Leukemias Flashcards
(86 cards)
1
Q
Number of peripheral WBCs in leukemia
A
- Increased (malignant leukocytes in blood
- rare cases –> normal/decreased
2
Q
Acute lymphoblastic leukemia/lymphoma (ALL) age
A
children
less common in adults but worse prognosis
3
Q
Acute lymphoblastic leukemia/lymphoma (ALL) is associated with
A
Down syndrome
4
Q
T cell ALL can present as
A
Mediastinal mass (presenting as SVC-like syndrome)
5
Q
Acute lymphoblastic leukemia/lymphoma (ALL)-better prognosis if
A
t(12;21)
6
Q
Acute lymphoblastic leukemia/lymphoma (ALL)
Peripheral blood and marrow have increased:
A
Lymphoblast
7
Q
Acute lymphoblastic leukemia/lymphoma (ALL)
Responsiveness to therapy
A
It is the most responsive to therapy
8
Q
Acute lymphoblastic leukemia/lymphoma (ALL) may spread to
A
CNS and testes
9
Q
Acute lymphoblastic leukemia/lymphoma (ALL) markers
A
- TdT+ (pre-T and pre-B cells)
2. CD10+ (pre-B cells only)
10
Q
Small lymphocytic lymphoma (SLL) / chronic lymphocytic leukemia (CLL) age
A
more than 60
11
Q
Most common adult leukemia
A
CLL
12
Q
CLL markers
A
CD20 and CD5 B cell neoplasm
13
Q
Smudge cells in the peripheral smear leukemia
A
CLL
14
Q
CLL - Anemia
A
Autoimmune hemolytic anemia
15
Q
SLL vs CLL
A
CLL has increased peripheral blood lymphocytosis or bone marrow involvement
16
Q
Hairy cell leukemia
Age and type of cells
A
Adults males
Mature B cells (with hair-like cells projections) –> fuzzy appearing on LM
17
Q
Hair cell leukemia cells morphology/presentation
A
Cells with filamentous, hair-like cells projections (fuzzy appearing on LM)
presents with massive splenomegaly
18
Q
Hair cell leukemia - aspiration?
A
dry because of Marrow fibrosis
19
Q
Hair cell leukemia cells detection
A
- Stains TRAP (Tatrate-resistant acid phosphate)
2. Flow cytometry (largely replaced TRAP)
20
Q
Hair cell leukemia treatment
A
- Cladribine
2. Pentostatin
21
Q
AML age
A
Median onset 65
22
Q
Auer robs
A
Perioxidase + cytoplasmic inclusions seen mostly in M3 AML
23
Q
AML - peripheral smear
A
Increased circulating myeloblasts
24
Q
AML risk factors
A
- Prior exposure to alkylating chemotherapy
- Radiation
- Myeloroliferative disorders
- Myelodysplastic
- Down syndrome
25
AML common presentation
DIC
26
AML M3 treatment
If t(15;17).. M3 subtype respond to all trans retinoic acid (vit A), inducing differentiation of meyloblasts
27
CML age
45-85
| Median 64
28
Philadelphia ch
t(9;22)
| BCR-ABL
29
CML cells
Mature and Maturing granoulocytes
Neutrophils
Metamyelocytes
Basophils
30
CML clinical finding
Splenomegaly
31
Blast crisis
CML transform to AML or ALL
32
CML respond to
Imatinib (bcr-al tyrosine kinase inhibitor)
33
CML vs leukemoid reaction
CML has very low LAP as a result of low activity in malignant neutrophils (vs benign neutrophilia)
34
t8;14
Burkitt lymphoma (c-myc)
35
t9;22
Philadelphia (BCR-ABL)
- CML
- rarely ALL
36
t11;14
Mantle (cyclin d1)
37
t14;18
Follicular lymphoma (BCL-2)
38
t12;21
Better prognosis ALL
39
t15;17
M3 AML subtype respond to vit A
40
Langerhans cell histiocytosis
Collective group of proliferative disorders of dendritic (langerhans) cells
41
Langerhans cell histiocytosis present in a child as
1. Lytic bone lesions
2. Skin rash
3. Recurrent otitis with a mass involving the mastoid bone
42
Langerhans cell histiocytosis - T cells activation
Cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation
43
Langerhans cell histiocytosis markers
S-100 (mesodermal origin)
| CD1a
44
Histological feature of Langerhans cell histiocytosis
Birdeck granules (tennis racket or rob shape on EM) are characteristic
45
Leukemic cells can infiltrate
1. Liver
2. Spleen
3. Lymph nodes
Skin (leukemia curtis)
46
Jak 2 is involved in
Hematopoietic growth factor signaling
47
Jak2 gene mutation is often found in (an the exactly mutation)
Chronic myeloproliferative disorder (except CML)
| V617F JAK2 mutation
48
Polycythemia vera - JAK2 mutation
+
49
CML - JAK2 mutation
-
50
Essential thrombocytosis - JAK2 mutation
+ (30-50%)
51
Myelofibrosis - JAK2 mutation
+ (30-50%)
52
Polycythemia vera definition/symptoms
A form of 1ry polycythemia --> disorder oh high Hct
1. Intense itching after hot shower (due to increased basophils)
2. Erythromelalgia (rare, severe, burning pain and red blue discoloration) due to blood clots in vessel extremities
53
Secondary polycythemia is via
Natural or artificial increase in EPO levels
54
Polycythemia vera-number of cells
RBCs - increased
Platelets - increased
WBCs - increased
55
Essential thrombocytosis - number of cells
RBCs - -
Platelets - increased
WBCs - -
56
Essential thrombocytosis definition / symptoms
massive proliferation of megakaryocytes and platelets
symptoms: 1. Bleeding 2. Thrombosis
3. Erythromelalgia may occur
57
essential thrombocytosis - bone marrow and blood smear
bone marrow: Enlarged megakaryocytes
| blood smear: markedly increased number of platelets, which may be large or otherwise abnormally formed
58
Myelofibrosis - definition
Obliteration of bone marrow due to increased fibroblast activity in response to proliferation of monoclonal cell lines
(pathoma: neoplastic prolif of mature myeloid cells, esp megak --> PDGF --> marrow fibrosis)
59
Myelofibrosis - RBCs morphology
Teardrop
60
Myelofibrosis - aspiration
Dry tap
61
Myelofibrosis often associated with ...... (A clinical manifestation)
Massive splenomegaly
62
Polycythemia vera / plasma volume
Increased
63
Leukemias - definition and course
unregulated growth and differentation of WBCs in bone marrow --> marrow failure --> anemia, infections, hemorrhage
64
Small lymphocytic lymphoma (SLL) / chronic lymphocytic leukemia (CLL) - course
- often asymptomatic
- progress slowly
- Richter transformation: transformation into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma
65
Richter transformation?
SLL/CLL transformation into an agressive lymphoma, most commonly diffuse large B-cell lymphoma
66
Langerhans histiocytosis - types and malignancy and age
1. Leterrer Siwe --> malignant --> rapidly fatal --> under 2
2. eosinophilic granuloma --> benign --> adolescent
3. Hand Shuller Christian --> Malignant --> children
67
Leterrer Siwe - presentation
skin rash + cystic skeletal defects + Multiple organs may ivolved in infants under 2 --> rapidly fatal
68
eosinophilic granuloma - presentation
pathologic fractures in adolescents but no skin involvement
69
eosinophilic granuloma - biopsy
Langerhans cells with mixed inflammatory cells, inducing numerous eosinophils
70
Hand Shuller Christian - presentation
scalp rash + lytic skull defects + Diabetes insipidus + exopthalmus in children
71
Chronic myeloproliferative disorders - definition and types
malignant hematopoietic neoplasms with varying impacts on WBCs and myeloid cell lines. types:
1. Polycethemia vera
2. CML
3. Essential thrombocythemia
4. Myelofibrosis
72
Polycythemia vera - respond to
Aspirin
73
Chronic myeloproliferative disorder that responds to apirin
Polycythemia vera
74
Chronic myeloproliferative disorders - types and correlation with Philadelphia Ch
1. Polycethemia vera -
2. CML +
3. Essential thrombocythemia -
4. Myelofibrosis -
75
Myelofibrosis - number of cells
```
decreased RBCs (and teardrop)
variable WBCs
varable platelets
```
76
Chronic myeloproliferative disorders - types and correlation with
1. Polycethemia vera +
2. CML + in 30-50%
3. Essential thrombocythemia + in 30-50%
4. Myelofibrosis -
77
CML - number of cells
decreased RBCs
Increased platelets
increased WBCs
78
types of polyceythemia
1. relative
2. Appropriate absolute
3. inappropriate absolute
4. Polycythemia vera
79
types of polyceythemia and EPO levels
1. relative: -
2. Appropriate absolute: increased
3. inappropriate absolute : increased
4. Polycythemia vera: decreased
80
types of polyceythemia and plasma volume
1. relative: decreased
2. Appropriate absolute: -
3. inappropriate absolute: -
4. Polycythemia vera: increased
81
types of polyceythemia and RBC mass
1. relative: -
2. Appropriate absolute: increased
3. inappropriate absolute: increased
4. Polycythemia vera: very increased
82
types of polyceythemia and O2 saturation
1. relative: normal
2. Appropriate absolute: decreased
3. inappropriate absolute: normal
4. Polycythemia vera: normal
83
Relative polycythemia - associations
1. dehydration
| 2. burns
84
Appropriate absolute polycythemia - associations
1. lung disease
2. congenital heart disease
3. high altitude
85
Inappropriate absolute polycythemia - associations
1. malignancy: renal, hepatocellular etc
2. hydronephrosis
(ECTOPIC EPO secretions)
86
Polycythemia vera - EPO (and mechanism)
low --> due to negative feedback
