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Hematology And Oncology > Leukemias > Flashcards

Leukemias Flashcards

1
Q

Number of peripheral WBCs in leukemia

A
  • Increased (malignant leukocytes in blood

- rare cases –> normal/decreased

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2
Q

Acute lymphoblastic leukemia/lymphoma (ALL) age

A

children

less common in adults but worse prognosis

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3
Q

Acute lymphoblastic leukemia/lymphoma (ALL) is associated with

A

Down syndrome

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4
Q

T cell ALL can present as

A

Mediastinal mass (presenting as SVC-like syndrome)

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5
Q

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

A

t(12;21)

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6
Q

Acute lymphoblastic leukemia/lymphoma (ALL)

Peripheral blood and marrow have increased:

A

Lymphoblast

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7
Q

Acute lymphoblastic leukemia/lymphoma (ALL)

Responsiveness to therapy

A

It is the most responsive to therapy

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8
Q

Acute lymphoblastic leukemia/lymphoma (ALL) may spread to

A

CNS and testes

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9
Q

Acute lymphoblastic leukemia/lymphoma (ALL) markers

A
  1. TdT+ (pre-T and pre-B cells)

2. CD10+ (pre-B cells only)

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10
Q

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

A

more than 60

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11
Q

Most common adult leukemia

A

CLL

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12
Q

CLL markers

A

CD20 and CD5 B cell neoplasm

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13
Q

Smudge cells in the peripheral smear leukemia

A

CLL

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14
Q

CLL - Anemia

A

Autoimmune hemolytic anemia

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15
Q

SLL vs CLL

A

CLL has increased peripheral blood lymphocytosis or bone marrow involvement

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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

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17
Q

Hair cell leukemia cells morphology/presentation

A

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

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18
Q

Hair cell leukemia - aspiration?

A

dry because of Marrow fibrosis

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19
Q

Hair cell leukemia cells detection

A
  1. Stains TRAP (Tatrate-resistant acid phosphate)

2. Flow cytometry (largely replaced TRAP)

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20
Q

Hair cell leukemia treatment

A
  1. Cladribine

2. Pentostatin

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21
Q

AML age

A

Median onset 65

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22
Q

Auer robs

A

Perioxidase + cytoplasmic inclusions seen mostly in M3 AML

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23
Q

AML - peripheral smear

A

Increased circulating myeloblasts

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24
Q

AML risk factors

A
  1. Prior exposure to alkylating chemotherapy
  2. Radiation
  3. Myeloroliferative disorders
  4. Myelodysplastic
  5. Down syndrome
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25
Q

AML common presentation

A

DIC

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26
Q

AML M3 treatment

A

If t(15;17).. M3 subtype respond to all trans retinoic acid (vit A), inducing differentiation of meyloblasts

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27
Q

CML age

A

45-85

Median 64

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28
Q

Philadelphia ch

A

t(9;22)

BCR-ABL

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29
Q

CML cells

A

Mature and Maturing granoulocytes
Neutrophils
Metamyelocytes
Basophils

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30
Q

CML clinical finding

A

Splenomegaly

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31
Q

Blast crisis

A

CML transform to AML or ALL

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32
Q

CML respond to

A

Imatinib (bcr-al tyrosine kinase inhibitor)

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33
Q

CML vs leukemoid reaction

A

CML has very low LAP as a result of low activity in malignant neutrophils (vs benign neutrophilia)

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34
Q

t8;14

A

Burkitt lymphoma (c-myc)

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35
Q

t9;22

A

Philadelphia (BCR-ABL)

  • CML
  • rarely ALL
36
Q

t11;14

A

Mantle (cyclin d1)

37
Q

t14;18

A

Follicular lymphoma (BCL-2)

38
Q

t12;21

A

Better prognosis ALL

39
Q

t15;17

A

M3 AML subtype respond to vit A

40
Q

Langerhans cell histiocytosis

A

Collective group of proliferative disorders of dendritic (langerhans) cells

41
Q

Langerhans cell histiocytosis present in a child as

A
  1. Lytic bone lesions
  2. Skin rash
  3. Recurrent otitis with a mass involving the mastoid bone
42
Q

Langerhans cell histiocytosis - T cells activation

A

Cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation

43
Q

Langerhans cell histiocytosis markers

A

S-100 (mesodermal origin)

CD1a

44
Q

Histological feature of Langerhans cell histiocytosis

A

Birdeck granules (tennis racket or rob shape on EM) are characteristic

45
Q

Leukemic cells can infiltrate

A
  1. Liver
  2. Spleen
  3. Lymph nodes
    Skin (leukemia curtis)
46
Q

Jak 2 is involved in

A

Hematopoietic growth factor signaling

47
Q

Jak2 gene mutation is often found in (an the exactly mutation)

A

Chronic myeloproliferative disorder (except CML)

V617F JAK2 mutation

48
Q

Polycythemia vera - JAK2 mutation

A

+

49
Q

CML - JAK2 mutation

A

-

50
Q

Essential thrombocytosis - JAK2 mutation

A

+ (30-50%)

51
Q

Myelofibrosis - JAK2 mutation

A

+ (30-50%)

52
Q

Polycythemia vera definition/symptoms

A

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
Q

Secondary polycythemia is via

A

Natural or artificial increase in EPO levels

54
Q

Polycythemia vera-number of cells

A

RBCs - increased
Platelets - increased
WBCs - increased

55
Q

Essential thrombocytosis - number of cells

A

RBCs - -
Platelets - increased
WBCs - -

56
Q

Essential thrombocytosis definition / symptoms

A

massive proliferation of megakaryocytes and platelets

symptoms: 1. Bleeding 2. Thrombosis
3. Erythromelalgia may occur

57
Q

essential thrombocytosis - bone marrow and blood smear

A

bone marrow: Enlarged megakaryocytes

blood smear: markedly increased number of platelets, which may be large or otherwise abnormally formed

58
Q

Myelofibrosis - definition

A

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
Q

Myelofibrosis - RBCs morphology

A

Teardrop

60
Q

Myelofibrosis - aspiration

A

Dry tap

61
Q

Myelofibrosis often associated with …… (A clinical manifestation)

A

Massive splenomegaly

62
Q

Polycythemia vera / plasma volume

A

Increased

63
Q

Leukemias - definition and course

A

unregulated growth and differentation of WBCs in bone marrow –> marrow failure –> anemia, infections, hemorrhage

64
Q

Small lymphocytic lymphoma (SLL) / chronic lymphocytic leukemia (CLL) - course

A
  • often asymptomatic
  • progress slowly
  • Richter transformation: transformation into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma
65
Q

Richter transformation?

A

SLL/CLL transformation into an agressive lymphoma, most commonly diffuse large B-cell lymphoma

66
Q

Langerhans histiocytosis - types and malignancy and age

A
  1. Leterrer Siwe –> malignant –> rapidly fatal –> under 2
  2. eosinophilic granuloma –> benign –> adolescent
  3. Hand Shuller Christian –> Malignant –> children
67
Q

Leterrer Siwe - presentation

A

skin rash + cystic skeletal defects + Multiple organs may ivolved in infants under 2 –> rapidly fatal

68
Q

eosinophilic granuloma - presentation

A

pathologic fractures in adolescents but no skin involvement

69
Q

eosinophilic granuloma - biopsy

A

Langerhans cells with mixed inflammatory cells, inducing numerous eosinophils

70
Q

Hand Shuller Christian - presentation

A

scalp rash + lytic skull defects + Diabetes insipidus + exopthalmus in children

71
Q

Chronic myeloproliferative disorders - definition and types

A

malignant hematopoietic neoplasms with varying impacts on WBCs and myeloid cell lines. types:

  1. Polycethemia vera
  2. CML
  3. Essential thrombocythemia
  4. Myelofibrosis
72
Q

Polycythemia vera - respond to

A

Aspirin

73
Q

Chronic myeloproliferative disorder that responds to apirin

A

Polycythemia vera

74
Q

Chronic myeloproliferative disorders - types and correlation with Philadelphia Ch

A
  1. Polycethemia vera -
  2. CML +
  3. Essential thrombocythemia -
  4. Myelofibrosis -
75
Q

Myelofibrosis - number of cells

A 
decreased RBCs (and teardrop)
variable WBCs 
varable platelets
76
Q

Chronic myeloproliferative disorders - types and correlation with

A
  1. Polycethemia vera +
  2. CML + in 30-50%
  3. Essential thrombocythemia + in 30-50%
  4. Myelofibrosis -
77
Q

CML - number of cells

A

decreased RBCs
Increased platelets
increased WBCs

78
Q

types of polyceythemia

A
  1. relative
  2. Appropriate absolute
  3. inappropriate absolute
  4. Polycythemia vera
79
Q

types of polyceythemia and EPO levels

A
  1. relative: -
  2. Appropriate absolute: increased
  3. inappropriate absolute : increased
  4. Polycythemia vera: decreased
80
Q

types of polyceythemia and plasma volume

A
  1. relative: decreased
  2. Appropriate absolute: -
  3. inappropriate absolute: -
  4. Polycythemia vera: increased
81
Q

types of polyceythemia and RBC mass

A
  1. relative: -
  2. Appropriate absolute: increased
  3. inappropriate absolute: increased
  4. Polycythemia vera: very increased
82
Q

types of polyceythemia and O2 saturation

A
  1. relative: normal
  2. Appropriate absolute: decreased
  3. inappropriate absolute: normal
  4. Polycythemia vera: normal
83
Q

Relative polycythemia - associations

A
  1. dehydration

2. burns

84
Q

Appropriate absolute polycythemia - associations

A
  1. lung disease
  2. congenital heart disease
  3. high altitude
85
Q

Inappropriate absolute polycythemia - associations

A
  1. malignancy: renal, hepatocellular etc
  2. hydronephrosis
    (ECTOPIC EPO secretions)
86
Q

Polycythemia vera - EPO (and mechanism)

A

low –> due to negative feedback

Hematology And Oncology (21 decks)

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