QUALITATIVE LEUKOCYTE ABNORMALITY Flashcards
1
Q
give me three plasma cell
A
- Russel bodies/grape bodies
- Dutcher bodies
- Flame cell
2
Q
5 Lymphocytes
A
- Reactive lymphocyte
TYPE1,2,3 - Basket cell
- Hair cell
- Sezary cell
- Reed-sternberg cell
3
Q
Nucleus
A
- hyposegmentation
- hypersegmentation
4
Q
- deposition of accumulates substances
- remnants of cellular structures
A
Cytoplasm
5
Q
Number of cells in pelted hurt anomaly
A
63-93%
6
Q
Number of affected cells in pseudo pelger huet anomaly
A
<38%
7
Q
More than 5 lobes
A
Pelger huet anomaly
8
Q
Pelger huet anomaly other term
A
- Pince nez
- peanut
- dumb bell
9
Q
- Autosomal dominant
- lamin b receptor
A
True pelger huet anomaly
10
Q
Heterozygous:
Homozygous:
A
-Clinically normal
- cognitive impairment, heart defects, skeletal abnormalities
11
Q
E.g. increase blast forms
A
Pseudo pelger huet anomaly
12
Q
Acquired form of nuclear hyposegmentation
A
Pseudo pelger huet anomaly
13
Q
Hypersegmentation of neutrophils (>5 lobes)
A
Undritz anomaly
14
Q
Associated with megaloblastic anemia - vitamin b 9 or b 12 deficiency
A
Undritz anomaly / neutrophil hypersegmentation
15
Q
- no megaloblastic anemia
- no clinical problems; non pathological
A
Hereditary neutrophil hypersegmentation
16
Q
- CXCR4 mutation
-neutropenia - pyknotic neutrophil nucleus
A
Myelokathexis
17
Q
- inactivated X chromosome
- lyonization
- normally seen in females
- kleinfelter syndrome
A
Barr body
18
Q
Barr body has three variants
A
- drumstick- not follow
- racquet - hollow center
-sessile nodule - no filament
19
Q
- granulocytes with large, darkly staining
Metachromatic granules - resemble toxic granulation
- granules: mucopolysaccharides
- seen in gargoyles and other mucopolysaccharidosis
A
Alder-Reilly anomaly
20
Q
s/s: mental retardaFon, clouding of the corners of
the eye, dwarfism
- leukocyte funcFon is not notably affected
- Heart failure – usual cause of death
- Treatment: _________, ______
A
Gargoylism/ hurler syndrome/ MPS 1
Stem cell transportaFon, enzyme
therapies
21
Q
large prominent dark primary granules found in band and segmented neutrophils or monocytes
A
. Toxic GranulaHon
22
Q
Represent precipitaFon of ribosomal
protein (RNA) due to metabolic toxicity
within the cells
A
Toxic Granulation
23
Q
- Azurophilic (primary) granules are peroxidaseposiFve
- found in burns, severe infecFons, cancer
(malignancy), hematoma, Fssue undergoing necrosis, or as a result of drug therapy - extent of toxic granulaFon is usually graded on a
scale of 1+ to 4+ being the most severe
A
Toxic Granulation
24
Q
- rare, autosomal dominant disorder
- MYH9 gene mutaFon on chr 22q12-13
A
. May-Hegglin Anomaly
25
- Macrothrombocytopenia, large Dohle-like body
inclusions in neutron, eo, baso and monocytes
- inclusions are made up of dense fibrils thought to
be mRNA
May-Hegglin Anomaly
26
- Most paFents are asymptomaFc but some may
have mild bleeding tendencies due to
thrombocytopenia
May-Hegglin Anomaly
27
Large, hypogranular platelets, thrombocytopenia
(approx. 40-80 x10^9/L; Prolonged BT
May-Hegglin Anomaly
28
- light blue crescent shape or round inclusion
- usually dohle like bodies
-giant platelet
May-Hegglin Anomaly
29
- intracytoplasmic pale blue, round inclusion, seen
near the periphery of the cytoplasm of neutrophils
but may also be seen in monocytes or lymphocytes
- aggregates of RER (remnants of rRNA)
- may be seen in conjuncFon with toxic granulaFon
- may also be seen normally but in small amounts
- associated with burns, infecFous disease, scarlet
fever, aplasFc anemia, but nonspecific because it
can also be seen in pregnancy
Dohle Bodies
30
composed of precipitated
myosin heavy chains
* May-Hegglin anomay
31
– consists of lamellar rows of
RER
*True Dohle bodies –
32
QualitaFve disorders of monocytes-macrophages
are manifested as lipid storage diseases
- Macrophage: prone to accumulate undegraded
lipid products à expansion of reFculoendothelial
Fssue
- MonocyFc disorders: Gaucher disease and
Niemann-Pick disease
Lipid Storage Disease
33
- deficient acFvity of an enzyme necessary for the degradaFon of dermatan sulfate, heparan sulfate,
keratan sulfate and/or chondroiFn sulfate
- results in serious physical and cogniFve problems
- shortened survival rate
MUCOPOLYSACCHARIDOSES
34
- lipid metabolism is defecFve
- includes Gaucher’s and Neimann-Pick disease
SPHINGOLIPIDOSES
35
- deficiency of B-glucocerebrosidase (gene 1q21-q22)
A. Gaucher’s Disease
36
- leading to accumulaFon of glucocerebroside
(lungs, liver, spleen, brain)
- autosomal recessive
- most common lysosomal lipid storage diseases
- abundant fibrillary blue-gray cytoplasm with
striated or wrinkled appearance (“onion skin-like”)
- B-glucosidase (glucocerebrosidase) is available to
confirm diagnosis
- treatment: Enzyme replacement therapy with
recombinant glucocerebrosidase
A. Gaucher’s Disease
37
- autosomal recessive - deficiency of the enzyme sp
hingomyelinase
- recessive mutaFons in the SMPD1 gene
– a
deficiency of acid Sp
hingomyelinase (ASM) and a
subsequent buildup of the sphingomyelin in the
liver, spleen, lungs - defecFve NPC1 and NPC2 genes
B. Niemann-Pick Disease
38
- adult form of Niemann Pick Disease and Chronic
GranulocyFc Leukemia
- accumulaFon of phosphoshingolipids in cytoplasm
- hisFocytes filled with lipid-rich granules that stain
Blue-green with polychrome stain (Giemsa, Wright
Stain)
Sea-blue HisHocytosis
39
- rare, autosomal recessive disease of immune
dysregulaFon
- mutaFon in the CHS1 LYST gene on chr 1q42.1-2
* Encodes for protein that regulates the
morphology and funcFon of lysosomerelated organelles
Chediak-Hegashi Granules
40
derived from fusion of primary granules which are
peroxidase posiFve
- rod-shaped or needle-shaped
- seen in myeloblasts and promyelocytes
* Acute MyeloblasFc Leukemia
* Acute PromyelocyFc Leukemia
Auer Rods
41
- reflects phagocytosis
* Autophagy
* Phagocytosis of pathogens
- EDTA sample is >2 hours old
- seen in fungal and bacterial sepsis
. Toxic Vacuoles
42
- most prominent cytoplasmic alteraFon in
eosinophil
- emptying of primary and secondary granules into
the phagosome
- basophil degranulaFon is hard to assess
DegranulaHon
43
- normally formed during extravasaFon
- in the blood smear:
* Normal for monocytes
* Rare and toxic for neutrophils
- Amoeboid structures (usually granule-free)
- can be pathologic or arFfactual
* Kawasaki Disease
Pseudopods
44
actual osmoFc swelling due to glass adhesiveness
- sFmulated neutrophils also exhibit swelling
- hypovitaminosis b12 and b9 also cause swelling
- large neutrophils are termed, Macropolycyte
. Cytoplasmic Swelling
45
- shrunken nuclei (nuclear water loss)
- seen in sepFc condiFons and poor preparaFon
techniques
Pyknosis
46
- early infecFon, toxemia and myeloproliferaFve
disorders
Ringed Nuclei
47
neutrophils or monocytes engulfing other cell’s
nuclei
- normal segmented neutrophil or another
phagocyFc cell with the engulfed homogenous and
swollen nucleus of either a neutrophil or a
lymphocyte
- in vitro phenomenon: classic outdated test for
Lupus
* Blood sample of a known px + blood sample
of px suspected with lupus erythematosus
or other autoimmune diseases
LE Cell
48
- monocytes or macrophages ingesFng another
cell’s unaltered nucleus
- usually seen as an arFfact in blood smear
preparaFon
Tart Cell
49
- aggregates of immunoglobulins in the plasma cell
- aka: Morula cell, Berry cell, Grape cell, Moq cell
- eosinophilic cytoplasmic inclusion
- round, glassy, transparent bodies
- found in gammopathies
1. Russel Bodies / Grape Cell
50
nvaginaFon of intracellular immunoglobulin
aggregates to the nucleus
- PAS posiFve (Periodic Acid Schiff)
- firstly associated with Waldenstrom’s
Macroglobulinemia
* Gammopathies
2. Dutcher Bodies
51
- atypical plasma cell characterized by the fiery fingers formed by cytoplasmic projecFons
- cytoplasm stains a bright-red/red-purple color due
to increased IgA
- contains increased quanFFes of glycogen or intracellular deposits of amorphous maqer
- seen in IgA Myeloma and other plasma cell dyscrasia
3. Flame Cell
52
- aka: Variant, Atypical, transformed, effector,
Plasmacytoid, Turk cells, Downey, Immunoblasts
- reacFve changes occur as lymphocytes are sFmulated when interacFng with anFgens in peripheral lymphoid organs
- variaFon in the N:C raFo, nuclear shape and
chromaFn paqern; Nucleoli may be visible
1. ReacHve Lymphocyte
53
______________ are not commonly encountered on blood film
- True plasma cells
54
used to classify different types of reacFve or variant lymphocytes
Downey Lymphocyte ClassificaHon
55
Downey Lymphocyte ClassificaHon - seen in:
- seen in:
* InfecFous Mononucleosis and other viral
infecFons
* Toxoplasmosis
* Severe anemia
* Chronic infecFon
56
- Turk’s irritaFon cell / Plasmacytoid lymphocytes
- B cell in origin
Type I
57
TYPE 1
seen in:
* Severe anemia
* Chronic infecFon
* Rubella
58
TYPE 1 cytoplasm
- Cytoplasm: moderately basophilic, vacuolated,
foamy appearance
- nucleus: indented or oval nucleus, dense blocks or
chromaFn
59
- InfecFous Mononucleosis Cell
- T cell in nature
- Cytoplasm: ballerina skirt appearance, resembling
a fried egg / flared skirt, basophilia at periphery
- Nucleus: round mass of chromaFn; nuclear
banding (EDTA)
TYPE 2
60
- Transformed / ReFcular Lymphocytes
- Cells in transiFon
- Cytoplasm: vacuolated with abundant basophilia,
and a clear perinuclear area
- Nucleus: finely reFculated nuclear chromaFn,
nucleoli usually visible
Type III
61
- aka: Smudge cell or Shadow cells of Grumpecht
- fragile cells that are damaged during smear
preparaFon
- increased in Chronic LymphocyFc Leukemia (CLL)
- PrevenFon: Add 22% bovine albumin in the blood
sample
2. Basket Cell
62
lymphocytes with hair-like projecFons
- B cell origin
- posiFve for TRAP (Tartrate-resistant acid
phosphatase)
- Hairy Cell Leukemia
3. Hair Cell
63
- lymphoid cells with prominently folded,
cerebriform nuclei
- T lymphocytes in origin
- seen in Sezary Syndrome and Mycosis Fungoides
4. Sezary Cell
64
- large, bilobed nucleus or 2 nuclei with eosinophilic
nucleoli and abundant cytoplasm (Owl’s eye appearance)
- derived from apoptoFc germinal center B cells
- pathognomonic of Hodgkin’s Lymphoma
5. Reed-Sternberg Cell
65
