• Mobilization refers to increasing the number of stem cells in blood. Mobilization of stem cells will be done by giving two intravenous doses of cyclophosphamide 2g/m2 (one each day for two days). The patient will also receive Mesna and hyperhydration for bladder protection. Five days following mobilization they will receive filgrastim (G-CSF) 10 mcg/kg/day for five days given as an injection under the skin. Filgrastim is a medicine given to further increase the number of stem cells in the blood. Blood counts will be monitored daily.

• Leukapheresis is the procedure by which the stem cells are separated from the rest of the blood. This is done by removing blood through a catheter and putting it through a machine that removes the stem cells; the blood is then returned to the patient. This usually takes 2-3 hours a day for 2-5 days. The stem cells are then processed, frozen and stored until it is time for transplant.

• Conditioning refers to the administration of cyclophosphamide and Antithymocyte Globulin (ATG). Cyclophosphamide 50 mg/kg/day will be given intravenously once daily for 4 days. ATG 2.5 mg/kg/day will be given intravenously, once a day for 3 days. They will also be given methylprednisone 1mg/kg to reduce the side effects caused by ATG.

• Autologous stem cell infusion will occur at least 48 hours after the last dose of cyclophosphamide. The autologous stem cells previously collected and frozen will be thawed and infused similar to a blood transfusion. The patients will be in the hospital to receive the stem cell infusion. After the infusion of stem cells, they will continue to be hospitalized so they can be monitored and treated for complications. After discharge from the hospital, patients will be followed in the outpatient clinic frequently until they are stable to return to their local oncologist.

1. Age between 16 and 60 years
2. Diagnosis of systemic sclerosis according to ARA-criteria
3. Diffuse scleroderma with disease duration </= 4 years since development of the skin thickening plus a modified Rankin skin score >/= 15 plus major organ involvement as defined as:

a. DLCO and/or (F)VC </= 70% and evidence of interstitial lung disease
b. Renal involvement = any of the following: Hypertension, urinalysis abnormalities, new renal insufficiency
c. Cardiac involvement = any of the following: reversible congestive heart failure, atrial or ventricular rhythm disturbances such as recurrent episodes of atrial fibrillation or flutter
d. Informed Consent

1. Pregnancy or unwillingness to use contraception during study

2. Concomitant severe disease:

a. respiratory
b. renal
c. cardiac
d. liver failure
e. psychiatric disorders
f. other neoplasms or myelodysplasia
g. uncontrolled hypertension
h. uncontrolled acute or chronic infection, including HIV, HTLV-1,2 positivity

3. Previous treatment with TLI, TBI or alkylating agents including cyclophosphamide

4. Significant exposure to bleomycin, tainted rapeseed oil, vinyl chloride, trichlorethylene or silica: eosinophilic myalgia syndrome; eosinophilic fasciitis

Research Coordinator: Dawn Garrett, L.P.N., CTC
Phone: 813-979-7227,
Email garretdl@moffitt.usf.edu

Principal Investigator: Karen K. Fields, M.D.
Co-Investigator: Frank Vasey, M.D.
Co-Investigator: Teresa Field, M.D., Ph.D.
Co- Investigator: Melissa Alsina, M.D.
Co-Investigator: Steven Golstein, M.D.
Co- Investigator: William Dalton, M.D.
Co- Investigator: Clayton Smith, M.D.
Co-Investigator: Daniel Sullivan, M.D.
Co-Investigator: Thomas Loughran, M.D.

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