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Chapter 10: Unrelated Donor HSCT 211 Transplant Therapy Once the patient and donor meet the transplant center’s eligibility criteria, the patient will be scheduled for the transplant admission. The exact timing and therapeutic plan may vary depending upon the hematopoietic stem cell source (marrow versus peripheral blood versus cord blood), degree of donor and patient HLA mismatch, age of patient, presence of specific end-organ dysfunction, the stage of the disease (aplastic anemia versus MDS versus acute leukemia), institutional preferences, and other personal factors (school, employment, etc). Preparative therapy The pre-transplant (or preparative) therapy most often used in 2008 in the United States consists of fludarabine (FLU), cyclophosphamide (CY), and total body irradiation (TBI). The purpose of the preparative therapy is to destroy the diseased marrow and to suppress the patient’s immune system so that the hematopoietic stem cells from the donor have less chance of being rejected. Pre-transplant therapy in FA patients is significantly reduced compared to transplant patients without FA, due to the unique hypersensitivity to alkylating agents and irradiation of FA patients. While lower dose therapy in FA recipients of sibling donor HSCT has been successful, such therapy is not sufficient in recipients of alternate donor HSCT due to high risk of graft rejection. The side effects of FLU, CY, and TBI are outlined in Table 7. Graft-versus-host disease (GvHD) prophylaxis GvHD results when the immune system of the donor recognizes the patient as “foreign” and tries to reject the foreign tissues. GvHD occurs after HSCT because the donor immune system is transplanted along with the hematopoietic stem cells responsible for marrow
212 <strong>Fanconi</strong> <strong>Anemia</strong>: Guidelines for Diagnosis and Management Table 7: Preparative Therapy Side Effects Total Body Irradiation • Sterility • Fluid retention • Temporary painful swelling of the parotid gland (located in the jaw area), as in mumps • Lung scarring • Hair loss • Sores in the mouth • Nausea, vomiting, and diarrhea • Fever • Dry skin and darkening of the skin • Cataracts • Hormone deficiencies (such as low thyroid hormone levels) • Cancer Cyclophosphamide • Hemorrhagic cystitis (bleeding from the urinary bladder), which sometimes can be prevented by intravenous fluid and with the drug Mesna. • Heart muscle injury • Nausea, vomiting, and diarrhea • Fluid retention • Sores in the mouth • Hair loss • Skin rash • Sterility Fludarabine • Infection • Nausea, vomiting, and diarrhea • Confusion, coma, rapidly progressive brain injury • Kidney insufficiency and failure • Mouth Sores recovery and reconstitution of the blood cells. While GvHD can occur in all patients undergoing an
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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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