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Flashcards in Hematological Malignancies Deck (88):
1

What is CA considered invasive?

Breaks the epithelium/BM into the circulation

2

What are the six main properties of tumor cells?

1. Self sufficiency of growth
2. Insensitivity to anti-growth
3. Tissue invasion and metastasis
4. Limitless replicative potential
5. Sustained angiogenesis
6. Evade apoptosis

3

What are cancers that begin in the skin or in tissues that line or cover the internal organs?

Carcinomas

4

What are cancers that begin in bone, cartilage, fat, muscle, blood vessel, or other CT?

Sarcomas

5

What are cancers that start in blood-forming tissue such as bone marrow, and causes large number of abnormal blood cells to be produced and enter the blood

Leukemia

6

What are cancers that being in the cells of the immune system?

Lymphoma and myelomas

7

How do you distinguish between lymphomas and leukemias?

how/where the first present

8

What is the difference between acute lymphoblastic leukemia vs acute lymphoblastic lymphoma?

Both the same disease, but leukemia starts in the blood

9

What is the difference between acute and chronic leukemia?

Acute = generally lethal within weeks w/o treatment

Chronic= can survive years without treatment

10

What are myeloid/myelogenous/myeloproliferative cancers?

Tumors involving the RBC, platelets, or their progenitors

11

What are lymphoid/lymphoblastic/lymphocytic/lymphoproliferative cancers?

Tumors involving the B, T, or NK cells

12

What are the five different hematological categories?

1. Acut leukemias
2. Chronic myeloproliferative
3. Myelodysplastic syndrome
4. Lymphomas & related lymphoid neoplasms
5. Plasma cell neoplasms

13

What are the two types of acute leukemias?

Acute myeloblastic (AML)
Acute lymphoblastic (ALL)

14

What are the symptoms of acute leukemias?

Pancytopenia d/t tumor cells displacing the normal blood elements in the bone marrow

15

What is the cause of the pancytopenia seen in leukemias?

Bone marrow replacement by tumors

16

What are the features of AML?

undifferentiated myeloid blast accumulating in the bone marrow

17

What is the most common leukemia in the adult?

AML

18

How do you subtype AMLs?

Clinical context
Cytogenetics
Molecular features

19

What is the origin of ALL-T?

T lymphocytes

20

What is the origin of ALL-B?

B lymphocytes

21

What is the origin of acute, monocytic leukemia?

Monocytes

22

What are the six types of AML classifications?

1. AML with recurrent genetic abnormalities
2. AML with multilineage abnormalities
3. AML and MDS: therapy related
4. AML NOS
5. AML of ambiguous lineage
6. MDS-RA with excess blasts

23

What are the two types of acute lymphoblastic leukemia? Which is more common?

1. B-ALL (85% of all cases)
2. T-ALL

24

In what age group is ALL most common in?

children

25

Are ALL tumors aggressive or slow growing?

Aggressive

26

What are the four major chronic myeloproliferative disorders?

-polycythemia
vera
-primary myelofibrosis
-essential thrombocythemia
-chronic myelogenous leukemia

27

What are diseases in which the bone marrow makes too many red blood cells, platelets, or certain white blood cells?

myeloproliferative disorders

28

What is the cause of polycythemia vera? Prognosis?

JAK2 mutation, causing a high level of funtional RBCs

median survival less than1 year without treatment

29

What is the treatment for polycythemia vera?

Blood removal

Chemo

30

What is essential thrombocythemia vera? Cause? Prognosis?

JAK2 or MPL mutation, causing an overproduction of megakaryocytes

Many pts asymptomatic for 50+ years

31

What is primary myelofibrosis? Causes? Prognosis?

JAK2 or MPL mutation causing fibrosis and atypical megakaryocytes

Median survival time 1-8 years

32

What is the chromosomal translocation that occurs with CML?

chr 9 and 22 cause philidelphia chromosome, and BCR-ABL Y kinase

33

What is the pathophysiology of CML?

Overgrowth of granulocytic and megakaryocytic precursors in the bone marrow

34

What is the end result of untreated CML? How long does this take to occur in untreated pts?

acute leukemia within 5 years

35

What is the treatment for CML?

Imatinib or stem cell transplant

36

What is myelodysplastic syndrome?

a type of cancer in which the bone marrow does not make enough healthy blood cells, and there are abnormal cells in the blood or bone marrow.

37

What can myelodysplastic syndrome lead to?

Myeloid leukemia

38

What is the virus that is believed to help cause lymphoid malignancies?

EBV

39

What is the pathophysiology of myeloma?

Clones of plasma cells

40

What are the cell types, special characteristics, diagnostic criteria for acute lymphoblastic leukemia (ALL)?

Immature pre B cells or B cells

Aggressive replacement of bone and brain tissue in children

Characteristic cells on sample

41

What are the cell types, special characteristics, diagnostic criteria for chronic lymphoblastic leukemia (CLL)?

mature B cells
Nonaggressive in old people

Lymphocytes no blood film

42

What are the cell types, special characteristics, diagnostic criteria for lymphoma?

B cells

Associated with EBV infx. Tend to cause solid lesions

heavy chain rearrangement

43

What are the cell types, special characteristics, diagnostic criteria for multiple myeloma?

Plasma cells

Common for olds

Presence of plasma cells in marrow and osteolytic lesions on radiographs

44

What are the cell types, special characteristics, diagnostic criteria for T-cell malignancy?

T-cells

rare- may be caused by HTLV1 infx

can behave either as leukemia or lymphoma

45

What are the two types of lymphomas?

Hodgkin's
Non-Hodgkin's

46

What are the two types of lymphomas and lymphoid neoplasms?

Lymphomas
Chronic lymphocytic leukemia

47

What is the major factor in determining the difference between Hodgkin and non-hodgkin's lymphoma?

reed-sternberg cells (found in Hodgkin's)

48

What are Reed-Sternberg cells?

large cells with two or more nuclei, each of which contains a large eosinophilic nucleoli

Found in Hodgkin's lymphoma

49

large cells with two or more nuclei, each of which contains a large eosinophilic nucleoli = ?

Reed-Sternberg cells

50

What are the three types of Non-Hodgkin's lymphoma?

Indolent
Aggressive
Very aggressive

51

Where does Hodgkin's lymphoma usually arise from?

In one lymph node group, and spread in a predictable way

52

How do you treat Hodgkin's lymphoma?

radiation and chemo

53

What is the cell origin, implicated oncogene, and usual clinical behavior of Follicular lymphoma?

Germinal B cells

BCL-2

Indolent

54

What is the cell origin, implicated oncogene, and usual clinical behavior of extranodal marginal zone lymphoma?

Post-germinal center B cell
NFkappaB

Very indolent

55

What is the cell origin, implicated oncogene, and usual clinical behavior of Diffuse large B cell lymphoma?

germinal or post germinal center B cell

BCL-2 (or 2)

Aggressive

56

What is the cell origin, implicated oncogene, and usual clinical behavior of Burkitt's lymphoma?

Germinal B cell

c-MYC

Very aggressive

57

True or false: chronic lymphocytic leukemias are associated with one or two types of chromosomal translocations

False--not associated

58

What is the median age of diagnosis of chronic lymphocytic leukemia?

70

59

What are the histological findings of CLL?

Increased number of small, round lymphocytes

Smudge cells

60

true or false: most patients diagnosed with CLL are asymptomatic at diagnosis

True

61

What is the prognosis for CLL?

5-11 years

62

What are the six major classifications of plasma cell disorders?

1. multiple myeloma
2. Plasmacytoma
3. Primary amyloidosis
4. Monoclonal gammopathy of uncertain significant (MGUS)
5. POEMS syndrome
6. Waldenstrom's

63

What is multiple myeloma?

B cell proliferation, with tumor cells producing full or partial monoclonal igs, with lytic bone lesions

64

Lytic bone lesions are found in what type of CA?

Multiple myeloma

65

Rouleaux formation = ?

RBC stacking found in multiple myeloma

66

What is solitary plasmacytoma?

Solitary mass consisting of a clonal plasma cell tumor, but WITHOUT monoclonal Ig in the serum (or low levels)

67

Solitary plasmacytoma has a risk for progressing to what?

Multiple myeloma

68

What is primary amyloidosis?

clonal expansion of plasma cells in the bone marrow, that secrete monoclonal light chains

leads to deposits of amyloid in tissues

69

What is M protein?

Igs produced by plasma cells in multiple myeloma (can be IgM or others)

70

what is monoclonal gammopathy of uncertain significance (MGUS)? What is the risk of this?

Detection of monoclonal ig protein in the serum or urine without evidence for malignancy of the plasma cells or B lymphocytes

Risk for progression to multiple myeloma

71

What is POEMS syndrome?

Plasma cell issue, causing:
Polyneuropathy
Organomegaly
Endocrinopathy
Monoclonal protein
Skin changes

72

What is Waldenstrom's macroglobulinemia/lymphoplasmacytic lymphoma?

IgM secreting

Associated with hyperviscosity

73

How do you diagnose leukemias?

PBS and/or bone marrow biopsy

74

How do you diagnose lymphomas?

Lymph node biopsy

75

How do you diagnose plasma cell neoplasms?

Bone marrow biopsy

76

What are the four techniques employed when diagnosing leukemias/lymphomas from PBS or bone marrow biopsy?

Morphology
Immunophenotyping
histochemistry
Cytogenetics

77

Auer rod on microscopy = ?

pathognomonic for acute myelogenous leukemia

78

What is the immuno marker for acute promyelocytic leukemia?

CD 13+
CD33+
CD34-
HLA-DR-
CD9+

79

What are the B cell markers?

CD19, 20, ***

80

What are the T cell markers?

CD 3, 4 ***

81

What are cytogenetics?

karyotyping or FISH

82

What are the four phases of drug trials?

1= get safe dosing
2= does it work?
3 = larger group, same a 2
4 = ongoing

83

What is induction therapy?

Initial treatment given for a cancer, with the goal being to induce remission of the disease

84

What is consolidation therapy?

Treatment given following induction therapy, with the goal being to eliminate any undetectable cancer cells

85

What is maintenance therapy?

Therapy given after pt has achieved remission. goal being to maintain the remission, and reduce the risk of relapse

86

What is adjuvant therapy?

Cancer therapy given in addition to the primary therapy to assist with the treatment goal

87

What is palliative therapy?

Therapy that lessens the symptoms and improve the quality of life in patient without curing the disease

88

What is salvage therapy?

Treatment given after other therapies have failed