Hematological Malignancies Flashcards
1
Q
What is CA considered invasive?
A
Breaks the epithelium/BM into the circulation
2
Q
What are the six main properties of tumor cells?
A
- Self sufficiency of growth
- Insensitivity to anti-growth
- Tissue invasion and metastasis
- Limitless replicative potential
- Sustained angiogenesis
- Evade apoptosis
3
Q
What are cancers that begin in the skin or in tissues that line or cover the internal organs?
A
Carcinomas
4
Q
What are cancers that begin in bone, cartilage, fat, muscle, blood vessel, or other CT?
A
Sarcomas
5
Q
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
A
Leukemia
6
Q
What are cancers that being in the cells of the immune system?
A
Lymphoma and myelomas
7
Q
How do you distinguish between lymphomas and leukemias?
A
how/where the first present
8
Q
What is the difference between acute lymphoblastic leukemia vs acute lymphoblastic lymphoma?
A
Both the same disease, but leukemia starts in the blood
9
Q
What is the difference between acute and chronic leukemia?
A
Acute = generally lethal within weeks w/o treatment
Chronic= can survive years without treatment
10
Q
What are myeloid/myelogenous/myeloproliferative cancers?
A
Tumors involving the RBC, platelets, or their progenitors
11
Q
What are lymphoid/lymphoblastic/lymphocytic/lymphoproliferative cancers?
A
Tumors involving the B, T, or NK cells
12
Q
What are the five different hematological categories?
A
- Acut leukemias
- Chronic myeloproliferative
- Myelodysplastic syndrome
- Lymphomas & related lymphoid neoplasms
- Plasma cell neoplasms
13
Q
What are the two types of acute leukemias?
A
Acute myeloblastic (AML) Acute lymphoblastic (ALL)
14
Q
What are the symptoms of acute leukemias?
A
Pancytopenia d/t tumor cells displacing the normal blood elements in the bone marrow
15
Q
What is the cause of the pancytopenia seen in leukemias?
A
Bone marrow replacement by tumors
16
Q
What are the features of AML?
A
undifferentiated myeloid blast accumulating in the bone marrow
17
Q
What is the most common leukemia in the adult?
A
AML
18
Q
How do you subtype AMLs?
A
Clinical context
Cytogenetics
Molecular features
19
Q
What is the origin of ALL-T?
A
T lymphocytes
20
Q
What is the origin of ALL-B?
A
B lymphocytes
21
Q
What is the origin of acute, monocytic leukemia?
A
Monocytes
22
Q
What are the six types of AML classifications?
A
- AML with recurrent genetic abnormalities
- AML with multilineage abnormalities
- AML and MDS: therapy related
- AML NOS
- AML of ambiguous lineage
- MDS-RA with excess blasts
23
Q
What are the two types of acute lymphoblastic leukemia? Which is more common?
A
- B-ALL (85% of all cases)
2. T-ALL
24
Q
In what age group is ALL most common in?
A
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
