Leukemia chpt 13 dsa Flashcards

1
Q

Acute Leukemia- essentials of diagnosis

A
  • short duration of sx’s, including fatigue, fever, bleeding
  • cytopenias or pancytopenia
  • > 20% blasts in bone marrow
  • blasts in peripheral blood in 90% of pts
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2
Q

AML- favorable cytogenetics

A
  • t(8;21))

- inv 16

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3
Q

AML- unfavorable cytogenetics

A

-monosomy 5 or 7

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4
Q

Acute promyelocytic leukemia (APL)- cytogenetics

A
  • t(15;17)

- highly curable!- ATRA (all-trans-retinoic acid) and ATO (arsenic trioxide) in induction

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5
Q

Acute lymphoblastic leukemia (ALL)- better prognosis cytogenetics

A
  • hyperdiploidy of 4, 10, 17

- t(12;21)

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6
Q

ALL- worse prognosis cytogenetics

A
  • hypodiploidy
  • t(9;22)
  • t(4;11)
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7
Q

acute leukemia- sx and signs

A
  • ill only for days-wks
  • bleeding (thrombocytopenia)- skin and mucosal surfaces
  • infection (neutropenia when < 500)
  • gum hypertrophy
  • bone pain
  • hyperleukocytosis (elevated blast count)- causes impaired circulation- HA, confusion, dyspnea
  • pale, purpura and petechiae
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8
Q

acute leukemia- lab findings

A

-pancytopenia with circulating blasts- hallmar!

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9
Q

DIC

A
  • fibrinogen level reduced
  • prothrombin time prolonged
  • D-dimers elevated
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10
Q

Auer rod

A
  • eosinophilic need-like inclusin in cytoplasm

- in AML!!!

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11
Q

AML- express what?

A
  • CD 13, 33
  • myeloperoxidase
  • tdT
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12
Q

acute leukemia- differential diagnosis

A
  • AML must be distinguished from other myeloproliferative disorders, CML, and myelodysplastic syndromes
  • ALL
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13
Q

AML- treatment

A

-anthracycline plus cytarabine- remission in 90% of pts (

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14
Q

AML- post-remission therapy

A
  • chemotherapy or allogeneic stem cell transplantation
  • if favorable- chemotherapy alone or with autologous transplant
  • if unfavorable- allogeneic stem cell transplantation
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15
Q

ALL- treatment

A
  • chemotherapy including daunorubicin, vincristine, prednisone, and asparaginase
  • if philadelphia chromosome positive- give a tyrosine kinase inhibitor (dasatinib)
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16
Q

ALL- remission induction therapy

A

less myelosuppressive than tx for AML

  • CNS prophylaxis so that meningeal sequestration of leukemic cells dont develop
  • either chemotherapy or chemotherapy and stem cell transplantation
  • <39 yo- better outcomes with pediatric protocols`
17
Q

AML- prognosis

A
  • 80% of pts < 60 yo achieve complete remission
  • 50% cured using risk-adapted post-remission therapy
  • older pts- complete remission 50%
18
Q

ALL- prognosis

A

-<29 yo- excellent outcomes after chemotherapy and risk-adated intestinfication and transplantation

19
Q

AML- induction, consolidation, maintenance

A
  • I- 3 + 7 (anthracycline and Ara-C)
  • C-High dose Ara-C (HDAC)
  • M- promyelocytic leukemia only (ATRA)
20
Q

ALL- induction, consolidation, maintenance

A
  • I- vincristine/prednisone plus others
  • C- mult agents plus CNS prophylaxis (MTX or ARA-C)
  • M- 6-MP and MTX
21
Q

Chronic Myeloid Leukemia (CML)- essentials of diagnosis

A
  • elevated white blood count
  • left-shifted myeloid series but with a low percentage of promyelocytes and blasts
  • BCR/ABL gene
22
Q

CML- cytogenetics

A

-BCR/ABL gene (chrom 9 and 22)

23
Q

CML- sx and signs

A
  • 55 yo
  • fatigue, night sweats, fevers
  • splenomegaly (abd fullness)
  • white blood count usually > 100,000
  • progresses to accelerated and blast phases (>20% blasts in bone marrow)
24
Q

CML- lab findings

A
  • elevated white blood count
  • peripheral blood- myeloid series is left shifted
  • hallmark- PCR Test- BCR-ABL gene
25
Q

CML- diff diagnosis

A
  • differentiated from reactive leukocytosis (due to infection)- white blood count < 50,000, no splenomegaly, no BCR/ABL
  • distinguish from other myeloproliferative diseases
26
Q

CML- tx

A
  • tyrosine kinase inhibitors (imatinib, nilotinib, dasatinib)- remission should occur within 3 months; major cytogenetic response within 3 months (<35% of metaphases contain philadelphia chrom)
  • Imatinib mesylate- 98% control of chronic phase dz
  • nilotinib and dasatinib-inc the rate of major molecular response
27
Q

CML- tx- tyrosine kinase inhibitors should be monitored with?

A
  • PCR assay
  • if a consistent inc in BCR/ABL or those with a suboptimal molecular response- should be switched to a diff TK inhibitor and undergo ABL mutation testing
  • T315I mutation in abl- resistant to imatinib, dasatinib, nilotinib, bosutinib- but sensitive to ponatinib (vascular thrombotic complications)
28
Q

CML- advanced stage (accelerated phase or blast crisis)- tx

A

-tyrosine kinase inhibitor alone or with myelosuppressive chemotherapy

29
Q

CML- prognosis

A

-pts with good molecular responses to TK inhibitor therapy- excellent prognosis 100% survival at 9 yrs

30
Q

Chronic Lymphocytic Leukemia (CLL)- essentials of dx

A
  • B cell lymphocytosis > 5000

- coexpression of CD19, CD5 on lymphocytes

31
Q

CLL- signs and sx’s

A
  • median age at 70 yo
  • lymphadenopathy and spleen enlargement (50%)
  • usually indolent
32
Q

CLL- Rai classification, survival in months

A
  • 0- lymphocytosis- 150 months
  • 1- plus lymphadenopathy- 101
  • 2- hepatosplenomegaly- 72
  • 3- anemia- 22
  • IV- thrombocytopenia- 11
  • 0-1- low risk
  • 2- intermediate risk
  • 3-4- high risk
33
Q

CLL- lab findings

A
  • lymphocytosis
  • CD19, 5, 23
  • 17p- worst prognosis
  • 11q- bad prognosis
  • 13q- better prognosis
  • hypogammaglobulinemia- in 50% of pts
34
Q

CLL- complications

A
  • Richter syndrome (transforms to diffuse large B-cell lymphoma)
  • autoimmune hemolytic anemia
35
Q

CLL- sx and complications that are an indication for tx

A
  • fatigue, symptomatic lymphadenopathy, anemia, or thrombocytopenia
  • autoimmune hemolytic anemia- rituximab, prednisone, or splenectomy
36
Q

Hairy Cell Leukemia

A
  • indolent B-cell leukemia
  • BRAF V600E mutation
  • CD 19, 20, 11c, 103
  • TRAP positive
37
Q

hairy cell leukemia- clinical manifestations

A
  • pancytopenia and splenomegaly

- cytopenia due to bone marrow failure due to leukemic infiltration and hypersplenism

38
Q

hairy cell leukemia- tx, prognosis

A
  • cladribine- complete remission in 80% of pts
  • Deoxycoformycin (adenosine deaminase inhibitor)- complete remission 80%, more SEs)
  • 90% of treated pts live past 10 yrs