Hematology-Oncology MKSAP/Board Review – Flashcards

-Bone marrow fails to produce blood cells (hypocellular bone marrow +pancytopenia) -Fanconi anemia: Congenital aplastic anemia, autosomal recessive/X-linked. Accompanied by skin defects, short stature, hypogonadism, microcephaly -Bone marrow bx for diagnosis -Association with PNH -Pts with severe aplastic anemia <40 yo should be offered HSCT. Immunosuppresion for those with less severe disease or who are not candidates for transplant (antithymocyte globulin +cyclosporine).

-Severe anemia, lack of reticulocytosis, absence of erythroid precursors in the bone marrow -Possible causes: Presence of new drugs/toxins, CT to rule out thymoma, peripheral blood or bone marrow flow cytometry, parvovirus B19, HIV, autoimmune conditions -Treatment aimed at underlying cause, or immunosuppresive if primary disorder

-Rheumatoid arthritis, splenomegaly, neutropenia

-Risk Factors: DNA damage from previous chemo/rads, antecedent MDS or myeloproliferative disorder, Down syndrome, bone marrow failure syndromes (aplastic anemia, PNH) -fatigue, dyspnea, easy bleeding, gingival hypertrophy, leukemia cutis (violaceous, nontender cutaneous plaques), Sweet syndrome (acute, neutrophilic dermatosis) -Circulating myeloblasts, Auer rods, DIC (especially with APL) -Diagnosis: >20% blasts in the blood or bone marrow -Poor prognostic factors include advanced age, antecedent history of MDS/myeloproliferative disorder, treatment-related disease, high risk cytogenetics -ATRA for APL (15;17) translocation, arsenic trioxide is another option with good cure rates, QT-interval prolongation a possible complication. Both agents can cause a differentiation syndrome with fever, dyspnea +/- pulm infiltrates, edema, hypotension. Treat with dex and hold treatment if severe. -Cytarabine +anthracycline for treatment

-Symptoms occur in days to weeks (fatigue, dyspnea, bleeding, infection-related fever). Lymphadenopathy and hepatosplenomegaly. CNS involvement more common with ALL than AML (intrathecal chemoprophylaxis routinely used in ALL). ->25% lymphoblasts on bone marrow exam -Poor prognosis: Advanced age, adverse cytogenetics (hypodiploidy, translocations involving the MLL gene and Philadelphia chromosome), B-cell disease, high circulating leukocyte count at diagnosis (>30,000) -Tumor lysis syndrome common (IVFs+allopurinol +/-rasburicase-->test for G6PD as can induce hemolysis in these patients)

-Used for hematologic malignancies, congenital/acquired bone marrow failure syndromes -Stem cell mobilization achieved with high doses of G-CSF which leads to increased numbers of CD34 + stem cells in circulation -Patients undergo conditioning for succesful engraftment (myeloablative cytotoxic chemo +/- total body irradiation). Diffuse alveolar hemorrhage and hepatic venoocclusive disease may occur (especially when total body irradiation is used). -Immunosuppressive therapy required to facilitate engraftment and reduce risk of graft-versus-host disease in allogeneic HSCT -Opportunistic infections: Pneumocystis jirovecii, aspergillosis, respiratory viruses, HSV, VZV, CMV.

-Lytic bone lesions, hypercalcemia, renal failure, anemia -Rouleaux formation