MORPHOLOGIC ABNORMALITIES OF LEUKOCYTES WITHOUT ASSOCIATED IMMUNODEFICIENCY

Question 1

decreased nuclear segmentation and distinctive coarse chromatin clumping
pattern

Answer 1

Pelger-Huët Anomaly

Question 2

affects all leukocytes

Answer 2

Pelger-Huët Anomaly

Question 3

nuclei may appear round, ovoid, or peanut
shaped

Answer 3

pelger-Huët (PH)

Question 4

haracteristic spectacle-like
(“pince-nez”) morphology with
the nuclei attached by a thin
filament

Answer 4

pelger-Huët (PH)

Question 5

associated with severe bacterial infections, HIV, tuberculosis, and mycoplasma pneumonia

Answer 5

Pseudo- or Acquired Pelger-Huët Anomaly

Question 6

NUMBER OF CELLS AFFECTED
>68%

Answer 6

TRUE Pelger-Huët Anomaly

Question 7

NUMBER OF CELLS AFFECTED <35%

Answer 7

PSEUDO PHA

Question 8

hyposegmentation

Answer 8

Pelger-Huët Anomaly

Question 9

decrease segmentation usually bilobed or unilobed

Answer 9

Pelger-Huët Anomaly

Question 10

Pelger-Huët Anomaly function

Answer 10

NORMAL DESPITE THE ABNORMAL FORMATION

Question 11

CHANGES ARE OBVIOUS IN MATURE NEUTROPHILS

Answer 11

Pelger-Huët Anomaly

Question 12

Pelger-Huët Anomaly is a mutation in

Answer 12

lamin -beta receptor gene

Question 13

round oval, duble shaped, pair of eyeglasses

Answer 13

Pelger-Huët Anomaly

Question 14

Drugs known to induce PSEUDO- Pelger-Huët Anomaly

Answer 14

-mycophenolate
-mofetil
-valproate
-sulfisoxazole
-ganciclovir
-ibuprofen
-chemotherapies such as paclitaxel and docetaxel

Question 15

granulocytes with large, darkly
staining metachromatic cytoplasmic
granules (large azurophilic granules)

Answer 15

Alder-Reilly Anomaly

Question 16

Leukocyte function is not affected in

Answer 16

Alder-Reilly Anomaly

Question 17

composed primarily of partially digested mucopolysaccharides

Answer 17

Alder-Reilly Anomaly

Question 18

hunter’s and hurler’s diseases

Answer 18

Alder-Reilly Anomaly

Question 19

characterized by variable thrombocytopenia, giant platelets, and large Döhle body-like inclusions in neutrophils, eosinophils,
basophils, and monocytes

Answer 19

May-Hegglin Anomaly

Question 19

mutation in the MYH9 gene on chromosome 22q12-13.3 most patients are asymptomatic, few have a mild bleeding tendencies

Answer 19

May-Hegglin Anomaly

Question 20

pink or rod shaped structures. These are fused primary granules (peroxidase positive)

Answer 20

auer rods

Question 21

found on myeloid and monocytic series only

Answer 21

auer rods

Question 22

bundles of auer rods that is mainly associated with M3 acute promyelocytic anemia

Answer 22

faggot cells

Question 23

found in cases of AML and AMML
ML (Acute Myeloid Leukemia) and AMML (Acute Myelomonocytic Leukemia)

Answer 23

auer rods

Question 24

single or multiple blue inclusions

Answer 24

dohle bodies

Question 25

aggregates of free ribosomes of ER are cytoplasmic inclusions consisting of remnants of RNA arranged in parallel rows

Answer 25

dohle bodies

Question 26

seen in severe infections and toxic states confused with May-Hegglin

Answer 26

dohle bodies

Question 27

-mutations in genes that code for the production of lysosomal enzymes. -classified according to the under degraded macromolecule that accumulates in the cell

Answer 27

Lysosomal Storage Disorders

Question 28

Lysosomal Storage Disorders

Answer 28

Mucopolysaccharidoses Gaucher Disease Niemann-Pick Disease

Question 29

- deficient activity of an enzyme necessary for the degradation of dermatan sulfate, heparan sulfate, keratan sulfate, and/or chondroitin sulfate

Answer 29

✓Mucopolysaccharidoses

Question 30

-The most common of the lysosomal lipid storage diseases. - defect or deficiency in the catabolic enzyme beta-glucocerebrosidase - three subtypes (type 1 – most common)

Answer 30

Gaucher Disease -

Question 31

- deficiency of lysosomal hydrolase enzyme acid sphingomyelinase

Answer 31

✓Niemann-Pick Disease

Question 32

deficiency of lysosomal hydrolase enzyme acid sphingomyelinase is due to the mutation of what gene?

Answer 32

SMPD1 GENE

Question 33

macrophage has foamy appearance

Answer 33

pickle cell in Niemann-Pick Disease