lymphoid leukemias

Question 1

absolute lymphocytosis?

Answer 1

>

4000. reactive/benign due to mono, viruses, stress, whooping cough.
      neoplastic due to CLL, leukemic lymphoma, sezary syndrome, hairy cell, A-T cell lymphoma, t cell large granular Lymphoma

Question 2

ALL? tx?

Answer 2

kids. no preleukemic state. 85% are B cell, 15% T cell.
treatment: CNS prophylaxis is important. multiple stages. different experimental drugs (BiTE, chimeric antigen receptors)

Question 3

B-ALL? good vs bad prognosis?

Answer 3

CD 10+, TdT, CD 19, 20, 22. t(12,21) = most. t(9, 22) = worse. good prognosis: t(12, 21), hyperdiploidy, 2-10 yo
bad prognosis: t(9, 22), MLL gene, hypodiploidy, WBC > 100k, under 2 or over 10 yo, CSF involvement

Question 4

treatment for t(9, 22) translocation ALL?

Answer 4

dasatinib = 1st line for adult patients w ph+ ALL

Question 5

T-ALL?

Answer 5

mediastinal mass; adolescents. CD3+, CD1a+

Question 6

CLL/SLL? good vs bad prognosis?

Answer 6

CD 5, CD 23 +. soccer ball cells, smudge cells, gingersnap cells.
good prognosis: low Rai, mutated IGHV gene, 13q deletion
bad prognosis: high Rai, unmutated IGHV, CD38, 17p deletion (p53)

Question 7

CLL/SLL treatment?

Answer 7

FCR (fludarabine, rituximab, cyclophosphamide)

Question 8

Hairy Cell Leukemia?

Answer 8

CD 11c, CD 22. splenomegaly dt red pulp infiltration. pancytopenia. lymphocytes w hairy projections and/or lymphocytosis. TRAP +. dry tap. indolent.

Question 9

Sezary Syndrome?

Answer 9

CD 4+ T cell neoplasm. 3: erythroderma, general LAD, circulating sezary cells. cerebriform nuclei. related to mycosis fungioides (patches, plaques on skin, truncal).

Question 10

adult T cell leuk/lymphoma?

Answer 10

CD4+. HTLV1 associated. japan, carribbean, africa. flower cells. hypercalcemia.

Question 11

large granular lymphocytic leukemia

Answer 11

CD 8+ CTLs or NK cells. pink granules. indolent. associated with autoimmune, RA.