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What type of transplants commonly have PTLD as a secondary malignancy? - correct answer Allo HSCT with T-cell depleted graft Therapies associated with therapy-related acute leukemia and MDS - correct answer 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 - correct answer neurofibromatosis type 1, Fanconi anemia Risk factors for PTLD - correct answer 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 - correct answer younger age at HSCT, TBI, chronic GVHD, increasing time from transplant, infections, genetic predisposition, lifestyle factors Treatment options for PTLD - correct answer EBV-specific cytotoxic T cells, mAbs targeted to B cells (rituximab), combination chemotherapy Treatment options for secondary MDS/acute leukemia - correct answer conventional chemo or allo transplant. Poor outcomes. QOL domains - correct answer Physical, Functional, Psychological, Social, Spiritual/existential, Multiple interactions between domains Altered sexual health in male HSCT survivors - correct answer 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 - correct answer 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 - correct answer POF (premature ovarian failure), TBI, chronic GVHD Risk factors for infertility - correct answer pre-HSCT antineoplastic therapy, exposure to alkylating agents, TBI or pelvic irradiation, older age Pregnancy risks in HSCT survivors - correct answer 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 - correct answer Pneumococcal, Diptheria-tetanus, Pertussis, Meningococcal, Inactivated polio Hep B, Recombinant Hep A, Influenza, Measles Mumps and Rubells, HPV When can HSCT survivors get vaccines? - correct answer 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? - correct answer Nonsquamous call carcinoma Interventions/treatment for vaginal chronic GVHD - correct answer topical steroids, topical cyclosporine, vaginal dilation Prevention of vaginal chronic GVHD - correct answer 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 - correct answer infertility, impaired sexual development, short stature
Umbilical cord blood transplant - correct answer Easy access to cell source. Limited number of cells. Delayed time to engraftment, causing increased infection rates Goal of therapy for Nonmalignant disease - correct answer Cell line replacement (e.g., chronic granulomatous disease, sickle-cell, aplastic anemia) Goal of therapy for Malignant disease - correct answer tumor ablation Graft vs tumor effect - correct answer 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 - correct answer 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 - correct answer half-matched related Allogeneic transplant pros and cons - correct answer 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 - correct answer infusion of lymphocytes from original donor in setting of relapsed disease after allo transplant. Induces GVT effect. Increased risk of inducing GVHD Peripheral blood stem cell advantages - correct answer faster engraftment, can be done outpt, collection is well tolerated, decreased early regimen-related toxicity in allos, shorter hospitalization, increased immunologic function compared to bone marrow, no anethesia necessary for donor
Peripheral blood stem cell disadvantages - correct answer may require central line for collection, collection may take several days, lots of potential side effects from apheresis, contains more CD34+ cells than bone marrow (related to GVHD in allos), increased risk of chronic GVHD in allos. bone marrow stem cell advantages - correct answer harvesting complete in a few hours, generally well tolerated and it outpt procedure, decreased risk of GVHD in allos bone marrow stem cell disadvantages - correct answer requires anethesia, surgical risks (infections, bleeding, pain, bone damage), longer time to engraftment UCB stem cell advantages - correct answer ease of access to cord blood units, short time until available for use, simple collection process w/ no harm to infant or mother, lower risk of GVHD, decreased risk of viral disease transmission UCB stem cell disadvantages - correct answer increased risk of passage for genetic abnormalities, limited use d/t number of stem cells in unit, delayed post-transplant immune reconstitution, decreased GVT effect, increased risk of graft failure, impossible to get more donor cells if needed, cost $$ G-CSF (filgrastim) - correct answer glycoprotein that stimulates production of hematopoietic cells by binding to certain cell surface receptors. Administered via daily SQ injections and collection begins after 4-5 days of G-CSF. Side effects: bone pain, HA, fatigue, muscle aches, N/V, stomach pain Chemomobilization - correct answer combining chemo with G-CSF, improves CD34+ yields. causes brief, predicable increase in circulating stem cells because the chemo agents cause a reduction in the production of blood cells, which stimulates hematopoietic recovery. Cyclophosphamide, paclitaxel, etoposide, cytarabine. Chemomobilization advantages - correct answer improved mobilization, potentially fewer apheresis procedures, more rapid engraftment, increased survival, may reduce risk of tumor cell contamination of the graft
characteristics of TLS - correct answer hyperkalemia, hyperuricemia, hyperphosphatemia, hypocalcemia, acute renal failure Minor ABO incompatability - correct answer MINOR: O --> A/B/AB recipient is A, B, or AB; donor is O donor O blood has antibodies to A, B, AB risk for acute or delayed hemolytic reactions delayed hemolysis would occur once the graft produces anti-A and anti-B antibodies to the recipient's current blood type helpful to reduce the plasma in the cellular product Major ABO incompatability - correct answer MAJOR: A/B/AB --> O recipient is O, donor is A, B, or AB recipient already has antibodies to A and B, causing risk for acute hemolysis. helps to red-cell deplete the cellular product and give significant IVF support to promote excretion
