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

Pathoma Chapter 6: Part 2 Flashcards

1
Q

What are myeloproliferative disorders (MPDs)?

A

neoplastic proliferation of mature cells of myeloid lineage

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2
Q

When do MPDs usually present?

A

late adulthood (50-60 y/o)

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3
Q

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

A

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

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4
Q

Complications with MPDs include what?

A
  • increased risk for hyperuricemia and gout due to high turnover of cells
  • progression to marrow fibrosis or transformation to acute leukemia
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5
Q

What are some types of MPDs?

A
  • chronic myeloid leukemia (CML)
  • polycythemia vera (PV)
  • essential thrombocytopenia
  • myelofibrosis
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6
Q

What is CML caused by?

A

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

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7
Q

What is characteristically increased in CML?

A

basophils

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8
Q

Cause of CML?

A

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

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9
Q

Treatment for CML?

A

Imatinib, which blocks tyrosine kinase activity

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10
Q

Progression of CML?

A

-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

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11
Q

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

A

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

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12
Q

What is polycythemia vera (PV) caused by?

A

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

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13
Q

Clinical symptoms of PV?

A

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

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14
Q

Treatment for PV?

A

1) phlebotomy
2) hydroxyurea

without treatment, death usually occurs in 1 year

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15
Q

How is PV distinguished from reactive polycythemia?

A

1) In PV, EPO levels are decreased and SaO2 is normal

2) In reactive polycythemia, SaO2 is low and EPO is increased

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16
Q

What is essential thrombocythemia (ET) caused by?

A

neoplastic proliferation of mature myeloid cells, especially platelets

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17
Q

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

A

Jak2 kinase mutation

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18
Q

T or F. ET commonly progresses to acute leukemia

A

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

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19
Q

What is myelofibrosis caused by?

A

neoplastic proliferation of mature myeloid cells, especially megakaryocytes

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20
Q

What is the root cause of marrow fibrosis in myelofibrosis?

A

megakaryocytes produce excess platelet- derived growth factor (PDGF)

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21
Q

What is common on a smear in myelofibrosis?

A

tear drop RBCs

22
Q

Painful LAD is indicative of what?

A

acute infection

23
Q

Painless LAD is indicative of what?

A
  • chronic inflammation
  • metastatic carcinoma
  • lymphoma
24
Q

What are lymphomas caused by?

A

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

25
Q

Subtypes of lymphoma?

A

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

26
Q

NHL is further divided based on what?

A

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

27
Q

Subtypes of NHL?

A

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

28
Q

High yield subtypes of small B cell NHL?

A
  • follicular lymphoma
  • mantle cell lymphoma
  • marginal zone lymphoma
  • small lymphocytic lymphoma
29
Q

What is follicular lymphoma?

A

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

30
Q

Patient population in follicular lymphoma?

A

late adulthood (with painless LAD)

31
Q

Root cause of follicular lymphoma?

A

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
Q

Treatment for follicular lymphoma?

A

rituximab or low-dose chemo

33
Q

Follicular lymphoma can progress to what?

A

diffuse large B-cell lymphoma (lymph nodes enlarge)

34
Q

How is follicular lymphoma distinguished from reactive follicular hyperplasia?

A
  • disruption of normal lymph node architecture
  • lack of tangible body macrophages in germinal centers
  • Bcl2 expresison in follicles
  • monoclonality
35
Q

What is mantle cell lymphoma?

A

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

36
Q

Patient population in mantle cell lymphoma?

A

late adulthood (with painless LAD)

37
Q

Root cause of mantle cell lymphoma?

A

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
Q

What is marginal zone lymphoma?

A

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

39
Q

What is marginal zone lymphoma associated with?

A
  • Hashimoto thyroiditis
  • Sjogren syndrome
  • H pylori gastritis
40
Q

What is Burkitt lymphoma?

A

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

41
Q

What is Burkitt lymphoma associated with?

A

EBV

42
Q

How does burkitt lymphoma classically present?

A

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
Q

What drives Burkitt lymphoma?

A

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

44
Q

What is diffuse large B-cell lymphoma?

A

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

clinically aggressive

45
Q

How does diffuse large B-cell lymphoma arise?

A

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

46
Q

How does diffuse large B-cell lymphoma present?

A

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

47
Q

What is Hodgkin lymphoma (HL)?

A

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

48
Q

HL is classically positive for?

A

CD15, CD20

49
Q

RS cells secrete cytokines. What can this lead to?

A
  • occasionally results in ‘B’ symptoms (fever, chill, weight loss, and night sweats)
  • attract reactive lymphocytes, plasma cells, macrophages, and eosinophils
  • possibly fibrosis
50
Q

Subtypes of HL:

A
  • nodular sclerosis (most common)
  • lymphocyte-rich (best prognosis)
  • mixed cellularity(abundant eosinophils via IL-5)
  • lymphocyte-depleted (most aggressive type)
51
Q

Lymphocyte-depleted HL is common in what patient population?

A
  • elderly

- HIV+

Heme Week 2 (18 decks)

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