Pathoma Chapter 6: Part 2 Flashcards Preview

Heme Week 2 > Pathoma Chapter 6: Part 2 > Flashcards

Flashcards in Pathoma Chapter 6: Part 2 Deck (51):
1

What are myeloproliferative disorders (MPDs)?

neoplastic proliferation of mature cells of myeloid lineage

2

When do MPDs usually present?

late adulthood (50-60 y/o)

3

T or F. in MPDs cells of all myeloid lineages are increased.

T. Subtypes are classified based on the major cell type produced

4

Complications with MPDs include what?

- increased risk for hyperuricemia and gout due to high turnover of cells
- progression to marrow fibrosis or transformation to acute leukemia

5

What are some types of MPDs?

- chronic myeloid leukemia (CML)
- polycythemia vera (PV)
- essential thrombocytopenia
- myelofibrosis

6

What is CML caused by?

neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors

7

What is characteristically increased in CML?

basophils

8

Cause of CML?

t(9,22) Philadelphia chromosome which generates a BCR-ABL fusion protein with increased tyrosine kinase activity

9

Treatment for CML?

Imatinib, which blocks tyrosine kinase activity

10

Progression of CML?

-splenomegaly common and enlarging spleen suggest progression to accelerated phase of the disease and can transform to acute leukemia (ALL 1/3 of times and AML 2/3 of times- because mutation is in a pluripotent stem cell) shortly after

11

How is CML distinguished from a leukomoid reaction (reactive neutrophilic leukocytosis)?

1) Negative leukocyte alkaline phosphatase (LAP stain) in CML
2) Increased basophils in CML
3) t(9,22) presence

12

What is polycythemia vera (PV) caused by?

proliferation of mature myeloid cells- especially RBCS (granulocytes and platelets also increased)

13

Clinical symptoms of PV?

due to hyper viscosity of blood:
1) blurry vision and headache
2) flushed face due to congestion
3) itching after bathing (histamine mediated)
4) risk of venous thrombosis

14

Treatment for PV?

1) phlebotomy
2) hydroxyurea

without treatment, death usually occurs in 1 year

15

How is PV distinguished from reactive polycythemia?

1) In PV, EPO levels are decreased and SaO2 is normal
2) In reactive polycythemia, SaO2 is low and EPO is increased

16

What is essential thrombocythemia (ET) caused by?

neoplastic proliferation of mature myeloid cells, especially platelets

17

PV and ET (and myelofibrosis sometimes) are both associated with what?

Jak2 kinase mutation

18

T or F. ET commonly progresses to acute leukemia

F, hardly ever progress and no significant risk for hyperuricemia or gout

19

What is myelofibrosis caused by?

neoplastic proliferation of mature myeloid cells, especially megakaryocytes

20

What is the root cause of marrow fibrosis in myelofibrosis?

megakaryocytes produce excess platelet- derived growth factor (PDGF)

21

What is common on a smear in myelofibrosis?

tear drop RBCs

22

Painful LAD is indicative of what?

acute infection

23

Painless LAD is indicative of what?

- chronic inflammation
- metastatic carcinoma
- lymphoma

24

What are lymphomas caused by?

noeplastic proliferation of lymphoid cells that forms a mass; may arise in lymph node or extranodal tissue

25

Subtypes of lymphoma?

-Hodgkins (40%) and non-Hodgkins (60%)

26

NHL is further divided based on what?

cell type (B vs T cell), pattern of cell growth expression of surface markers, and cytogenetic translocations

27

Subtypes of NHL?

1) small B cell (many types)
2) intermediate sized B cell (Burkitt lymphoma)
3) large B cell (diffuse large B-cell lymphoma)

28

High yield subtypes of small B cell NHL?

- follicular lymphoma
- mantle cell lymphoma
- marginal zone lymphoma
- small lymphocytic lymphoma

29

What is follicular lymphoma?

neoplastic proliferation of small B cell (CD20+) that form follicle-like nodules

30

Patient population in follicular lymphoma?

late adulthood (with painless LAD)

31

Root cause of follicular lymphoma?

t(14,18) translocation in which BCL2 on chrome 18 translocates to the Ig heavy chain locus on chrome 14 resulting in over-expression of BCL2

32

Treatment for follicular lymphoma?

rituximab or low-dose chemo

33

Follicular lymphoma can progress to what?

diffuse large B-cell lymphoma (lymph nodes enlarge)

34

How is follicular lymphoma distinguished from reactive follicular hyperplasia?

- disruption of normal lymph node architecture
- lack of tangible body macrophages in germinal centers
- Bcl2 expresison in follicles
- monoclonality

35

What is mantle cell lymphoma?

neoplasitc proliferation of small B cell (CD20+) that expands the mantle zone

36

Patient population in mantle cell lymphoma?

late adulthood (with painless LAD)

37

Root cause of mantle cell lymphoma?

t(11,14) - Cyclin D1 gene on chrom 11 translocates to Ig heavy chain locus on chrome 14 causing over-expression of cyclin D1

38

What is marginal zone lymphoma?

neoplasitc proliferation of small B cell (CD20+) that expands the marginal zone

39

What is marginal zone lymphoma associated with?

- Hashimoto thyroiditis
- Sjogren syndrome
- H pylori gastritis

40

What is Burkitt lymphoma?

neoplastic proliferation of intermediate-sized B cells (CD20+)

41

What is Burkitt lymphoma associated with?

EBV

42

How does burkitt lymphoma classically present?

as an extranodal mass in a child or young adult
i. african form usually involves the jaw
ii. sporadic form usually involves the abdomen

43

What drives Burkitt lymphoma?

t(8,14) translocation of c-myc (8) to the Ig heavy chain locus (14) promoting cell growth

44

What is diffuse large B-cell lymphoma?

neoplastic proliferation of large B cells (CD20+); most common form of NHL

clinically aggressive

45

How does diffuse large B-cell lymphoma arise?

sporadically or from transformation of a low-grade lymphoma (e.g. follicular lymphoma)

46

How does diffuse large B-cell lymphoma present?

in late adulthood as an enlarging lymph node or an extranodal mass

47

What is Hodgkin lymphoma (HL)?

neoplastic proliferation of Reed-Sternberg (RS) cells, which are large B cells with multi lobed nuclei and prominent nucleoli (owl-eyed)

48

HL is classically positive for?

CD15, CD20

49

RS cells secrete cytokines. What can this lead to?

- occasionally results in 'B' symptoms (fever, chill, weight loss, and night sweats)
- attract reactive lymphocytes, plasma cells, macrophages, and eosinophils
- possibly fibrosis

50

Subtypes of HL:

- nodular sclerosis (most common)
- lymphocyte-rich (best prognosis)
-mixed cellularity(abundant eosinophils via IL-5)
- lymphocyte-depleted (most aggressive type)

51

Lymphocyte-depleted HL is common in what patient population?

- elderly
- HIV+