Term
|
Definition
| Megakaryocytes are present in normal to increased numbers, generally with a shift towards younger megakaryocytes |
|
|
Term
| when should iron chelation start in pts with thalassemia? |
|
Definition
|
|
Term
| The protein which is abnl in CDA-1 |
|
Definition
| codanin-1, involved in nuclear membrane activity |
|
|
Term
| Bleeding d/o which is associated with "extreme thrombocytosis" in MP d/o. |
|
Definition
|
|
Term
| 70% of infants with ALL have a transloaction ivolving this chromosome, and this gene. |
|
Definition
|
|
Term
| Fusion genes associated with inv(16)? |
|
Definition
|
|
Term
| Main side effect of EPO alfa = ? |
|
Definition
|
|
Term
| T or F: EPO spares most chemo pts from getting transfused. |
|
Definition
| False: 6 pts must be treated with EPO to prevent one transfusion. |
|
|
Term
| When using EPO< what should the ferritin and transferrin levels be maintained? |
|
Definition
| Ferritin > 100, transferrin > 20%. |
|
|
Term
| Mutations in Mpl cause a rare pediatric disorder. |
|
Definition
|
|
Term
| the primary site of thrombopoietin production in vivo |
|
Definition
|
|
Term
|
Definition
| Increased plt turnover (NOT decreased production--TPO levels are normal or high) |
|
|
Term
| TPO levels in aplastic anemia = ? |
|
Definition
|
|
Term
| Most important side effect of the currently tested exogenous TPO = ? |
|
Definition
| Anti-TPO antibodies formed in 4%, with subsequent t-penia. |
|
|
Term
| How does recombinant IL-11 work? |
|
Definition
| IL-11 promotes CFU-Meg growth |
|
|
Term
| What is the role of Oprelvkin in SCT? |
|
Definition
| Oprelvekin does not significantly enhance platelet recovery or decrease the need for platelet transfusions following autologous stem cell transplantation. |
|
|
Term
| What is the role of oprelvekin in chemo-induced t-penia? |
|
Definition
| oprelvekin is effective for primary prevention of chemotherapy-induced thrombocytopenia in patients receiving moderately dose-intensive chemotherapy |
|
|
Term
| Main 3 side effects of oprelvekin = ? |
|
Definition
| fluid retention, atrial tachyarrhythmias, myalgias/arthralgias. NOTE: fever is NOT a side effect. |
|
|
Term
| Does GCSF have an effect on long-term survival of AML patients? |
|
Definition
|
|
Term
| What is CAM- and what does it do? |
|
Definition
| vascular cell adhesion molecule, which keeps cells in marrow; neutrophil granules contain elastase and cathepsin, and these proteases cleave vascular cell adhesion molecule-1 from the surface of bone marrow stromal cells. Release of this tether facilitates the egress of hematopoietic cells from the bone marrow into the blood. |
|
|
Term
| When is GCSF indicated in outpt chemotx regimens? |
|
Definition
| When risk of febrile NTP >= 40% |
|
|
Term
| How do patients with Kostman syndrome present? |
|
Definition
| Patients with Kostmann syndrome present in infancy with neutrophils <200/mL, an arrest in myeloid maturation at the promyelocyte–myelocyte stage in the marrow, and recurrent infections. |
|
|
Term
| Side effect of GMCSF that is NOT common in GCSF = ? |
|
Definition
| Fever (22%). Also, fluid retention/dyspnea. |
|
|
Term
| Pts with this disease should NOT receive filgrastim. |
|
Definition
|
|
Term
| A dose of peg-filgrastim is equal to a how many does of filgrastim? |
|
Definition
| a single dose of pegfilgrastim provides neutrophil support comparable with that provided by 11 daily injections of filgrastim |
|
|
Term
| 4 adverse cytogenetic findings in adult ALL. |
|
Definition
| 9;22, 4;11, trisomy 8, hypodiploidy (esp < 45 chromos) |
|
|
Term
| a WBC count higher than this protends badness for ALL patients. |
|
Definition
|
|
Term
| ALL in infancy with poor prognosis is often associated with this chromosomal abnormality. |
|
Definition
|
|
Term
| What phenotype is t(1;19) associated with? |
|
Definition
|
|
Term
|
Definition
| With modern treatment, the t(1;19), associated with a pre-B phenotype, no longer has an unfavorable prognosis. |
|
|
Term
| treatment of choice for Ph-positive ALL in first CR for all age and WBC risk groups |
|
Definition
|
|
Term
| Clinical features associated with a higher rate of CNS relapse (4) |
|
Definition
| BTLP: mature b-cell, T-cell, high LDH level, high proliferative index. |
|
|
Term
| When should imatinib be used in ALL? |
|
Definition
| Only in relapsed, PH+ patients, and even then, responses (if any) tend to be short-lived. |
|
|
Term
| defect which sometimes results in fatal clostridium infection |
|
Definition
|
|
Term
| infections caused by catalase-positive organisms including Staphylococcus aureus and Aspergillus species |
|
Definition
| chronic granulomatous disease |
|
|
Term
| This disorder is caused by mutations in one of the components of the NADPH oxidase system that results in a failure to generate the oxidative burst within the neutrophil |
|
Definition
| chronic granulomatous disease |
|
|
Term
| x-linked gene involved in CGD |
|
Definition
|
|
Term
| sex of most CGD patients = ? |
|
Definition
|
|
Term
| abnormal nitroblue tetrazolium (NBT) test |
|
Definition
| chronic granulomatous disease |
|
|
Term
| mildly neutropenic, recurrent infections, blood smear shows large, blue-staining granules in cytoplasm |
|
Definition
|
|
Term
| Neoplastic disease which is more common in FMF patients. |
|
Definition
| Because the chronic recurrent inflammatory attacks also cause persistent elevations of serum amyloid A (AA) protein, patients with FMF are at high risk of developing complications of AA amyloidosis, especially in the kidneys. |
|
|
Term
| Tx for FMF (prophylactic) = ? |
|
Definition
|
|
Term
| 2 malignancies that can result in monocytosis. |
|
Definition
|
|
Term
| fever, lymphadenopathy, hepatosplenomegaly, rash, neurologic signs, cytopenias, coagulopathy (especially hypofibrinogenemia), hypertriglyceridemia, hyperferritinemia and abnormal liver function tests |
|
Definition
|
|
Term
| mutations involving the PRF1 gene, encoding perforin, which leads to this disease |
|
Definition
|
|
Term
| Tx options for HLH (6) include? |
|
Definition
| intravenous immunoglobulin, cyclosporine A, etoposide, cyclophosphamide, splenectomy and plasmapheresis |
|
|
Term
| cells that express surface CD1a and cytoplasmic S-100 |
|
Definition
| Langerhans cell histiocytosis |
|
|
Term
| long-standing LCH lesions have this histologic appearance |
|
Definition
| foamy macrophages and fibrosis |
|
|
Term
| three markers expressed on Langerhans cells (co-expressed) |
|
Definition
|
|
Term
| chromo abnormalities in LCH = ? |
|
Definition
|
|
Term
| isolated bony involvement resulting in ear drainage is more common in children and pulmonary disease occurs predominantly in adults |
|
Definition
|
|
Term
| this endocrine d/o is found in 30% of Langerhans patients |
|
Definition
|
|
Term
| treatment for single-site LCH disease |
|
Definition
| local therapy: surgery, curettage, XRT |
|
|
Term
| treatment for systemic mastocytosis patients with painful/unstable skeletal lesions |
|
Definition
|
|
Term
| treatment for systemic mastocytosis with HSM, portal HTN, marrow suppression, and NO bony cxs |
|
Definition
|
|
Term
| mastocytosis and HES with this fusion gene respond to imatinib |
|
Definition
| FIP1L1-PDGFRA fusion gene |
|
|
Term
| eo count req'd for dx of HES |
|
Definition
|
|
Term
| what sex gets HES more often? |
|
Definition
|
|
Term
| this is the major cause of death in HES |
|
Definition
|
|
Term
| how is diagnosis of eosinophilic infiltration of heart made? |
|
Definition
|
|
Term
| initial tx of choice in hypereosinophilic syndrome |
|
Definition
|
|
Term
| most sensitive way to dx CML = ? |
|
Definition
| RT-PCR of peripheral blood |
|
|
Term
| % blood or marrow blasts in blast-phase CML = ? |
|
Definition
|
|
Term
| conditioning regimen for CML x-plant |
|
Definition
| cyclophosphamide/busulfan |
|
|
Term
| How long after completion of anticoagulation should one wait before doing protein C, S, AT-II tests? |
|
Definition
|
|
Term
| Yearly incidence of DVT for pt on OCP and having FVL mutation (homozygous)? |
|
Definition
|
|
Term
| What is the antibody in cold agglutinin disease? In PCH? |
|
Definition
| cold agg dz = IgM, PCH = IgG |
|
|
Term
| What is the antigen in cold agglutinin disease? |
|
Definition
|
|
Term
| Of the AIHAs (3 types), which does NOT respond to steroids? |
|
Definition
|
|
Term
| Of the AIHAs (3 types), which does respond to splenectomy? |
|
Definition
|
|
Term
| Goal ferritin level in hereditary HC? |
|
Definition
|
|
Term
| Type IIa VWD has been associated with what physiologic problem? |
|
Definition
|
|
Term
| Pregnant women with thrombosis (or high-risk for same) should get what regimen? |
|
Definition
| LMWH q12h for pregnancy and until 6 weeks post. Factor Xa activity should be followed. |
|
|
Term
| Intermediate risk (for thrombosis) in pregnant women should be treated with this regimen. |
|
Definition
| LMWH @ prophylactic dose (40 daily) or heparin at prophylactic dose (5000-10000 bid) for pregnancy and for 6 weeks post. |
|
|
Term
| FVL (heterozygous) and PT 20210 pts should get this prophylaxis during pregnancy. |
|
Definition
| Observation only; no tx unless prior hx of clot. |
|
|
Term
|
Definition
|
|
Term
| What bacteria most often contaminates platelets? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| These three childhood disorders greatly increase the risk of MDS. |
|
Definition
| Schwachman-Diamond, congenital neutropenia, MDS. |
|
|
Term
| Progressive macrocytic anemia in early childhood, normal cellularity of the bone marrow with markedly decreased or absent erythroid precursors, reticulocytopenia. |
|
Definition
|
|
Term
| Treatments for Diamond-Blackfan anemia (3). |
|
Definition
| Steroids, x-fusions, allo-SCT. |
|
|
Term
| Anemia, splenomegaly, gallstones, multinucleated erythroblasts. |
|
Definition
| congenital dyserythropoetic anemia |
|
|
Term
|
Definition
|
|
Term
| Three things considered in the MDS IPSS score = ? |
|
Definition
| BKC (blasts, karyotype, cytopenias) |
|
|
Term
| low-risk (zero) IPSS score in MDS = survival for how long? |
|
Definition
|
|
Term
| high-risk (2.5) IPSS score in MDS - survival for how long? |
|
Definition
|
|
Term
| 4 cytogenetic markers of good px in MDS = ? |
|
Definition
| normal, 5q-, Y abnormalities, 20q |
|
|
Term
| 4 cytogenetic markers of poor px in MDS = ? |
|
Definition
| 5-, 7-, trisomy 8, complex karyotype |
|
|
Term
| what % of 5q- pts will become overt MDS? |
|
Definition
|
|
Term
| What % of MDS patients will respond to EPO? |
|
Definition
|
|
Term
| This is the serum EPO level associated with good response to EPO supplementation in MDS. |
|
Definition
|
|
Term
| Who should be considered for x-plant in MDS? (3) |
|
Definition
| Patients with advanced MDS (int-2 and high by IPSS), a sibling donor and a good performance status |
|
|
Term
| What special consideration needs to be given to Fanconi patients undergoing x-plant for MDS? |
|
Definition
| Conditioning regimen needs to be modified, as chromos are very susceptible to toxins. |
|
|
Term
| How does azacytidine work? |
|
Definition
| Inhibition of DNA methylation. |
|
|
Term
| which chromo is alpha globin gene on? |
|
Definition
|
|
Term
| which chromo is beta globin gene on? |
|
Definition
|
|
