[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
Acute Monocytic Leukemia is a proliferation of what cells? Where do these cells infiltrate?
Monoblasts Patients present with gum infiltration
26
Acute Megakaryoblastic Leukemia is a proliferation of which cells? This is associated with which disease?
Megakaryoblasts Association with Down syndrome (before the age of 5) *Recall that ALL is associated with Down Syndrome AFTER the age of 5
27
Define: Myelodysplastic Syndrome
Cytopenias with hypercellular bone marrow Abnormal maturation with increased blasts (<20%)
28
Define: Chronic Leukemia
Neoplastic proliferation of mature circulating lymphocytes
29
CLL cells express?
CD5 and CD50
30
CLL is a neoplastic proliferation of which cells?
Naive B-Cells
31
Smudge cells are classically seen in what disease?
CLL
32
How does CLL cause hypogammaglobulinemia?
Naive B cells do not mature to become plasma cells, which produce immunoglobulins
33
Hairy Cell Leukemia is a neoplastic proliferation of?
Mature B Cells
34
Hairy Cell Leukemia cells are positive for which enzyme?
Tartrate Resistant Acid Phosphatase (TRAP)
35
Treatment: Hairy Cell Leukemia
2-CDA | Adenosine deaminase inhibitor; allowing adenosine to accumulate to toxic levels
36
Adult T-Cell Leukemia Lymphoma is a neoplastic proliferation of which cells?
Mature CD4+ T Cells
37
Myeloproliferative disorders are defined by?
Neoplastic proliferation of mature cells of myeloid lineage
38
Chronic Myeloid Leukemia is a neoplastic proliferation of?
Granulocytes, characteristically basophils
39
CML is caused by?
t(9;22) causes a BCR-ABL fusion with increased tyrosine kinase activity
40
Treatment: CML
Imatinib | Blocks tyrosine kinase activity
41
In patients with CML, what does splenomegaly indicate?
Suggests accelerated phase of the disease; transformation to acute leukemia follows shortly after
42
Polycythemia Vera is a neoplastic proliferation of?
RBCs
43
Polycythemia Vera is associated with what mutation?
JAK2 Kinase Mutation
44
Polycythemia Vera causes an increased risk of venous thrombosis, leading to this disease entity of which it is also the most common cause of
Budd-Chiari Syndrome
45
Treatment: Polycythemia Vera Secondline Treatment
Phlebotomy 2nd Line: Hydroxyurea
46
This tumor classically causes reactive polycythemia
Renal Cell Carcinoma | Due to production of ectopic EPO
47
Essential Thrombocythemia is a neoplastic proliferation of?
Platelets
48
Essential Thrombocythemia is associated with what mutation?
JAK2 Kinase
49
Myelofibrosis is a neoplastic proliferation of?
Megakaryocytes
50
Myelofibrosis is associated with which mutation?
JAK2 Kinase
51
How does Myelofibrosis cause marrow fibrosis?
Overproduction of PDGF
52
What peripheral blood smear finding can be seen in Myelofibrosis?
Teardrop Cell (RBC trying to squeeze past a fibrosed bone marrow)
53
Follicular Lymphoma is composed of?
Neoplastic Small B cells (CD20+)
54
Follicular Lymphoma is caused by the translocation of?
t(14,18) of IgH -> Bcl2
55
What is Bcl2's function in the body?
Inhibition of Apoptosis
56
How can you differentiate Follicular Lymphoma from Follicular Hyperplasia?
1. Disruption of normal LN Architecture 2. Lack of tingible body macrophages in GC 3. Expression of Bcl2 4. Monoclonality
57
Mantle Cell Lymphoma is caused by neoplastic proliferation of?
Neoplastic small B cells (CD20+) that expand the mantle zone
58
What is the clinical presentation of Mantle Cell Lymphoma?
Presents in late adulthood with painless LAD
59
What translocation causes Mantle Cell Lymphoma?
t(11;14) Cyclin D1 on C11 translocates to IgH on C14
60
Function: Cyclin D1
Allows progression of cell cycle from G1/S transition
61
Marginal Zone Lymphoma is commonly associated with these diseases
Chronic Inflammatory States Hashimoto's Thyroiditis Sjogren Syndrome H. pylori gastritis
62
Burkitt Lymphoma is associated with this disease
Epstein-Barr Virus
63
Burkitt Lymphoma is caused by translocation of?
t(8;14) c-myc -> IgH translocation
64
Function: c-myc
Promotes cell growth
65
This is the most common form on Non-Hodgkin Lymphoma
Diffuse Large B-Cell Lymphoma
66
Key Hallmark of a Hodgkin Lymphoma
Reed-Sternberg Cell
67
Key Markers for Reed-Sternberg Cells
CD15+ and CD30+
68
What do Reed-Sternberg Cells secret?
Cytokines
69
Most common subtype of Hodgkin Lymphoma
Nodular Sclerosis
70
Classic presentation of Nodular Sclerosis HL
Enlarging cervical neck / Mediastinal LN in a young female
71
Which subtype of Hodgkin Lymphoma has the best prognosis?
Lymphocyte-Rich
72
Which subtype of Hodgkin Lymphoma has the worst prognosis?
Lymphocyte-Depleted
73
Most common primary malignancy of bone
Multiple Myeloma
74
What is the pathophysiology behind Multiple Myeloma?
B-Cells produce osteoclast activating factor causing punched out lesions and hypercalcemia Neoplastic plasma cells also produce immunoglobulin
75
What is the reason behind the increased risk of infection in patients with Multiple Myeloma?
Monoclonal antibody lacks antigenic diversity Infection is the most common cause of death
76
What is a Bence-Jones Proteinuria?
Free light chain excreted in urine
77
What causes Waldenstrom Macroglobulinemia
B-cell lymphoma with monoclonal IgM production
78
Treatment: Waldenstrom Macroglobulinemia
Plasmapheresis (IgM removal)
79
What are Langerhans Cells
Specialized dendritic cells found predominantly in skin
80
Function: Langerhans Cells
Present antigen to naive T-Cells