from the natural granulocyte stimulating factor due to its lack in glycosylation and the presence of an extra N-terminal methionine. It has proved to be effective in the treatment of severe neutropenia.

Ref: Goodman and Gilman’s The Pharmacological Basis of Therapeutics, 11th Edition, Pages 1429-32; Immunopharmacology By Manzoor M. Khan, Pages 49-50


Q. 6

A child with recurrent severe bacterial infections is diagnosed of having Kostmann’s syndrome. Treatment is:

 A

Antithymocyte globulin + cyclosporine

 B

Antithymocyte globulin + cyclosporine – C31-CSF

 C

G-CSF

 D

GM-CSF

Q. 6

A child with recurrent severe bacterial infections is diagnosed of having Kostmann’s syndrome. Treatment is:

 A

Antithymocyte globulin + cyclosporine

 B

Antithymocyte globulin + cyclosporine – C31-CSF

 C

G-CSF

 D

GM-CSF

Ans. C

Explanation:

Kostmann syndrome is the severe neutropenia with maturation defect in the marrow progenitor cells.
Recombinant G-CSF will increase neutrophil counts in most patients and has the potential to prolong life expectancy.
Hematopoietic stem cell transplantation may be considered for patients with severe complications, especially those with severe congenital neutropenia.

 

 

 

Ref: Ambruso D.R., Hays T., Goldenberg N.A., Nuss R. (2012). Chapter 30. Hematologic Disorders. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

 

Quiz In Between


Q. 7

Filgrastim is used in the treatment of –

 A

Anemia

 B

Neutropenia

 C

Malaria

 D

Filaria

Q. 7

Filgrastim is used in the treatment of –

 A

Anemia

 B

Neutropenia

 C

Malaria

 D

Filaria

Ans. B

Explanation:

Ans. is ‘b’ i.e., Neutropenia

o Filgrastim is a recombinant human granulocyte colony stimulating factor (G-CSF) effective in the treatment of severe neutropenia.


Q. 8

Drug of choice for Neutropenia due to cancer chemotherapy is –

 A

Vitamin B-12

 B

IL 11

 C

Filgrastim

 D

Erythropoietin

Q. 8

Drug of choice for Neutropenia due to cancer chemotherapy is –

 A

Vitamin B-12

 B

IL 11

 C

Filgrastim

 D

Erythropoietin

Ans. C

Explanation:

Ans. is ‘c’ i.e., Filgrastin

o Filgrastim is a recombinant human granulocyte colony stimulating factor (G-CSF) effective in the treatment of severe neutropenia.


Q. 9

Kostmann’s syndrome-treatment is –

 A

Anti-thymocyte globulin + cyclosporin

 B

Anti- thy mocyte globulin + cyclosporin + GM-CSF

 C

G-CSF

 D

GM-CSF

Q. 9

Kostmann’s syndrome-treatment is –

 A

Anti-thymocyte globulin + cyclosporin

 B

Anti- thy mocyte globulin + cyclosporin + GM-CSF

 C

G-CSF

 D

GM-CSF

Ans. C

Explanation:

Ans. is ‘c’ i.e., G-CSF

Kostmann’s syndrome (severe congenital neutropenia)

  • Kostmann’s syndrome,an autosomal recessive disorder, is an inherited disorder of the bone marrow in which there is arrest of maturation of neutrophils at promyelocyte stage.

o There is congenital neutropenia and neutrophils are often completely absent in the blood at time of diagnosis. o Because of neutropenia, these patients suffer from severe infections e.g., omphalitis (infection of navel), Pneumonia, Skin abscesses, otitis media.

o Kostmann’s syndrome is believed to be caused due to defect in receptor of granulocyte colony stimulating factor (GCSF) on neutrophils (granulocytes). The purpose of this receptor is binding of the granulocyte to the cytokine (GCSF) in order to give signal to the cell to mature and multiply.

Patients with Kostamann’s syndrome produce GCSF but because of the defet in GCSF receptor the response of neutrophils to normal amounts of GCSF in the blood is reduced. However, they can respond if the amount of GCSF is increased –> These patients will respond to higher dose of GCSE

Quiz In Between



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