onc

Question 1

Wiskott-Aldrich syndrome

Answer 1

B and T cell dysfunction, atopic dermatitis, and thrombocytopenia. associated with leukemia/lymphoma and autoimmunity. mutation that makes T cells unable to reorganize actin cytoskeleton. low IgG and IgM. X-linked recessive

Question 2

ALL epidemiology

Answer 2

most common childhood cancer, esp. 2-6 years of age

associated with radiation, chemo, trisomy 21, bloom syndrome, immunodeficiency

Question 3

signs and symptoms of ALL

Answer 3

fever and bone/joint pain (refusal to bear weight)

signs: pallor, bruising, HSP, LAD

Question 4

diagnosis of ALL

Answer 4

CBC showing anemia and thrombocytopenia
WBC count is variable (may be low, high, or normal)
blasts may be seen
confirmation is by bone marrow evaluation showing marrow replacement by lymphoblasts

Question 5

prognostic ALL factors

Answer 5

good prognosis: age 1-9, female, white, WBC <50,000, hyperploidy (more than 53 chromosomes in leukemic cells), no organ involvment, CALLA positive, no chromosome translocation

Question 6

How is ALL classified?

Answer 6

cell morphology- L1 is most common in childhood. L1 symphoblasts are small and have indistinct nucleoli.
Also classified by immunophenotype: t cells 25%, B cells 5%, rest are pre-B cells. most are CALLA positive.x

Question 7

Treatment of ALL

Answer 7

1. induction. give intrathecal MTX
2. consolidation: systemic chemoc. continue intrathecal MTX, and may consider cranial irradiation. cranial irradiation often avoided in kids under 5.
3. maintenance: daily and periodic chemo for up to 3 years.
   bone marrow transplant for very high risk kids or relapse

Question 8

acute myelogenous leukemia: associated conditions

Answer 8

Downs, fanconia anemia, neurofibromatosis. may also be a secondary malignancy

Question 9

Signs and symptoms of AML

Answer 9

fever, HSM, bruising and bleeding, gingival hypertrophy, bone pain. LAD and testicular invovlement are rare

Question 10

lab findings of AML

Answer 10

pancytopenia or leukocytosis, DIC

Question 11

treatment of AML

Answer 11

“very intensive myeloablative therapy”
bone marrow transplant once in remission
prognosis is better for AML associated with Down’s

Question 12

CML

Answer 12

least common type of childhood leukemia
may be adult type (esp. in adolescents- much more common) with 9:22 translocation, or juvenile CML, seen in kids <2. juvenile CML does not have philidelphia chromosome.

Question 13

clinical features of adult CML

Answer 13

nonspecific symptoms, massive splenomegaly, extremely high WBC count (>100,000)

Question 14

clinical features of juvenile CML

Answer 14

fever, chronic eczema-like facial rash, LAD, petechia/purpura, moderate leukocytosis

Question 15

treatment of CML

Answer 15

bone marrow transplant. often fatal regardless, esp. with juvenile CML. imatinib.