Term
| Clinical triad of t-penia, large plts, neutrophil inclusions |
|
Definition
|
|
Term
| severe t-penia, large plts, interstitial nephritis, nerve deafness |
|
Definition
|
|
Term
| history of unexplained fever and inflammatory episodes with neutrophilia, synovitis, lymphadenopathy and mild splenomegaly |
|
Definition
| Familiam Mediterranean Fever (mutation in MEFV gene) |
|
|
Term
|
Definition
|
|
Term
| two genetic predispositioning conditions that can lead to JMML |
|
Definition
| Noonan syndrome, neurofibromatosis type 1 |
|
|
Term
| clinical triad of veno-occlusive disease |
|
Definition
| weight gain, hepatomegaly, ascites |
|
|
Term
| two cytogenetics findings that give good prognosis to kids with ALL |
|
Definition
|
|
Term
|
Definition
| bad prognosis ALL in kids |
|
|
Term
| this group ethnic gets factor XI deficiency |
|
Definition
|
|
Term
|
Definition
| CMML, which may respond to imatinib |
|
|
Term
|
Definition
| acute megakaryocytic leukemia |
|
|
Term
| shortened euglobin lysis time |
|
Definition
| antiplasmin deficiency (homozygous) |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| regular flow results for HDz |
|
Definition
| cd15 pos, cd30 pos, cd45 neg |
|
|
Term
| cd15 neg, cd30 neg, cd45 pos |
|
Definition
| nodular lymphocyte predominant HDz |
|
|
Term
|
Definition
| age > 60, Ldh > ULN, PS > 1, extranodal sites > 1, stage > 2 |
|
|
Term
|
Definition
|
|
Term
| what is the anti-cd-52 moab and its main side effect? |
|
Definition
| alemtuzumab (campath); opportunistic infections (esp CMV) |
|
|
Term
| treatment for t-cell CLL = ? |
|
Definition
|
|
Term
| large hemangiomas and comsumptive coagulopathy ion a neonate |
|
Definition
| Kasabach-Merritt syndrome |
|
|
Term
| no megs in the marrow, and mutation in c-mpl gene, low plts |
|
Definition
| amegakaryocytic thrombocytopenia |
|
|
Term
| sever postnatal t-penia, but if pt survives the first year, they will do well |
|
Definition
| TAR (thrombocytopenia-absent radius syndrome) |
|
|
Term
| triad of immunodeficiency, eczema, and moderate thrombocytopenia with small platelets |
|
Definition
|
|
Term
| mild thrombocytopenia, circulating "giant" platelets, and bleeding |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| VWD with normal R:co and RIPA, normal multimers |
|
Definition
|
|
Term
| decreased binding of VWF to F VIII |
|
Definition
|
|
Term
| this disease is worse in pts with blood type O |
|
Definition
|
|
Term
| thrombopoetin receptor = ? |
|
Definition
|
|
Term
| mutation in ET, PV, and MF = ? |
|
Definition
|
|
Term
|
Definition
| megakaryocytic hyperplasia |
|
|
Term
| plt upper limit threshold for dx of ET |
|
Definition
|
|
Term
| most common secondary malignancy in HDz |
|
Definition
|
|
Term
|
Definition
|
|
Term
| chromosomes damaged by alkylators |
|
Definition
|
|
Term
| chromosomal abnormality in topoisomerase secondary leukemias |
|
Definition
|
|
Term
| a mutation which changes the termination codon |
|
Definition
|
|
Term
| this technique probes for the EXISTENCE of specific genes by restriction enzymes in DNA |
|
Definition
|
|
Term
| this probes for EXPRESSION of genes in RNA |
|
Definition
|
|
Term
| this robes for existence of specific proteins in cell |
|
Definition
|
|
Term
| are cytogenetics sensitive enough to r/o MRDz? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| what is the preferred technique for precise measurement of MRDz? |
|
Definition
|
|
Term
| a mouse with a gene placed in it is called? |
|
Definition
|
|
Term
| a mouse bred to have null alleles in a gene is called? |
|
Definition
|
|
Term
| how are FVL and sickle found in patients (what molecular technique)? |
|
Definition
|
|
Term
| 190 kDa protein = what disease? |
|
Definition
|
|
Term
| on which chromosome is HLA found? |
|
Definition
|
|
Term
| On which chromo is beta-2-microglobulin found? |
|
Definition
|
|
Term
| what % of people have a sibling match? |
|
Definition
|
|
Term
| how does survival for 5/6 matched transplants compare with 6/6 transplants? |
|
Definition
|
|
Term
| what is the clear, defined benefit of peripheral SCT over BMT? |
|
Definition
|
|
Term
| how is incidence of GVHD changed in SCT pts compared with BMT? |
|
Definition
| incidence is equivalent (no difference) |
|
|
Term
| with which type of x-plant is DLI not possible? |
|
Definition
|
|
Term
| survival for MM pt after auto x-plant = ? |
|
Definition
|
|
Term
|
Definition
| poor risk ALL (treat adults with x-plant) |
|
|
Term
| what group of aplastic anemia pts gets transplanted imediately on diagnosis? |
|
Definition
| young pts, 40 years old or less |
|
|
Term
| 3 markers of sickle disease badness (indications for x-plant) |
|
Definition
| 1) high WBC 2) low hgb 3) early dactylitis |
|
|
Term
|
Definition
|
|
Term
|
Definition
| indomethacin, chloramphenicol, diclofenac, clopidogrel, butazones |
|
|
Term
reticulated
pigmentation, dystrophic
nails, leukoplakia |
|
Definition
|
|
Term
| small head, brown skin, deformed thumbs |
|
Definition
|
|
Term
| sensitivity to DEB and MMC (clastogens) |
|
Definition
|
|
Term
Failure to thrive, diarrhea
(pancreatic insufficiency),
metaphyseal
chondrodysplasia/
dwarfism |
|
Definition
| Schwachman-Diamond syndrome |
|
|
Term
| marrow findings = increased fibrosis and amorphous eosinophilic material composed of the cytoplasm of the malignant cells |
|
Definition
|
|
Term
| condition characterized by an increased number of circulating T cells bearing the CD57 activation marker of effector/cytotoxic T cells |
|
Definition
| t cell large granular lymphocytic leukemia |
|
|
Term
| up to what age can Fanconi anemia be diagnosed |
|
Definition
|
|
Term
| in what disease state (specific) are ALIPs found? |
|
Definition
|
|
Term
| treatment for people < 40 y/o with severe AA |
|
Definition
|
|
Term
| acute intermittent prophyria is manifest how? |
|
Definition
| neurovisceral disease (abd pain, psychosis) |
|
|
Term
| when are porphyria specimens best collected? |
|
Definition
| during an acute exacerbation |
|
|
Term
| What specific gene is affected in AIP? |
|
Definition
|
|
Term
| barbiturates, other anticonvulsants, sulfonamides, oral contraceptives, and alcohol can all bring on an attack |
|
Definition
|
|
Term
| two urine tests for porphyria |
|
Definition
|
|
Term
| which porphyria excretes coprotoporphyrin in the stool? |
|
Definition
| hereditary coproporphyria |
|
|
Term
| which neurovisceral porphyria does not have stool findings? |
|
Definition
|
|
Term
| Porphyria cutanea tarda is caused by a partial deficiency in this enzyme |
|
Definition
| UROD (uroporphyrinogen decarboxylase) |
|
|
Term
| The enzyme defect on protoporphyria. |
|
Definition
|
|
Term
| In erythropoetic protoporphyria, the protoporphyrins accumulate in what three areas? |
|
Definition
|
|
Term
| What causes seizures in patients with porphyria? |
|
Definition
|
|
Term
| How do aminocaproic acid and tranexamic acid work? |
|
Definition
| Plasminogen activator inhibitors. |
|
|
Term
| What percent of patients who have surgery within 1 month of a DVT will recur? |
|
Definition
|
|
Term
| What percent of TKR pts will have DVT? |
|
Definition
|
|
Term
| Treatment for PTLD < 1 year after transplant. |
|
Definition
| withdraw immunosuppressive drugs |
|
|
Term
| What is the most common clinical disease caused by "passenger lymphocytes" in solid organ transplant? |
|
Definition
| autoimmune, DAT-positive, hemolytic anemia |
|
|
Term
| The preferred test for a pregnant woman suspected of pulmonary embolism |
|
Definition
| V/Q scan (less radiation than PA-gram) |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| prolymphocytic leukemia is positive for this CD marker. |
|
Definition
|
|
Term
| negative for cd 5/10/20 = ? |
|
Definition
|
|
Term
| Name the disease state: t(9;11) |
|
Definition
|
|
Term
|
Definition
| CML or MDS/AML (alkylators) |
|
|
Term
|
Definition
|
|
Term
|
Definition
| bad in myeloma, good in CLL |
|
|
Term
| 15;17 - how do you treat this? |
|
Definition
| ATRA/dauno > dauno consolidation x 2, then atra/orals x 2 years |
|
|
Term
|
Definition
|
|
Term
|
Definition
| extranodal marginal zone lymphoma |
|
|
Term
|
Definition
| extranodal MZL or mantle cell |
|
|
Term
|
Definition
|
|
Term
|
Definition
| lymphoplasmacytic lymphoma |
|
|
Term
| latency period for alkylator-induced leukemia = ? |
|
Definition
|
|
Term
|
Definition
| topoisomerase-induced leukemia (AML) |
|
|
Term
| latency for topoisomerase-induced leukemias = ? |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| southern blot has mostly been replaced by this technique |
|
Definition
|
|
Term
| what causes fibrinogen to become fibrin? |
|
Definition
|
|
Term
| Two causes of basophilic stippling |
|
Definition
| lead poisoning, thalassemias |
|
|
Term
|
Definition
| G6PD deficiency, oxidant (drug-induced) hemolysis |
|
|
Term
|
Definition
| postsplenectomy, sideroblastic anemia |
|
|
Term
| What does myeloperoxidase stain? |
|
Definition
| Primary granules of neutrophils and secondary granules of eosinophils. |
|
|
Term
| Specific esterase - what does it stain? |
|
Definition
| granulos and mast cells, NOT monos |
|
|
Term
| What does toluidine blue stain? |
|
Definition
|
|
Term
| CD235a positive leukemia = ? |
|
Definition
|
|
Term
| Which leukemia is positive for surface Ig? |
|
Definition
| Burkitt's. All of the rest of ALLs are negative for surface Ig. |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| three unusual CD markers on myeloma = ? |
|
Definition
|
|
Term
| CD5 neg, CD 10/20 positive |
|
Definition
|
|
Term
|
Definition
| natural killer cell lymphoma (i.e., lethal midline granuloma) |
|
|
Term
| F 10a converts what into what? |
|
Definition
| prothrombin into thrombin |
|
|
Term
| What converts fibrinogen into fibrin? |
|
Definition
|
|
Term
| What factor catalyzes dissolution of fibrin clot? |
|
Definition
|
|
Term
| What are the four contact factors? |
|
Definition
| factors XI and XII, prekallikrein and HMWK |
|
|
Term
| What does the protamins sulfate test look for? |
|
Definition
| Heparin contamination of a sample causing false elevation of PTT. |
|
|
Term
| This inhibitor can arise after use of bovine thrombin (fibrin glue). |
|
Definition
|
|
Term
| These three drugs prolong the thrombin time. |
|
Definition
| Heparin, argatroban, lepirudin. |
|
|
Term
| Chemo agent which can cause hypofibrinogenemia. |
|
Definition
|
|
Term
| What does reptilase do to a normal human plasma sample? |
|
Definition
| Cleaves fibrinopeptide A from fibrinogen, causing fibrin formation (clot). |
|
|
Term
| If you use these cards and like them, let me know! |
|
Definition
|
|
Term
|
Definition
|
|
Term
| VWDz with undetectable multimers |
|
Definition
|
|
Term
| An abnormality of the platelet receptor Ib. |
|
Definition
| pseudo-VWD or platelet-type VWD |
|
|
Term
| Three causes of bleeding with nl coag and plt studies. |
|
Definition
| factor XIII deficiency, a2-antiplasmin deficiency and PAI-1 deficiency |
|
|
Term
| Pts with MTHFR mutation must have this problem as well to be hypercoagulable. |
|
Definition
|
|
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
|
|
