Non-Malignant Leukocyte Disorders

Question 1

vacuolation in neuts

Answer 1

increased lysosomal activity

Question 2

heavy granulation in neuts

Answer 2

enzymes are more active

Question 3

when do we see hypersegmented neuts

Answer 3

severe B12 deficiency or severe myeloblastic anemia

Question 4

describe hypogranular neutrophils

Answer 4

• bluer cytoplasm
• no granules in cell = incapable of fighting infections
• we can also see similar patterns in immature cells
• may be hard to differentiate between lymphs
• MDS or anything that overworks BM

Question 5

how is karyorrhexis different from hypersegmentation

Answer 5

no filaments connecting nucleus; seen in MDS

Question 6

Pelger-Huet Anomaly

Answer 6

• autosomal dom
• laminopathy (nuclear envelop diseases)
• B-lamin receptor mutation -> abnormal shape of nucleus
• cell function is normal
• hyposegmentation
• inherited = unilobed; but acquired through disease like MDS or AML = pseudo

Question 7

Alder-Reilly Anomaly

Answer 7

• recessive, rare
• mucopolysaccharides accumulated in lysosomes = dark inclusions
• reason why need two toxic changes
• can sometimes see in monos and lymphs UNLIKE toxic granulation
• cell function not affected

Question 8

Chediak-Higashi

Answer 8

• CHS1 LYST gene mutation (lysosome trafficking regulator)
• lysosomes containing fused granules
• abnormal granules; unable to release contents = abnormal cell function
• impaired chemotaxis
• impaired degranulation and release of enzyme
• impaired destruction of bacteria/foreign material

Question 9

clinical findings associated w Chediak-Higashi

Answer 9

• recurrent bacterial infections
• neutropenia
• melanocytes affected bc also have lysosome organelles
• platelet granules also affected so bleeding tendencies

Question 10

treatment for Chediak-Higashi

Answer 10

prophylactic antibiotics; BM transplant only cure

Question 11

May-Hegglin Anomaly

Answer 11

• autosomal dom
• MYH9 mutation => abnormal myosin heavy chain IIA
  -meg maturation and PLT fragmentation
• excess chains precipitate as large blue cytoplasmic inclusions that look like Dohle bodies
• giant PLTS; low PLT counts
• cell function not impaired too much besides inclusions
• pts may have bleeding episodes

Question 12

chronic granulomatous disease

Answer 12

• defect in phagocyte NADPH oxidase
• cells canot produce H2O2 and ther superoxides
• cell = normal morph; abnormal function
• pts see recurring infections and formation of granulomas around infectious agents

Question 13

screen test for chronic granulomatous disease

Answer 13

nitroblue tetrazolium
- clear to deep blue for NORMAL cells
- abnormal = stays clear

Question 14

this is due to lack of respiratory burst

Answer 14

chronic granulomatous disease

Question 15

T or F. In chronic granulomatous disease, granules are absent

Answer 15

F! normal morphology on smear but function is abnormal bc granules may be emmpty
so base diagnosis off pt symptomology and history of infections

Question 16

describe what is happening in a leukemoid rxn

Answer 16

• 2ry leukocytosis due to drugs, infections, etc.
• WBC >50
• toxic changes, left shift, normal RBC & PLT counts
• LAP score increased

Question 17

How to differentiate a leukemoid rxn from CML

Answer 17

• there are no blasts in a leukemoid rxn
• LAP score increased in rxn, but in CML = decreased due to clonal abnormality
• PHIL chromosome pos for CML
• high WBC count will go away if just a rxn