Term
|
Definition
| layer in middle of centrifuged blood. Top to bottom: platelets, lymphocytes, monocytes, granulocytes |
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Term
|
Definition
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Term
|
Definition
|
|
Term
|
Definition
| ABO genes are all on chromosome 9, H and h are on 19. H can be converted into A,B, or H (not converted by O) hh produces no protein (Oh) |
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Term
|
Definition
| can happen when bacteria deacetylate N acetylgalactosamine to galactosamine |
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Term
|
Definition
| coded on chromosome 1 as two domains: RhD and RhCE. |
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Term
|
Definition
| traffics Rh to the membrane. If defective, then Rh null, causing spherocytosis, stomatocytosis, and anemia (rare) |
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Term
|
Definition
| mutation in intramembrane or cytoplasmic regions of RhD, causing reduced insertion. |
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|
Term
| Non expressed (Rh negative) D+ phenotypes (Del) |
|
Definition
| low level of D expression undetected by lab tests, may cause complications if their blood is transfused to Rh- pts |
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Term
|
Definition
| false D+, truly Rh neg, with D- like antigen. present in 1:950 blacks |
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Term
|
Definition
| no Rh expression! HPA1a is the most important. they also have ABO antigen |
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Term
|
Definition
| human neutrophil antigen, HNA-3a is the most clinically important. cause of acute resp failure after transfusion if anti HNA-3a serum given to HNA-3a recipient |
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Term
|
Definition
| anti human globilin serum, allows cross linking |
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Term
|
Definition
| always unexpected, largely developed in conjunction with pregnancy. 5-20% of HLA alloimmunized patients have coexisting platelet specific antibodies. Platelet transfusions for pts with anti-HLa should lack corresponding HLA antigens. |
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Term
|
Definition
| use plasma with same ABO as pt or that lacks ABO Abs to pt's red cells. Any Rh is fine. O is universal plasma RECIPIENT! however, giving incompatible plasma can still cause poor reactions |
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Term
|
Definition
| chloroquine, quinine, mefloquine, primaquine |
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Term
|
Definition
| pyrimethamine, sulfas, proguanil |
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|
Term
| mitochondrial poison antimalarials |
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Definition
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Term
|
Definition
|
|
Term
| artemisinin antimalarials |
|
Definition
| wormwood derivatives, variable solubility by form. short T/2 |
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|
Term
| antimalarial active against hepatic form |
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Definition
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|
Term
| antimalarial active against hepatic and erythrocytic forms |
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Definition
| atovaquone and proguanil together are malarone |
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|
Term
| chloroquine concentrations in body |
|
Definition
| high Vd due to concentration in liver, low plasma conc, 250x plasma conc in infected RBCs |
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Term
|
Definition
| acts as base in cytoplasm, preventing acidic proteases from breaking down hemoglobin for food and also blocking heme polymerase from making hemozoin, allowing heme to build up to toxic levels for parasite. |
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Term
|
Definition
| hemolysis for G6PD deficiency, also GI Sx (nausea and vomiting) |
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Term
|
Definition
| VERY fast acting, lower Vd than chloroquine, highly active against schizonts of all 4 malarial species. IV quinine not FDA approved in US, but stereoisomer quinidine approved for cardiac. Has some cardiac side fx. |
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|
Term
|
Definition
| GI, Cinchonism (headache, visual disturbances, dizziness, tinnitus), hemolysis in G6PD def, hypoglycemia 2' to increased insulin secretion, cardiac fx |
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Term
|
Definition
| chloroquine resistant areas, but not suitable for parenteral due to intense local pain. Good against falciparum and vivax. |
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|
Term
| Mefloquine contraindications |
|
Definition
| pregnant women, children under 5 kgs, pts with Hx of seizure, depression, psychosis, cardiac issues, other quinolones |
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|
Term
| primaquine mechanism of action |
|
Definition
| kills hepatic stages of vivax and ovale, erythrocytic stages of falciparum, w/ no action on blood schizontes via redox cycles whose intermediates interfere at mitochondrion |
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|
Term
| primaquine contraindications |
|
Definition
| G6PD deficiency (more severe than other quinolones) and pregnancy |
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|
Term
| atovaquone and proguanil indications |
|
Definition
| used TOGETHER in Tx and prophylaxis, good for MDR malaria, active against schizonts but not hypnozoites, also blood stages of vivax, ovale, and malariae |
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Term
|
Definition
| converted to cycloguanil (analogous to pyrimethamine) by CYP450, blocks plasmodial DHFR and inhibits purine synth |
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Term
|
Definition
| like ubiquinone, irreversibly binds cyt. BC1 to inhibit mitochondrial e- transport, collapses mitochondrial membrane potential |
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Term
|
Definition
| activated by iron, makes radicals to alkylate proteins and heme in food vacuole |
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Term
|
Definition
| first line Tx for falciparum, not prevention (short T/2), used in combination w/ lumefantrine (Coartem) |
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|
Term
| doxycycline use against malaria |
|
Definition
| effective against MDR falciparum, not liver stages, useful alone for prophylaxis. SLOW acting, used w/ reduced dose quinine |
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|
Term
| chloroquine OR mefloquine dosing regimen |
|
Definition
| weekly doses 2 wks before travel- 4 wks after travel |
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|
Term
| doxycycline dosing regimen (malaria) |
|
Definition
| daily 2d before travel-4 wks after travel |
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|
Term
| Malarone (aatovaquone + proguanil) dosing regimen (malaria) |
|
Definition
| daily 2 days before travel- 1 wk after travel |
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|
Term
| Tx for incorrect Rh transfusion (immediate) |
|
Definition
| IV Anti RhD IgG (note that the IM preparation given IV may cause a serious rxn) |
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|
Term
| difference btwn lymphoma and leukemia |
|
Definition
| lymphomas occur primarily in tissues, leukemias occur primarily in the blood and bone marrow, but they may involve the same basic cell types. |
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Term
|
Definition
| origin of most B cell lymphomas. proliferating immunoblasts in the lymph nodes are selected for if they have most affinity for Ag. Highest affinity ones become centrocytes w/ cleaved nucleus, which become plasma or memory B cells. |
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Term
|
Definition
| cancer of lymphocytes that spreads in an orderly fashion from one node to the next, characterized by Reed Sternberg giant cells |
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Term
|
Definition
| immediate IgE mediated allergy/asthma/hayfever |
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Term
|
Definition
| IgG/M antibody mediated diseases attaching to tissue or tissue receptors |
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Term
|
Definition
| immune complexes form with aggregates of Ab and soluble antigen, causing vasculitis. pathogenicity depends on size, charge, Ig isotype, and tissue affinity. |
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Term
|
Definition
| delayed, T-cell mediated. Cytokines released by T cells may cause damage, or cytotoxic T cells may cause damage directly. |
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Term
|
Definition
| type 2 hypersensitivity w/ anti acetylcholine receptor Ab. blocks transmission of nerves to muscles--> flaccid paralysis |
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|
Term
|
Definition
| basement membranes in glomeruli or alveoli are attacked by auto Abs |
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|
Term
| 3 most important cytokines in asthma |
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Definition
|
|
Term
|
Definition
| mutation in AIRE causes autoimmune polyendocrinophath, candidiasis, and ectodermal dystropy. AKA APS-1 |
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|
Term
|
Definition
| CD25 (IL-2Ra) and forkhead family txn factor FOXP3 |
|
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Term
|
Definition
| immune dysfunction polyendocrinopathy enteropathy Xlinked, diarrhea in infancy, dermatitis, type 1 DM, thyroiditis, die in first 2 years of life from FOXP3 mutation |
|
|
Term
| Activation induced death of lymphocytes |
|
Definition
| as immune response fades, activated T cells without antigen bound will bind one another via Fas and FasL, causing them both to die. |
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|
Term
| infections indicative of granulocyte def. |
|
Definition
| skin, lymph node, or deep tissue infs w/ S. aureus, G-s like Serratia, and fungi |
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|
Term
|
Definition
| X linked recessive, recurrent infs w/ S. aureus, G-, fungi. Deficiency in neutrophil ability to kill w/ NADPH oxidase. |
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|
Term
| Clinical phenotype of Hyposplenism |
|
Definition
| sepsis 2' to S. pneumo, H. inf. type b, other encapsulated bacteria. Spleen normally makes IgM to these spp. w/ marginal zone B cells |
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Term
|
Definition
| deletion of 22q11.2. Variable w/ craniofacial dysmorphisms, development, cardiovascular, parathyroid hypocalcemia, T cell deficiency, learning disabilities. |
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Term
|
Definition
| clinical syndrome w/ severe T and B cell deficiencyies. may come from a maturation defect of T cells, or defects in common IL receptor or other signalling (TCR) |
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|
Term
|
Definition
|
|
Term
| Sx of B cell deficiencies |
|
Definition
| recurrent normal infs, GI Sx, granulocytopenia, JRA-like arthritis |
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|
Term
|
Definition
| most common 1' immune def, variable genetics, likely failure to switch to IgA. other Igs nl. May be asymptomatic, atopic dz, recurrent infs, or autoimmune dz. |
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|
Term
|
Definition
| common variable immune deficiency; late onset in life, impaired antibody response and Bcell maturation |
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|
Term
| IFN gamma receptor deficiency |
|
Definition
| only causes atypical mycobacteria infs |
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|
Term
|
Definition
| dementia, psychiatric sx, cerebellar sx, involuntary movement, death. irreversible. |
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|
Term
|
Definition
|
|
Term
|
Definition
| PrP coded on chromosome 20, glycosylated, lots of alpha helix. Folding of the alpha helices into beta sheets causes the dz. |
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|
Term
|
Definition
| nl cellular protein, sensitive to proteolytic degredation and sensitive to chemical and physical inactivation |
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|
Term
|
Definition
| pathological form of PrP, resistant to degredation, name comes from Scrapie in sheep. |
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|
Term
|
Definition
| Creutzfeld-Jakob Disease is TSE in humans |
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|
Term
|
Definition
| some people have a genetic predisposition in their PrPs to misfold and cause a TSE, thus familial CJD |
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|
Term
|
Definition
| misfolding of PrP happens all on its own sometimes, which is the most common form |
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Term
|
Definition
| CJD or TSE caused by eating contaminated human brain |
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|
Term
| polymorphism linked to TSE |
|
Definition
| homozygosity for met at codon 129 is very closely linked to TSE |
|
|
Term
|
Definition
| early onset, median age of death: 28. Psych and sensory Sx begin early, no EEG changes, 13 months for total duration of illness, Florid prion plaques seen, prion detectable in lymphoid tissues. |
|
|
Term
| Characteristics of classic CJD |
|
Definition
| late onset, median age of death: 68. Psych and sensory Sx begin later in illness, EEG changes commonly seen. 4 months for whole illness. Florid plaques usually absent, no PrP in lymphoid tissues. |
|
|
Term
| Chagas vertical transmission |
|
Definition
| crosses placenta and causes as much as 15% of stillbirth in Brazil |
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|
Term
|
Definition
| Chagoma: hardened, red, small tumor at site of parasite entry. Ramona's sign: unilateral bipalpebral edema if port of entry is conjunctiva. Non specific fever, malaise, lymphadenopathy. |
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|
Term
|
Definition
| myocarditis/cardiomyopathy, meningioencephalitis in young pts, dementia, megacolon, megaesophagus |
|
|
Term
|
Definition
| x-rxn w/ host antigens causes autoimmunity, direct damage to neurons, heart, and GI, and inflammation cause dz |
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|
Term
| most important aspect of host defense to T. cruzi |
|
Definition
| cell mediated response is key. IgG and IgM can cause complement-mediated lysis |
|
|
Term
| Tx of acute and chronic Chagas |
|
Definition
| Azoles: Nifurtimox and Benznidazole. Chronic complications can require organ transplant |
|
|
Term
|
Definition
| selectively sensitizes neutrophils to normal stimuli, allows them to kill better, anti-apoptotic for granulocytes, and allows release of NPs from marrow, also encourages growth of NPs |
|
|
Term
|
Definition
| Warts, hyperglobulinemia, immunodeficiency, and myelocathexis; caused by constant CXCR4 signal (sends NPs to bone marrow) |
|
|
Term
|
Definition
| responseible for lymphocyte, granulocyte, and stem cell homing to bone marrow. Sympathetically regulated |
|
|
Term
|
Definition
| absolute neutrophil count= WBC x (%SEGs + %Bands) |
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|
Term
|
Definition
|
|
Term
|
Definition
| <300 typically, although may not necessarily be problematic |
|
|
Term
| pus indicates what about neutrophils |
|
Definition
| indicates adequate production and migration of NPs generally |
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|
Term
|
Definition
| leukocytes localized to margins of vasculature; released by epinephrine |
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|
Term
|
Definition
| makes H2O2, NADPH dependent |
|
|
Term
|
Definition
| makes HOCl in presence of H2O2 |
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|
Term
|
Definition
|
|
Term
|
Definition
| autoimmune dz of adrenal glands |
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|
Term
|
Definition
| autoimmune dz of thyroid gland |
|
|
Term
| Vertical transmission of Grave's dz |
|
Definition
| IgG crosses placenta, causing Graves in fetus, but plasmapheresis may cure this |
|
|
Term
|
Definition
| auto Ab to receptors for thyroid hormone that block negative feedback, causing hyperthyroidism |
|
|
Term
|
Definition
| inflammation makes the BBB permeable to leukocytes and blood ptns. activated T cells specific for CNS ag attack microglia or dendritic cells in brain, causing mast cell activation, complement activation, Ab release, and cytokines --> demyelination |
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|
Term
|
Definition
| important for T and B cell activation induced cell death. defects can cause autoimmune lymphoproliferative dz |
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|
Term
|
Definition
| lupus like dz 2' to defective clearance of immune complexes |
|
|
Term
| ways that infs may cause autoimmunity |
|
Definition
| self-reacting T cells may be stimulated by APCs that upregulate stimulatory molecules in response to microbe. Also, self-reactive T cells may cross react with bacterial peptide. Also, mild infs may broadly activate immune sys, including autoimmune activity |
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|
Term
|
Definition
| models multiple sclerosis when injected with myelin basic protein and Freund's adjuvant to induce T cells against myelin (experimental autoimmune encephalomyelitis) |
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|
Term
|
Definition
| genetically susceptible to autoimmune DM |
|
|
Term
|
Definition
| anti-a4 integrin Ab that prevents leukocytes from migrating across BBB |
|
|
Term
| CMV vertical transmission |
|
Definition
| most common virus transmitted to fetus, 1/150 children born w/ congenital CMV. 1/750 has permanent disability 2' to CMV. |
|
|
Term
|
Definition
| between 50-85% of adults are seropositive- inf is lifelong. recurrences allow transmission |
|
|
Term
| pathologic hallmark of CMV |
|
Definition
| Cytomegaly; enlarged cells seen in cell culture w/ viral inclusion bodies |
|
|
Term
| heterophile negative mononucleosis |
|
Definition
| caused by CMV in immunocompetent pt, less serious than EBV mono. |
|
|
Term
|
Definition
| intrauterine growth restriction, prematurity, microcephaly, jaundice, petechiae, hepatosplenomegaly, pneumo |
|
|
Term
| CMV defense against immune sys |
|
Definition
| produces protein inhibiting synth of ligand for NK activating receptors, also downregulates MHC 1 on infected cells and MHC2 on APCs, inhibits TH1 by encoding IL10 analogue |
|
|
Term
|
Definition
| fomivirsen, gancyclovir, foscarnet, and cidofovir |
|
|
Term
| early indicator of west nile virus |
|
Definition
|
|
Term
|
Definition
| serology w/ antibodies, also nucleic acid amplification technique (NAAT) detects viral RNA; very sensitive |
|
|
Term
|
Definition
| 1% encephalitis or meningits, 40% of pts develop neuroinvasive dz after organ transplant (due to immunosuppresion) |
|
|
Term
|
Definition
| IFN dependent response, neutralizing Abs, complement mediated lysis, cell mediated res |
|
|
Term
|
Definition
| Primary: seroconversion. latent: virus establishes in T cells, MPs, epithelial cells, etc. no viral prolif, no cell damage, no dz. controlled by CD8s. Recurrent: usually due to suppression of immune sys w/ aids, transplant, immune def, etc. CMV may shed w/ fluids in primary and recurrent phases. |
|
|
Term
| nomenclature of chemokines |
|
Definition
| based on disulfide bridges; C is one bridge, CC is two adjacent, CXC is two w/ AAs between. R is receptor |
|
|
Term
|
Definition
| allows B cells to enter lymph nodes, binds CXCR5 receptor which is necessary for AIDS. |
|
|
Term
| General role of selectins |
|
Definition
| bind carbs, initiate leukocyte/ET interaction |
|
|
Term
| General role of integrins |
|
Definition
| bind to cell-adhesion molecules and ECM, strong adhesion. |
|
|
Term
| General role of Ig superfamily |
|
Definition
| ICAMs work in cell adhesion, ligand for integrins |
|
|
Term
| series of neutrophil interactions that lead to extravasation |
|
Definition
| E selectin binds sLeX, CXCL8R binds the gradient of IL-8, and finally LFA-1 binds ICAM1 which is firm and allows extravasation toward the gradient of IL8 or other chemokine |
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|
Term
|
Definition
| SIRS in the presence of an infection; more broadly, the body's abnormal, generalized inflammatory rxn to inf caused by microbial products |
|
|
Term
|
Definition
| systemic inflammatory response syndrome; 2 or more of the following: temp above 38C, temp below 36C, HR>90, Resp >20, WBC >12K or <4K or >10% bands |
|
|
Term
|
Definition
| culturable bacteria present in blood |
|
|
Term
|
Definition
| SIRS with infection and organ failure distant from site of infection (esp. CNS, renal, pulmonary dysfn) |
|
|
Term
|
Definition
| sepsis + hypoTN not responsive to fluid Tx |
|
|
Term
|
Definition
| multi organ system dysfunction ; 3 or more organ systems failing; hemostasis unmaintainable w/o Tx |
|
|
Term
| cytokines that can directly mimic septic shock |
|
Definition
|
|
Term
|
Definition
| increases survival of sepsis w/ these goals: adequate oxygenation, adequate BP, adequate perfusion w/ fluid Tx and vasoactive Tx, also transfusion Tx for anemia |
|
|
Term
|
Definition
| silent (decoy) chemokine receptor that prevents cell activation to check chemokine signaling |
|
|
Term
| tumors expressing oxysterols |
|
Definition
| downregulate expression of CCR7, inhibiting dendritic cells from migrating to lymph nodes to present tumor Ag there |
|
|
Term
| HIV surface binding proteins |
|
Definition
| GP120, GP41 bind CD4 and CCR5 or CXCR4 |
|
|
Term
|
Definition
| release of cell-free virus, exosome transmission, or cell to cell direct transfer; most rapid, 100-1000x more efficient, evades defenses. |
|
|
Term
| HIV -cytokine interaction |
|
Definition
| HIV integrates into T cell genome; when activated by cytokines or at TCR, then viral proteins are made |
|
|
Term
| primary lytic target of HIV |
|
Definition
| activated and memory TH1 effector T cells, esp. in lymphoid tissues. It especially attacks HIV-specific CD4 T cells |
|
|
Term
|
Definition
| within first 14 days, 50% of all memory CD4 T cells dead, mostly mucous associated, esp. GALT |
|
|
Term
|
Definition
| about 10 billion virions produced, mostly eliminated daily |
|
|
Term
|
Definition
| 3 drugs acting on separate targets make resistance far less likely than with just 1 or 2 drugs |
|
|
Term
| why HIV cannot be eliminated with current therapies |
|
Definition
| even when serum load is eliminated, infected memory T cells may lie dormant indefinitely |
|
|
Term
|
Definition
| CD317 inhibits HIV release from infected cell. HIV Vpu inhibits tetherin activity |
|
|
Term
|
Definition
| T cell is activated by APC, PD-1 is expressed on T cell and is turned off by PD-L on APC, making T cells anergic. |
|
|
Term
|
Definition
| causes scarring in lymphoid tissues, decreased marrow pdxn of lymphoid precursors, thymic output of T cells, and destroys lymph node architecture impairing clonal expansion |
|
|
Term
|
Definition
| infects microglial cells which produce cytokines that cause adjacent neurons to apoptose |
|
|
Term
| Sx of acute retroviral syndrome or acute HIV |
|
Definition
| fever, fatigue, rash, headache, adenopathy, pharyngitis, myalgia, N,V,D, Night sweats, meningitis, oral ulcers, gastric ulcers |
|
|
Term
|
Definition
| HIV viral load test; western blot and elisa detect Abs that may not yet be present |
|
|
Term
|
Definition
| wasting, thrush, fever >2wks, diarrhea >1mo, idiopathic thrombocytopenic purpura, oral hairy leukoplakia, herpes zoster |
|
|
Term
|
Definition
| low platelets, HIV testing is indicated. may be only symptom. |
|
|
Term
|
Definition
| same as men plus recurrent yeast infs, cervical dysplasia, carcinoma in situ of cervix, and PID |
|
|
Term
| test of chronic symptomatic HIV |
|
Definition
| western blot is a confirmatory test for chronic HIV |
|
|
Term
|
Definition
| useful for screening; high sensitivity, lower specificity |
|
|
Term
|
Definition
| opportunistic infection, CD4<200, CD4<15% |
|
|
Term
| drugs that directly act on HIV virus |
|
Definition
| nucleoside RT inhibitors, non-nucleoside RT inhibitors, protease inhibitors, integrase inhibitors |
|
|
Term
| drugs that affect host cell interxn w/ HIV |
|
Definition
| fusion inhibitors, co-receptor antagonists |
|
|
Term
|
Definition
| emtricitabine, nucleoside RT inhibitor w/ no major toxicity |
|
|
Term
|
Definition
| tenofovir; nucleoside RT inhibitor, may cause Fanconi syndrome and greater loss of bone density |
|
|
Term
|
Definition
| FTC, TDF (emtricitabine and tenofovir) |
|
|
Term
|
Definition
| Atazanavir (ATV) and Darunavir (DRV) given with ritonavir that suppresses CYP450 3A4 to boost their serum levels |
|
|
Term
| non-nucleoside RT inhibitors |
|
Definition
| Efavirenz (EFV) has severe CNS side efects that should resolve shortly, also rash, hypersensitivity, teratogenicity, upregulates CYP450 3A4 |
|
|
Term
|
Definition
| raltegravir (RAL) has no common or severe side fx, but is a 2x daily drug and only needs 1 viral mutation to develop resistance. Rifampin increases required dose. |
|
|
Term
|
Definition
| 2 nucleoside RT inhibitors plus one other class |
|
|
Term
| physical manifestations of HIV associated lipidemia |
|
Definition
| central fat accumulation (not just subq), hollowed cheeks, buffalo hump on neck, prominent leg veins, thinning legs |
|
|
Term
| neoplastic manifestations of EBV |
|
Definition
| Burkitt's lymphoma, nasopharyngeal carcinoma, lymphoproliferative dz (lymphoma in immunosuppressed, hemophagocytic lymphohistiocytosis also) and x-linked lymphoproliferative syndrome, Hodgkin's lymphoma, gastric CA, T cell lymphoma |
|
|
Term
|
Definition
| fever, lymphadenopathy, pharyngitis; looks like strep throat or acute HIV |
|
|
Term
|
Definition
| looks for agglutination reaction to Ags of sheep or horse erythrocytes on latex beads; positive in 80-90% of mono cases, represents nonspecific B cell activation |
|
|
Term
| common manifestations of mono |
|
Definition
| splenomegaly, palatal petechiae, hepatomegaly |
|
|
Term
| uncommon manifestations of mono |
|
Definition
| hemolytic anemia, thrombocytopenia, aplastic anemia, myocarditis, hepatitis, genital ulcers, rash |
|
|
Term
|
Definition
| ampicillin will cause serious rash |
|
|
Term
|
Definition
| occurs in children 3-14, endemic in malarial areas, most common childhood tumor in Africa, EBV and malaria inf are important cofactors |
|
|
Term
| DDx of infectious mononucleosis syndrome |
|
Definition
| CMV, toxoplasma gondii, acute HIV inf., EBV, strep throat |
|
|
Term
|
Definition
| a fusion of ch22 and 9 that produces the BCR-ABL fusion protein, an oncogene that can cause CML. |
|
|
Term
| normal kappa:lambda light chain ratio |
|
Definition
| 2:1. disturbances of this ratio may indicate clonal dz |
|
|
Term
|
Definition
| leukemia cells early in differentiation, indicative of acute leukemia |
|
|
Term
|
Definition
| a tyrosine kinase receptor for TPO and EPO; not necessarily the 1' cause of MPNs, but a contributing factor when it is permanently switched on. present in 30-50% of myelofibrosis |
|
|
Term
|
Definition
| demonstrates myelofibrosis as black hairy looking mess in marrow |
|
|
Term
| histology of essential thrombocytosis |
|
Definition
| fluffy megakaryocytes in clusters in marrow; otherwise normocytic |
|
|
Term
| EPO levels in erythrocytosis |
|
Definition
| a high EPO level indicates that the primary problem is not in the marrow (2') whereas a low EPO suggests PV |
|
|
Term
|
Definition
| A MPN characterized by overgrowth of RBCs; acquired clonal HSC dz. Increased WBCs, platelets, and spleen size. decreased EPO, causes clots. 95% w/ JAK-2 mutation |
|
|
Term
|
Definition
| myeloproliferative neoplasms; polycythaemia vera, ess. thrombocytosis, myelofibrosis, and also chronic myelomonocytic leukemia is similar |
|
|
Term
|
Definition
| Imatinib (gleevec) is a BCR-ABL blocker, binding the ATP cleft |
|
|
Term
| non-specific cytotoxic Tx for CML |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| PV, congenital EPOr abnormality |
|
|
Term
| 2' congenital erythrocytosis |
|
Definition
| VHL, HIF2a, PHD mutations w/ increased EPO, 2,3 DPG def, high affinity Hbopathy |
|
|
Term
| 2' acquired erythrocytosis |
|
Definition
| hypoxia, renal EPO over pdxn, EPO producing tumors |
|
|
Term
| Polycythaemia Vera Dx criteria |
|
Definition
| severely elevated Hb, JAK2 mutation, marrow trilineage hyperplasia, suppressed EPO, spontaneous EEC |
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Term
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Definition
| asymptomatic-> hyperviscosity Sx-> leukocytosis, thrombocytosis + clotting-> MF -> acute leukemia |
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Term
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Definition
| 30-50% w/ JAK2 mutation, may be 2' to PV or ET, presents w/ splenomegaly, anemia. HSC transplant cures |
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Term
| histology findings of myelofibrosis |
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Definition
| PBS shows leukoerythroblastosis (immature cells pushed into circulation; teardrop RBCs, nucleated RBCs, early myelooid cells) |
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Term
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Definition
| antithymocyte globulin--> major lympopenia, side fx: thrombocytopenia, cytokine release syndrome, serum sickness, allergy |
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Term
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Definition
| anti-CD25 monoclonal ab for pts w/ low-moderate risk of rejection in induction phase. Only targets activated (CD25+) T cells w/ minimal side fx |
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Term
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Definition
| binds cyclophilin, which complexes calcineurin to disrupt signal transduction. Side fx are nephrotoxicity, HTN, hyperlipidemia, gingival hyperplasia, hirsutism, and tremor, diabetes, hemolytic uremic syndrome |
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Term
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Definition
| binds FKV12, inhibits target of rapamycin; side fx: hyperlipidemia, thrombocytopenia, impaired healing, proteinuria, pneumonitis. Not useful in induction phase. Decreased risk of CMV, some antineoplastic fx |
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Term
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Definition
| inhibits inosine monophosphate dehydrogenase and purine synth. Side fx: diarrhea, leukopenia, anemia |
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Term
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Definition
| monoclona ab against CD20 on B cells, not plasma cells |
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Term
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Definition
| violent illness, organ must be removed; preformed Ab attacks transplant vessels |
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Term
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Definition
| antibody mediated rejection; 2' to circulating antidonor Ab, may cause ATN-like histology (type I), capillary involvement (II) or arterial (III) all w/ C4d |
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Term
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Definition
| SUCROSE FREE IVIG, plasmapheresis, rituximab |
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Term
| Tcell mediated rejection Tx |
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Definition
| solumedrol pulse; if failed, thymoglobulin 1-2wks |
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Term
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Definition
| CMV (treatable), EBV: --> post transplant lymphoproliferative dz. BK virus |
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Term
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Definition
| post transplant lymphoproliferative dz; caused by EBV and immunosuppression, Tx: stop immunosuppression, add rituximab |
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Term
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Definition
| dsDNA non-enveloped virus -> URI Sx, decline of renal fn. |
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Term
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Definition
| cells in urine w/ viral inclusions: "decoy cells" (nonspecific). Also PCR |
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Term
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Definition
| -7: chemo/radiation. -3: start GVHD prophylaxis w/ immune suppression. 0: HSC graft. |
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Term
| graft vs leukemia/lymphoma effect |
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Definition
| donor cells recognize cancerous host cells as foreign and kill them, helping cure |
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Term
| advantage of G-CSF mobilized peripheral blood HSCs |
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Definition
| is about a week faster than marrow aspirated from iliac crest |
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Term
| G-CSF affects which interaction |
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Definition
| CXCR4 and CXCL12 is blocked by G-CSF, mobilizing HSCs to periphery |
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Term
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Definition
| Skin: maculopapular rash Liver: elevated transaminases, cholestasis GI: nausea, vomiting, diarrhea |
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Term
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Definition
| 100 days is the cutoff between the two |
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Term
| histological GI hallmark of GVHD |
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Definition
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Term
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Definition
| immunosuppression w/ calcineurin inhibitor (cyclosporin or tacrolimus), also methotrexate or mycophenolate; T-cell depletion of graft w/ CD34+ selection, ATG |
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Term
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Definition
| anti-thymocyte globulin isolated from horses or rabbits. |
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Term
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Definition
| X linked recessive dz w/ eczyma, thrombocytopenia, immune def, bloody diarrhea. May cause autoimmune dz and cancer. B and T immunodeficiency, all curable w/ HSC transplant |
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Term
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Definition
| lethal congenital absence of antigen specific B and T cell responses, curable w/ HSC transplant, no conditioning or GVHD prophylaxis required |
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Term
| cord blood HSC transplant |
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Definition
| readily available, more tolerant to HLA mismatch, less severe GVHD, no risk to donor, longer recovery and lower success rate |
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