MODULE 6 UNIT 2: WHITE BLOOD CELL ANOMALIES Flashcards by Arriane Camacho

Owing to the fact that white blood cells are a heterogenous population, anomalies of white blood cells can be classified according to the

type of cell, cell parts affected and function

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A. NUCLEAR ABNORMALITIES

Hyposegmentation
Hypersegmentation
Barr body (Sex chromatin)

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B. CYTOPLASMIC ABNORMALITIES

Alder-Reilly Bodies
Auer Rods
Chediak-Higashi granules
Dohle Bodies
Toxic granules
Toxic vacuoles

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C. ABNORMALITIES OF CELLS EXHIBITING PHAGOCYTOSIS

LE cell
Tart Cell

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D. FUNCTION ABNORMALITIES

Job syndrome
Lazy Leukocyte Syndrome
Chronic granulomatous disease
Leukocyte Adhesion Disorder- I (LAD-I)

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E. ABNORMALITIES ASSOCIATED WITH LYMPHOCYTES

Atypical lymphocytes
Basket cell/ Smudge cell
Hairy cell
Sezary cell

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F. ABNORMALITIES ASSOCIATED WITH PLASMA CELLS

Flame Cell
Russell Bodies
Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
Dutcher’s Bodies

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G. MONOCYTE/ MACROPHAGE LYSOSOMAL STORAGE DISORDERS

Lipid Storage Diseases
Mucopolysaccharidosis

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Lipid Storage Diseases

Gaucher Disease
NiemannPick Disease
Fabry Disease
Tay-Sachs disease
Sandhoff disease
Sea Blue Histiocytes

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Mucopolysaccharidosis

MPS I- Severe
MPS I- Attenuated
MPS II
MPS III
MPS IV

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 benign anomaly of neutrophils

Hyposegmentation

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 nucleus fails to segment properly

Hyposegmentation

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 bilobed nuclei

Hyposegmentation

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o Dumbbell-shaped
o Spectacle-shaped
o Peanut-shaped

Hyposegmentation

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o “Pince-nez”

Hyposegmentation

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 abnormality in the maturation of the neutrophils (abnormality in DNA synthesis)

Hypersegmentation

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 6 or more lobed nucleus

Hypersegmentation

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o seen in the nuclear material

Appendage

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o represents the second X chromosome in females o may be seen in 2-3% of neutrophils in females

Appendage

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o NOT FOUND IN NORMAL MALES

Barr body

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o a small, well-defined, round projection of nuclear chromatin

Barr body

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o connected to the nucleus of the neutrophil by a single, fine strand of chromatin

Barr body

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 large purple-black coarse cytoplasmic granules

Alder-Reilly Bodies

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 accumulations of degraded mucopolysaccharides

Alder-Reilly Bodies

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 may be found in all leukocytes in cases of its diseases

Alder-Reilly Bodies

 may resemble toxic granules

Alder-Reilly Bodies

used to differentiate Alder-Reilly Bodies

o Cetyl Trimethyl Ammonium Bromide (CTAB) Test

occurrence of white turbidity

Alder-Reilly Bodies

 pink or red rod-shaped cytoplasmic structures

Auer Rods

 formed from the fusion of primary granules

Auer Rods

 normally found in the younger myeloid precursors

Auer Rods

 When seen in mature cells, they are associated with AML or AMML

Auer Rods

 giant red, blue to grayish round inclusions

Chediak-Higashi granules

 deficient in enzymes for phagocytosis

Chediak-Higashi granules

 giant lysosomal granules o lymphocyte o Neutrophil o monocyte

Chediak-Higashi granules

 peroxidase (+)  Sudan Black B (+)

Chediak-Higashi granules

 Amato Bodies

Dohle Bodies

 single or multiple blue cytoplasmic inclusions in the neutrophil

Dohle Bodies

 aggregates of free ribosomes or rough endoplasmic reticulum

Dohle Bodies

 often confused with May-Hegglin anomaly

Dohle Bodies

leukocyte inclusions are composed of precipitated myosin heavy chains

Dohle Bodies

 large purple to black azurophilic granules

Toxic granules

 thought to be primary granules

Toxic granules

 often present with Dohle bodies and toxic vacuoles

Toxic granules

 ↑ ALP activity

Toxic granules

 large purple to black azurophilic granules

Toxic vacuoles

 thought to be primary granules

Toxic vacuoles

 ↑ ALP activity

Toxic granules

 often present with dohle bodies and toxic granules

. Toxic vacuoles

 large empty white areas within cytoplasm

Toxic vacuoles

 represent end-stage phagocytosis

Toxic vacuoles

 neutrophil with large purple homogenous round inclusion with nucleus wrapped around

LE cell

 appear smooth and evenly stained

LE cell

 a monocyte with ingested lymphocyte

Tart Cell

 appears rough & unevenly stained

Tart Cell

 neutrophils exhibit normal random activity (chemokinesis) but abnormal directional activity (chemotaxis)

Job syndrome

Random locomotion

Chemokinesis

Directed locomotion brought about by CHEMOTAXINS (chemotactic factors such as endotoxins and other bacterial products, cytokines, and lymphokines)

Chemotaxis

Locomotion through unruptured walls of the blood vessels

Diapedesis

 neutrophils have abnormal random and directional activity

Lazy Leukocyte Syndrome

 inability of phagocytes to kill ingested microorganisms due to impaired NADPH oxidase/ Respiratory burst

Chronic granulomatous disease

 diagnostic test: Nitroblue tetrazolium dye test

Chronic granulomatous disease

 caused by decreased or truncated β2 integrin, needed for neutrophil adhesion to endothelial cells and recognition of bacteria

Leukocyte Adhesion Disorder- I (LAD-I)

 caused by decreased amount or function of selectin ligands and defective leukocyte recruitment.

Leukocyte Adhesion DisorderII (LAD-II)

 brought about by defective protein Kindlin-3, needed for β integrin activation and leukocyte rolling

Leukocyte Adhesion DisorderIII (LAD-III)

 Failed response to external signals that would normally result in leukocyte activation

Leukocyte Adhesion DisorderIII (LAD-III)

 reactive lymphocytes, variant lymphocytes, transformed lymphocytes, and leukocytoid lymphocytes

Atypical lymphocytes

 can be referred to as DOWNEY CELLS (Classification by Dr. Hal Downey) of different types

Atypical lymphocytes

▪ Turk’s Irritation cells/ Plasmacytoid lymphocytes

Type I

▪ Characterized with a large block of chromatin

Type I

▪ IM cell

Type II

▪ Characterized by a round mass of chromatin; “BALLERINA SKIRT” appearance

Type II

▪ Seen in Infectious Mononucleosis

Type II Atypical lymphocytes

• Causative agent: Epstein-Barr virus

Infectious Mononucleosis

• Characterized by lymphocytosis often mistaken as monocytosis

Infectious Mononucleosis

T lymphocytes reacting to EBV-infected B lymphocytes

Atypical lymphocytes

▪ Vacoulated, “Swiss cheese” appearance, “Moth-eaten” appearance

Type III

 white blood cells that have degenerated nucleus or ruptured cell

Basket cell/ Smudge cell

 are B lymphocytes with hair like cytoplasmic projections surrounding the nucleus

Hairy cell

associated with Human T-lymphotropic virus Type II

Hairy cell

 stain positive with tartrate resistant acid phosphatase (TRAP)

Hairy cell

 a lymphocyte with T cell characteristics with nucleus

Sezary cell

 grooved and have brain like convolutions (“cerebri” form)

Sezary cell

 a plasma cell with red to pink cytoplasm associated with increase in immunoglobulin

Flame Cell

 individual globules of immunoglobulins seen in plasma cells

Russell Bodies

 are plasma cell that contains vacuoles and large protein globules

Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell

 occurs as the accumulation of Russell bodies

Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell

 intranuclear protein inclusions occurring in the plasma cell

Dutcher’s Bodies

Gaucher Disease Deficient Enzyme

βglucocerebrosidase

Gaucher Disease Accumulation of:

Glucocerebroside

Gaucher Disease Morphologic Appearance

CYTOPLASM  Wrinkled/ Crumpled  “Cat-Scratch” OTHERS  Clinical Triad used in Diagnosis  Hepatomegaly  Gaucher cells in BM  ↑ serum phosphatase

NiemannPick Disease Deficient Enzyme

Sphingomyelinase

NiemannPick Disease Accumulation of:

Sphingomyelin

NiemannPick Disease Morphologic Appearance

CYTOPLASM  Foamy

Fabry Disease Deficient Enzyme

α-galactosidase

Fabry Disease Accumulation of:

Ceramide trihexose

Fabry Disease Morphologic Appearance

---

Tay-Sachs disease Deficient Enzyme

Hexosaminidase A

Tay-Sachs disease Accumulation of:

GM2 ganglioside

Tay-Sachs disease Morphologic Appearance

CYTOPLASM  Vacuolated

Sandhoff disease Deficient Enzyme

Hexosaminidase A Hexosaminidase A & B

Sandhoff disease Accumulation of:

Glycolipid & ganglioside

Sandhoff disease Morphologic Appearance

CYTOPLASM  Vacuolated

Sea Blue Histiocytes Deficient Enzyme

Unknown

Sea Blue Histiocytes Accumulation of:

Unknown

Sea Blue Histiocytes Morphologic Appearance

CYTOPLASM  Blue-green cytoplasm

MPS I- Severe Deficient Enzyme: α-l-iduronidase Accumulated substance: Name:

Dermatan sulfate Heparan sulfate Hurler Syndrome

MPS I- Attenuated Deficient Enzyme: α-l-iduronidase Accumulated substance: Name:

Dermatan sulfate Heparan sulfate Scheie Syndrome

MPS II Deficient Enzyme: Iduronate sulfatase Accumulated substance: Name:

Dermatan sulfate Heparan sulfate Hunter Syndrome

MPS III Deficient Enzyme: Heparan-N-sulfate aNacetylglucosamini dase Acetyl–coenzyme A:aglucosaminide N-acetyltransferase Accumulated substance: - - Name: - -

-Heparan sulfate -Heparan sulfate -Sanfilippo Syndrome Type A -Sanfilippo Syndrome Type B

MPS IV Deficient Enzyme: Galactose-6sulfatase Accumulated substance: Name:

Keratan sulfate Chondroitin-6sulfate Morquio syndrome Type A

MODULE 6 UNIT 2: WHITE BLOOD CELL ANOMALIES Flashcards

(111 cards)