Neutropenia

Question 1

At what ANC is infection risk the greatest?

Answer 1

< 200 /mcL

- infection risk increases greatly with ANC < 500/mcL

Question 2

Which populations commonly have mild congenital asymptomatic neutropenia?
Do you treat them?

Answer 2

Blacks, Yemenite Jews, Jordanian Arabs

- not associated with increased infections, requires no therapy

Question 3

What is the ANC range for mild congenital asymptomatic neutropenia?

Answer 3

ANC between 1000 and 1500/mcL

Question 4

What is autoimmune neutropenia?

Answer 4

• An acquired abnormality; may be isolated or assoc with autoimmune diseases (ex: SLE)
• may detect ANCA
• neutropenia not severe enough to develop frequent infections
• may have spontaneous remissions in pts with isolated form

Question 5

Drugs that can cause neutropenia:

Answer 5

• chemo agents (alkylating agents, anti-metabolites)
• cephalosporins, TMP-SMX (severe neutropenia in rare cases)
• Chloramphenicol = agranulocytosis
• Carbamazepine, phenytoin (anti-epileptics)
• Amiodarone, procainamide (anti-arrythmics)
• naproxen (NSAIDs)
• propylthiouracil
• gold salts

Question 6

When does drug-induced neutropenia resolve?

Answer 6

Usually after discontinuation of drug (self-limited); in rare cases may be prolonged after drug withdrawal

Question 7

Infectious causes of neutropenia:

Answer 7

• Viral: HIV, CMV, EBV
• Rickettsial
• overwhelming infection/sepsis from Strep pneumo or Neisseria meningitidis

Question 8

What is cyclic neutropenia?

What is a potential treatment?

Answer 8

• A disorder in stem cell regulation leading to recurrent neutropenia and infectious risk typically every 2-4 weeks
• G-CSF may be effective at the time of the nadir neutrophil count

Question 9

What is non-immune chronic idiopathic neutropenia?

Answer 9

• A diagnosis of exclusion

- rarely requires treatment

Question 10

What are the things to look for in Felty’s syndrome?

Answer 10

• Rheumatoid arthritis, splenomegaly, neutropenia

- may present similarly to large granular lymphocytosis

Question 11

What is the approach for neutropenia?

Answer 11

• Treating the underlying cause
• Drug withdrawal
• management of any vitamin deficiencies (ex: B12, folate)
• treatment of autoimmune disorders or viral syndromes
• management of any resulting infections
• G-CSF

Question 12

What is a possible treatment for severe congenital neutropenia?

Answer 12

G-CSF; effective in increasing neutrophil counts and decreasing infectious risk
- pts at high risk for myelodysplasia and acute myeloid leukemia