IM hemat ~anemia

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Author:
TerryZ
ID:
249812
Filename:
IM hemat ~anemia
Updated:
2013-12-06 07:05:28
Tags:
internal medicine IM hematologic hemat Leukemia ALL CLL AML CML lymphoma Hodgkins multiple myeloma MM
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IM
Description:
Internal medicine - hematologic other than anemia
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  1. Leukemia - general
    • Def: neopl prolif of abnl WBCs; x nl cells
    • Cls: WBC type
    •         myelogenous: granulo/monocytes
    •         lymphoblastic: lymphocytes
    •       WBC maturity
    •         acute: rapid progr, immature cells
    •         chron: slow progr, mature cells
  2. AML - facts
    • Who: Adults (80% of adult leukemia)
    • RF: rad, myeloprolif, Down's, chemo
    • Resp: <ALL
    • APL: t(15:17), pancytopenia
    •    tx: ATRA + chemo STAT
  3. ALL - facts
    • What: neopl of early L precurs; -blasts
    • Who: MC malign of <15yo in US
    • Prog: most responsive to tx
    • Poor: <2, >9, WBC>10^5, cns involv
    • /CNS RF: B-cell, /LDH, rapid cell proliph
  4. AML/ALL S/Sx
    • anemia: fatig, palor, DOE, F, jt/bone P
    • /infect: PNA, UTI, cellul, pharyng, esoph
    • Bleed: TCP-epistax,punct,petech,ecchy
    •      plt<20 - oral/GI hemorrh
    • Organ: spleno/hepatomegaly,lymphaden
    • CNS: diffuse/focal (mening, seiz)
    • ALL: testicular,ant mediast mass(T-cell)
    • AML: skin nodules, soft tissue masses
  5. ALL/AML - Dx
    • WBC: 1K-100K;//blast;anem,G/TCP,Lyte
    • Bx: marrow-for dx (blasts repl marrow)
    • Auer rods: AML only (in malig cells)
    • /LDH, uric acid: .
  6. ALL/AML - Tx
    • Emerg: blood cx, abx; ?blood/plt transf
    • Chemo: aggr,combo,/dose wks;mo-yrs
    • ALL: 75%child rem,surv15yr+,50%cure
    • AML: BMT best chance remission/cure
  7. CLL - facts
    • MC: leukemia 50+, /CLL 60+, U.S
    • Def: monoclon prolif abnl L; xAb-plasma
    • Prog: least aggressive leukemia
  8. CLL - S/Sx
    • Asymp: @ dx; routine CBC
    • LN: painless lymphadenopathy
    • Splenomegaly: .
    • Infect: freq skin/resp
    • Adv: fatig, wt, palor, rash, bruis, boneP, bleed, abd P
  9. CLL - Dx
    • WBC: 50-200K
    • Cells: anemia, TCP, NP
    • Smear: WBCsMature/small;smudgeCells
    • Flow cytom: MspikeB-cellsCD5,19,20,23+
    • Bx: marrow-infiltr (?)
  10. CLL - Tx
    • Chemo: sx-atic, inf; -survival
    • Observe: until sxs develop
    • Prog: dep on #LNs, +/- anem/TCP
  11. CML - facts
    • D: neopl, clonal prolif myeloid stem cells
    • Occ: >40yo, ^50-60; MC myeloprolif
    • Crs: C-course/yrs -> A-blast crisis
    • Gen: Phily chrom(90%)}actv tyroKinase
    •        ~Phily} surv, tx resp
  12. CML - S/Sx
    • MC: asympt, 85% chronic phase (CBC)
    • Const: F, night sweats, anorex, wt
    • Penias: recurInf, bruise/bld, ncnc anem
    • Org: SplenMeg(LUQP),hepMeg,lymphAd
    • Adv: accelerated/blast
  13. CML - Dx
    • //WBC: 50-200K, leftShift}granulocytes
    • Few: blasts, promyelocytes
    • Eosinophilia: .
    • Smear: myel, metamyel, bands, sego
    • Alkϕ: activity in leukocytes
    • Thrombocytosis: .
    • Bx: leukemic cells in marrow
    • /Uric a., LDH: .
    • FISH: for bcr-abl gene
    • rtPCR: Philly chrom
  14. CML - Tx
    • Imatinib: PO TK inhib} dysfnl bcr-abl
    • Mon: CBC/1-3w; bx/6m; bcr-abl&bx/1y
    • Prog: in blast crisis = poor
    • Stem cell Xplant: young, resist TKI,
    •        fail imatinib; blast-crisis/advanced
  15. Hodgkin's lymphoma - age
    Bimodal: 15-30, >50
  16. Hodgkin's lymphoma - subtypes
    • Lymphocyte predom: 5%, RS, /B
    • Nodular scler: 70%, >W, collag env RS
    • Mixed cell: 25% /RS, pleomorph
    • Lymphocyte deplet'n: <1%, prog
  17. Hodgkin's lymphoma/NHL - Staging
    • Basis: PE, CT, bone marrow bx
    • Ann Arbor Stages
    • I: single LN
    • II: 2+LN, same side diaphragm
    • III: both sides diaphragm
    • IV: Extralymph sites
    •    A: No sxs
    •    B: F, wt loss, night sweats
  18. Hodgkin's lymphoma - S/Sx
    • MC: painless lymphadenopathy
    • LNs: SClav, cerv, axil, mediast
    • B sxs: F, nt sweats, wt loss
    • Pruritis: worse at night, no relief
    • Cough: 2/2 mediast LN
    • F, intermit: Pel-Ebstein 40-40.5/7-10d
  19. Hodgkin's lymphoma - Dx
    • LN bx: Reed Sternberg cells
    • Inflam infilt: plasma, eos, blasts, T&B
    • CXR/CT: LN involvement
    • Marrow bx: .
    • Lab: /L, /E, /ESR
  20. Hodgkin's lymphoma - Tx
    • ABVD: adria/bleomycin,vinblast,dacarbaz
    • IFRT: involved field radiation tx
    • I & II: ABVD x2-4, IVRT OR ABVD x6
    • III&IV: ABVD + IVRT (more cycles)
    • Stem Cell: transplant
  21. Non-Hodgkin's lymphoma - facts
    • B/T cell: malign xform & growth(B 85%)
    • Prog: LN->blood/marrow
    • MC: primary tumor in GI tract
    • Occ: 2x HL
  22. Non-Hodgkin's lymphoma - RFs
    • HIV/AIDS
    • Immunosuppression
    • EBV, HTLV-1, H. pylori
    • AI dz (Hashimotos, Sjogrens -> MALT)
  23. Non-Hodgkin's lymphoma - classif'n
    • Indolent/LG: MC sm lymphocytic lymphoma, related  to CLL, follicular, cleaved, P-less peripheral LAP
    • Intermed: diffuse, L-cell; locally invasive, large extranodal mass
    • Hi-grade: lymphobl L->T-cell, aggres/fast
    •               Burkitt's L->B-cell, African, EBV
    • Misc Ls: Mycosis fungioids, eczemoid skin lesions, cribriform-shaped Lcytes
  24. Non-Hodgkin's lymphoma - S/Sx
    • LAD: P-less, firm, mobile, rapid, SCA
    • B-sxs: <HL
    • Abd: HSM, abd P, fullness
    • Marrow: recur inf, anem/TCP
    • Misc: SVC obstr,resp,boneP,skin lesions
  25. Non-Hodgkin's lymphoma - Dx
    • LN bx: >1cm >4wks ~infect
    • CXR: hilar/mediastinal LAP
    • CT: extent spread, resp to tx
    • Labs: LDH, B2 microglob
    • /alkΦ: bone/liver involv
    • /LFT, bili: liver involv
    • CBC, Lytes, renal fn, marrow bx
  26. Non-Hodgkin's lymphoma - Tx
    • Indolent: ~cure, 5yr Surv 75%
    •              observ, chemo, rad
    • Interm/high: ?cure w/ agress tx,
    •              <complete -> <2yrs
    • Aggressive: combo chemo + rad
    •   cyclophos, doxy, vincrist, predn, ritux
    • Xplant: bone marrow,stem cell(relapsed)
    • Low-grade: rare cure: surv 5-7 yrs
    • Int-grade: 50%cure aggres surv 2yrs
    • hi-grade: 70%cure aggress else  2mo
  27. Non-Hodgkin's lymphoma - Prog
    • Internat'l Progn Ndx
    • Points:       1           0
    • Age:       >60       <60
    • LDH:      elevated   nml
    • ECOG:     2-4         0-1
    • AAS:      III-IV       I-II
    • #xNode:   1+          0
    • ------------------------------
    • Risk  Points   5-yr survival
    • Low    0-1         73%
    • Int     2, 3     51%, 43%
    • High    4-5        26%
  28. MM - facts
    • Def: neoplast prolif plasma->IgG/A
    • Occ: >50, AA 2x
    • Prog: marrow repl w/malig plasm}penia
  29. MM - S/Sx
    • Skel: bone P 2/2 osteolytic les'ns, fxs,
    •  vert collapse (low back, ribs, jaw) short
    • Anem: NN {infiltration & renal fail
    • Renal fail: Bence Jones prot casts, HCa
    • Recur inf: \nl Ig. 70% die inf(lung/UTI)
    • Cord compr: {plasmacytoma, fx
    •    med emerg->MRI + steroids
    • CRAB: /Ca, RF, Anem, Bone P
  30. MM - Dx
    • Electrophoresis: S/U prot - M-prot spike
    • XR: bone lesions
    • marrow bx: 10%+ abnl cells
    • HCa: bone destruction
    • /T prot: HGlob
    • Periph smear: rouleaux
    • //ESR: . 
    • //free lt chains: Urine,Bense Jones prot
    • Penia: Leuko, TCP, anemia
    • /Cr: .
  31. MM - Tx
    • HCT: autologous hematopoietic cell xfer
    • Chemo: lenalidomide, dexamethasone
    • Rad: no resp to chemo, disabling P

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