Hematology: Leukemia and Non-malignant Leukocyte Flashcards

1
Q

abnormal uncontrollable proliferation of one or more hematopoietic stem cell in the BM and blood

A

Leukemia

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2
Q

3 manifestations of leukemia

A

bleeding
anemia
infection

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3
Q

classification of leukemia that is sudden in onset

acute or chronic?

A

acute

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4
Q

classification of leukemia that is variable wbc and higher proliferative state

A

acute

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5
Q

hematopoietic malignancy classification that divides acute leukemia to lymphblastic (L1 to L3) and Myeloblastic (M0 to M7)

A

FAB Classification

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6
Q

classification that is standard for diagnosis now

A

WHO

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7
Q

microscopic study of evaluation of chemical constituents within individual cells

A

Cytochemistry

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8
Q

PAS
Sudan Black
Toluidine blue
Iron stains
(PIS T)

are what classification of cytochemical stains

A

Non Enzymatic stains

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9
Q

cytochemical stain increased in pregnancy, infections, polycythemia, aplastic anemia

A

LAP

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10
Q

Is EDTA used in LAP stain? Yes or No.

A

No. It inhibits reaction.

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11
Q

Normal score of LAP

A

15-170

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12
Q

strong blue precipitated staining is graded what in LAP score

A

3+

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13
Q

deep blue with no visible cytoplasm

A

4+

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14
Q

stain that differentiates AML from ALL

A

MPO

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15
Q

AML is ___ on MPO.

Positive or negative?

A

positive

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16
Q

stains primary granules in granulocytes and monocytes

A

MPO

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17
Q

DIFFERENCE of SBB and MPO

A

In SBB smears does not have to be fresh

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18
Q

stains specific for granulocytic cells and stains black

A

Specific Esterase (NASDA)

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19
Q

differentiates monocytic leukemia from granulocytic leukemia

A

Non-Specific esterase

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20
Q

difference btwn nonspecific and specific esterase

A

NSE =
monocytes are +
granulocytes are -

SE =
monocytes are -
granulocytes are +

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21
Q

NSE that stains esterase in the monocytes and megakaryocytes

A

Alpha Naphtyl Acetate Esterase

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22
Q

ANBE NSE stains color _____.

A

dark red

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23
Q

ANAE NSE stains color ______.

A

red brown/orange red

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24
Q

PAS stains mycoproteins , glycoproteins and HMW CHONS present in almost all blood cell type except ____.

A

normoblasts

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25
Q

red purple stain in blocks in cytoplasm

A

PAS

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26
Q

normoblasts in PAS only stain positive in ______ and _______.

A

erythroleukemia, thalassemia

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27
Q

discrete purplish to dark red color stain

A

AP

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28
Q

marker for hairy cell leukemia

A

TRAP

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29
Q

only _______ is resistant to tartrate.

A

ACP isoenzyme 5

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30
Q

TdT (Terminal deoxyribonuecleotidyl Transferase) is positive in _____.

A

Lymphoblasts

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31
Q

Cytochemical stains AML is positive in

A
  1. MPO
  2. SBB
  3. SE
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32
Q

+ in erythroleukemia

A

PAS

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33
Q

Specific esterase

Positive cells: _____.
Negative cells: _____.

A

Positive
GRANULOCYTIC

Negative
MONOCYTIC AND LYMPHOCYTIC

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34
Q

What stains are positive for all cells?

A

PAS and ACP

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35
Q

pregnancy, infection, aplastic anemia, polycythemia (PIPA)in LAP

A

INCREASED

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36
Q

CML reaction in LAP

A

DECREASED

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37
Q

lamin β-receptor gene mutation

A

Pelger-Huet Anomaly

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38
Q

PHA is inherited. True or False

A

True. Autosomal Dominant.

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39
Q

the failure of neutrophils to segment (hyposegmentation) with coarseness of chromatin; sometimes also affects other WBCs

A

Pelger-huet Anomaly

40
Q

pince-nez spectacle
appearance

A

Heterozygous PHA

41
Q

WBC function is normal (asymptomatic)
normal granulation
63-93% of cells affected

PHA or PSEUDO PHA?

A

PHA

42
Q

Seen in
MDS, AML, CMPDs, patients
receiving chemotherapy, severe infections, cancer metastizing to the bone, drugs

A

pseudo pelger-huet anomaly

43
Q

Entire cell is greatly enlarged and
nuclear material fills the cell with long thin filaments

A

NEUTROPHILS HYPERSEGMENTATION

44
Q

NORMAL granulocyte production,
IMPAIRED release;
component of WHIM
found in hereditary NH

A

Myelokathexis

45
Q

Autosomal recessive with dense blue black granules in WBC

A

Alder Reilly Anomaly

46
Q

Alder Reilly Anomaly is seen in what disease/s

A

Hunters and Hurlers disease
Maroteaux-Lamy polydystrophic dwarfism

47
Q

Neutrophilia, Dohle bodies and left shift

toxic granules or alder reilly?

A

toxic granules

48
Q

Round projection of nuclear chromatin connected to the neutrophil nucleus by a fine strand of chromatin (drumstick appearance)

nonpathogenic and represents x chromosomes in females

A

barr bodies

49
Q

Very large reddish-purple or greenish-gray
granules in all WBCs

cell granules normal in content but abnormally packaged

also a rare and fatal disorder in children

A

CHEDIAK HIGASHI

50
Q

albinism, mental retardation, poor resistance to infections

A

chediak higashi

51
Q

Remnants of REC containing RNA

Seen in infections, poisoning, burns, following
chemotherapy, pregnancy

A

Dohle bodies

52
Q

Autosomal dominant, disordered production of myosin heavy chain. Characterized by:

Leukopenia
Thrombocytopenia
Gray blue spindle shaped inclusions (resemble dohle bodies but larger)

A

MAY HEGGLIN ANOMALY

53
Q

Seen in all WBC (granulocytes and monocytes)

Dohle bodies vs May Hegglin

A

May Hegglin

54
Q

Seen in all neutrophils

Dohle bodies vs May Hegglin

A

Dohle bodies

55
Q

chemokinesis is normal but chemotaxis is not; characterized by boils and furuncles

A

Job’s syndrome

56
Q

both chemotaxis and chemokinesis are abnormal

neutropenia, but BM granulocytes are normal

A

lazy leukocyte

57
Q

Mutation(s) in NADPH oxidase genes

Sex-linked recessive (disease of male infants) and Autosomal recessive

A

CHRONIC GRANULOMATOUS DISEASE (CGD)

58
Q

Phagocytes ingest but can’t kill catalase (+) organisms due to lack of respiratory b

A

CHRONIC GRANULOMATOUS DISEASE (CGD)

59
Q

yellow formazan reduced to blue insoluble blue formazan

A

NBT Test

60
Q

Bacterial killing is slow but complete

Benign condition, patients rarely troubled by infections

A

MYELOPEROXIDASE (MPO) DEFICIENCY

61
Q

aka glycosaminoglycan (GAG) storage diseases

Deficiency of specific enzymes involved in the
degradation of mucopolysaccharides

A

MUCOPOLYSACCHARIDOSES (MPSS)

62
Q

SUBSTANCES stored in MPS III (San Filippo)

A

heparan sulfate only

63
Q

Deficient enzyme in MPS II-severe

A

Iduronate sulfatase

64
Q

Also called Hurler syndrome

A

MPS I-severe

65
Q

MPS I deficient enzyme

A

a-l-iduronidase

66
Q

gargoylism
Metachromatic Reilly bodies
diagnose by use of assays

A

MPS

67
Q

Result from the lack of a functional enzyme
required for breakdown of lipids that have been
ingested by phagocytes

A

LIPIDOSES

68
Q

GAUCHER DISEASE TYPE II IS AUTOSOMAL DOMINANT. TRUE OR FALSE.

A

TRUE. only lipidoses that is not autosomal recessive.

69
Q

Deficiency in β-glucocerebrosidase

hepatomegaly, Gaucher cells in BM, markedly elevated serum ACP

A

GAUCHER’S DISEASE

70
Q

Diagnostic biomarker of Gaucher’s disease

A

chitotriosidase

71
Q

juvenile form; life expectancy is short of Gaucher’s disease

A

Type III

72
Q

infantile or cerebral form; neurologic deterioration, death within first few years of life type of Gaucher’s

A

Type II

73
Q

Crumpled tissue paper or cigarette butt cell

Fibrillar blue-grey cytoplasm with striated or wrinkled appearance (onion skin-like)

Cells strongly PAS (+)

A

gaucher’s cell

74
Q

Deficiency in α-sphingomylinase

Most prevalent form develops in infancy and
resembles type II Gaucher disease

A

NIEMANN-PICK DISEASE

75
Q

Also foam or bubble cell

macrophages with lipid filled lysosomes that appear as vacuole

A

NIEMANN-PICK CELL

76
Q

Deficiency in Hexominidase A

High incidence in Ashkenazi, Jewish population

Vacuolated and foamy lymphocytes

A

TAY-SACHS DISEASE

77
Q

Found in association with ↑ tissue stores of
phospholipids/glycolipids

Accumulation of histiocytes filled with lipid-rich
granules in the spleen and BM

Hepatosplenomegaly and thrombocytopenia

A

SEA-BLUE HISTIOCYTOSIS

78
Q

Defective development of thymus, failure of T
cell development

A

NEZELOF’S SYNDROME

79
Q

Almost indistinguishable from Nezelof’s

Not of genetic origin, cause is unknown

A

DIGEORGE’S SYNDROME

80
Q

ALL classes of Ig are either absent or extremely low

Absent B cells, T cells normal

Prone to bacterial infections

A

INFANTILE SEX-LINKED (BRUTON) AGAMMAGLOBULINEMIA

81
Q

One or combination of Ig is either missing entirely or synthesized only in small quantities

A

COMMON VARIABLE HYPOGAMMAGLOBULINEMIA

82
Q

Depletions of T, B and NK cells

A

SCID

83
Q

Migration, adhesion, and activation of T-cells, B
cells and NK cells are affected

SEX LINKED

A

WISKOTT-ALDRICH SYNDROME

84
Q

Increased LAP score with rare blasts
normal platelets

NLR or CML?

A

NLR

85
Q

Presence of immature neutrophils, nucleated RBCs and teardrop RBCs

Commonly associated with myelophthisis
and primary myelofibrosis

A

LEUKOERYTHROBLASTIC REACTION

86
Q

Mechanism: Increase outflow from BM and shift from marginal pool to circulating pool

A

NEUTROPHILIA

circuLating = neutrophiLia

87
Q

mild left shift

A

band and metamyelocyte

88
Q

MARKED left shift

A

blast

89
Q

seen in
Bacterial infection – TB, SBE, syphilis

A

monocytosis

90
Q

Stem cell disorders – aplastic anemia

A

monocytopenia

91
Q

definitive test for IM

A

EBNA

92
Q

Indicative of depressed locomotion
Caused by bacterial and therapeutic agents

A

pseudopods

93
Q

what type of neutrophil nuclear alteration is Metastatic carcinoma or after irradiation

A
94
Q

Downey classification Type II size

A

15-25 um

95
Q

irregular or scalloped type of downey’s class

A

Type II

96
Q

fine chromatin; highly visible nucleoli type of downey’s class

A

Type III