tx

Question 1

agranulocytosis

Answer 1

o Broad spectrum antibiotics.

o GCSF administration to stimulate production of granulocytes from marrow precursors.

Question 2

Hemophagocytic Lymphohistocytosis (HLH)

Answer 2

• Immunosuppressive drugs and ‘mild’ chemotherapy.

- Germline mutations or persistent/resistant disease: HSC transplantation

Question 3

C/SLL (chronics,small lymphocytic leukemia)

Answer 3

• Gentle chemotherapy.
• Immunotherapy with Abs to surface proteins (CD20).
• HSC transplant is offered to young pts.
• BTK inhibitors.

Question 4

multiple myeloma (plasma cell myeloma)

Answer 4

Proteasome inhibitors: Degrade unwanted proteins (cells are prone to accumulation of misfolded, unpaired Ig chains). Induce cell death and retard bone resorption (thru stromal cells).

Thalidomide

Bisphosphonates: Inhibit bone resorption, can exacerbate renal complications of disease, ensure pt is adequately hydrated! Follow Cr.

HSC transplant prolongs life but is not curative.

Question 5

-lymphoplasmacytic lymphoma:

Answer 5

Low dose chemo and immunotherapy (antiCD20) may control tumor growth for a bit

Question 6

mantle cell lymphoma (MCL)

Answer 6

HSC and proteasome inhibitors are now being used with potential.
chemo not helpful

Question 7

hairy cell leukemia

Answer 7

Exceptionally sensitive to gentle chemo = long lasting remission.

Relapse in 5 yrs but respond well to reTx with same agents.

BRAF inhibitors = excellent tumor response in pts who fail chemo

Question 8

Anaplastic Large Cell Lymphoma

Answer 8

chemo cure: 75-80%

recombinant antiCD30 abs

Question 9

Extranodal NK/T-cell Lymphoma

Answer 9

Respond well to radiation.

Resistant to chemo

Question 10

Hodgkin Lymphoma

Answer 10

o . Low stage 1st cured with involved field radiotherapy: increased risk of radiotherapy induced malignancies (lung cancer, melanoma, breast cancer).
o 2° tumors reduced by using minimal radiation and less genotoxic chemo w/o loss of therapeutic efficacy.
o AntiCD30 Abs produce excellent response in pts failing conventional methods

Question 11

AML

Answer 11

• 80% of t(15:17) are cured with ATRA + arsenic salts.
• T(8:21), inv(16) w/o KIT mutation = good prognosis with chemo.
• Follow MDS or genotoxic therapy, in older adults, or returned AML = dismal prognosis for these high risk.
• HSC transplant is performed with ‘high-risk’ disease

Question 12

Myelodysplastic syndromes (MDS)

Answer 12

• HSC transplant in young pts.
• Older pts receive abx and transfusions.
• Thalidomide-like drugs and DNA methylation inhibitors.

Question 13

Chronic Myelogenous Leukemia (CML)

Answer 13

• 90% of pts achieve remission with BCR-ABL inhibitors

- HSC may be offered to young pts (75% cured)..

Question 14

Polycythemia Vera (PCV or PV)

Answer 14

phlebotomy

Question 15

Essential Thrombocytosis (ET)

Answer 15

gentle chemo to suppress thrombopoiesis

Question 16

Primary Myelofibrosis (PM)

Answer 16

• Difficult :/

- JAK2 inhibitors for splenomegaly and s/s and HSC in young pts.

Question 17

Multifocal Multisystem Langerhans Cell Histiocytosis (letterer-siwe Disease)

Answer 17

intensive chemo