BMTCN TEST QUESTIONS WITH VERIFIED CORRECT ANSWERS

What type of transplants commonly have PTLD as a secondary malignancy? Allo HSCT with T-cell depleted graft Therapies associated with therapy-related acute leukemia and MDS alkylating agents (eg. Cytoxan, cisplatin, melphalan, busulfan, ifos) , radiation, topoisomerase II inhibitors (eg. doxorubicin, etoposide, mitoxantrone) Genetic predispositions that increase risk of primary and secondary cancers neurofibromatosis type 1, Fanconi anemia Risk factors for PTLD T-cell depleted graft, mismatched related or unrelated donor. Busulfan, ATG, mAbs, TBI patient w/ primary immunodeficiency, acute or extensive GVHD, EBV Risk factors for secondary solid tumors younger age at HSCT, TBI, chronic GVHD, increasing time from transplant, infections, genetic predisposition, lifestyle factors Treatment options for PTLD EBV-specific cytotoxic T cells, mAbs targeted to B cells (rituximab), combination chemotherapy Treatment options for secondary MDS/acute leukemia conventional chemo or allo transplant. Poor outcomes. QOL domains Physical, Functional, Psychological, Social, Spiritual/existential, Multiple interactions between domains Altered sexual health in male HSCT survivors damage to the hypothalamic-pituitary-gonadal axis: elevated FSH, elevated LH, low testosterone levels; cavernosal arterial insufficiency causing ED Altered sexual health in female HSCT survivors alkylating agents and radiation cause infertility and premature ovarian failure, elevated FSH and LG, low estradiol, menopausal symptoms Risk factors for vaginal alterations post transplant POF (premature ovarian failure), TBI, chronic GVHD Risk factors for infertility pre-HSCT antineoplastic therapy, exposure to alkylating agents, TBI or pelvic irradiation, older age Pregnancy risks in HSCT survivors increased risk of preterm birth and low birth weight, cardiac decompensation during pregnancy 2/2 prior anthracycline exposure. Similar rate of spontaneous abortion to general population. Recommended vaccines Pneumococcal, Diptheria-tetanus, Pertussis, Meningococcal, Inactivated polio Hep B, Recombinant Hep A, Influenza, Measles Mumps and Rubells, HPV When can HSCT survivors get vaccines? inactive vaccines start at 6 months post HSCT, live vaccines start at 2 years post HSCT (in absence of ongoing immunosuppression and GVHD) Most common secondary malignancy in pediatric patients who received radiatios? Nonsquamous call carcinoma Interventions/treatment for vaginal chronic GVHD topical steroids, topical cyclosporine, vaginal dilation Prevention of vaginal chronic GVHD patient education, topical estrogen, early initiation of HRT, vaginal dilation in absence of sexual activity, regular gyn exams Side effects of ovarian failure in prepubescent females infertility, impaired sexual development, short stature Marker that shows immune recovery CD4 Myeloid progenitor cell mature into: Megakaryocytes (produce plts), Erythrocytes, Mast cells, Myeloblasts, neutropils, basophils, macrophages, monocytes, erythrocyte, eosinophils, myeloid dendritic cells, tissue mast cell Lymphoid progenitor cells mature into: NK cells, small lymphocytes, which differentiate into B cells and T cells innate immunity occurs naturally, uses phagocytes that release inflammatory mediators and NK cells acquired immunity response of either B cells or T cells to antigens Syngeneic transplant Identical twin source. No need for immunosuppression. No graft-versus-tumor effect Matched sibling/related transplant HLA-identical relative. No potential stem cell contamination. Continued access to cells. Only 25% of population has a sibling match. Risk of GVHD. Mismatched related transplant HLA-nonidentical relative. No potential stem cell contamination. Increased number of potential donors. Increased risk of GVHD. Increased risk of graft failure d/t HLA disparity Matched unrelated transplant HLA-identical unrelated donor. No potential stem cell contamination. Increased risk of GVHD. Limited number of non-caucasian donors. Waiting period to identify donor. Mismatched unrelated transplant HLA-nonidentical donor. No potential stem cell contamination. Increased risk of GVHD. High treatment-related mortality. Umbilical cord blood transplant Easy access to cell source. Limited number of cells. Delayed time to engraftment, causing increased infection rates Goal of therapy for Nonmalignant disease Cell line replacement (e.g., chronic granulomatous disease, sickle-cell, aplastic anemia) Goal of therapy for Malignant disease tumor ablation Graft vs tumor effect promoted by withdrawal of immunosuppressant therapy, promoted by DLI, decreased in absence of acute GVHD, associated with higher rates of cancer relapse. Autologous transplant pros and cons Pro: easily available source of stem cells, decreased risk of side effects, early engraftment, low risk of GVHD Con: contamination of stem cells by undetectable disease cells, no change of immunologic effect of GVT to assist with control of relapse haploidentical transplant half-matched related Allogeneic transplant pros and cons Pro: disease-free stem cell source, potential for GVT effect Con: increased risk of side effects r/t polypharmacy (i.e. immunosuppressants), GVHD, lifestyle changes r/t ongoing risks of side effects DLI infusion of lymphocytes from original donor in setting of relapsed disease after allo transplant. Induces GVT effect. Increased risk of inducing GVHD