Pathoma Chapter 6: Part 1 Flashcards Preview

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Flashcards in Pathoma Chapter 6: Part 1 Deck (47):
1

Myeloid stem cells give rise to what?

- Erythroblasts (RBCs)

- Myeloblasts (neutrophils, basophils, eosinophils)

- Monoblasts (monocytes)

- Megakaryoblasts (Megakaryocytes)

2

What is a normal white cell count?

4.4-11.3 K/ul

3

What are some causes of neutropenia?

- drug toxicity (chemo with alkylating agents)
- severe infection (gram- species)

4

How do chemo alkylating agents cause neutropenia?

they damage stem cells resulting in decreased production

5

How do severe infections cause neutropenia?

increased movement of neutrophils into tissue results in decreased circulating neutrophils

6

What are some causes of lymphopenia?

- immunodeficiency (DiGeorge or HIV)
- High cortisol state (Cushing's of exogenous corticosteroids)
- Autoimmune destruction (SLE)
- Whole body radiation

7

How does a high cortisol state cause lymphopenia?

it induces apoptosis of lymphocytes

8

What are some causes of eosinophilia?

- type I hypersensitivity
- parasitic infections
- Hodgkin lymphoma

9

What are some causes of basophilia?

- acute myeloid leukemia

10

What are some causes of lymphocytosis?

- viral infections
- Bordetella pertussis infection (via lymphocytosis promoting factor secreted by the bacteria which prevents them from leaving blood to enter lymph)

11

What is acute leukemia?

neoplastic proliferation of blasts; defined as an accumulation of 20+% of blasts in the bone marrow or peripheral blood

12

Why is it called "acute" leukemia?

increased blasts crowd-out normal hematopoiesis, resulting in an acute presentation

13

Clinical presentation of acute leukemia?

- anemia (fatigue)
- thrombocytopenia (bleeding)
- possible neutropenia (infection)

14

T or F. WBC is usually elevated in acute leukemia

T. due to blasts entering the blood stream

15

What is acute lymphoblastic leukemia defined as?

accumulation of 20+ % of lymphocytes in the bone marrow

16

How are lymphoblasts identified?

via positive nuclear staining for TdT, a DNA polymerase

TdT is absent in myeloid blasts and mature lymphocytes

17

ALL is common in what patient population?

usually kids (associated with down syndrome)
presents after 5 y/o typically

18

Subclasses of ALL?

B-ALL (most common) and T-ALL based on surface markers

19

How is B-ALL identified?

usually characterized by lymphoblasts (TdT) that express CD10, CD19, and CD20.

20

Clinical course for B-ALL?

requires prophylaxis to scrotum and CSF- great response

21

Prognosis of B-ALL?

based on cytogenetic abnormalities
i. t(12,21)-kids- has a good prognosis
ii. t(9,22)-adults- has a poor prognosis (philadelphia)

22

What is T-ALL characterized by?

lymphoblasts (TdT+) that express markers ranging from CD2 to CD8 (e.g. CD3/4/7).

don't express CD10

23

What patient population is common for T-ALL?

usually presents in teenagers as a mediastinal (thymic) mass (called acute lymphoblastic lymphoma because the malignant cells form a mass)

24

What is acute myeloid leukemia defined as?

20+% of immature myeloid precursors (myeloblasts) in the bone marrow

25

How are myeloblasts identified?

positive cytoplasmic staining for MPO (crystals of MPO commonly seen as Auer rods)

26

Patient population for AMLs?

older adults (avg 50-60 y/o)

27

High-yield subtypes of AML (based on cytogenetics, lineage of immature myeloid cells, and surface markers):

- Acute promyelocytic leukemia (APL)
- Acute monocytic leukemia
- Acute megakaryoblastic leukemia

28

What causes APL?

t(15,17) PML-RARa. The fusion protein causes enhancement of RARa function, which is to disrupt myeloid differentiation, but proliferation is unaffected so promyeloblasts accumulate

29

What is a common risk associated with APL?

DIC

30

What is the treatment for APL?

ATRA (vitamin A derivative) which binds the receptor and causes the blasts to mature (and eventually die)

31

What causes acute monocytic leukemia?

-proliferation of mono blasts that usually lack MPO
- these blasts characteristically infiltrate gums

32

What causes acute megakaryocytic leukemia?

-proliferation of megakaryoblasts that usually lack MPO

33

What is acute megakaryocytic leukemia associated with? When does it present?

Down syndrome (usually arise BEFORE age 5)

34

What else can AML arise from?

-pre-existing dysplasia (myelodysplastic syndromes)

35

How does myelodysplastic syndromes usually present?

- cytopenias
- hyper cellular bone marrow
- increased blasts (but less than 20%)

36

Outcome of myelodysplastic syndrome patients?

most die from infection or bleeding, though some progress to acute leukemia

37

What causes chronic leukemia?

neoplastic proliferation of mature circulating lymphocytes (high WBC count)

38

Patient population for chronic leukemia?

insidious onset in older adults

39

What causes chronic lymphocytic leukemia (CLL)?

neoplastic proliferation of naive B cells that co-express CD5 and CD20 (most common leukemia overall)

40

What are some complications with CLL?

- hypogammaglobinemia
- autoimmune hemolytic anemia
- Transformation to diffuse large B-cell lymphoma (Richter transformation)

41

Hairy cell leukemia (HCL) patients are positive for what?

TRAP (tartrate-resistant acid phosphatase)

42

Clinical features of HCL?

-splenomegaly due to accumulation of hairy cells in red pulp
- dry tap on bone marrow aspiration (due to marrow fibrosis)
- no lymphadenopathy

43

How is HCL treated?

excellent response to 2-CDA (Cladribine), an adenosine deaminase inhibitor

44

What is adult T-cell leukemia/lymphoma (ATLL) caused by?

neoplastic proliferation of mature CD4+ T cells

associated with HTLV-1

45

Clinical presentation of ATLL?

- rash (skin infiltration)
- generalized lymphadenopathy with hepatosplenomegaly
- lytic (punched out) bone lesions with hypercalcemia

46

Normal counterpart in mycosis fungicides (chronic leukemia type)?

mature CD4 T cells

47

When MF spreads to include peripheral blood it is called _____.

Sezary syndrome