Clinical Flashcards
(97 cards)
1
Q
This is the early bone marrow hematopoietic cell.
A
Blast cell
2
Q
What happend to bone marrow to cause acute leukemia?
A
Failure
3
Q
What are the 3 markeres for AML?
A
CD13, CD33, MPO
4
Q
Do mainly kids or adults get AML?
A
Adults
5
Q
What 2 conditions can make u eligable for stem cell transplant for the treatment of AML?
A
< 65 y/o
HLA matching a sibling donor
6
Q
What is the main detereminant for therapy for AML?
A
age
7
Q
What is the regiment for AML therapy?
A
4 blocks, each about 1 wk
8
Q
What is the treatment if a hemorrhagic syndrome develops in promyelocytic variant AML?
A
platelets with FFP
9
Q
What drug can cause the prolfieration of N0 that follows differentiation of promyelocytes from the bone marrow?
A
ATRA
10
Q
What is the treatment of ATRA syndrome?
A
Dexamethasone
11
Q
What do u treat the elderly with in AML over 70?
A
supportive care with or without gentle single-drug chemo
12
Q
What is the treatment options for relapsed AML?
A
Further chemo
stem cell transplant if tolerable and have a donor
arsenic trioxide for relapsed M3 variant
13
Q
This is teh small # of leukemic cells that remain in the pt during treament, or during remission.
A
Minimal residual disease (MRD)
14
Q
What can MRD do?
A
cause relpase in cancer and leukemia
15
Q
Are chronic or acute leukemias harder to treat?
A
Chronic
16
Q
What % of leukemias are CML?
A
15%
17
Q
What is the gene mutation in CML?
A
t(9;22)
18
Q
What age range is at risk for CML?
A
40-60
19
Q
Which organ is enlarged in CML?
A
Spleen
20
Q
What is the drug that is used to treat CML by blocking tyrosine kinase activity?
A
Imatinib (glivec)
21
Q
What is the optimal response to imatinib?
A
complete cytogenic response
22
Q
If the pt has a suboptimal response to imatinib in the Tx of CML, what other 2 drugs can u give?
A
Dasatinib
Nilotinib
23
Q
What do Dasatinib and Nilotinib do to help Tx CML?
A
overcome resistance to imatinib or early allogenic stem cell transplant
24
Q
What is the overall survival rate in CML pts in the Tx with imatinib?
A
89%
25
What does hydroxyurea do to help CML pts?
it can control and maintain the white cell count in chronic phase
26
How long do u have to take hydroxyurea in the Tx of CML?
Forever.
27
After the white cell count has been controlled by hydroxyurea, what has been used to keep the white cell count low?
a-interferon
28
Waht is the only established curative treatment for CML?
Allogenic stem cell transplant
29
What are the 2 reservations for the use of stem cell transplant for CML Tx?
if u fail with imatinib and are < 65 y/o
30
This is when there is 20% or more blasts in the marrow and may occur rapidly over days or weeks.
Acute transofrmation
31
What are the features of the accelerated phase?
anemia, thrombocytopenia and an increase in basophils, eosinophils or blast cells in the blood and marrow
32
What are the 3 main myeoloproliferative neoplasms (MPN)?
PV
Pimary myelofibrosis
ET
33
What mutation is common in all MPN's?
JAK2 tyrosine kinase
34
What is the normal role of JAK2 tyrosine kinase?
acts as a signal transduction molecule for the EPO and TPO receptors.
35
This is the disease state in which the proportion of blood volume that is occupied by red blood cells increases.
Polycythemia
36
This is the form of polycythemia where an increased hct due to a decreased plasma volume and normal RBC mass.
Relative polycythemia
37
This is the form of polycythemia when the hct levels are greater than 18 g/dL in males and 16.5 g/dL in females.
Absolute polycythemia
38
This is a myeloproliferative disorder of RBC's that results from activating mutations of JAK2 tyrosine kinase.
PV
39
What are the main clinical manifestations of PV?
postbathing pruritis, fatigue, erythromyelelgia (acral dyesthesias and ertythema), headache, dizziness, unexplained wt loss, joint symptoms, dyspnea, and epigastric distress.
40
What are the main treatment options for PV?
Phelbotomy, low dose ASA, hydroxyurea, INFa, and radiophosphorus.
41
This is a clonal hematologic disorder in which pts present with thrombocytosis and sometimes leukocytosis. It’s from the overproduction of platelets by megakryocytes in the bone marrow
ET
42
What are the clinical manifestations to ET?
bleeding and thrombosis (counterinuitive lol), erythromelalgia, possible splenomegaly
43
What is teh treatment for low risk ET?
ASA
44
What are the 2 ways u can treat high risk ET?
```
Platelet apheresis (in emergencies)
Cytoreductive therapy (hydroxyurea, anagrelide, INFa)
```
45
This is the condition where there is progressive reactive fibrosis (scarring) of the bone marrow occurs --> blood forms in sites other than the bone marrow (like liver or spleen) --> enlargement of liver/spleen
Primary myelofibrosis (PMF)
46
What is the main organ problem seen on exam in PMF?
Massive splenomegaly
47
What 2 things do u see on smear in PMF?
nucleated RBC's and teardrop cells
48
This is the presence of too many mast cells and CD34+ mast cell precursors in a person’s body.
Mastocytosis
49
What mutation causes mastocytosis?
KIT tyrosine kinase
50
What are the 2 clinical presentations of mastocytosis?
Skin lesions and allergic Sx
51
What is the DOC for mastocytosis?
imatinib
52
What is the cause of priamry and secondary myelodysplasia?
primary- unknown
| secondary- post cancer Tx
53
Usually there arent Sx in myelodysplasia, but what are the Sx if there are any?
Infections or easy bruising/bleeding
54
Who is at risk for ALL?
kids 3-7
55
What are the 3 manifestatiosn from bone marrow failure in ALL?
Anemia
Neutropenia
Thrombocytopenia
56
What is the morphology of the RBC's in ALL?
normochromic, normocytic anemia
57
How many chromosomes characterizes hyperdiploid?
> 50
58
Does hyperdiploid cells in ALL have a good or bad prognosis, when compared to hypodiploid?
Good
59
High dose systemic methotrexate given IV, intrathecal methotrexate or cytosine arabinoside or cranial irradiation are all used to treat what?
Cranial disease
60
What is the maitenance therapy for ALL?
Daily oral mercaptopurine + once weekly oral methotrexate for 2 years
61
In addition to oral therapy for ALL, what IV therapy is added monthly for ALL?
IV vincristine with a short course of oral corticosteroids
62
What is the best treatment for ALL relapse?
Allogenic stem cell transplant
63
What is it called when there is a condition that resembles CLL but does not meet criteria for CLL + does not require treatment?
MBL
64
For the following indicators, what the results of them for a BAD prognosis in CLL?
```
Stage
Sex
Lymphocute doublign time
Bone marrow biospy appearance
Chromsomes
VH immunoglobulin gene
ZAP expression
CD38 expression
CLLU.1 expression
LDH
```
```
Stage- Binet B, C
Sex- male
Lymphocute doublign time- rapid
Bone marrow biospy appearance- diffuse
Chromsomes- trisomy 12, del 17p, del 11q23
VH immunoglobulin gene- unmutated
ZAP expression- high
CD38 expression- +
CLLU.1 expression- high
LDH- raised
```
65
Since cure rates in CLL are rare, what is the approach to therapy?
Conservative, aiming for Sx control
66
When is chlorambucil given in CLL to induce remission?
given for several months
67
The use of Rituximab and other monoclonal AB's in CLL is used for what?
used in resistant and relapsed disease
68
What is teh indication for the use of corticosteroidds in CLL?
bone marrow failure
69
This is the conditon which is like CLL but there is a majority of prolymphocytes in the blood (big suckers) and they have a nucleolus.
B-PLL
70
What are the 2 drugs for Hairy cell leukemia?
2-chlorodeoxyadenosine
| Deoxycoformycin
71
What do the lymphocytes look like in LGL-L?
abundant cytoplasm and large azurophilic granules
72
Which 2 WBC's are transformed in LGL-L?
T or NK
73
What age do u get LGL-L?
~50
74
What is the clinical presentation of LGL-L?
cytopenia, esp neutropenia
75
What happens to the ESR and CRP in HL?
They're raised
76
What is characterized by stage I HL?
node involvement in one lymph node area
77
What is characterized by stage II HL?
disease involving two or more lymph nodal areas confined to one side of the diaphragm
78
What is characterized by stage III HL?
disease involving lymph nodes above and below the diaphragm
79
What is characterized by stage IV HL?
involvement outside the lymph node areas and refers to diffuse or disseminated disease in the bone marrow, liver and other extranodal sites.
80
What should men do before Tx to HL?
freeze their sperm
81
What is the treatment for stage 1 and 2A of HL?
radiotherapy alone
82
What is the treatment for stage 3 and 4 of HL?
Chemo
83
What is the combo chemo regimen for HL?
Adriamycin, bleomycin, vinblastine and dacarbazine (ABVD) is given in 6 cycles (or 4 following complete remission)
84
What is the cure rate for each stage of HL?
I- 90%
II- 90%
III- 80%
IV- 65%
85
Which form of NHL (low or high grade) is easier to cure and often curable?
High grade
| the worse the better?
86
This is the term for the presence of a monoclonal immunoglobulin band in the serum
Paraproteinemia
87
What type of MM is when there's plasma cells in the bone marrow (plasma cell count is >10%) but there is no evidence of tissue damage?
Asymptomatic (smouldering) MM
88
What does the CRAB acronym stand for in the Dx of MM?
hyperCalcemia
Renal impairment
Anemia
Bone disease
89
What is the Tx for MM in pts that are 65-70 and need intensive therapy?
severeal corses of chemo, allogenic stem cell trasnplant
90
What is the non-intensive therapy for 70+ pts with MM?
monthly oral alkylating gane MELPHALIN
91
What drug is the 1st line therapy and management of relapsed disease in MM?
Thalidomide
92
When is Bortezomib used in the Tx of MM?
Refractory dz
93
What are pts with MGUS at risk for later in life?
Myeloma or lymphoma
94
What is the stain used to identify amyloidosis?
Congo red stain
95
What do u see under polarized light after staining with Congo red for amyloidosis?
Red-green birefringence
| Apple-green
96
What is the msot common cause of polycythemia?
Hyperviscosity syndrome
97
What pts are at risk for hyperviscosity syndrome?
myeloma, Waldenstrom's macroglobulinemia, CML, or acute leukemias
