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Hematology/Oncology Flashcards

1
Q

Heparin to Warfarin bridge

A

Prot C and S w/ shorter t1/2
II, VII, IX, X longer t1/2

Transient paradoxic hypercoag before anticoag

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2
Q

Heparin vs LMWH

A

Heparin: activ antithromb; ↑PTT
Antidote: protamine sulfate

LMWH: esp inhib Xa; No eff on PTT
No eff of prot sulf

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3
Q

Warfarin

A

Inhib synth vitK-dep coag fact
↑PT
Reversal: rapid w/ FFP; slow vitK
! teratogen

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4
Q

tPA

A

Help conv plasminog to plasmin (breaks fibrin)
↑PT; ↑PTT
If toxicity, give aminocap acid

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5
Q

Factor Xa inhibitors

Factor IIa inhibitors

A 
No monitoring (PT, PTT)
No reversal agent
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6
Q

Hemophilia (PE)

A 
X-linked; A=VIII; B=IX; C=XI
Rare acquir (autoimm, post-part)

Young boy; spont hge tissue/joint (intracereb/GI/…)
Hge after surg, trauma, dental proced
If not ttt, arthropathy/joint destruction

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7
Q

Hemophilia (dg, ttt)

A

Dg: ↑PTT; normal PT/BT
Mixing study (#1): correct PTT
Specific fact assay (accur)

ttt: if sever bld or fact ≤1% (immediat transfu missing fact; cryoprecip)
If not sev and >1% (DDAVP releases VIII from endoth C)

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8
Q

von Willebrand disease (PE)

A

AD; #1 inherit bld ds
Defect or deficiency in vWF + ↓VIII
Types: 1 (mild-mod defic); 2 (qualit defect); 3 (No funct)

Child; recurr + prolong mucosal bld + bld after proced
⊕ fam Hx; sympt worse w/ ASA

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9
Q

von Willebrand disease (dg, ttt)

A

Dg: ↑BT; may ↑PTT (by ↓VIII); normal PT/PC
Accur: Ristocetin cofact assay + vWF Ag level

ttt: DDAVP (mild-mod); OCPs (if menorrhg); Avoid ASA and plt fct inhib

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10
Q

Bernard-Soulier syndrome

Glanzmann thrombasthenia

A

B-S: def GpIb (binds plt to endoth vWF)

G-T: def GpIIb/IIIa (binds 2 plts by fibrinogen)

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11
Q

ADP receptor inhibitors

GpIIb/IIIa inhibitors

A

ADP-R inh: clopidog/prasug/ticlopidine
No externaliz of GpIIb/IIIa

GpIIb/IIIa inh: abciximab/eptifibatide/tirofiban

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12
Q

Hypercoagulable states (etiologies)

A

Thrombophilias or prothrombotic states
Genet, acquir, physiol
3 main causes but same PE

  • F.V Leiden: f.V resist to inactiv by activ prot C (#1 hered thromboemb ds)
  • HIT: immuno react to heparin; Ab activates plt; bld clots + rapid ↓50% PC
  • APS: ass w/ SLE/RA; art+ven thrombi; spont abort
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13
Q

Hypercoagulable states (PE, dg)

A

Recurr thromb (DVT, PE, art thromb, MI, stroke, abort)
HIT: hospit ptt on anticoag then ↓PC
F.V.L: young caucas + pers/fam Hx thromb
APS: middle-age women + recurr miscarr/thromb

Dg: r/o acqu causes before hered workup
Hered: 2 abNl values while asympt/unttt + similar values in 2 fam memb
HIT: PF4 Ab + serotonin release assay
APS: lupus anticoag + anticardiol Ab

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14
Q

Hypercoagulable states (ttt)

A

DVT/PE: heparin then 3-6mo warfarin (1st epis); 6-12mo (2nd); lifelong (next)
If anticoag CI (hge, sev HTN, recent trauma) or refract: IVC filter

HIT: !d/c hep; start argatroban/lepirudin
F.V.L: avoid OCPs

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15
Q

Protein C or S deficiency (PE)

A

Skin or tissue necrosis after Warfarin

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16
Q

Disseminates intravascular coagulation (RF)

A

Acquir; deposit of fibrin in Vx → thromb + end-org dam
Deplet coag fact + plts → bleed

Ass w/ obstetr complic; sepsis; neopl; ApML; pancreatitis; massiv trauma; drug react; ARDS; …

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17
Q

Disseminates intravascular coagulation (PE, dg, ttt)

A

Acute: bld from venipunct/in org; ecchym, petech
Chronic: bruis, mucos bld; thrombophleb; renal dysfct; neuro sympt

Dg: clinic+lab; #dg liv ds (but DIC w/ VIII is depressed)
ttt: underl cause; transfuse RBCs/plts/FFP; manage shock

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18
Q

Thrombotic thrombocytopenic purpura (PE)

A

Inherit or acquir (after inf)

vWF-cleav enz def (ADAMTS-13 def) → large vWF → plts microthrombi → end-org dam
RBCs fragm by microT → hemolysis

TTP if 3/5⊕: ↓PC; microang hemol anemia; neuro chang; impair renal fct; fever

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19
Q

Thrombotic thrombocytopenic purpura (dg, ttt)

A

Dg: schistoc + ↓PC + ↑creat
HUS: simil to TTP; child w/ EcoliO157H7; renal fail, hemol anemia, ↓PC, No neuro sympt

ttt: #1 plasma exchange; steroids to ↓thromb
CI for plt transfu

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20
Q

3 causes of microangiopathic hemolytic anemia

A

HUS
TTP
DIC

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21
Q

Idiopathic thrombocytopenic purpura (PE, etiologies)

A

IgG against plts → plt/Ab complex destr by spleen → ↑plt prod by BM
#1 immuno ds in middle-age women
Ass w/ leukemia/lymphoma/SLE/HIV/HCV

Acute (aft viral inf; 2-6yo) or chronic (20-40yo, esp women)
Asympt; minor mucocut bld, easy bruis, petech, hematuria, melena

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22
Q

Idiopathic thrombocytopenic purpura (dg, ttt)

A 
Dg: of exclusion; r/o causes of thrombocytop then labs (Nl RBC morpho)
BM biopsy (only if atyp or >60yo): ↑megakaryoc

ttt: No ttt (if plt >30K + no bld)
CS or IVIG: if plt <30K or bleed/sympt
If refract: splenectomy +/- rituximab +/- TPO recept agonist

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23
Q

9 #dg of thrombocytopenia

A 
HIT
HUS
TTP
ITP
Medications
Splenomegaly
Hereditary (Wiskott-Aldrich sd)
Chemotherapy
Other (malignancy)
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24
Q

Iron deficiency anemia (etiologies, PE)

A

Normocytic then microcytic An
↑demand (pgncy, growth) or ↓iron (malnutr/malabs, bleed)
Esp toddlers, ado girls, women of childB age

Fatig, dyspn, tachycard, pica; asympt if slowly
Glossitis; conj pallor; cheilosis; koilonychia

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25
Iron deficiency anemia (dg, ttt)
Dg: CBC; ↓iron, ↑TIBC/transferrin, ↓ferritin, ↑RDW; periph bld smear (hypochromic+microc w/ ↓reticulocyte count) ttt: oral iron x4-6mo (!N, abdo pain, const/diarr) CI antacids w/ iron IV iron dextran (! anaphylaxis)
26
Anemia of chronic inflammation/disease
Chronic infl, inf, malign, RA, SLE Body hides iron (limit bact prolif) Normocytic then microcytic An Dg: ↓iron, ↓TIBC/transf, ↑ferritin
27
Sideroblastic anemia
Microcytic An Def in heme metab (hered; alcoh, lead poison, chloramph, INH, malign) Dg: ↑iron; basoph stippl (periph smear); ringed sideroblasts (BM)
28
Megaloblastic anemia (etiologies, PE)
Macrocytic anemia Impaired DNA synth (vitB9 def, vitB12 def, MTX, 6-MP) -B12: intest malabs (Crohn/celiac/gastrect/panc insuff), pernicious An, vegetarian, Diph latum -B9: alcoh, ↓diet folate, malabs, phenytoin Fatig, pallor, diarr, ↓appet, headac B12: demyelin (subacute comb degen of cord)
29
Megaloblastic anemia (dg, ttt)
Dg: CBC, smear w/ hypersegm neutroph (≥6 lobes) MMA+homocysteine (↑both in B12 but MMA Nl in B9) BM (giant+hyperseg neutro); Schilling test (for B12) ttt: underl cause
30
Hemolytic anemia (etiologies)
Normocytic An BM unable to compens for ↑destruc - Intrinsic: memb def (spheroc), enz def (G6PD def), HbC, SCD, parox noct hemoglobinuria - Extrinsic: autoimm, microangiop (HUS/TTP/DIC), macroangiop (mechan heart valv), inf (malaria), hypersplenism
31
Hemolytic anemia (PE, dg, ttt)
Pallor, fatig, tachycard, tachypn Dg: jaund, ↑ind bili, ↑reticuloc, ↑LDH, ↓haptogl ttt: underl cause; CS in immuno; iron to replace loss If severe, splenectomy or transfusions
32
G6PD deficiency
X-linked; hemolyt An after oxidant stress Episod dark urin + jaund Trigger: sulfa drug, antimalar, inf, fava beans, metab acid Dg: CBC, smear (bite cell, Heinz); G6PD level after 1mo ttt: avoid trigg
33
Paroxysmal nocturnal hemoglobinuria
Def in GPI-anchor molec → No CD55/CD59 on RBCs → No protec ag complem-med hemolysis Dg: An, dark urin, venous thromb, abdo pain; flow cytom w/o CD55/59 (accur)
34
Hereditary spherocytosis
AD def in spectrin/ankyrin Spherical RBCs destroyed by spleen (extravasc hemol) Dg: CBC + smear; osmotic fragil test (accur) ttt: splenectomy (+pneumo/meningo/haemo vacc before)
35
Sickle cell disease (PE, complications)
AR; beta chain has Val (not Glu) Trigg: dehydr, deoxyg, high altit, inf, cold Dactylitis (child); An, jaund, pigm cholelith, ↑CO (CHF), delayed growth - Vaso-occlusion: isch org dam, splen infarc, acute chest sd, avasc hip necr, priapism, stroke - Splenic sequestr: hypovol; Nl/↑ plts - Aplastic crisis: by parvoB19; ↓plts
36
Sickle cell disease (dg, ttt)
Dg: CBC w/ smear (sickle C, Howell-Jolly); Hb electroph (accur) ttt: hydroxyurea (↑fetal Hb); if ineff, chronic transfu Cholecystec if lithias; folate suppl; vaccines; penicill in <5yo -VasoO crisis: pain manag, O2, IV hydr, AB -Acute chest sd: aggr hydr, spirom, transfu
37
Autoimmune hemolytic anemia
AutoAb: warm (IgG ass w/ SLE, CLL, drugs) and cold (IgM ass w/ EBV, mycopl) Dg: direct Coombs ttt: no ttt (mild); steroids (severe warm); avoid cold+/-Rituximab (severe cold)
38
Aplastic anemia
BM cells destruc → No bld cells Hered (Fanconi An); autoimm; viral (HIV, parvoB19); toxins; radiation; drugs Pancytop: pallor, weak, ↑inf, petech, bruis, bleed Dg: CBC; BM biopsy w/ hypocell + fat (accur) ttt: transfu + SCT; ttt inf aggress; if no donor, immunosupress to prevent autoimm BM destr
39
Thalassemias (etiologies, PE)
Heredit; ↓/No prod of nl globin Alpha: mut of ≥1 of 4 genes for alpha-Hb Beta: mut of ≥1 of 2 genes for beta-Hb Africa, middle east, Asia PE depends on nb of genes; asympt; fatig w/ microcyt An + Nl iron studies
40
Thalassemias (dg, ttt)
Dg: Hb electroph and DNA studies ttt: most, no ttt Beta-T major (0/2 beta) and HbH ds (1/4 alpha): transfu-dep + iron chelator
41
Polycythemias (etiologies, PE)
Erythrocytosis: -absolute (↑RBC prod: hypoxia, lung ds, smok, altit; neopl, EPOtumor; PCV) or relative (↓plasma, hypovol and hemoconcentr) ↑Ht; ↓tissu bld flow+O2; ↑card work Hyperviscosity sd: easy bleed/bruis; blurred vis*; neuro def; prurit aft warm bath; thromb; CHF; HSM
42
Polycythemias (dg, ttt)
Dg relative erythro: ↑Ht, ↑/Nl EPO, O2 often ↓ compared to PCV ttt relative eryth: underl cause; phlebotomy (↓sympt)
43
Polycythemia vera
Clonal prolif of a pluripotent marrow stem cell All marrow cell lines ↑, esp RBCs Older ptt Dg: CBC, ↑RBCs, ↑WBCs, ↑plts; ↓EPO, Nl O2, ↑Ht >60% JAK2 mutation⊕ ttt: phlebotomy + aspirin (↓sympt + prev thromb); hydroxyurea + IFN (↓cells)
44
Febrile nonhemolytic transfusion reaction
Cytokine format* in storage Fever/chill, malaise; aft 1-6h ttt: stop transfu + give acetamino
45
Allergic transfusion reaction
Ab ag donor prot; usual aft plasma-contain prod Prominent urticaria ttt: antihist; if severe, stop transfu + give epinephrine
46
Hemolytic transfusion reaction
Preformed (acute) or formed (delayed) Ab ag donor RBCs ABO mismatch; Rh and Duffy Ag Fever/chilld, N, flush, burn at IV site, tachycard, hypoTN direcly aft transfu ttt: stop transfu immediately + vigorous IV fluids + monitor urine
47
Porphyria (etiologies, dg, ttt)
Inherit ds, abNl heme prod Accumul of porphyrins Acute intermitt porph; erythropoietic porph; porphyria cutanea tarda Dg: urin+plasm porphyrin lev; mutat*/enz analysis (accur) ttt: avoid trigg; sympt ttt in attacks + high dose glucose
48
Porphyria (PE)
Photodermatitis, neuropsych, visceral sympt (colic abdo pain), seizures Tachycard, skin erythema, blisters, areflexia -Erythrop porph: hemolyt An, urine pink/black Attacks ass w/ ↑heme synthesis (fasting, chemic expo, alcoh, barbit, OCPs) -Acute interm porph: abdo pain, red urin, polyneurop, psychol disturb; by CYP450 inducers/alcoh/fasting
49
Acute leukemias (PE)
Clonal disord of blasts → unregul WBCs in BM BM overwhelmed → anemia/fatig, inf, hge/petech/purpu DIC (AML); bone pain (ALL) Child + adult; rapid onset+progr Leukemic infiltr: HSM; swoll/bld gums; skin; CNS
50
Acute leukemias (dg)
Dg: CBC w/ smear (AML: myeloblasts; ALL: lymphoblasts); BM biopsy (accur) + flow cytom AML: Auer rods, myeloperoxidase ⊕ ↑WBCs but dysfct or neutropenia If WBCs >100K, leukostasis (vaso-occl)
51
Acute leukemias (ttt)
AML/ALL: chemotherapy; if unfavor genetics, BMT Leukostasis sd: hydroxyu +/- leukapheresis Poor pg: -ALL (<1 or >10yo; WBC>50K; t(9:22); CNS) -AML (>60yo; ↑LDH; complex karyotype)
52
CLL (PE)
``` Dysfct lymphocytes in BM/bld/LN/spleen/liv Older adults (65yo; M=2F) ``` Asympt; fatig, malaise, inf Lymphadenopathy; SMG
53
CLL (dg)
Dg: CBC+diff; smear (lymphocytosis + smudge cells) Flow cytom w/ CD5 on B cells (accur) Granulocytop, anem, thrombocytop, hypogammaglobulin Rarely BM done
54
CLL (ttt)
Palliative; ttt given when symptomatic (recurr inf/sev LNpathy/SMG/An/thrombocytop) Chemoth: fludarabine + chlorambucil No cure; ds-free intervals w/ adequate ttt
55
CML (PE)
Clonal prolif myeloid progen cells + BCR-ABL transloc Philad chrm t(9,22); middle-aged ptt ↑granulocytes and basophils; maybe ↑RBCs and plts Asympt; anem, SMG w/ LUQ pain, anorex/fev/chil/↓weight 3 phases: chronic, accelerated, blast crisis
56
CML (dg)
Philad chrm (accur) by PCR/FISH CBC (WBCs >100K to 500K); ↓LAP ↑ LDH, uric ac, B12 #dg: leukemoid reaction (↑neutro w/ left shift; ↑LAP)
57
CML (ttt)
Chronic: imatinib (Gleevec) Young ptt: allogen SCT Blast crisis: chemotherapy; dasatinib + SCT
58
Leukemia based on age (~)
<13yo: ALL 13-40yo: AML 40-60yo: CML >60yo: CLL
59
Hairy cell leukemia (PE)
Ds of well-diff B lymphoc Older men ``` Pancytop, BM infiltr, SMG Weak, fatig, petec, bruis, ing, abdo pain, ↓weight Rare LNpathy (#CLL) ```
60
Hairy cell leukemia (dg, ttt)
``` Dg: CBC w/ smear (hairy C w/ TRAP stain) Flow cytom (accur) ``` ttt: cladribine; splenectomy; IFN-alpha
61
Non-Hodgkin lymphoma (PE)
#1 hematopoietic neopl Group of mature B (85%) and T cell neopl >50yo; aggress in child ``` Painless periph LNpathy + extranodal (#HL) B sympt (fev, night swt, ↓weight) ```
62
Non-Hodgkin lymphoma (dg, ttt)
Dg: excisional LN biopsy; Ann Arbor staging CSF exam if HIV, neuro sympt, 1*CNS lymphoma ttt: based on histopath (not stage) → radioth, chemoth, x2 Low grade: palliative High-grade: aggress ttt
63
4 types of B cell NHL
- Follic: adult; low grd; painless wax/wan LN; radioth if local - DLBC: middle-age/elder; interm grd; single rapid grw mass; R-CHOP ttt - Burkitt: child/ado; high grd; starry sky; jaw/Afr; abdo/USA; EBV; t(8;14); aggress ttt - Mantle cell: elder men; rare
64
2 types of T cell NHL
- Adult T-cell: adult; high grd; progr to ALL; cutan les*; by HTLV; ass w/ IVDA - Mycosis fungoides: adult; skin; eczema-like + prurit; cerebriform cells; progr to Sezary sd (leukemia)
65
Tumor lysis syndrome
Hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalemia; renal insuff Prevent: gd hydrat + allopur or rasbur
66
Hodgkin lymphoma (PE)
Predomin B-cell; ass w/ EBV Bimodal age: 30yo (nodular sclerosing type); 60yo (lymphocyte-depleted type) Above diaphragm (cervic LNpathy); if under D = dissem Systemic B symptoms, prurit, HSMG Rare: Pel-Ebstein fevers and alcohol-induced pain
67
Hodgkin lymphoma (dg, ttt, pg)
Dg: excisional LN biopsy (Reed-Sternb C = giant B C) Stage: nb of nodes, if B sympt, if both sides of diaph ttt: stage-dep; chemoth and radiation 5-yr pg: stage I/II 90%; III 84%; IV 65% Lymphocyte-predomin HL: best pg
68
Multiple myeloma (RF, PE)
Clonal prolif of plasma C w/ excess monoclon Ig (A/G) or Ig fragm (kappa/lambda light chains) Elder RF: radiation; MGUS; petrolium; pesticides Bone pain/pathol fx; anem; hyperCa; renal abnl Inf (monoclon Ig ineffect); ↑monoclon M prot
69
Multiple myeloma (dg, ttt)
Dg: serum prot electroph (IgG/IgA spike) BM biop (>10% monoclon CD138+ plasma cells) CBC w/ smear (rouleaux); ↑prot:album Urinal (Bence Jones prot); skeletal survey ttt: autolog BMT (if <70yo); melphalan+prednis (if >70yo)
70
Cryoglobulinemia vs cold agglutinins
IgM Ab Cryog: in HCV, joint pain, renal involv Cold agg: in EBV, mycoplasma, Waldenstrom macroglob; numbness on cold expo,
71
Waldenstrom macroglobulinemia (PE)
B cells; malign monoclonal gammopathy ↑IgM; hyperviscosity sd Coag abNl, cryoglobul, cold agglutinin ds, amyloidosis Lethargy, ↓weight, Raynaud phen, neuro prob, organomeg, org dysfct MGUS is precursor
72
Waldenstrom macroglobulinemia (dg, ttt)
Dg: BM biopsy/aspirate (abNl plasma cells w/ Dutcher bodies); prot electroph+immunofixation; ↑ESR, uric ac, LDH, ALP ttt: chemo; plasmapheresis
73
Amyloidosis (PE)
Extracell deposit of amyloid prot fibrils Elder PE: dep on type, amount, distrib (esp kidney, heart, liv) Alzheimer ds: deposit just in brain
74
Amyloidosis (dg, ttt)
Dg: tissue biopsy w/ Congo red (apple green birefr) (accur) Immunohistochemistry ttt: 1* amyl: experimental chemoth + autologous SCT 2* amyl: underl cause
75
5 types of amyloidosis
- AL: monoclonal light-chain fragm; ass w/ MM + Walden macroglob - AA: amyloid A; ass w/ chron infl ds/inf/neopl - Dialysis related: Beta2-microglobulin, pt on long-term dialysis - Heritable: deposit of abnl gene prod (transthyretin) - Senile-systemic: deposit of Nl transthyretin
76
Neutropenia (etiologies)
ANC <1500/mm3 Acquired or intrinsic ↓prod; migrat* from Vx; ↑destruct; utilization Adult: #1 by inf; #2 by drugs; leuk/lymph; aplast anem; B12/B9 def
77
Neutropenia (PE)
↑R of inf Acute: S aureus; Pseudom; E coli; Proteus; Klebs sepsis Chron + AI: recurr sinusitis; stomatitis; gingivitis; perirectal ing +/- SMG
78
Neutropenia (dg)
Dg: CBC w/ smear; Hx (drug/toxin/ing/AI/neopl) Follow w/ CBC+ANC If ↓plts/RBCs, BM biopsy/aspirate Immuno eval; ANA lev; collagen vasc ds
79
Neutropenia (ttt)
ttt inf Neutropenic fever = emergency; broad AB (cefepime) +/- antifungal G-CSF shortens durat* of neutrop Rare: IVIG; allogen BMT
80
Eosinophilia (etiologies, PE)
AEC >350/mm3 Trigg: overprod of cytokines/chemokines 1* or 2* to allergy, parasitic inf Hx travel, med, atopic, leuk/lymph, diet Hypereosino sd: fever, anem, cardiac findings), other org
81
Eosinophilia (dg, ttt)
Dg: CBC w/ diff CSF w/ eosino: drug react* or inf (coccidio, helminth) Hematuria w/ eosino: schistosom ttt: cardiac find, eos >100K, drug react w/ systemic sympt → early dg + steroids + stop off agent
82
3 types of tissue transplantation
- Autologous: ptt to him/herself - Allogenic: donor to genet diff ptt; ABO and HLA match; but match + immsuppr and still get rejection - Syngeneic: btw/ identical twins (genet identical)
83
3 types of solid organ transplant rejection
-Hyperacute: min; preform Ab; Vx thrombi; tissu isch Prevent (check ABO); ttt (cytotoxic agent) - Acute: 5d-3mo; T-cell med; tissu destr (↑GGT/ALP/LDH/ BUN/creat); No prevent; confirm w/ biopsy then ttt (CS, OKT3, tacrolimus, MMF) - Chronic: mos-ys; imm react* to fibrosis; gradual loss of fct; No prevent; No ttt (biopsy to r/o treatable acute react*)
84
GVHD
Specif to allogeneic BMT Donor T cells attack host tissues Acute (<100d) or chronic (>100d) Esp skin, liv, GI (by minor histocomp Ag) Skin changes, cholest liv dysfct, obstr lung ds, GI probl ttt: high-dose CS
85
Typical posttransplant immunosuppression and infection prophylaxis
Imm: prednisone, MMF, tacrolimus Proph: TMP-SMX, ganciclovir, ketoconazole

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