[5] White Blood Cell Disorders Flashcards

1
Q

Common causes of Neutropenia

A
Drug Toxicity (Chemotherapy)
Severe Infection
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2
Q

Common causes of Lymphopenia

A

Immunodeficiency

High cortisol state (Cortisol causes apoptosis of lymphocytes)

Autoimmune destruction

Whole body radiation

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3
Q

What is the marker used to detect decreased Fc receptors in immature neutrophils?

A

CD16

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4
Q

Common causes of Eosinophilia

A

Allergic Reactions
Parasitic Infections
Hodgkin Lymphoma (due to an increased IL-5)

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5
Q

Basophilia is classically associated with which disease?

A

CML

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6
Q

Common causes of Lymphocytic Leukocytosis

A

Viral Infections

Bordetella pertussis

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7
Q

Causative agent of Infectious Mononucleosis

A

EBV

CMV is a less common cause

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8
Q

What part of the lymph nodes do B cells reside?

A

Cortex

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9
Q

What part of the lymph nodes do T cells reside?

A

Paracortex

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10
Q

Screening test for EBV

A

Monospot Test

Usually turns positive within 1 week after infection

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11
Q

Define: Acute Leukemia

A

Neoplastic proliferation of blasts

>20% blasts in the bone marrow

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12
Q

What is the key marker to identify lymphoblasts?

A

TDT+ (DNA Polymerase) in the Nucleus

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13
Q

What is the key marker to identify myeloblasts?

A

Myeloperoxidase

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14
Q

What is an Auer Rod?

A

Crystallization of myeloperoxidase, indicating that the blast is a myeloblast

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15
Q

ALL is commonly associated with this genetic disease

A

Down Syndrome (After the age of 5)

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16
Q

What is the most common type of ALL?

A

B-ALL

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17
Q

What are the classic surface markers of B-ALL

A

CD10
CD19
CD20

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18
Q

This subtype of B-ALL has a good prognosis and commonly seen in kids

A

t(12;21)

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19
Q

This subtype of B-ALL has a poor prognosis and commonly seen in adults

A

t(9;22)

Also called the PH ALL (Philadelphia)

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20
Q

What are the classic surface markers of T-ALL

A

CD2-CD8

Blasts do NOT express CD10

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21
Q

How can you subclassify AML?

A
  1. Cytogenetic abnormalities
  2. Lineage of myeloblasts
  3. Surface markers
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22
Q

What is the pathophysiology behind Acute Promyelocytic Leukemia?

A

t(15;17)

Retinoic acid receptor disrupted causes promyelocytes to accumulate

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23
Q

Why is Acute Promyelocytic Leukemia considered an emergency?

A

Promyelocytes contain numerous Auer rods, which is a risk for DIC

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24
Q

Treatment for Acute Promyelocytic Leukemia

A

All Trans Retinoic Acid (ATRA) causes blasts to mature

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25
Q

Acute Monocytic Leukemia is a proliferation of what cells?

Where do these cells infiltrate?

A

Monoblasts

Patients present with gum infiltration

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26
Q

Acute Megakaryoblastic Leukemia is a proliferation of which cells?

This is associated with which disease?

A

Megakaryoblasts

Association with Down syndrome (before the age of 5)

*Recall that ALL is associated with Down Syndrome AFTER the age of 5

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27
Q

Define: Myelodysplastic Syndrome

A

Cytopenias with hypercellular bone marrow

Abnormal maturation with increased blasts (<20%)

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28
Q

Define: Chronic Leukemia

A

Neoplastic proliferation of mature circulating lymphocytes

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29
Q

CLL cells express?

A

CD5 and CD50

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30
Q

CLL is a neoplastic proliferation of which cells?

A

Naive B-Cells

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31
Q

Smudge cells are classically seen in what disease?

A

CLL

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32
Q

How does CLL cause hypogammaglobulinemia?

A

Naive B cells do not mature to become plasma cells, which produce immunoglobulins

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33
Q

Hairy Cell Leukemia is a neoplastic proliferation of?

A

Mature B Cells

34
Q

Hairy Cell Leukemia cells are positive for which enzyme?

A

Tartrate Resistant Acid Phosphatase (TRAP)

35
Q

Treatment: Hairy Cell Leukemia

A

2-CDA

Adenosine deaminase inhibitor; allowing adenosine to accumulate to toxic levels

36
Q

Adult T-Cell Leukemia Lymphoma is a neoplastic proliferation of which cells?

A

Mature CD4+ T Cells

37
Q

Myeloproliferative disorders are defined by?

A

Neoplastic proliferation of mature cells of myeloid lineage

38
Q

Chronic Myeloid Leukemia is a neoplastic proliferation of?

A

Granulocytes, characteristically basophils

39
Q

CML is caused by?

A

t(9;22) causes a BCR-ABL fusion with increased tyrosine kinase activity

40
Q

Treatment: CML

A

Imatinib

Blocks tyrosine kinase activity

41
Q

In patients with CML, what does splenomegaly indicate?

A

Suggests accelerated phase of the disease; transformation to acute leukemia follows shortly after

42
Q

Polycythemia Vera is a neoplastic proliferation of?

A

RBCs

43
Q

Polycythemia Vera is associated with what mutation?

A

JAK2 Kinase Mutation

44
Q

Polycythemia Vera causes an increased risk of venous thrombosis, leading to this disease entity of which it is also the most common cause of

A

Budd-Chiari Syndrome

45
Q

Treatment: Polycythemia Vera

Secondline Treatment

A

Phlebotomy

2nd Line: Hydroxyurea

46
Q

This tumor classically causes reactive polycythemia

A

Renal Cell Carcinoma

Due to production of ectopic EPO

47
Q

Essential Thrombocythemia is a neoplastic proliferation of?

A

Platelets

48
Q

Essential Thrombocythemia is associated with what mutation?

A

JAK2 Kinase

49
Q

Myelofibrosis is a neoplastic proliferation of?

A

Megakaryocytes

50
Q

Myelofibrosis is associated with which mutation?

A

JAK2 Kinase

51
Q

How does Myelofibrosis cause marrow fibrosis?

A

Overproduction of PDGF

52
Q

What peripheral blood smear finding can be seen in Myelofibrosis?

A

Teardrop Cell (RBC trying to squeeze past a fibrosed bone marrow)

53
Q

Follicular Lymphoma is composed of?

A

Neoplastic Small B cells (CD20+)

54
Q

Follicular Lymphoma is caused by the translocation of?

A

t(14,18) of IgH -> Bcl2

55
Q

What is Bcl2’s function in the body?

A

Inhibition of Apoptosis

56
Q

How can you differentiate Follicular Lymphoma from Follicular Hyperplasia?

A
  1. Disruption of normal LN Architecture
  2. Lack of tingible body macrophages in GC
  3. Expression of Bcl2
  4. Monoclonality
57
Q

Mantle Cell Lymphoma is caused by neoplastic proliferation of?

A

Neoplastic small B cells (CD20+) that expand the mantle zone

58
Q

What is the clinical presentation of Mantle Cell Lymphoma?

A

Presents in late adulthood with painless LAD

59
Q

What translocation causes Mantle Cell Lymphoma?

A

t(11;14)

Cyclin D1 on C11 translocates to IgH on C14

60
Q

Function: Cyclin D1

A

Allows progression of cell cycle from G1/S transition

61
Q

Marginal Zone Lymphoma is commonly associated with these diseases

A

Chronic Inflammatory States

Hashimoto’s Thyroiditis
Sjogren Syndrome
H. pylori gastritis

62
Q

Burkitt Lymphoma is associated with this disease

A

Epstein-Barr Virus

63
Q

Burkitt Lymphoma is caused by translocation of?

A

t(8;14)

c-myc -> IgH translocation

64
Q

Function: c-myc

A

Promotes cell growth

65
Q

This is the most common form on Non-Hodgkin Lymphoma

A

Diffuse Large B-Cell Lymphoma

66
Q

Key Hallmark of a Hodgkin Lymphoma

A

Reed-Sternberg Cell

67
Q

Key Markers for Reed-Sternberg Cells

A

CD15+ and CD30+

68
Q

What do Reed-Sternberg Cells secret?

A

Cytokines

69
Q

Most common subtype of Hodgkin Lymphoma

A

Nodular Sclerosis

70
Q

Classic presentation of Nodular Sclerosis HL

A

Enlarging cervical neck / Mediastinal LN in a young female

71
Q

Which subtype of Hodgkin Lymphoma has the best prognosis?

A

Lymphocyte-Rich

72
Q

Which subtype of Hodgkin Lymphoma has the worst prognosis?

A

Lymphocyte-Depleted

73
Q

Most common primary malignancy of bone

A

Multiple Myeloma

74
Q

What is the pathophysiology behind Multiple Myeloma?

A

B-Cells produce osteoclast activating factor causing punched out lesions and hypercalcemia

Neoplastic plasma cells also produce immunoglobulin

75
Q

What is the reason behind the increased risk of infection in patients with Multiple Myeloma?

A

Monoclonal antibody lacks antigenic diversity

Infection is the most common cause of death

76
Q

What is a Bence-Jones Proteinuria?

A

Free light chain excreted in urine

77
Q

What causes Waldenstrom Macroglobulinemia

A

B-cell lymphoma with monoclonal IgM production

78
Q

Treatment: Waldenstrom Macroglobulinemia

A

Plasmapheresis (IgM removal)

79
Q

What are Langerhans Cells

A

Specialized dendritic cells found predominantly in skin

80
Q

Function: Langerhans Cells

A

Present antigen to naive T-Cells