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Chapter 10: Unrelated Donor HSCT 209 leukemia) suggest that when compared to bone marrow, there is greater risk of graft failure and slower recovery with cord blood and greater risk of chronic GvHD with unmodified peripheral blood. Recent data suggest that 6/6 or 5/6 HLA-matched cord blood results are similar to those with 8/8 HLA-matched marrow. Currently, the vast majority of FA patients have received marrow. There is relatively less experience using cord blood and peripheral blood to draw conclusions about the best stem cell source. While UCB clearly extends the availability of HSCT to those lacking an 8/8 HLA-matched adult volunteer donor, it is not yet known whether a 6/6 matched cord blood is superior. Data thus far suggest a better outcome with 6/6 matched cord blood but larger patient numbers are required before a recommendation can be made. Exclusion criteria Alternate donor HSCT is not the appropriate treatment for all patients. While exclusion criteria may differ among transplant centers, usually patients will be considered ineligible for transplant if the transplant evaluation indicates that the patient has: • Active uncontrolled infection • HIV seropositivity • Active extramedullary leukemia • History of epithelial malignant solid tumors within two years of HSCT • Severe end-organ dysfunction (variable) • Karnofsky performance status
210 <strong>Fanconi</strong> <strong>Anemia</strong>: Guidelines for Diagnosis and Management Table 6: Laboratory Evaluations to Determine Eligibility for Alternate Donor HSCT Diagnosis • Confirmatory diepoxybutane (DEB) or mitomycin C (MMC) chromosome fragility test (if mosaic, test skin fibroblasts) Complementation Group and Genotype • Determination of complementation group and genotype (desirable but not required) Hematologic • Complete blood count and differential • Bone marrow aspiration and biopsy with cytogenetic evaluation Hepatic • Liver enzymes, total bilirubin • Ultrasound (to determine presence of adenomata, liver size) • Abdominal CT (as indicated) Renal / Bladder • Serum electrolytes and creatinine • 24-hour creatinine clearance or glomerular filtration rate (GFR) • Ultrasound (to determine presence of renal dysplasia, hydronephrosis, abnormal bladder) Cardiac • Electrocardiogram (EKG) • Echocardiogram with left ventricular ejection fraction (heart function) Infectious Disease • Chest radiograph • Chest CT with high resolution inspiratory/expiratory films to rule out occult infection is performed at some centers. • Sinus CT to rule out infection • Panorex to rule out major dental problems Cancer Evaluation (patients with biallelic BRCA2 mutations) • Abdominal CT (to rule out kidney cancer) • MRI of the head (to rule out brain cancer)
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Chapter 4 Gastrointestinal, Hepatic
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Chapter 7 Endocrine Disorders in Fa
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Chapter 14 The Adult Fanconi Anemia
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