acute leukemia Flashcards
1
Q
FAB
CD 13, 33, 14, 4, 11b, 11c, 64, 36, 68
A
FAB M4
2
Q
- Is primarily a disease of childhood and adolescence, accounting for 25% of childhood cancers and up to 75%ofchildhood leukemia (most common type of leukemia inchildren)
- Peak incidence in children is between 2 and 5 years of age; although rare in adults, risk increases with age; most adult patients are older than 50 years of age
- The subtype is an important prognostic indicator for survival; adults have a poorer outlook: 80% to 90% experience complete remission, but the cure rate is less than 40%
- Lymphoblasts stain PAS and TdT (+) ;
- Sudan Black B (SBB) and Myeloperoxidase (MPO) (-)
A
acute lymphoblastic anemia (ALL)
3
Q
- SB (+)
- MP (+)
- PAS (-)
- NASDCA (-)
- a-NA (-)
- acid phos (-)
A
M1
4
Q
- Characterized by the presence of at least >20% (WHO) or >30% (FAB) blasts, of which at least 50% must be of megakaryocyte origin ; Rarest type of AML
- Cells are stained with Alpha Naphthyl acetate esterase (ANAE) and PAS
- Markers present: von Willebrand factor, CD41 (gp IIb) , CD42b (gp Ib), CD61 (gp IIIa)
A
FAB M7 (acute megakaryoblastic leukemia)
5
Q
FAB type
- patients with leukemia secondary to Burkitt’s lymphoma; t(8;14) → myc oncogene
- blast: large
- nuclear shape: regular oval to round
- nucleoli: prominent, basophilic
- cytoplasm: prominent, vacuoles
A
L3
6
Q
- Similar to t(8;21) (q22;q22.1); RUNX1/RUNX1T1 mutation in WHO classification
- A common variant that presents with <90%, myeloblasts and >10% maturing cells of neutrophil lineage, and <20% precursors with monocytic lineage
- Auer rods are often present
- Most blasts stain with MPO,SBB, and CAE
- Markers present: CD13, CD33, CD117, CD34
- FAB M1 and M2 account for 50% of AML cases
A
FAB M2 (w/ maturation)
7
Q
- Although morphology is the first tool used to distinguish ALL from AML, (?) and genetic analysis are the most reliable indicators of a cell’s origin
- Because both B and T cells are derived from lymphoid progenitors, both usually express CD34, terminal deoxynucleotidyl transferase (TdT), and human leukocyte antigen, DR subregion (HLA-DR)
A
immunophenotyping
8
Q
- One of the major changes in the 2017 WHO classification is the removal of acute erythroleukemia (erythroid/myeloid type)– most of these cases will now be classified as MDS with excess blasts
ACUTE ERYTHROLEUKEMIA
* Aka known as Erythemic myelosis, Di Guglielmo’s syndrome
- Characterized by >20% (WHO) or >30% (FAB) marrow myeloblasts and >50% dysplastic marrow normoblasts/erythroblasts
Markerspresent:
* Erythroblasts: CD45, Glycophorin A (CD71)
- Myeloblasts: CD13, CD15, CD33, CD117
- Myeloblasts stain positive with SBB, MPO, CAE ; erythroid cells are PAS-POSITIVE
PURE ERYTHROID LEUKEMIA
* characterized by >80% erythroid cells ; >30% proerythroblasts
- RBC precursors have significant dysplastic features ; presence of ringed sideroblasts, Howell-Jolly bodies and other inclusions
A
FAB M6 (acute erythroleukemia)
9
Q
- SB (++)
- MP (++)
- PAS (-)
- NASDCA (+)
- a-NA (-)
- acid phos (-)
A
M2
10
Q
FAB
CD 13, 33, 117
A
- FAB M0
- FAB M1
- FAB M2
11
Q
immunophenotype
- t(4;11) , CD10 (CALLA → common ALL antigen)
- CD34, CD19, CD10, vytoplasmic CD22, TdT
A
intermediate (common) B-ALL
12
Q
- affects all ages, but increases with older age (>60 yrs)
- may resemble acute infection at presentation
- requires 20% blasts in blood or marrow for diagnosis
- key myeloid antigens : MPO, CD13, CD33, CD117, ans CD14/CD64
- recurrent cytogenetic abnormalities that characterize defined subtypes
- classification made by morphology, cytogenetics, flow cytometry, cytochemistry
A
acute myeloid leukemia
13
Q
AML
CLINICALFINDINGS:
* Infiltration of (?) into the gums and other mucosal sites and skin
- (?) is seen in half of AML patients, but (?) is rare
- (?) is also common at presentation
- During induction chemotherapy, especially when the WBC count is quite elevated, (?) may occur
A
- malignant cells
- splenomegaly, lymph node enlargement
- hypokalemia
- lumor lysis syndrome
14
Q
WHO ALL
have abnormal gene rearrangements, none of the abnormalities is clearly associated with specific biologic features
A
T-lymphoblastic leukemia/lymphoma (T-ALL)
15
Q
ALL
- Predominance of (?) (>20% for (?); >30% for (?)) in about 50% of patients (lymphoblasts with lymphocytes and smudge cells)
- Granulocytopenia
IMMUNOPHENOTYPING:
* B-CELL ALL: CD(?), CD (?),CD (?), CD(?), TdT
* T-CELL ALL: CD(?), CD(?), CD(?), CD(?), CD(?), CD(?), TdT
A
- blast cells
- WHO, FAB
- 34, 19, 10, 22
- 2, 3, 4, 5, 7, 8
16
Q
- May comprise >90% myeloblasts, and fewer than 10% of the leukocytes show maturation to the promyelocyte stage or beyond
- Atleast 3% of blasts give positive results with myeloperoxidase (MPO) or Sudan black B (SBB) stains ; also stain with CAE
- Markerspresent: CD13, CD33, CD117, CD34
- Presence of chloroma → green appearance of tissue using MPO
- Auer rods maybe present
A
FAB M1 (w/o maturation)
17
Q
- SB (-)
- MP (-)
- PAS (+)
- NASDCA (+)
- a-NA (+++)
- acid phos (-)
A
M6
18
Q
immunophenotype
- t(9;22) →Philadelphia chromosome
- MostmatureBcell ALL; TdTnegative, CD34 variable
- 15%ofchildhoodand 10%ofadult B cell ALL
- CD34, CD19, cytoplasmic CD22, cytoplasmic u, TdT (variable)
A
pre-B-ALL
19
Q
- SB (-)
- MP (+)
- PAS (+)
- NASDCA (+)
- a-NA (+++)
- acid phos (-)
A
M7
20
Q
- t(7;11)
- mostoften in teenaged males with a mediastinal mass, elevated peripheral blast counts, meningeal involvement, and infiltration of extra marrow sites
- CD2, CD3, CD4, CD5, CD7, CD8, TdT
A
T-ALL
21
Q
FAB
CD 13, 33, 117, 14, 4, 11b, 11c, 64, 68, 36
A
FAB M5
22
Q
FAB type
- children
- blast: small
- nuclear shape: indistinct
- nucleoli: scant
- cytoplasm: invisible
A
L1
23
Q
FAB
CD 41, 61, 36, 13, 33
A
FAB M7
24
Q
immunophenotype
- 5%inchildren and 11%in adults
- CD34, CD19, cytoplasmic CD22, TdT
A
early (pro/pre-pre) B-ALL
25
* Account for less than 5% of AML, and patients are generally either infants or older adults
* Auer rods typically are absent, and there is no clear evidence of cellular maturation
* Markers present: CD13, CD33, CD34, CD117
* Cells yield NEGATIVE RESULTS with the cytochemical stains myeloperoxidase (MPO) and Sudan black B (SBB)
FAB M0 (minimally differentiated)
26
# FAB type
* older children and adults
* blast: large, heterogenous
* nuclear shape: indented, prominent
* nucleoli: large, abundant
* cytoplasm: moderately clefted
L2
27
* the most common type of leukemia in adults, and the incidence increases with age
* is less common in children
acute myeloid leukemia (AML)
28
* Characterized by >20% (WHO) or >30% (FAB) marrow monoblasts ; account for 10% of AML cases
* In these leukemias, which are divided into monoblastic and monocytic based on the degree of maturity of the monocytic cells present
* Schilling’s type/AML poorly differentiated/Acute monoblastic leukemia/AML without maturation
* Seen in children ; >80% monoblasts in the bone marrow
* Seen in middle-aged adults ; <80% monoblasts
* Cytogenetic abnormality: t(8;16)(p11;p13) has been associated with M5 (also M4)
* Markers present: CD14, CD4, CD11b, CD11c, CD 64
* Cells stain positive with NSE
FAB M5 (acute monocytic leukemia)
29
* Characterized by a significantly elevated WBC count and the PRESENCE OF MYELOID AND MONOCYTOID CELLS in the peripheral blood and bone marrow
* Characterized by >20% (WHO) or >30% (FAB) marrow myeloblasts with >20%-80% cells of monocytic origin (monoblast, promonocyte, monocyte); may have Auer rods
* Also called as NAEGELI’S TYPE ; accounts for 30% of AML cases
Markerspresent:
* Myeloid: CD13, CD33, CD34, CD117
* Monocytic: CD14, CD4, CD11b, CD11c, CD64, CD36, CD68, and lysozyme
* Myeloid cells stain with SBB, MPO, and CAE , monocytic cells with NSE (non-specific esterase)
FAB M4 (acute myelomonocytic leukemia)
30
* SB (+/-)
* MP (+/-)
* PAS (++)
* NASDCA (-)
* a-NA (++)
* acid phos (-)
M5
31
* GOLD STANDARD in classifying leukemias
* Based on: bone marrow morphology, cytochemical reactions, immunophenotyping, clinical manifestations, cytogenetics (detection of the abnormal gene)
* CRITERIA FOR DIAGNOSIS OF ACUTE LEUKEMIA: >20% of blasts in the blood is associated with leukemia
WHO classification
32
* SB (+/-)
* MP (+/-)
* PAS (-)
* NASDCA (+/-)
* a-NA (++)
* acid phos (-)
M4
33
# WHO ALL
is subdivided into nine subtypes that are associated with recurrent cytogenetic abnormalities
B-lymphoblastic leukemia/lymphoma (B-ALL)
34
* Refers to the rapid, clonal proliferation in the bone marrow of lymphoid or myeloid progenitor cells knownas lymphoblasts and myeloblasts
* When proliferation of blasts overwhelms the bone marrow, blasts are seen in the peripheral blood and the patient’s symptoms reflect suppression of normal hematopoiesis
* Onset is sudden, progression is rapid, and the outcome is fatal in weeks or months if left untreated
acute leukemia
35
differentiated based on morphology, including cell size, prominence of nucleoli, and the amount and appearance of cytoplasm
FAB classification
36
# FAB
glycophorin A in erythroids; CD 13, 33, 117 in myeloblasts
FAB M6
37
* Devised in the 1970’s
* Based on: Bone marrow morphology, cytochemical reactions, cytogenetics, immunophenotyping
* CRITERIA FOR DIAGNOSIS OF ACUTE LEUKEMIA: >30% of blasts in the blood is associated with leukemia
FAB classification
38
* Comprises 5% to 10% of AML cases ; occurs in all age groups but is seen most commonly in young adults
* Characterized by a differentiation block at the promyelocytic stage
* Thepresence of >30% abnormal promyelocytes with bundles of auer rods (faggot cells)
* Abnormal promyelocytes can be:
* Hypergranular → associated with DIC and absence of HLA
* Hypogranular/Microgranular → aPML-M3v ; 20-30% of APL cases; presence of “butterfly” or coin-on-coin nucleus
* Promyelocytes stain positive with SBB, MPO, and CAE
* Cytogenetic abnormality involves balanced translocation between chromosomes 15 and 17 t(15;17) ; fusion of PML gene and retinotic acid receptor alpha (RARa) or the PML-RARa gene
* 95% of APL cases have the PML-RARa protein
* Treated with all-trans-retinoic acid (ATRA) and arsenic trioxide
FAB M3 (acute promyelocytic leukemia)
