Leukemias Flashcards

1
Q

What is the most common cancer in children?

A

ALL, acute lymphocytic leukemia

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2
Q

What are B-ALL and T-ALL?

(cell type, markers)

A

B-ALL (B cell acute lymphocytic leukemia):

  • leukemia of immature B cells (lymphoblasts)
  • TdT+, CD10, CD19, CD20

T-ALL (T cell acute lymphocytic leukemia/lymphoma):

  • leukemia/lymphoma of immature T cells (lymphoblasts) with a common thymic mass
  • TdT+, variable CD1-8
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3
Q

What genotypic factors are associated with B-ALL?

A

-misc. translocations; most common t(12;21) RUNX1/ETV6

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4
Q

What genotypic factors are associated with T-ALL?

A
  • increased NOTCH1 function
  • misc. translocations
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5
Q

What are the common demographic factors of B-ALL?

A

Age: peak at 3y/o, uncommon after age of 15

Race/ethnicity: whites/hispanics 3x more than blacks

Gender: ~equal

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6
Q

What are the common demographic factors T-ALL?

A

Age: peak in adolescence, uncommon in adults

Race/ethnicity: whites/hispanics 3x more than blacks

Gender: males > females

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7
Q

What is the common presentation of B-ALL?

A
  • aggressive in progression (days to weeks)
  • decreased bone marrow function; ie., fatigue (anemia), fever/infections (neutropenia), and bleeding (thrombocytopenia)
  • mass effects (pain around lesion)
  • HA and vomiting from meningeal spread
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8
Q

What is the common presentation of T-ALL?

A
  • aggressive in progression (days to weeks)
  • decreased bone marrow function; ie., fatigue from anemia, infections, and bleeding
  • mass effects (pain around lesion) **thymic mass**, possible compression of medistinal structures
  • HA and vomiting from meningeal spread
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9
Q

What is the course/prognosis of ALL?

A

Aggressive course

Good prognosis:

  • remission: 95%
  • cure: 75-85%
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10
Q

What factors provide a favorable outcome in ALL?

A
  • age between 2-10 y/o
  • low WBC
  • hyperploidy
  • chromosomal trisomy (4, 7, or 10)
  • t(12;21)
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11
Q

What factors provide an unfavorable outcome in ALL?

A
  • less than 2 y/o
  • greater than 10 y/o
  • peripheral blast count of >100,000
  • pressence of t(9;22) BCR-ABL “Philadelphia chromosome”
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12
Q

What is the most common leukemia in adults?

A

CLL

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13
Q

What are CLL and SLL? (cell type and key features)

A

CLL (chronic lymphocytic leukemia):

  • leukemia of naive, mature B cells
  • **CD5** and CD20 positive
  • “smudge cells”

SLL (small lymphocytic lymphoma)

-CLL involing lymph node mass

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14
Q

What genotypic factors are associated with CLL/SLL?

A
  • deletions (11q, 13q, and 17p)
  • trisomy 12q
  • no translocations
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15
Q

What is the common presentation of CLL/SLL?

A
  • older adults, median 60 y/o
  • typcially asymptomatic/nonspecific

-decreased immune function

-lymphadenopathy and hepatosplenomegaly common in those that are symptomatic

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16
Q

What are potential complications of CLL/SLL?

A
  • decreased immune fucntion/hypogammaglobulinemia -> increased infections
  • autoimmune hemolytic anemia/thrombocytopenia (10-15%)
  • progression to diffuse large B-cell lymphoma (Richter transformation)
17
Q

What factors provide an unfavorable outcome in CLL/SLL?

A
  • deletions of 11q and 17p
  • lack of somatic hypermutation
  • expression of ZAP-70
  • NOTCH1 mutations
18
Q

What is the course/prognosis of CLL/SLL?

A
  • indolent course
  • variable prognosis, death from complications or years to decades of survival
19
Q

What is hairy cell leukemia?

A
  • rare B cell leukemia
  • BRAF mutation
  • “hairy” cells on microscopy
  • cells accumulate in red pulp of spleen (normally leukemias are in white pulp)
  • “dry tap” on marrow aspiration due to fibrosis
20
Q

What is the common presentation of hairy cell leukemia?

A

-older males

-pancytopenia

-splenomegaly (accumulation in red pulp)

21
Q

What is ATLL? (cell type and key features)

A

adult T cell lymphoma

  • CD4 T cells
  • occurs in adults with HTLV-1
22
Q

What is the common presentation of ATLL?

A
  • only adults
  • typically Japanese, West African, or Carribean (HTLV-1 is endemic to these regions)
  • skin lesions
  • hypercalcemia with lytic bone lesions
23
Q

What is the course/prognosis of ATLL?

A

aggressive course

poor progonsis even with treatment (months to a year)

24
Q

Compare ATLL and MM.

A

both have lytic “punched-outbone lesions and hypercalcemia

ATLL will present with rash, MM will not

25
Q

What is mycosis fungoides/Sé​zary syndrome?

A
  • cancer of mature CD4 T cells
  • infiltrate skin

-aggregates of cancer cells in skinproduces characteristicsPautrier microabscesses

-cerebriform nuclei Sé​zary cells in blood

26
Q

What is the common presentation of mycosis fungoides/Sézary syndrome?

A
  • adults
  • rash or erythema with skin plaques and nodules
27
Q

What is the course/prognosis of mycosis fungoides/Sé​zary syndrome?

A

indolent course

great prognosis, responds well to treatment

frequently will relapse but still responds well