ALL, CLL, HCL

Question 1

ALL lymphocytes characterized as….

Answer 1

very immature

Question 2

when does the last L in ALL stand for leukemia rather than lymphoma?

Answer 2

when the malignancy is primarily in the bone marrow

Question 3

when does the last L in ALL stand for lymphoma?

Answer 3

1) there is a nodal mass

2) less than 25% of bone marrow is lymphoid blasts

Question 4

who gets ALL?

Answer 4

mostly kids 6 and under

Question 5

ALL T cell vs B cell prevalence

Answer 5

more than 80% B cell

Question 6

peripheral blood smear in ALL

Answer 6

• maybe blasts

- cytopenias common

Question 7

bone marrow cytometry in ALL

Answer 7

• TdT positive, myeloperoxidase/buterate negative
• B cell: CD10 and CD19 positive
• T cell: CD3 and CD7 positive

Question 8

ALL adult cytogenetics

Answer 8

• philadelphia chromosome t(9;22)

Question 9

ALL treatment: induction goal

Answer 9

• ablation of bone marrow with goal of remission
• prolonged cytopenias like in AML
• multi-drug due rapid resistance

Question 10

ALL treatment: consolidation

Answer 10

• high rate of relapse
• chemo for 6-12 months
• sometimes up to two years of maintenance chemo

Question 11

ALL CNS prophylaxis

Answer 11

• XRT

- chemo across BBB

Question 12

autologous vs allogeneic bone marrow transplant in ALL patients

Answer 12

autologous - more common in children

allogeneic - more common in adults and high risk children

Question 13

survival in childhood vs adult ALL

Answer 13

childhood - 80%

adult - less than 50%

Question 14

ALL aggressiveness

Answer 14

high grade/aggressive cancer

Question 15

CLL aggressiveness

Answer 15

low grade/indolent

Question 16

CLL vs SLL

Answer 16

CLL - chronic lymphocytic leukemia is primarily in the bone marrow and peripheral blood
SLL - small lymphocytic lymphoma is primarily nodal

both same disease

Question 17

median age of diagnosis of CLL

Answer 17

65

Question 18

immunophenotypic profile of CLL

Answer 18

• CD19, CD23, CD5 positive
• monoclonal B cells (either kappa or lambda)
• very low density, surface Ig or CD20

Question 19

CLL diagnosis

Answer 19

1) either lymphocytosis or adenopathy

2) cell markers

Question 20

CLL risk scale

Answer 20

0-IV

0 - low risk, lymphocytosis in blood and marrow only
I-II - intermediate risk, lymphadenopathy, hepato/splenomegaly
III-IV - high, anemia and thrombocytopenia

Question 21

IgVH gene mutation in CLL is good or bad?

Answer 21

• good!
• derived from more mature cell
• mean survival is longer than 10 years

Question 22

cell markers associated with poor prognosis in CLL

Answer 22

• CD38

- ZAP70

Question 23

FISH for CLL and prognoses

Answer 23

• 17p (p53 mutation) - worst prognosis
• deletion 11q
• trisomy 12
• deletion 13q - better prognosis

Question 24

CLL treatment

Answer 24

• oral or IV chemo

- monoclonal antibodies

Question 25

what is the only potential cure of CLL

Answer 25

allogeneic transplant

Question 26

potential chemo options for CLL

Answer 26

alkylating agents: chlorambucil, cyclophosphamide
nucleoside analogues: fludarabine, pentostatin
monoclonal antibodies: rituximab
combination agents: bendamustine

Question 27

survival in patients refractory to fludarabine

Answer 27

less than 1 year

Question 28

autoimmune complications of CLL

Answer 28

• autoimmune hemolytic anemia
• immune-mediated thrombocytopenia
• granulocytopenia