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HEMATOLOGY (Topnotcher QT) > LEUKOCYTE DISORDERS > Flashcards

LEUKOCYTE DISORDERS Flashcards

memorization

1
Q

Redistribution of the blood pools causes a short-term increase in the total WBC count and the absolute number of neutrophils in the circulating granulocyte pool; caused by exercise, stress, pain and pregnancy

A

Shift/physiologic pseudoneutrophilia

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2
Q

Blood picture mimics chronic myelogenous leukemia; benign, specific response to specific agent or stimulus; WBC count can increase to 50-100x10^9/L; increased LAP score; there is a shift to the left with toxic changes to neutrophils

A

Neutrophilic Leukemoid Reaction

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3
Q

Morphologically normal, but functionally abnormal because of enzyme deficiency (NADPH oxidase) that results in an inability to degranulate which causes inhibited bacteriocidal function

A

Chronic granulomatous disease (CGD)

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4
Q

what is the treatment for CGD?

A

granulocyte concentrate

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5
Q

Both morphologically and functionally abnormal leukocytes; WBCs unable to degranulate and kill invading bacteria; abnormal fusion of primary and secondary neutrophilic granules; leukocytes have gigantic granules that are peroxidase positive

A

Chediak-Higashi syndrome

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6
Q

5 or more lobes in the neutrophil; associated with megaloblastic anemia

A

Hypersegmentation

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7
Q

Nucleus is hyperclumped and does not mature past the two-fold stage’ nucleus has a dumbbell or peanut-shape; “pince-nez” appearance; morphologically abnormal but functionally normal

A

Pelger-Huet Anomaly

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8
Q

What is the difference of Pelger-Huet anomaly and Pseudo Pelger-Huet Anomaly?

A

In Pseudo PH Anomaly - round nucleus

In Pelger-Huet anomaly - Dumbbell shape nucleus

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9
Q

Which of the following is not characterized by giant plaetelets?

A. Bernard-Soulier Syndrome
B. Gray Platelet
C. Wiskott-Aldrich Syndrome
D. MHA

A

C. Wiskott-Aldrich Syndrome

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10
Q

Large crystalline, Dohle-like inclusions in the cytoplasm of neutrophils on Wright’s stain; gray blue spindle (cigar) shaped; Morphologically abnormal but functionally normal; giant platelets, thrombocytopenia, and clinical bleeding

A

May-Hegglin anomaly

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11
Q

Large azurophilic granules appear in the cytoplasm of all or only one cell line; granules contain degraded mucopolysaccharides due to enzyme defect

A

Alder-Reily Anomaly

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12
Q

Most common lipid disorder; deficiency in glucocerecrosidase causes glucocerebroside to accumulate in the macrophages of the bone marrow, spleen and liver; Crumpled tissue paper appearance of macrophage

A

Gaucher disease

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13
Q

Deficiency in sphingomyelinase causes sphingomyelin to accumulate in macrophages in multiple organs and bone marrow; foamy appearance of macrophages (foam cells)

A

Niemann-Pick Disease

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14
Q

What enzyme is deficient in Niemann-Pick disease?

a. Sphingomyelinase
b. glucocerebrosidase
c. Arylsulfatase A
d. Hexosaminidase A

A

A. Sphingomyelinase

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15
Q

What enzyme is deficient in Gaucher disease?

a. Sphingomyelinase
b. glucocerebrosidase
c. Arylsulfatase A
d. Hexosaminidase A

A

B. glucocerebrosidase

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16
Q

What enzyme is deficient in Fabry’s disease?

a. Alpha galactosidase
b. Cerebroside beta galactosidase
c. Hexosaminidase A and B
d. Hexosaminidase A

A

A. Alpha galacosidase

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17
Q

What enzyme is deficient in Tay Sach disease?

a. Alpha galactosidase
b. Cerebroside beta galactosidase
c. Hexosaminidase A and B
d. Hexosaminidase A

A

D. Hexosaminidase A

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18
Q

What enzyme is deficient in Krabbe disease?

a. Alpha galactosidase
b. Cerebroside beta galactosidase
c. Hexosaminidase A and B
d. Arylsulfatase A

A

B. Cerebroside beta galactosidase

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19
Q

causative agent of IM

A

Epstein-Barr virus

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20
Q

what is the target cell of EBV?

A

B-cells

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21
Q

Infectious Mononucleosis is also known as “_____”

A

the kissing disease

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22
Q

Is IM positive or negative for heterophile antibody test?

A

yes. positive

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23
Q

Symptoms similar to IM; most commonly transmitted infection from mother to fetus/ most common congenital infection; negative for heterophile antibody test; no reactive lymphocytes

A

Cytomegalovirus

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24
Q

Associated with adenovirus and coxsackie A virus; Contagioyus mostly affecting young children; lymphocytosis with no reactive lymphocytes

A

Infectious lymphocytosis

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25
Q

Defines acute leukemia as >30% bone marrow blasts; easier to use and is still widely taught

A

French-America-British (FAB) Classification

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26
Q

Defines acute leukemia as >20% bone marrow blasts; standard for diagnosis

A

World Health Organization (WHO) Classification

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27
Q

WHO Classification is based on: (enumerate)

A
  1. Cellular morphology
  2. Cytochemical stains
  3. Immunologic probes of cell markers (CD markers)
  4. Cytogenetics
  5. Molecular abnormalities
  6. Clinical syndrome
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28
Q

FAB Classification is based on: (enumerate)

A
  1. Cellular morphology
  2. Cytochemical stain results
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29
Q

Stains siderotic granules, pappenheimer bodies and hemosiderin

A

Perl’s Prussian blue stain

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30
Q

Stains ALP present in the neutrophil

A

Leukocyte Alkaline Phsphatase (ALP)

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31
Q

LAP is also known as “_____”

A

KAPLOW COUNT

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32
Q

LAP score is increased in CML, PNH, Sickle cell anemia, IM, PA and decreased in PV, last trimester of pregnancy, infections with neutrophilia

True or False

A

False

LAP is increased in
1. PV
2. Last trimester of pregnancy
3. infections with neutrophilia

LAP is decreased in
1. CML
2. PNH
3. Sickle cell anemia
4. IM
5. PA

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33
Q

Stains peroxidase present in granulocytes and monocytes; recommended specimen is fresh blood smear from a capillary puncture

A

Myeloperoxidase stain (MPO)

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34
Q

MPO is used to differentiate:

A

Used to differentiate blasts of acute myelogenous leukemias (AMLs) from acute lymphoblastic and monocytic leukemias (ALLs)

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35
Q

These are lysosomes that can be seen in AMLs when stained with MPO

A

Auer Rods

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36
Q

Stains lipids present in granulocytes and monocytes

A

Sudan Black B

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37
Q

SBB is used to differentiate _____ and ______ from ______

A

SBB is used to differentiate Acute myelogenous leukemias and MYELOMONOCYTIC Leukemias from acute lymphocytic leukemia

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38
Q

Stains mucoproteins, glycoproteins, and high molecular weight carbohydrates;

A

Periodic Acid Schiff stain

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39
Q

PAS stain is used in the diagnosis of?

A

DiGuglielmo’s Syndrome (FAB M6)

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40
Q

In PAS stain, L1 and L2 produces what pattern?

A

Block pattern

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41
Q

Stains esterases in granulocytes and mast cell granules

A

Naphthol AS-D chloroacetate esterase

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42
Q

Naphthol AS-D chloroacetate esterase is also known as

A

Specific esterase

43
Q

Specific esterase is used to differentiate ______ from _______

A

Specific esterase is use to differentiate granulocytic cells from monocytic cells

44
Q

Stains esterases present in the monocytic cells, macrophages, megakaryocytes, and platelets

A

a-Naphthyl Acetate Esterase

45
Q

a-naphthyl Butyrate Esterase is also known as

A

Non-specific esterase

46
Q

Monocytes stains _____ in non-specific esterase

A

Red-brown

47
Q

Marker for HAIRY CELL LEUKEMIA

A

Tartrate-resistant ACP (TRAP)

48
Q

Binds with acid mucopolysaccharides in blood cells; useful for the recognition of mast cells and tissue basophils

A

Toluidine blue

49
Q

Screening procedure for the detection of chronic granulomatous disease (CGD)

A

Nitroblue Tetrazolium Neutrophil Reduction Test (NBT test)

50
Q

Recommended specimen for NBT test

A

Heparinized whole blood

51
Q

Positive result in NBT indicates a normal result while the negative result indicates that the patient has CGD

True or false

A

True

52
Q

A marker for primitive lymphoid cells; used to differentiate ALL from AML

A

Termimal Deoxyribonucleotidyl Transferase (TdT)

53
Q

TdT is present in ___% of cases of ALL

A

TdT is present in 90% cases of ALL

54
Q

Small lymphoblast, homogenous; Most common ALL in children

A

FAB L1

55
Q

large lymphoblast, heterogenous; Most common ALL in Adult

A

FAB L2

56
Q

Most common Leukemia in Adult

A

AML

57
Q

leukemic phase of BURKITT’S LYMPHOMA

A

FAB L3

58
Q

What cells are affected in FAB L3?

A

B-cell

59
Q

Blast exhibits myeloid markers; DOES NOT STAIN

A

FAB M0 - w/o differentiation

60
Q

What test principle should be used to identify FAB M0 w/o differentiation?

A

FLOW CYTOMETRY

61
Q

FAB M0 is detected through CD markers ______

A

CD 13
CD 33
CD 34
CD 117

62
Q

> 90% of myeloblasts, may have Auer rods

A

FAB M1 (AML W/O MATURATION)

63
Q

FAB M1 is positive in stains:

A

SBB
MPO
Specific esterase

64
Q

<90% marrow myeloblasts

A

FAB M2 (AML WITH MATURATION)

65
Q

FAB M2 is positive in stains

A

SBB
MPO
Specific esterase

66
Q

> 30% marrow PROMYELOCYTES with BUNDLES OF AUER RODS

A

FAB M3

67
Q

FAB M3 is also known as:

A

Hypergranular PROMYELOCYTIC leukemia OR
Acute PROMYELOCYTIC leukemia

68
Q

these are promyelocytes with AUER RODS

A

Faggot cells

69
Q

Acute myeloproliferative disorder that is associated with DIC

A

FAB M3/ Acute promyelocytic leukemia

70
Q

FAB M3 is positive in stains ____, _____, ______, and negative in _______

A

positive (+) SBB, MPO, Sp. Esterase
negative (-) non. sp. esterase

71
Q

> 30% marrow MYELOBLASTS OF MONOCYTIC ORIGIN

A

FAB M4

72
Q

FAB M4 is also known as:

A
  • NAEGELI SYNDROME
  • Acute MYELOMONOCYTIC Leukemia
73
Q

FAB M4 is positive in stains:

A

positive (+)
SBB
MPO
Sp. Esterase
Non-Sp. Esterase

74
Q

FAB M5 is also known as:

A

SCHILLING LEUKEMIA
Acute MONOCYTIC Leukemia

75
Q

FAB M5 is positive in stains _____ and negative in ______

A

positive (+) Non-Sp. Esterase
negative (-) MPO, SBB, SPE

76
Q

FAB M6 is also known as:

A

Di Guglielmo’s Syndrome
Acute ERYTHROLEUKEMIA

77
Q

FAB M6 is positive in what stain?

A

PAS (+)
Periodic Acid Schiff stain (+)

78
Q

FAB M7 is also known as:

A

Acute MEGAKARYOCYTIC leukemia

79
Q

Which FAB classification uses anti-vWF Abs and does not use stain to be identified?

A

FAB M7

80
Q

The only chronic myeloproliferative disorder that is negative for JAK2 V617F gene

A

Chronic Myelogenous Leukemia or
Chronic Granulomatous Leukemia

81
Q

In CGL/CML, LAP score is ________

A

decreased

82
Q

Philadelphia chromosome is a result of ______ OR ______ gene mutation

A

Philadelphia chromosome is a result of T (9, 22) and BCR/ABL gene mutation

T (9,22)
BCR/ABL gene mutation

83
Q

Plaetelets can reach up to >1000x10^9/L; JAK2V617F gene positive

A

Essential Thrombocythemia

84
Q

JAK2V617F gene positive; pancytosis and low EPO

A

Polycythemia Vera

85
Q

Increased presence pf smudge cells in the PBS; JAK2V617F gene positive

A

Chronic Lymphocytic Leukemia

86
Q

TRAP positive (ACP 5)

A

Hairy Cell Leukemia

87
Q

What cell is malignant in HCL?

A

B-cell

88
Q

cancer of mature plasma cells; abnormally increased IgG

A

Multiple Myeloma (MM)

89
Q

Protein that is present in urine of a patient with MM

A

Bence Jones Protein

90
Q

BJP in urine precipitates at _____C and dissolves at _____C

A

precipitates at 40-60C
dissolves at 100C

91
Q

Lymphoid malignancy characterized with increased IgM; Lymphoplasmacytic infiltration of the BM

A

Waldenstrom’s Macroglobulinemia

92
Q

The pathognomonic cell of Hodgkin’s Lymphoma:

A

Reed-Sternberg cells

93
Q

The most common cutaneous lymphoma

A

Mycosis Fungoides

94
Q

Mycosis Fungoides is a malignany of

A

T cells

95
Q

Cells present in Mycosis Fungoides are called:

A

Sezary cells - cancerous T cells

96
Q

lymphoid malignancy caused by HTVL-1

A

Adult T-cell leukemia

97
Q

Adult T-cell leukemia is characterized by the presence of:

A

Flower cells

98
Q

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS:

Stem cell leukemia

A

lymphoma, undifferentiatedA

99
Q

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS:

Acute lymphoblastic leukemia

A

lymphoma, poorly differentiated, lymphocytic

100
Q

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS:

Chronic lymphocytic leukemia

A

lymphoma, well-differentiated, lymphocytic

101
Q

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS:

Monocytic leukemia

A

Reticulum cell sarcoma

102
Q

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS:

Acute myelogenous leukemia

A

Chloroma

103
Q

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS:

Plasma cell leukemia

A

Myeloma

HEMATOLOGY (Topnotcher QT) (12 decks)

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