DT PACE, Tandem Autologous Transplant, Maintenance Therapy for Waldenstrom's Macroglobulinemia Patients
DT PACE, Tandem Autologous Transplant, Maintenance Therapy for Waldenstrom's Macroglobulinemia Patients
The purpose of this study is to find out what the response is and the side effects are with
 chemotherapy using a combination of drugs called D.T. PACE (Dexamethasone, Thalidomide,
 cis-Platinum, Adriamycin, Cyclophosphamide, and Etoposide) + Rituxan, followed by two
 autologous transplants.
Approximately 25 patients, male or female, age 18 and older, regardless of race or ethnicity,
 will participate in this study at UAMS (University of Arkansas for Medical Sciences) only.

 Participants will receive two courses of chemotherapy with a regimen called DT PACE +
 Rituxan. This regimen consists of 6 drugs: Dexamethasone, Thalidomide, cis-Platinum,
 Adriamycin, Cyclophosphamide, and Etoposide.
Inclusion Criteria:

 -  Patients must have a pathological diagnosis of Waldenstrom's Macroglobulinemia, with
 advanced and/or symptomatic disease requiring therapy. At least one of the following
 must be true: *Presence of cytopenias, defined as Hemoglobin < 11 gm%, or WBC <
 2000/µl, or platelets < 100,000 µl; *Presence of extensive (>3 cm) or symptomatic
 lymphadenopathy or hepatosplenomegaly; *Presence of B symptoms (night sweats, fever,
 weight loss of >10% of the baseline); *Presence of severe neuropathy, not otherwise
 explained; *Progressive disease (increase in "M" by > 50% while observed, and decrease
 in hemoglobin by >2 gm%,and/or decrease in platelets by >50,000/µl, and/or increase in
 lymphadenopathy); *Serum albumin <2.5 gm%; *Persistently elevated β-2M >3.0 in the
 absence of renal impairment or active infection.

 -  Hyperviscosity will be treated (in addition to plasmapheresis) only in the presence of
 any of the above

 -  All necessary baseline studies for determining eligibility must be obtained within 35
 days prior to registration.

 -  Patients must have a performance status of 0-2 based on SWOG criteria. Patients with a
 poor performance status (3-4), based solely on bone pain, will be eligible.

 -  Patients must not have significant co-morbid medical conditions or uncontrolled life
 threatening infection.

 -  Patients with recent (< 6 months) myocardial infarction, unstable angina, difficult to
 control congestive heart failure, uncontrolled hypertension, or difficult to control
 cardiac arrythmias are ineligible. Ejection fraction by ECHO must be > 50% and must be
 performed within 60 days prior to registration, unless the patient has received
 chemotherapy within that period of time (dexamethasone and thalidomide excluded), in
 which case the LVEF must be repeated.

 -  Patients must have a creatinine < 3 mg/dl and a creatinine clearance > 30 ml/minute.
 Patients with a creatinine clearance of 30-50 ml will only receive a 50% cisplatin
 dose.

 -  Patients must have adequate hepatic function defined as serum transaminases < 2 x ULN
 and direct bilirubin < 2.0 mg/dl.

 -  Patients must be able to receive full doses of DT PACE, in the opinion of the treating
 investigator, with the exception of: *Patients that have received prior adriamycin >
 450 mg/m2 and LVEF < 55%. Adriamycin will be omitted in these patients; *Patients with
 a creatinine clearance 30 - 50 ml/minute, who will receive 50% of the cisplatin dose.

 -  Patients must not have a history of chronic obstructive or chronic restrictive
 pulmonary disease. Patients must have adequate pulmonary function studies > 50% of
 predicted on mechanical aspects (FEV1, FVC, etc) and diffusion capacity (DLCO) > 50%
 of predicted. Patients unable to complete pulmonary function tests due to myeloma
 related pain or fracture must have a high resolution CT scan of the chest and must
 also have acceptable arterial blood gases defined as PO2 greater than 70.

 Exclusion Criteria:

 -  No prior malignancy is allowed except for adequately treated basal cell or squamous
 cell skin cancer, in situ cervical cancer, or other cancer for which the patient has
 been disease free for at least three years. Prior malignancy is acceptable provided
 there has been no evidence of disease within the three-year interval.

 -  Pregnant or nursing women may not participate. Women of childbearing potential must
 have a negative pregnancy test documented within one week of registration. Women/men
 of reproductive potential may not participate unless they have agreed to use an
 effective contraceptive method.

 -  All patients must be informed of the investigational nature of this study and must
 sign and give written informed consent in accordance with institutional and federal
 guidelines.