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HEMA LEC 2 > QUALITATIVE LEUKOCYTE ABNORMALITY > Flashcards

QUALITATIVE LEUKOCYTE ABNORMALITY Flashcards

1
Q

give me three plasma cell

A
  1. Russel bodies/grape bodies
  2. Dutcher bodies
  3. Flame cell
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2
Q

5 Lymphocytes

A
  1. Reactive lymphocyte
    TYPE1,2,3
  2. Basket cell
  3. Hair cell
  4. Sezary cell
  5. Reed-sternberg cell
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3
Q

Nucleus

A
  • hyposegmentation
  • hypersegmentation
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4
Q
  • deposition of accumulates substances
  • remnants of cellular structures
A

Cytoplasm

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5
Q

Number of cells in pelted hurt anomaly

A

63-93%

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6
Q

Number of affected cells in pseudo pelger huet anomaly

A

<38%

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7
Q

More than 5 lobes

A

Pelger huet anomaly

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8
Q

Pelger huet anomaly other term

A
  • Pince nez
  • peanut
  • dumb bell
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9
Q
  • Autosomal dominant
  • lamin b receptor
A

True pelger huet anomaly

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10
Q

Heterozygous:
Homozygous:

A

-Clinically normal
- cognitive impairment, heart defects, skeletal abnormalities

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11
Q

E.g. increase blast forms

A

Pseudo pelger huet anomaly

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12
Q

Acquired form of nuclear hyposegmentation

A

Pseudo pelger huet anomaly

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13
Q

Hypersegmentation of neutrophils (>5 lobes)

A

Undritz anomaly

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14
Q

Associated with megaloblastic anemia - vitamin b 9 or b 12 deficiency

A

Undritz anomaly / neutrophil hypersegmentation

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15
Q
  • no megaloblastic anemia
  • no clinical problems; non pathological
A

Hereditary neutrophil hypersegmentation

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16
Q
  • CXCR4 mutation
    -neutropenia
  • pyknotic neutrophil nucleus
A

Myelokathexis

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17
Q
  • inactivated X chromosome
  • lyonization
  • normally seen in females
  • kleinfelter syndrome
A

Barr body

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18
Q

Barr body has three variants

A
  • drumstick- not follow
  • racquet - hollow center
    -sessile nodule - no filament
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19
Q
  • granulocytes with large, darkly staining
    Metachromatic granules
  • resemble toxic granulation
  • granules: mucopolysaccharides
  • seen in gargoyles and other mucopolysaccharidosis
A

Alder-Reilly anomaly

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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

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21
Q

large prominent dark primary granules found in band and segmented neutrophils or monocytes

A

. Toxic GranulaHon

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22
Q

Represent precipitaFon of ribosomal
protein (RNA) due to metabolic toxicity
within the cells

A

Toxic Granulation

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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

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24
Q
  • rare, autosomal dominant disorder
  • MYH9 gene mutaFon on chr 22q12-13
A

. May-Hegglin Anomaly

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25
Q
  • Macrothrombocytopenia, large Dohle-like body
    inclusions in neutron, eo, baso and monocytes
  • inclusions are made up of dense fibrils thought to
    be mRNA
A

May-Hegglin Anomaly

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26
Q
  • Most paFents are asymptomaFc but some may
    have mild bleeding tendencies due to
    thrombocytopenia
A

May-Hegglin Anomaly

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27
Q

Large, hypogranular platelets, thrombocytopenia
(approx. 40-80 x10^9/L; Prolonged BT

A

May-Hegglin Anomaly

28
Q
  • light blue crescent shape or round inclusion
  • usually dohle like bodies
    -giant platelet
A

May-Hegglin Anomaly

29
Q
  • 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
A

Dohle Bodies

30
Q

composed of precipitated
myosin heavy chains

A
  • May-Hegglin anomay
31
Q

– consists of lamellar rows of
RER

A

*True Dohle bodies –

32
Q

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

A

Lipid Storage Disease

33
Q
  • 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
A

MUCOPOLYSACCHARIDOSES

34
Q
  • lipid metabolism is defecFve
  • includes Gaucher’s and Neimann-Pick disease
A

SPHINGOLIPIDOSES

35
Q
  • deficiency of B-glucocerebrosidase (gene 1q21-q22)
A

A. Gaucher’s Disease

36
Q
  • 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

A. Gaucher’s Disease

37
Q
  • 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
A

B. Niemann-Pick Disease

38
Q
  • 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)
A

Sea-blue HisHocytosis

39
Q
  • 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
A

Chediak-Hegashi Granules

40
Q

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

A

Auer Rods

41
Q
  • reflects phagocytosis
  • Autophagy
  • Phagocytosis of pathogens
  • EDTA sample is >2 hours old
  • seen in fungal and bacterial sepsis
A

. Toxic Vacuoles

42
Q
  • most prominent cytoplasmic alteraFon in
    eosinophil
  • emptying of primary and secondary granules into
    the phagosome
  • basophil degranulaFon is hard to assess
A

DegranulaHon

43
Q
  • 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
A

Pseudopods

44
Q

actual osmoFc swelling due to glass adhesiveness
- sFmulated neutrophils also exhibit swelling
- hypovitaminosis b12 and b9 also cause swelling
- large neutrophils are termed, Macropolycyte

A

. Cytoplasmic Swelling

45
Q
  • shrunken nuclei (nuclear water loss)
  • seen in sepFc condiFons and poor preparaFon
    techniques
A

Pyknosis

46
Q
  • early infecFon, toxemia and myeloproliferaFve
    disorders
A

Ringed Nuclei

47
Q

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

A

LE Cell

48
Q
  • monocytes or macrophages ingesFng another
    cell’s unaltered nucleus
  • usually seen as an arFfact in blood smear
    preparaFon
A

Tart Cell

49
Q
  • 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
A
  1. Russel Bodies / Grape Cell
50
Q

nvaginaFon of intracellular immunoglobulin
aggregates to the nucleus
- PAS posiFve (Periodic Acid Schiff)
- firstly associated with Waldenstrom’s
Macroglobulinemia
* Gammopathies

A
  1. Dutcher Bodies
51
Q
  • 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
A
  1. Flame Cell
52
Q
  • 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
A
  1. ReacHve Lymphocyte
53
Q

______________ are not commonly encountered on blood film

A
  • True plasma cells
54
Q

used to classify different types of reacFve or variant lymphocytes

A

Downey Lymphocyte ClassificaHon

55
Q

Downey Lymphocyte ClassificaHon - seen in:

A
  • seen in:
  • InfecFous Mononucleosis and other viral
    infecFons
  • Toxoplasmosis
  • Severe anemia
  • Chronic infecFon
56
Q
  • Turk’s irritaFon cell / Plasmacytoid lymphocytes
  • B cell in origin
A

Type I

57
Q

TYPE 1

A

seen in:
* Severe anemia
* Chronic infecFon
* Rubella

58
Q

TYPE 1 cytoplasm

A
  • Cytoplasm: moderately basophilic, vacuolated,
    foamy appearance
  • nucleus: indented or oval nucleus, dense blocks or
    chromaFn
59
Q
  • 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)
A

TYPE 2

60
Q
  • Transformed / ReFcular Lymphocytes
  • Cells in transiFon
  • Cytoplasm: vacuolated with abundant basophilia,
    and a clear perinuclear area
  • Nucleus: finely reFculated nuclear chromaFn,
    nucleoli usually visible
A

Type III

61
Q
  • 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
A
  1. Basket Cell
62
Q

lymphocytes with hair-like projecFons
- B cell origin
- posiFve for TRAP (Tartrate-resistant acid
phosphatase)
- Hairy Cell Leukemia

A
  1. Hair Cell
63
Q
  • lymphoid cells with prominently folded,
    cerebriform nuclei
  • T lymphocytes in origin
  • seen in Sezary Syndrome and Mycosis Fungoides
A
  1. Sezary Cell
64
Q
  • 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
A
  1. Reed-Sternberg Cell
65
Q
A

HEMA LEC 2 (8 decks)

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