Morphologic abnormalities of Leukocytes Flashcards

1
Q

decreased nuclear segmentation and distinctive coarse chromatin clumping pattern

A

Pelger-Huët Anomaly

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2
Q

affects all leukocytes

A

Pelger-Huët Anomaly

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3
Q

mutation in the lamin beta-
receptor gene.

A

Pelger-Huët Anomaly

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4
Q

pelger-Huët (PH) nuclei may appear

A

round, ovoid, or peanut shaped

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5
Q

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

A

Pelger-Huët Anomaly

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6
Q

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

A

Pseudo- or Acquired Pelger-Huët Anomaly

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7
Q

Pseudo- or Acquired Pelger-Huët Anomaly

Number of cells affected

A

True >68%
Pseudo PHA <35%

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8
Q

Pseudo- or Acquired Pelger-Huët Anomaly

TYPES OF WBC AFFECTED

A

True: All WBC lineages
Pseudo PHA: Neutrophil only

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9
Q

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

A

Alder-Reilly Anomaly

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10
Q

What is not affected in the Alder-Riley Anomaly

A

Leukocyte function

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11
Q

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

A

May-Hegglin Anomaly

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12
Q

May-Hegglin Anomaly is a mutation in the

A

MYH9 gene on chromosome 22q12- 13.3

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13
Q

most patients are asymptomatic, few have a mild bleeding tendencies

A

May-Hegglin Anomaly

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14
Q

mutations in genes that code for the production of lysosomal enzymes.

A

Lysosomal Storage Disorders

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15
Q

classified according to the under degraded macromolecule that accumulates in the cell

A

✓Mucopolysaccharidoses
✓Gaucher Disease
✓Niemann-Pick Disease

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16
Q

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

A

Mucopolysaccharidoses

17
Q

the most common of the lysosomal lipid storage diseases.

A

Gaucher Disease

18
Q

defect or deficiency in the catabolic enzyme beta-glucocerebrosidase

A

Gaucher Disease

19
Q

deficiency of lysosomal hydrolase enzyme acid sphingomyelinase

A

Niemann-Pick Disease