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Hematology And Oncology > Leukemias > Flashcards

Flashcards in Leukemias Deck (86):
1

Number of peripheral WBCs in leukemia

- Increased (malignant leukocytes in blood
- rare cases --> normal/decreased

2

Acute lymphoblastic leukemia/lymphoma (ALL) age

children
less common in adults but worse prognosis

3

Acute lymphoblastic leukemia/lymphoma (ALL) is associated with

Down syndrome

4

T cell ALL can present as

Mediastinal mass (presenting as SVC-like syndrome)

5

Acute lymphoblastic leukemia/lymphoma (ALL)-better prognosis if

t(12;21)

6

Acute lymphoblastic leukemia/lymphoma (ALL)
Peripheral blood and marrow have increased:

Lymphoblast

7

Acute lymphoblastic leukemia/lymphoma (ALL)
Responsiveness to therapy

It is the most responsive to therapy

8

Acute lymphoblastic leukemia/lymphoma (ALL) may spread to

CNS and testes

9

Acute lymphoblastic leukemia/lymphoma (ALL) markers

1. TdT+ (pre-T and pre-B cells)
2. CD10+ (pre-B cells only)

10

Small lymphocytic lymphoma (SLL) / chronic lymphocytic leukemia (CLL) age

more than 60

11

Most common adult leukemia

CLL

12

CLL markers

CD20 and CD5 B cell neoplasm

13

Smudge cells in the peripheral smear leukemia

CLL

14

CLL - Anemia

Autoimmune hemolytic anemia

15

SLL vs CLL

CLL has increased peripheral blood lymphocytosis or bone marrow involvement

16

Hairy cell leukemia
Age and type of cells

Adults males
Mature B cells (with hair-like cells projections) --> fuzzy appearing on LM

17

Hair cell leukemia cells morphology/presentation

Cells with filamentous, hair-like cells projections (fuzzy appearing on LM)
presents with massive splenomegaly

18

Hair cell leukemia - aspiration?

dry because of Marrow fibrosis

19

Hair cell leukemia cells detection

1. Stains TRAP (Tatrate-resistant acid phosphate)
2. Flow cytometry (largely replaced TRAP)

20

Hair cell leukemia treatment

1. Cladribine
2. Pentostatin

21

AML age

Median onset 65

22

Auer robs

Perioxidase + cytoplasmic inclusions seen mostly in M3 AML

23

AML - peripheral smear

Increased circulating myeloblasts

24

AML risk factors

1. Prior exposure to alkylating chemotherapy
2. Radiation
3. Myeloroliferative disorders
4. Myelodysplastic
5. 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