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Case conference IM R3 박미나
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최o남(F/66) ADM C.C: easy bruising P.I: 3년전 고혈압 진단 후 약물 복용하고 있는 것 외 특이 병력 없었으나. 약 2주 전부터 왼쪽 손에 점상 출혈 (petechia) 발생하였으며, 이후 하지와 복부에 동일 병변 발생하여 외부 개인 병원 방문하여 혈액 검사 결과 혈소판 감소 소견 보여 자세한 검사 위해 내원 PMHx: DM/HTN/Tbc/hepatitis(-/+/-/-) Norvasc 5mg qd op Hx (-) FHx: none, PHx: alcohol(-) smoking(-)
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Review of system weight loss(-), fever sense(-), chill(-), fatigue(+)
sweating(-) rash(-), itching(-), multiple petechia and purpura(+) headache(-), gum bleeding(-), sore throat(-) cough(-), sputum(-), hemoptysis(-) chest pain(-), palpitation(+), edema(-), chest discomfort(+) A/N/V/D/C(-/-/-/-/-) , jaundice(-), melena(-) frequency(-), dysuria(-), hematuria(-) dizziness(-), tremor(-), syncope(-)
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Physical examination 120/70mmHg -96회/min-20회/min-36.3°C
Alert mentality, chronic ill appearence Multiple petechia and purpura on both extremity and abdomen Pale conjunctiva, clear sclera No neck vein engorgement, no cervical LN enlargement CBS without crackle, RHB without murmur soft and flat abdomen, no hepatosplenomegaly no abdominal T/RT, normoactive bowel sound No pph pitting edema, no CVA tenderness
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Laboratory finding CBC/DC 2490/mm3 -10.2 g/dl –30.5 % -22K/mm3
(seg: 40%, lympho 52%, mono 7%) ( reticulocyte 1.29%, MCV fl, MCH 32.4pg) PTT 40C33, PT INR 1.20 Chemistry T/D bil /0.1mg/dL AST/ALT 25/23 U/L T-prot/Alb 6.2/3.5g/dL ALP/rGT 55/26 U/L BUN/Cr /0.7mg/dL Na/K/Cl 136/3.9/101 mEq/L Glucose mg/dL LD/CK /104U/L Ca /Mg /P /uric acid 9.1/2.7/2.3/0.9 mg/dL TIBC /iron 209 / 58 ug/dL, Fol 7.2ng/mL ,Fer 64ng/mL LAP score 198
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Chest X-ray
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Impression Plan Myelodysplastic syndrome r/o aplastic anemia
r/o acute leukemia r/o idiopathic thrombocytopenic purpura Plan Abdominal sonography Bone marrow aspiration and biopsy Autoimmune marker, viral marker Antiplatelet antibody
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Abdominal sono
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Chromosome study with BM: 46XX(35)
Bone marrow exam ( ) CBC/DC /mm g/dl –30.8 % -21K/mm3 (seg: 40%) Blast : 15.2% , cellularity : 60% => Myelodysplastic syndrome refractory anemia with excess blast II, C/W Chromosome study with BM: 46XX(35)
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날짜 WBC(mm3) Hgb(g/dl) PLT(mm3) Dfferential count 6/24 2490 10.2 22K Seg40%,lymph52%,mono7% 7/1 2150 16K Seg42%,lymph47%,blast1% 7/15 2690 10.3 19K Seg37%,lymph54%,blast2% 7/26 2650 10.6 14K Seg36%,lymph56%,blast1% 8/11 3150 10.1 7K Seg30%,lymph59%,blast5%
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Bone marrow exam (2005. 8. 12) CBC/DC 2360/mm3 -9
Bone marrow exam ( ) CBC/DC /mm g/dl -77K/mm3 (seg: 40%,blast: 8%) Blast : 21.0% , cellularity : 60% => Acute myelogenous leukemia(FAB M2), most probably FISH with BM : AML1/ETO rearrangement (-) PCR : AML1/ETO rearrangement (-) Chromosome study with BM: 46XX t(2:4)(p23:q35)(5) /46XX(15)
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Plan: chemotherapy Diagnosis: Acute myelogenous leukemia(M2)
Intermediate risk group Plan: chemotherapy (Induction+consolidation#3) => AI induction for AML ( ) Ara-C 150mg(D1-7)+ Idarubicin 15mg(D1-3)
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Clinical course 날짜 WBC(mm3) Hgb(g/dl) PLT(mm3) D/C GCSF 8/18(D1) 3000
9.3 67K Seg55% 8/24(D7) 330 9.8 30K Seg34% ↑ 8/29(D11) 250 8.7 24K Seg9.2% 9/2(D16) 500 10.7 39K Seg4% 9/9(D23) 620 7.4 61K Seg42% 9/10(D24) 1580 9.7 21K Seg50% 9/12(D26) 2850 15K Seg74% 9/14(D28) 1610 8.9 13K Seg68%
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piperacillin+tazobactam D18 + amphotericin D21 hickman catheter remove
D D16 +vancomycin piperacillin+tazobactam D18 + amphotericin D21 hickman catheter remove D16 periodontitis D18 blood culture: candida albicans
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Chromosome study with BM: 46XX(35)
Bone marrow exam ( ) CBC/DC /mm g/dl -13K/mm3 (seg: 69%) Blast : 0.2% , cellularity : 30% => Acute myelogenous leukemia(FAB M2), most probably Chromosome study with BM: 46XX(35)
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FAB morphologic guideline for MDS (1982)
: heterogenous, various cytogenic abnormality, widely variable clinical outcome WHO classification of MDS (2001) Lowering threshold for defining AML from 30%->20% blasts in BM, pph blood Division of low grade categories of RA and RARS into 5 entities Subdividing RAEB into two categories depending on number of blasts in blood and marrow Removing CMML from MDS category into myelodysplastic/ myeloproliferative disease <Elimination of FAB caterogy of RAEBT> FAB category of RAEBT = WHO category of MDS related AML (clinical, biologic, genetic, prognosis)
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MDS : hematologic, pathologic, and biological heterogeneity
Expectation for survival and leukemia evolution -> reflect natural Hx -> therapeutic and management decisions The International Prognostic Scoring System (IPSS) : BM blast pecentage, cytogenetic pattern, number of cytopenia
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MDS Secondary leukemia Primary curative Tx for MDS =>allogeneic SCT
MDS with RA and RARS => lower relapse rate, >50% long term disease free survival MDS with RAEB => less favorable, higher relapse risk Secondary leukemia < ys old: standard induction therapy =>intensive post-remission therapy (including BMT) =>overall outcome is inferior to primary MDS/AML > ys old reasonable performance status=>standard induction therapy => supportive care only (median survival 3-4months)
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