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Abnormal WBCs and Leukemia Flashcards

1
Q

Diapedesis

A

Passage of blood cells through intact capillary walls, usually accompanying inflammation

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2
Q

T Cell Markers

A

Tdt, CD4, CD8

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3
Q

B Cell Markers

A

Surface Ig, CD34, CD19

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4
Q

Hypersegmentation occurs in

A

megaloblastic anemia or in response to infection

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5
Q

Hyposegmentation occurs in

A

Pelger Huet Anomaly, Myeloproliferative Disorder (MPS) or Myelodysplastic Disorder (MDS)

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6
Q

Dohle Bodies

A

Light blue granular inclusions, which are aggregates of ER (RNA), associated with infection and May-Hegglin anomaly

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7
Q

Auer Rods

A

Red or orange needle-like inclusions, made of fused lysosomes. Associated with myelocytic leukemia

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8
Q

Russell Bodies

A

“Flame Cells” (stark cytoplasmic color gradient) and “Mott Cells” (extreme vacuolation)

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9
Q

Barr Bodies

A

“Drumstick” (tiny nuclear lobe) inclusions made of inactive X chromosomes. Seen in females and Klinefelters syndrome patients

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10
Q

Leukemoid Reaction

A

Leukemia-like response to chronic infection. Severe left-shift, usually >25 x10^9/L neutrophils. Distinguished with high LAP score, toxic granulation, and Dohle Bodies from Chronic Myelogenous Leukemia (CML).

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11
Q

Marginated Granulocyte Pool (MGP)

A

Neutrophils that are stuck to the inside of the vascular system and don’t come out w/ peripheral blood. Can cause pseudoneutropenia.

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12
Q

Chronic Granulomatous Disease

A

Rare, inherited. Abnormal oxidative metabolism in neutrophils. Results in frequent infections. Test with Nitroblue tetrazolium (NBT)

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13
Q

Chediak Higashi Anomaly

A

Rare, inherited. Usually fatal in infancy due to infection. Large, dark blue inclusions. Fusion of primary and secondary granules in neutrophils = abnormal lysosomes. Affects cell locomotion, de-granulation, and killing potential

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14
Q

May-Hegglin Anomaly

A

Rare, inherited. Large, round, pale blue inclusions. Associated with thrombocytopenia, giant platelets, bleeding.

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15
Q

Pyknotic Nucleus

A

Hyper-dense (almost black) nuclei. Indicates a cell part-way through apoptosis.

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16
Q

Pelger-Huet Anomaly

A

Benign inherited. “Pince nez” neutrophils, which function normally.

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17
Q

Alder-Reilly Anomaly

A

Large purple granules in WBC cytoplasm (looks like toxic gran but can be in lymphs). Caused by enzyme deficiency, but cells function normally.

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18
Q

Leukocyte Adhesion Deficiency

A

Rare, inherited. Absence of surface adhesion proteins on WBCs (integrins). High mortality rate from frequent infections.

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19
Q

Gaucher’s Disease

A

Lipid Storage Disorder. Deficiency of Glucocerebrosidase. Causes “tissue paper cell” with eccentric nucleus and “crinkly” looking cytoplasm

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20
Q

Nieman Pick Disease

A

Lipid Storage Disorder. Deficiency of sphingomyelinase. Causes “foamy” macrophages with tons of fat vacuoles. Often fatal by age 3.

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21
Q

Tay-Sachs Disease

A

Lipid Storage Disorder. Deficiency of B-hexoseamidase. Glycolipids and mucopolysaccharides in CNS. Often fatal by age 4.

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22
Q

Sea Blue Histiocyte Syndrome

A

Lipid Storage Disorder. Sea Blue staining of macrophages in spleen and marrow. Splenomegaly and decreased platelets. Usually benign.

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23
Q

Wiskott-Aldrich Syndrome

A

Normal B cell count, abnormal B cell function. Decreased antibody production.

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24
Q

DiGeorge Syndrome

A

Decreased T cells and Lymphoid tissue, normal B cells. Missing or dysfunctional thymus.

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25
Q

Dutcher Bodies

A

Vacuole-like inclusions in the nucleus of plasma cells, associated with multiple myeloma

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26
Q

Myeloproliferative Neoplasms (MPN) Symptoms and examples

A
  • Increased RBC, WBC, PLTs
  • Includes: Chronic Myelogenous Leukemia (CML), Chronic Neutrophilic Leukemia (CNL), Essential Thrombocythemia (ET), Polycythemia Vera (PV), Primary Myelofibrosis (PMF)
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27
Q

What mutation is most commonly associated with CML?

A

BCR-ABL1, aka the “Philadelphia Chromasome”, translocation between 9 and 22

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28
Q

CML Laboratory Presentation

A
  • VERY high WBC count (200-500k)
  • Left shift to blasts, no leukemic hiatus
  • Increased Eos/Basos
  • N/N Anemia
  • Increased PLT
  • Low LAP stain
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29
Q

CNL Laboratory Presentation

A
  • Neutrophilia
  • Slight left shift
  • No BCR/ABL1 (Phili chromosome)
  • RBCs and PLT Normal
  • High LAP stain
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30
Q

ET Laboratory Presentation

A
  • PLT > 1,000,000/cumm
  • Abnormal appearance and function of PLT
  • Increased WBCs
  • JAK2 Mutation
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31
Q

Polycythemia Vera (PV) etiology

A

RBC clonal stem cell defect allows proliferation without need for EPO

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32
Q

PV Laboratory Presentation

A
  • Increased RBCs (N/N, sludging)
  • HGB >18g/dL
  • Increased WBC and PLT
  • Increased LAP
  • Often a JAK2 Mutation
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33
Q

Relative Polycythemia

A

Increased HCT due to decreased plasma volume

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34
Q

Primary Myelofibrosis Etiology

A

Fibrotic tissue replaces cellular mass of the marrow

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35
Q

PMF Laboratory Presentation

A
  • N/N Anemia
  • nRBCs
  • Teardrop cells
  • Anisocytosis/poikilocytosis
  • Increased WBC w/ left shift and eos/basos
  • Decreased PLT
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36
Q

Myelofibrosis vs. Myelophithisic Anemia

A
  • High vs low WBCs
  • Fibrotic vs tumor cells in marrow
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37
Q

Chronic Eosinophilic Leukemia (CEL)

A
  • Increased WBC
  • N/N Anemia
  • Low PLT
  • Lots of Eos w/ left shift of Eos
38
Q

Basophilic Leukemia (BL)

A
  • Increased WBC
  • N/N Anemia
  • Decreased PLT
  • Lots of Basos w/ left shift of Basos
39
Q

Myelodysplastic Syndrome (MDS) Etiology

A
  • Abnormal maturation (dysplasia) in bone marrow
  • Activated oncogenes
  • Loss of tumor suppressor genes
  • Epigenetic and chromosome damage
40
Q

Mutations associated with MDS

A
  • Proto-oncogenes: JAK2, RAS, RUNX1
  • Tumor suppressor: TP53
41
Q

MDS Laboratory Presentation

A
  • Anemia (Macrocytes, dimorphic, basophilic stippling, H-J bodies)
  • Neutropenia w/ hyposegs and hypogran
  • Dysplasia in one or more cell lines
  • Increased Monos
  • Decreased T cells
42
Q

MDS vs Megaloblastic Anemia

A

B12/Folate normal vs Low

43
Q

Myeloperoxidase (MPO) Stain

A

Myeloid cells (Neutrophils and precursors, Eos, Monos)

44
Q

Sudan Black B (SBB) Stain

A

Strong on Granulocytes, Weak on Monos, Negative on Lymphs

45
Q

Chloroacetate Esterase (Specific Esterase) Stain

A

Granulocytes and mast cells

46
Q

Alpha Napthyl Esterase (Non-specific Esterase) Stain

A

Monocytes and myelomonocytes

47
Q

Periodic Acid Schiff (PAS) Stain

A

Glycogen, Strong positive in M6 ALL

48
Q

Tartrate Resistant Acid Phosphatase (TRAP) Stain

A

Hairy cells

49
Q

Terminal Deoxynucleotidyl Transferase (TDT) Stain

A

Immature lymphs, especially T cells

50
Q

Toluidine Blue Stain

A

Basophils and mast cells

51
Q

Leukocyte Alkaline Phosphatase (LAP) Stain

A
  • Strong in reactive myelocytes (Leukamoid reaction, PV, MM, PMF)
  • Weak in CML, PNH, sideroblastic anemia
52
Q

Difference between leukemia and lymphoma

A

Leukemia starts in the blood and bone marrow, lymphoma starts in the tissue (usually lymph). They often turn into each other as they progress.

53
Q

What marker is used to identify immature T and B cells?

54
Q

Common T cell markers

A

CD2, CD3, CD5, CD7

55
Q

Common B cell markers

A

CD19, CD22

56
Q

Categories of Acute Lymphoblastic Leukemia (ALL)

A

WHO:
- B cell ALL (most common)
- T cell ALL
FAB:
- L1-L3

57
Q

ALL Laboratory Presentation

A
  • N/N Anemia
  • Decreased PLT
  • Blasts (no Auer rods) with hiatus
  • Variable WBC count
58
Q

Lymphoblastic Lymphoma (LBL)

A

B or T cell neoplasm without bone marrow involvement

59
Q

FISH Testing

A

Fluorescent In-Situ Hybridization. Detects chromosomal abnormalities.

60
Q

CLL Laboratory Presentation

A
  • N/N Anemia
  • Decreased PLT
  • Decreased Neutrophils
  • Increased WBC
  • Increased Lymphs
  • Smudge Cells!!
61
Q

Hairy Cell Leukemia Laboratory Presentation

A
  • Pancytopenia
  • Hairy Cells (confirm with TRAP stain)
  • Fibrosis in bone marrow
62
Q

Multiple Myeloma Etiology

A

Malignant proliferation of plasma cells. Monoclonal gammopathy (mostly IgG).

63
Q

Multiple Myeloma Laboratory Presenation

A
  • +++ plasma cells in bone marrow
  • Abnormal plasma cells (flame cells, Dutcher bodies, mott cells with Russel bodies)
  • Monoclonal (M spike) on electrophoresis
  • Bence Jones protein in urine
  • Rouleaux!!
64
Q

Sezary Syndrome

A
  • T cell lymphoma
  • T helper cells with irregular nuclear outline and fine chromatin (clefts and folds)
65
Q

Hodgkin’s Lymphoma

A
  • Orderly progression in the lymph nodes
  • “Owl eye” cells in lymph nodes
  • Does not enter peripheral blood
  • Central lymph nodes
  • “Staging” used to treat
66
Q

Non-Hodgkin’s Lymphoma

A
  • Disorderly progression, jumping around
  • “normal” appearance of cells in lymph nodes
  • Can enter peripheral blood
  • Peripheral lymph nodes
  • “Grading” used to treat
67
Q

Lymph Node Structure

A
  • B cells in follicles of cortex
  • T cells in paracortex
  • Macrophages in sinuses
68
Q

Burkitt’s point mutation

A

8,14 translocation, associated with Non-Hodgkin’s Lymphoma

69
Q

Staging of Hodgkin’s Lymphoma

A

Stage I - 1 node
Stage II - >1 node, same side of diaphragm
Stage III - >1 node, both sides of diaphragm
Stage IV - disseminated disease

70
Q

Which Acute Leukemia is most common in children?

71
Q

AML Laboratory Presentation

A
  • N/N Anemia
  • Decreased PLT
  • Variable WBC
  • Blasts with Auer rods
  • Leukemic hiatus
72
Q

FAB vs WHO classification of acute leukemia

A

FAB: >30% blasts in bone marrow
WHO: >20% blasts in bone marrow

73
Q

FAB categories of AML

74
Q

M0

A

“Minimal differentiation”
- 90% blasts
Positive for:
- MPO/SBB (<3%)
- Specific esterase
- PAS
Negative for:
- Non-specific esterase

74
Q

M1

A

“Without maturation”
- 90% blasts
Positive for:
- MPO/SBB (>3%)
- Specific esterase
- PAS
Negative for:
- Non-specific esterase

75
Q

M2

A

“With maturation”
- 30-89% blasts
- 10% promyelocytes and myelocytes
- can have Auer rods
Positive for:
- MPO/SBB
- Specific esterase
- PAS
Negative for:
- Non-specific esterase

76
Q

M3

A

“With PML-RARA”
- >30% blasts
- Increased promyelocytes
- Auer rods
- Associated with DIC
- 15,17 translocation
Positive for:
- MPO/SBB (+++)
- Specific Esterase
- PAS
Negative for:
- Non-specific esterase

77
Q

M4

A

“Myelomonocytic leukemia”
- >30% blasts
- Increased monocytes
Positive for:
- MPO/SBB
- Specific Esterase
- PAS
- Non-specific Esterase

78
Q

M5

A

“Monoblastic leukemia”
- >30% blasts
- >80% monocytic lineage
Positive for:
- MPO/SBB (<20%)
- Specific Esterase
- PAS
- Non-specific Esterase (>80%)

79
Q

M6

A

“Pure erythroid leukemia”
- Digugliemo’s Syndrome
- >30% blasts
- >50% erythroid lineage
Positive for:
- PAS
Negative for:
- MPO/SBB
- Specific Esterase
- Non-specific Esterase

80
Q

M7

A

“Megakaryoblastic leukemia”
- >30% megakaryoblasts
- Dry tap (tons of fibrin in the marrow)
- Platelet blebbing
Positive for:
- PAS
- Acid phosphatase
Negative for:
- MPO/SBB
- Specific esterase
- Non-specific esterase

81
Q

M4e

A

“Eosinophilic variant”
- Same appearance as M4, plus increased Eos in bone marrow
- Inversion of chromosome 16
- Eos here are Specific esterase positive (unlike normal Eos)

82
Q

What are CD markers?

A

“Clusters of Differentiation”, a system of categorizing antigens which can be bound by antibodies produced by various companies to ensure that product names didn’t obscure what the antibodies were specific to

83
Q

Markers associated with myelocytic and monocytic cells

A

CD13 and CD33

84
Q

L1

A
  • Small homogenous blasts with fine chromatin and no nucleoli
  • Scant cytoplasm
  • Most common in children
85
Q

L2

A
  • Large blasts with fine chromatin and irregular nuclei (clefts) and nucleoli
  • Abundant cytoplasm
  • Adults
86
Q

L3

A
  • Large cells with fine chromatin and nucleoli
  • Very blue and vacuolated cytoplasm
  • Burkitt’s lymphoma
87
Q

Common Acute Lymphocytic Leukemia Antigen (CALLA)

88
Q

T vs B Cell Cytochemical Stain

A

T cells - Acid Phosphatase Pos
B cells - Surface Immunoglobulin (sIg) Pos

89
Q

9:22 Translocation

A

“Philadelphia Chromosome”, important CML

90
Q

15:17 Translocation

A

Diagnostic for M3

91
Q

8:21 Translocation

A

Better prognosis for AML

MLS (18 decks)

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