(F) L2.1 : LAP and acute myeloid leukemia Flashcards
1
Q
a disease of leukocytes in the blood and bone marrow; uncontrolled proliferation of a clone of malignant WBCs
A
Leukemia
2
Q
solid tumor neoplasms of lymphoid tissues
A
lymphoma
3
Q
2 main types of lymphoma
A
- Hodgkin
- Non-hodgkin
4
Q
form of cancer of the plasma cells
A
myeloma
5
Q
non malignant tumors are caused by
A
congenital genetic abnormalities
6
Q
General characteristics of hematologic neoplasm
(acquired / congenital) genetic disease
A
acquired
patient acquires the disease sometime in life due to consistent genetic
Non-malignant yung congenital
7
Q
General characteristics of hematologic neoplasm
type of infection as well as the treatment
A
Systemic infection, therefore treatment MUST also be systemic
ex; chemotherapy, radiation, supportive therapy, targeted therapies, hem
8
Q
General characteristics of hematologic neoplasm
of unkown cause
A
idiopathic
except environmental factors like radiation and chemical exposure
9
Q
Agents / Factors affecting occurence of leukemias and lymphomas
Matching type
a. Genetic and immunological factors
b. Genetic abnormalities and association
c. Viral agents
____1. Down syndrome, Fanconi anemia
____2. Translocation B and A interchange
____3. Age
____4. Mutations in a single gene
____5. Epstein B
A
ABAAC
10
Q
Agents / Factors affecting occurence of leukemias and lymphomas
types of exposure that can become factors in the occurence of leukemias and lymphomas (3)
A
environmental, chemical, and drug exposure
11
Q
- known as reactive neutrophilia
- inflammatory reaction which frequently resembles leukemia on bone marrow or blood smears
A
leukemoid reaction
12
Q
all but one is a characteristic of leukemoid reaction
* WBC count is elevated (>50,000 / mK)
* Presence of severe infection or inflammation
* Involves lymphocytes with some immature forms
* Increased leukocyte alkaline phosphatase (LAP) index
A
Involves neutrophils with some immature forms
13
Q
leukemoid reaction vs chronic myeloid leukemia
- normal count and morphology for eosinophils and basophils
- toxic granulation and Dohle bodies are often found in neutrophil morphology
- Normal platelet count and morphology
- Normal hemoglobin
- Elevated LAP score
- Normal genetics
A
Leukemoid reaction
14
Q
Leukemoid reaction vs Chronic Myeloid Leukemia
- Often elevated eosinophils and basophils with possible mixed granulation
- pseudo-Pelger-Huet forms in Neutrophil morphology
- Platelet count is elevated in early detection, decreased in late
- anemic hemoglobin
- decreased LAP score
- Positive for t(9;22); BCR-ABL [genetics]
A
chronic myeloid leukemia
15
Q
Leukemoid reaction vs Chronic Myeloid Leukemia
which one has a genetic abberation (presence of Philadelphia chromosome)
A
Chronic myeloid leukemia
16
Q
LAP matching type:
a. naphthol AS-MX
b. blue pigment (insoluble)
c. violet pigment (insoluble)
____1. Naphtol AS-MX phosphate - alkaline phosphatase
____2. Naphthol AS-MX + fast violet B salt
____3. Naphthol AS-MX + fast blue RR salt
A
ACB
17
Q
LAP
How many neutrophils and bands must be examined before giving a rating of - to 4+
A
100 neutrophils and bands
18
Q
LAP matching type
refreence range:
a. 12-180
b. 32-182
c. 15-170
____1. Fast Blue RR
____2. Rodak’s
____3. Fast violet B
A
BCA
19
Q
LAP matching type
a. 0-13
b. more than 100
____1. Leukemoid reaction
____2. Chronic myelogenous Leukemia
____3. Leukemia
____4. Polycytemia vera
A
BAAB
20
Q
LAP cytochemical stain scoring
a. 0
b. 1+
c. 2+
d. 3+
e. 4+
- none (%), no granules, no staining
- 80-100%, medium to large granules, strong staining
- 50-80%, small granules, moderate to strong staining
- 50% small granules, faint to moderate staining
- 100%, medium to large granules, brilliant staining
A
ADCBE
21
Q
LAP
staining used in LAP
A
cytochemical stains
22
Q
LAP
How to compute for the lap score?
A
(cells counted) (rating)
repeat that for all ratings and all of their products
23
Q
CML has a (increased/decreased) LAP score
explain why :>
A
increased
(despite having a lot of cells, they’re dysfuctional, = no acid phosphatase activity)
24
Q
2 categories leukemia is differentiated
A
a. clinical course of the disease
b. cell type
25
# types of leukemia
what are the two clinical courses of leukemia?
acute and chronic
26
# types of leukemia matching type
a. chronic
b. acute
* sudden onset, rapid progression, and fatal outcome of weeks to months if left untreated
* insidious onset with gradual and slow progression, reslting to longer survival even if left untreated
BA
27
# types of leukemia
what are the four major categories of chronic leukemia
a. Acute myeloid leukemia (AML)
b. Acute lymphoblastic leukemia (ALL)
c. Chronic myeloid leukemia / chronic myelogenous leukemia
d. Chronic lymphocytic leukemia (CLL)
28
what are the types of leukemia categorized by cell type
myeloid and lymphoid
29
# leukemia and lymphoma classification
a. French American British (FAB) classification
b. WHO classification
* more complicated
* based on molecular, morphologic, and clinical features
* based on morphology and cytochemistry
* Mostly based on how the leukemia cells will look under the microscope after routine staining
* Some of the elements from FAB were retained but gives emphasis on the molecular and cytogenetic changes involved in the type of leukemia
BBAAB
30
# acute myeloid leukemia
* most common in (adults / children)
* rare in adolescents, common in (blank)
* bone and joint pain are present in (25%/85%) of cases
* (hepatomegaly / splenomegaly)
* (common / rare) CSF involvement
* adult
* adolescents, children under 1 yr old
* 25%
* splenomegaly
* rare
31
# acute myeloid leukemia matching type
a. hyperuricemia
b. hyperphosphatemia
c. hypokalemia
d. hypocalcemia
* contributes to bone and joint pain (two answers)
* found as an early complication of patients with hematologic conditions or malignancy
* caused by rapid cell lysis
* CD
* A
* B
32
# what does this lab finding indicate?
WBC: 5 and 30 x 109/L
| May range: 1 - 200 x 10^9
leukocytosis
33
# Acute myeloid leukemia
a syndrome secondary to aggressive cancer treatments such as chemotherapy
causes:
* hyperuricemia / uricosria
* hyperphosphatemia
* hyperkalemia
* hypocalcemia
tumor lysis syndrome
34
# FAB classification of Acute Myeloid leukemia
1. AML, minimally differentiated
2. Acute myelomonocytic leukemia
3. AML with maturation
4. AML without maturation
5. Acute promyelocytic leukemia
M0, M4, M2, M1, M3
35
# FAB classification of acute myeloid leukemia
1. Acute megakaryocytic anemia
2. Acute erythroleukemia
3. Acute myelomonocytic leukemia with eosinophilia
4. Acute monocytic leukemia, poorly differentiated
5. Acute monocytic leukemia, well differentiated
M7, M6, M4eo, M5a, M5b
36
# FAB classification of acute myeloid leukemia
what is the prognosis of all types of AML?
INTERMEDIATE
| pls remeber this, I will not be including this in the other cards thanks
37
# FAB classification of acute myeloid leukemia
* myelocytic origin,
* 5% of AML cases (infant / older adults)
* No evidence of myeloid differentiation
* Auer Rods (N)
* Fewer than 10% of leukocyte show maturation to the promyelocytic stage
M0
38
# FAB classification of acute myeloid leukemia
* myelocytic origin
* 90% BLASTS
* blasts and promyeolocytes predominate without further maturatioin of myeloid cells CD13, CD33, CD117, and CD34
* most common among less than 18 months and middle aged adults (M46)
M1
( AML with minimal differentiation)
39
# FAB classification of acute myeloid leukemia
* > 20% blasts, at least 10% maturing cells of neutrophil lineage
* <20% precursors with monocytic lineage
* Auer Rods (P)
* 40% in 60 years old or older
M2
(AML without maturation)
40
# FAB classification of acute myeloid leukemia
* promyelocytic in origin
* 5-10% of AML cases
* all ages, mostly in young adults (M38)
* DIC; butterfly / coin-in-coin nuclie (bowtie)
* Treated with ATRA and arsenic trioxide
* Auer rods (P)
* Most aggressive acute anemia
M3 (Acute promyelocytic anemia)
41
# FAB classification of acute myeloid leukemia
* myelocytic monocytic origin
* 20% of promonocytes and monoblasts
* Naegeli type
* significant increased WBC
* common in adults
M4 (Acute myelomonocytic anemia)
42
# FAB classification of acute myeloid leukemia
* myelocytic monocytic origin
* marrow-predominance of abnormal eosinophilia
M4eo
(Acute meulomonocyte with Eosinophilia)
43
# FAB classification of acute myeloid leukemia
* monocytic origin
* 5% of AML cases
* most common in younger individuals
* Cutaneous and gingival infiltrations
* 80% monocytic and promonocytes
M5aa
(acute monocytic leukemia, poorly differentiated / Acure monoblastic)
44
# FAB classification of acute myeloid leukemia
* monocytic origin
* common in young adults M16
* middleage (peak 46)
M5b
(acute monocytic leukemia, well differentiated / acute monocytic)
45
# FAB classification of acute myeloid leukemia
* of erythrocytic myelocytic origin
* 80% erythroid, 30% are proerythroblasts
* Di Guglielmo syndrome
* the only pure erythroid leukemia present, very rapid course
* has plenty of NRBC, around 50% NRBC and 80% eryhtroid cells
M6 (acute erythroleukemia)
46
# FAB classification of acute myeloid leukemia
* megakaryocytic origin
* 20% blasts, 50% megakaryocytic
* characterized by cytopenias
M7 (Acute megakaryocytic leukemia)
47
# classification of acute myeloid leukemia based on staining results
MPO: -
SBB: -
NASDA: -
ANBE: -
ANAE: -
M0
48
# classification of acute myeloid leukemia based on staining results
MPO: +
SBB: +
NASDA: +
ANBE: -
ANAE: -
M1, M2, and M3
49
# classification of acute myeloid leukemia based on staining results
MPO: +
SBB: +
NASDA: +
ANBE: + (diffuse)
ANAE: + (diffuse)
M4
50
# classification of acute myeloid leukemia based on staining results
MPO: +
SBB: +/-
NASDA: -
ANBE: + (diffuse)
ANAE: + (diffuse)
M5
51
# classification of acute myeloid leukemia based on staining results
MPO: -
SBB: -
NASDA: -/+
ANBE: -
ANAE: -
M6
52
# classification of acute myeloid leukemia based on staining results
MPO: -
SBB: -
NASDA: -
ANBE: -
ANAE: + (localized)
M7
53
# classification of acute myeloid leukemia based on staining results
which cell line will be positive for MPO, SBB, and NASDA?
myeloid cell line
| MPO = myeloperoxidase
NASDA = naphthyl ASD chloroacetate esterase
54
# CD markers of subclassess matching type
1. CD13, CD33, CD34, CD117
2. CD13, CD33, CD15, CD117, CD 34, HLA Dr
3. CD13, CD 33 monoblast, CD14, CD4, CD11b, CD11c, CD64, CD36
4. CD14, CD4, CD11b, CD11c, C36, CD64, CD68
5. GLPHA, CD71
6. vwf Platelet membrane antigen, CD41 (GLLPB), CD42b (GPLB), CD61 (GPLLLA)
1. M0. M1 M3
2. M2
3. M3
4. M5
5. M6
6. M7
55
what is the most common genetic abbration in acute myeloid leukemia
translocation
56
# WHO classification of AML
WITH GENETIC ABERRATIONS
* favorable prognosis
* M2 FAB subtype
* full range of myelocytic maturation
* Auer rods easily found
* abnormal cytoplasmic granues
* pseudo Pelger-Huet cells seen
* most common translocationi in AML
* shows maturation in the neutrophil lineage
* has large blasts, heavily granulated
* immunophenotype of CD13+, CD33+
AML with t(8:21)(q22;q22)
CBFolETO fusion gene
57
# WHO classification of AML
WITH GENETIC ABERRATIONS
* favorable prognosis
* M4eo FAB subtype
* meyolocytic and monocytic differentiation
* abnormal eosinophilic precursors with abnormal basophilic granules
* shows monocytic and granulocytic diffrentiation
* immunophenotype of granulocytic and monocytic markers, and CD2+
AML with inv(16)(p13;q22)
CBFbeta / MYH1 1 fusion gene
58
# WHO classification of AML
WITH GENETIC ABERRATIONS
* intermediate prognosis
* M3, M3v FAB subtype
* Numerous Auer rods, ofthen in bundles within individual progranulocytes
* usually very prominent primary granules (M3 subtype)
* inconspicuous primary granules in microgranular variant;
* high incidence of DHC
* 5-10% AML
* abnormal promyelocytes predominate
* associated ith disseminated intravascular coagulationi
* strongly positive in MPO and SBB
AML with t(15;17)(q22;11-12)
RArolPML fusion gene
(Acute promyelocytic leukemia)
59
# WHO classificationi of AML
WITH GENETIC ABERRATIONS
* poor prognosis
* M4 and M5 FAB subtype
* usually some degree of monocytic differentiation
* associated with monocytic features
* rare in adults (more in infants)
* associated with MLL rearrangement
AML with t(11q23; v): diverse MLL fusion genes
60
# WHO classification of AML
* favorable prognosis
* variable FAB subtype
* detected by immunohistochemical staining for NPM
AML with normal cytogenetics and mutated NPM
61
# WHO classification of AML
AML with MDS-like features
* poor pronosis
* varable FAB subtype
* diagnosis is based on clinical history
| MDS= myelodysplastic syndrome
with prior MDS
62
# WHO classification of AML
AML with MDS like features
* poor prognosis
* variable FAB subtype
* maturing cells with dysplastic features typical of MDS
AML with multilineage dysplasia
63
# WHO classification of AML
AML with MDS like features
* poor prognosis
* variable FAB subtype
* associated with 5q-, 7q-, 20q-aberrations
AML with MDS-like cytogenetic aberrations
64
# WHO classification of AML
* very poor prognosis
* variable FAB subtype
* if following alkylator therapy or radiation therapy, 2-8 year latency period
* MDS like cytogenetic aberrations
* if following topoisomerase II inhibitor therapy, 1-3 year latency
* translocations involving MLL (11q23)
AML, THERAPY RELATED
65
Transcriptor factor - a proteni that binds to specific regions of DNA to control the activity of that specific gene
RA Ra (Retionoic acid receptor alpha)
66
the only leukemia associated with DIC
| DIC = disseminated intravascular coagulation
acute promyelocytic leukemia
67
# WHO classification of leukemia
* defined as acute leukemia with more than 20% blasts, dysplasia in 2 or more myeoloid cell lines generally including megakaryocytes
* may occu de-novo or follownig MDS
Acute myeloid leukemia with multilineage dysplasia
68
# FAB classification of leukemia
of myelocytic origin
a. M4
b. M2
B. M2 (AML with maturation)
of myelocytic origin: MO, M1, M2
| M4 = acute myelomonocytic leukemia
69
# FAB classification of leukemia
of intermediate prognosis
a. M7
b. M4eo
both (ALL TYPES ARE OF INTERMEDIATE PROGNOSIS)
70
# FAB classification of leukemia
of erythrocytic myelocytic origin
a. M6
b. M5aa
a. M6 (acute erythroleukemia)
only one of erythrocytic origin
71
# FAB classification of leukemia
of monocytic origin
a. M5aa
b. M5b
both
72
# FAB classification of leukemia
of megakaryocytic origin
a. M0
b. M4a
neither
only M7 is of megakaryocytic origin
73
# WHO classification of AML
Of poor prognosis
a. AML with t(15;17)(q22; 11-12)
b. AML with t(11q23;v)
b
74
# WHO classification of AML
of very poor prognosis
a. AML with t(8;21)(q22;q22)
b. AML with inv(16)(p13;q22)
neither
AML therapy related
a. favorable
b. favorable
75
* Trisonomy 21 or Down syndrome
* 10% of newborn with down syndrome w/ abnormal myelopeoiesis
* AML with megakaryocytic maturation (FAB AML7)
* 50-fold increase in the first 5 years of life ( they will develop these syndromes (in the first 5 years of their life)
myeloid prolifereations related to down syndrome
76
* Rare, aggressive type of neoplasm
Lesion seen in skin
Plasmacytoid dendritic cells (not plasma cells)
Skin, blood, then bone marrown (progresses)
Presentation: skin lesion that progresses to peripheral blood and bone marrow
Blastic plasmacytic neoplasm
77
* No clear differentiation to a single lineage (AUL)
* No lineage-spcific Ag or Ags of more than one lineage are expressed (MPAL)
* Mixed phenotype acute leukemia
ACUTE LEUKEMIAS OF AMBIGUOUS AGE
