Unit 4 Flashcards
(153 cards)
1
Q
What is a neoplasm
A
New growth that is usually cancer or malignant
2
Q
What are the 3 main groups of FAB classified Neoplasms
A
- Myeloproliferative disorders (MPD)
- Myelodysplastic syndromes (MDS)
- Acute Leukemia
3
Q
What blast percentage makes the FAB classification acute?
A
30%
4
Q
What are the 2 major components of bone marrow
A
Hematopoietic
vascular
5
Q
Normal expected cellularity range equation
A
100-patient’s age +- 10%
6
Q
In bone marrow examination what is the min cell differential
A
200
erythroid precursors and megakaryocytes are not included
7
Q
What is the normal myeloid to erythroid ratio (M:E)
A
2-4:1
8
Q
What are the types of Myeloproliferative disorders
A
Chronic Myelogenous leukemia (CML)
Polycythemia vera
9
Q
General characteristics of MPD
A
Middle age (50-70)
gradual onset
10
Q
What are MPD caused by
A
genetic defect in the HSC
11
Q
Panhypercellularity is seen in?
A
MPD
12
Q
What is CML
A
An unregulated proliferation of myeloid cells in the BM
13
Q
What is CML caused by
A
Philadelphia Chromosome
14
Q
What does myeloid refer to
A
granulocyte line of cells
15
Q
What are the phases of CML
A
Chronic Phase
Accelerated phase
Blast crisis
16
Q
What are the general characteristics of CML in the chronic phase
A
Middle age (50-70)
slow onset
symptoms gradually worsen
may suddenly become more aggressive
17
Q
Symptoms of CML in the chronic phase
A
weakness
weight loss
hepatosplenomegaly
anemia due to myelophthisis
infection
18
Q
CML in chronic phase BM lab findings
A
High WBC Count *low blast count
High PLT count
NC/NC anemia
19
Q
What will distinguish AML from CML
A
High PLT count is only seen in CML
20
Q
What is the M:E ratio seen in CML in the chronic phase
A
10-50:1
21
Q
What is the blast % in CML in the chronic phase
A
<20%
22
Q
What is happening in the BM in CML in the chronic phase
A
BM hyperplasia
Hypercellularity
23
Q
What are the lab findings in CML Accelerated phase
A
WBC elevates further
PLT count begins to drop
24
Q
Blast crisis in CML is analogous to what
A
Acute leukemia
25
What are the lab findings in CML Blast Crisis
LOW PLT count
Blast >20%
*in large clusters on smear
More severe left shift
26
Why does CML not become AML
CML is because of the Philadelphia Chromosome
27
What does the Philadelphia Chromosome lead to?
fusion of the BCR and ABL genes
28
What is the Philadelphia Chromosome translocation
t(9:22)
29
What is the result of the Philadelphia Chromosome in CML
Production of unregulated tyrosine kinase
30
What does unregulated Tyrosine Kinase do
Uncontrolled, fast cell division
Inhibits apoptosis
Impairs normal DNA repair
31
What are the treatment methods for CML
Leikapheresis
Tyrosine kinase inhibitors
Stem cell transplants
32
What is Polycythemia Vera
Unregulated proliferation of erythroid cells
33
What is frequently accompanied by pancytosis
PV
34
What is the EPO concentration in PV
Very low
35
What are the symptoms from the high blood viscosity in PV
Cardiovascular disease
Headaches
Thrombotic or hemorrhagic episodes
Hypertension
36
How many of PV patients develop acute leukemia
5-10%
37
What are the 3 types of PV
1. Primary
2. Secondary
3. Relative
38
What causes Primary PV
Issues from HSC
39
What is Secondary PV associated with
Elevated EPO levels
40
What causes Secondary PV
Environment
-Altitude, COPD etc
41
What causes Relative PV
Decrease in plasma volume
-severe dehydration
-burns
42
What is the EPO concentration in Relative PV
Not affected
43
What are the types of Myelodysplastic Syndrome
Refractory Anemia
Chronic Myelomonocytic Leukemia
Therapy related MDS
44
What causes MDS
Genetic defect in the HSC
45
What has a higher predisposition to acute leukemia than MPD
MDS
46
What are the clinical findings in MDS
At least one type of peripheral cytopenia
Progressive decline in blood cell counts
47
What is considered Preleukemia
MDS
-20-40% transform
48
What is found in peripheral blood in MDS
Megaloblastic Anemia
Decreased retics
Basophilic stippling
Howell Jolly bodies
nRBC
Aniso
Poiko
49
Lab findings for MDS
Left shift
Nuclear abnormalities
Monocytosis
Variable PLT counts
50
Characteristics of Refractory Anemia
MEGALOBLASTIC anemia
Reticulocytopenia
Low blast count
51
How many Chronic MyeloMonocytic Leukemias turn to AML
30%
52
What is Therapy Related MDS
Secondary to chemotherapy or radiotherapy
53
What is the disease progression in Therapy Related MDS
Pancytopenia
More severe thrombocytopenia
Increasing presence of blasts
Development of AML
54
What is Acute Leukemia
A malignant proliferation of leukocytes originating in the bone marrow
55
What are the classic triad symptoms for acute leukemia
Anemia
Infection
Hemorrhage
56
What are the main lab findings for Acute Leukemia
Highly variable WBC count
Marked thrombocytopenia
Severe anemia
>20% blasts
57
How are cell lineages Identifications classified by
Cytochemical stains
Cytogenetics
Flow Cytometry
58
What does MPO stain
Peroxidase enzyme in primary granules in the myeloid line
59
What is MPO stain used for
Differentiate AML from ALL
60
What does Sudan Black B stain
the phospholipid membrane that encases the primary and secondary granules
61
What is SBB used for
To differentiate AML from ALL
62
What does Specific Esterase stain
specific esterase enzyme in primary granules of myeloid cells
63
What is Specific Esterase used to stain
Myeloblasts
64
What does Non-Specific Esterase stain
Non-specific esterase enzyme in monocyte precursors
65
What is Combined Esterase used for
-Differentiate myeloblasts and monoblasts
- AML M4 vs AML M5
66
What is Tartrate Resistant Acid Phosphatase (TRAP) used for?
To diagnose Hairy Cell Leukemia
67
What is specific to cells of hairy cell leukemia
Isoenzyme 5
68
What enzyme is present in all leukocytes
Acid Phosphatase
69
What is TdT?
Terminal Deoxynucleotidyl Transferase
*a DNA polymerase
70
What is TdT used for?
Diagnose of ALL
71
What is AML M0
Minimally Differentiated
72
Microscopic findings for AML M0
NO auer rods
Blasts are negative for MPO/SBB
73
How are auer rods formed
Fusion of primary granules
74
When are Auer Rods seen
in Neoplastic myeloblasts and promyelocytes
75
If Auer Rods are seen what diagnosis is excluded
ALL
76
Auer Rod
77
What is AML M1
AML without maturation
78
AML M1 has what microscopic findings
<10% of granulocytes mature beyond the myeloblast stage
Blasts are poorly differentiated
79
What is AML M2
AML with Maturation
80
What is the most common AML
AML M2
about 30%
81
Common findings in AML M2
Myeloblasts are predominant
all stages of neutrophils
Auer Rods
82
What is AML M3
Acute Promyelocytic Leukemia (APL)
83
What is Faggot Cells
blasts that contain bundles of auer rods
84
what is the translocation in AML M3
t(15;17)
85
what does the AML M3 translocation do?
alters confirmation of retinoic acid receptors (RAR)
86
What is the normal RAR function
RAR is a nuclear receptor for retinoic acid (Vit A)
When RA binds to the RAR DNA transcription occurs and cells are allowed to mature
87
What stage do most cells not mature past in AML M3
Promyelocyte stage
88
What is the initial presentation of APL most often
Abnormal bleeding and DIC
89
What is the main complication from APL
Promyelocytes contain a large amount of tissue factor which starts the coagulation cascade
90
AML M3
91
AML M3
92
What is AML M4
Acute myelomonocytic leukemia
93
What is the 2nd most common AML
AML M4
25%
94
Findings in AML M4
Myeloid cells
Blast nucleus may be irregular
about 20% monocytic cells
auer rods may be present
95
What are the symptoms outside of the classic triad for AML M4
Gingival hypertrophy
Cutaneous lesions
Meningeal infiltration
96
AML M4
97
What are the two types of AML M5
M5a: acute monoblastic leukemia
M5b: acute monocytic leukemia
98
Symptoms in addition to classic triad for AML M5
More extensive extramedullary involvement than AMML
Liver, spleen, skin, gums, CNS, eyes
More aggressive: >40% die
99
What is AML M5a
Acute monoblastic leukemia (AMoL)
>80% monoblasts
100
What is AML M5b
acute moncytic leukemia
<80% monoblasts
More promonocytes and monocytes
101
AML M5a
102
AML: M5b
103
What are the 2 subtypes of AML M6
Pure Erythroid
Erythroid/myeloid
104
What are characteristics of AML M6
Anisopoikilocytes
High # of nRBC
nRBC may be megaloblastic, bizarre, and multinucleated
105
What is AML M6
Acute Erythroleukemia
106
What is AML M7
Acute megakaryoblastic leukemia
107
What happens in AML M7
proliferation of micromegakaryocytes and myeloblasts
108
What AML has an increased disposition for individuals with Down Syndrome (Trisomy 21)
AML M7
Acute Megakaryoblastic leukemia
109
What makes lymphoma different than leukemia
Lymphoma is hematologic cancer in lymphoid tissues while leukemia is in the BM
110
What are the Acute Lymphoid Neoplams
B cell Malignancy
T & NK cell Malignancy
111
What lymphoid neoplasms are more common
B cell malignancy
112
What is seen in B cell Malignancy
May involve plasma cells
Secrete excessive Ab
Hyper viscosity syndrome
Rouleaux
May produce autoAb
113
Which Acute Lymphoid Neoplasm is more aggresive
T & NK cell malignancy
114
Where is T & NK cell malignancy involved
Extranodal and extramedullary sites
*skin
*CNS
*Mediastinum
*Kidneys
115
What is ALL
Acute Lymphoblastic Leukemia
*Malignant lymphocytes
116
What is the most common malignancy of childhood
ALL
80% of childhood leukemia
117
Common complaints in ALL
Bone pain
Headache fatigue
118
Lab findings in ALL
Thrombocytopenia
NC/NC anemia
excessive lymphoblasts
119
What ALL subtype is the most common childhood leukemia
ALL L1
120
What is ALL L1
Precursor B-cell Leukemia
121
What is the most aggressive ALL
L2
122
What ALL is more common in adults
ALL L2
123
What mass can grow bc of ALL L2
Mediastinal mass from leukemic infiltration of the thymus
124
What is ALL L2
Precursor T-cell leukemia
125
What is ALL L3
Burkitt Leukemia
126
What cell lineage is ALL L3
B cell lineage
127
What are the chronic lymphoproliferative disorders
-B cell chronic lymphocytic leukemia
-Prolymphocytic leukemia
-Hairy cell leukemia
-Sezary's syndrome
128
What are the malignant lymphomas
Non-Hodgkin
Hodgkin
129
What is the plasma cell disorder
Multiple myeloma
130
What is Chronic Lymphocytic Leukemia
A B cell neoplasm
-accumulation of mature, nonfunctional memory B cells
131
What is the most common leukemia in adults
CLL
Chronic lymphocytic leukemia
132
What is Richter's Transformation
When CLL progresses to aggressive prolymphocytic leukemia
133
What is the % of CLL undergoing Richter's Transformation
10%
134
What are the symptoms of CLL
Swollen lymph nodes
infection
anemia
hepatosplenomegaly
135
What is seen in CLL
smudge cells
136
What is Prolymphocytic leukemia
Very aggressive B cell subtype
137
What are lab findings in PLL
Extremely elevated WBC count
>55% prolymphocytes
Severe anemia and thrombocytopenia
138
What is Hairy Cell Leukemia
A rare B-cell malignancy
139
What is seen in Hairy Cell Leukemia
Very significant splenomegaly
Extensive BM infiltration
140
What is Sezary's Syndrome
A late stage variant of cutaneous T cell Lymphoma (CTCL)
141
Sezary Cell
**look like a brain
142
What is the subtype of Sezary's Syndrome
Mycosis fungoides
143
Characteristics of Non-Hodgkin Lymphoma
Originates in lymph tissue
Spreads to extranodal sites
Normal cell morphology
Older onset
144
What are the B cell Non-Hodgkins lymphoma subtypes
Waldenstrom macroglobulinemia
Burkitt lymphoma
145
What is Waldenstrom macroglobulinemia
A plasma cell lymphoma which produces excessive IgM Ab
146
What syndrome is seen in Waldenstrom Macroglobulinemia
Hyper viscosity syndrome
*Protein buildup and poor circulation
*Visual impairments
*Headache
*Vertigo
*Deafness
*Rouleaux
147
What are the 3 subtypes of Burkitt Lymphoma
Endemic
Sporadic
Adult cases
148
What Burkitt Lymphoma is associated with EBV
Endemic
149
Characteristics of Endemic Burkitt
African pediatric
infiltrates facial bones
150
Characteristics of Sporadic Burkitt
North american Pediatrics
infiltrates abdominal organs
151
When is Adult Cases Burkitt seen
HIV patients
152
Characteristics of Hodgkin Lymphoma
Younger adults
localized malignancy - stays in lymph tissue
153
What is produces in Multiple myeloma
Monoclonal protein production
*partial production of Ab
