Leukemias Flashcards
What percentage of childhood leukemia is ALL?
77%
What is the peak age of ALL?
2-3 years.
What conditions predispose you to ALL?
Down syndrome. Bloom syndrome Ataxia Telangiectasia Fanconi's anaemia Diamond-Blackfan anaemia Schwachman-Diamond Syndrome Kostmann syndrome NF type 1 SCID Paroxysmal nocturnal haemoglobinuria Li-Fraumeni syndrome
What is the risk of leukemia in MCDA twins?
> 70%
What is the most important subtype to differentiate in ALL and why?
Burkitt leukemia (L3 type) or mature B-cell. Most rapidly growing cancer. Need different therapeutic approach
What are some favourable cytogenetics in ALL?
Trisomy 4, 10 and 17 (25%)
t (12;21) (20-25%) (TEL-AML1)
Hyperdiploidy (20-25%)
What are some unfavourable cytogenetics in ALL?
t (4;11) (2%)
t (9;22) (3%) (BCR-ABL)
Hypodiploidy (1%)
Which type of ALL present with mediastinal mass?
T-cell ALL
Which type of ALL most likely present before age 1?
Pro-B cell (CD10-)
Which type of ALL has highest leukocyte count?
T-cell ALL
What is the most common immunophenotype in ALL?
Pre B-cell ALL (CD 10+)
What are negative predictive factors for ALL?
Age of diagnosis (worse if >10 or 50)
Poor response to treatment (High MDR)
Cytogenetics t 4;11, t 9;22, or T-cell type, hypodiploidy, deletion of IKZF1 gene.
What are some clinical features that distinct AML from ALL?
Subcutaneous nodules/blueberry muffin lesions
Gingival infiltration (M4 and M5)
DIC (M3
Masses (chloromas/granulocytic sarcomas) (M2 with t 8;21 translocation)
What are some favourable prognostic features of AML?
M3
t (8;21)
t(15;17)
inv (16)
What are some unfavourable features of AML?
monosomy 7 and 5
5q-
11q23
What’s the treatment for acute promyelocytic leukemia?
all-trans-retinoic acid (treteinoin) (+anthracyclins and cytarabine)
What kind of leukemia often gives you massive splenomegaly?
CML
What is the treatment for CML with BCR-ABL gene rearrangement t (9;22)
Imatinib
Which leukemia can give you hyperuricemia and neurologic symptoms from hyperleukocytosis?
CML
What age group is affected by JMML?
<2 years.
How do JMML present?
Rash Lymphadenopathy Splenomegaly Haemorrhagic manifestations. Elevated leukocytes with monocytes, thrombocytopenia, anaemia with erythroblasts.
What’s in the bone marrow of JMML?
Myelodysplasic pattern with <30% of cells.
What conditions predispose you to JMML?
Noonan
NF-1
In infantile leukemia, are they more likely to get ALL or AML?
ALL
What are some poor prognostic features of ALL in infancy?
Rearrangement of MLL gene - t (4;11) - more prone to relapse.
What is leukemia cutis?
Subcutaneous nodules from leukemic cell infiltration. More common in infantile leukemia.
What is the 5 year survival of ALL?
80%
How many more times likely are Down syndrome kids more likely to develop acute leukemia?
15-20x
Which chemotherapeutic agent is Down syndrome children with leukemia sensitive to?
methotrexate and antimetabolites.
