Devita, Hellman, and Rosenberg's Cancer

LWBK1006-31 LWW-Govindan-Review December 12, 2011 19:43

446 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology Review

when compared to best supportive care alone. More recently, hypomethy-

lating agents have been used in the treatment of AML in the elderly. How-

ever, they have not been shown to be superior to treatment with low-dose

cytarabine. Treatment with tipifarnib, a farnesyl transferase inhibitor, did

not improve outcomes for elderly patients with AML when compared to

best supportive care alone. Treatment with decitabine is associated with

better response rate, but not improved survival, compared to low-dose

cytarabine.

Answer 31.14. The answer is D.

Patients with APL are at considerable risk of early morbidity and mortality

because of disseminated intravascular coagulation and bleeding compli-

cations. Aggressive management of coagulopathy is mandatory, with the

goal of maintaining the fibrinogen greater than 150, INR less than 1.5,

and platelet count greater than 30,000. Patients with WBC greater than

10,000/L are at a higher risk of differentiation syndrome once ATRA

is started. Typically, ATRA is held while daunorubicin is started until the

WBC is less than 10,000/L. Arsenic trioxide has demonstrated signifi-

cant efficacy in the setting of relapsed APL. Ongoing studies are evaluating

its efficacy during induction therapy in conjunction with ATRA. However,

it is not currently standard of care during induction therapy.

Answer 31.15. The answer is C.

Differentiation syndrome can be a life-threatening complication of APL

and ATRA treatment. As the granule-laden promyelocytes differentiate,

the granular products stimulate pulmonary edema and fluid retention.

Fevers and weight gain are common symptoms of differentiation syn-

drome. Treatment includes steroids and management of coagulopathy.

Differentiation syndrome may affect as many as 10% to 25% of patients

with APL.

Answer 31.16. The answer is B.

Headache is a common side effect from ATRA. Treatment options include

dose reduction and symptom management with analgesics. Unfortunately,

patients occasionally require drug discontinuation because of this side

effect.

Answer 31.17. The answer is C.

The greatest risk of relapse in APL is associated with an elevated WBC

greater than10,000/L on presentation. Patients with presenting WBC

less than 10,000/L achieve CR approximately 90% of the time.

Answer 31.18. The answer is D.

Clofarabine is being studied in relapsed AML, but it has not been eval-

uated in APL. Arsenic trioxide has demonstrated significant efficacy in

relapsed APL and in consolidation protocols. APL promyelocytes com-

monly express high levels of CD33, and gemtuzumab ozogamicin (anti-

CD33) has demonstrated efficacy in relapsed APL. APL is one of the few

cases in AML in which autologous transplantation has demonstrated sig-

nificant benefit, and it is often used in a salvage consolidation protocol.