Darren Low Wei Wen , Dennis Neo Wee Keong and A Senthil Kumar
A CASE OF ACUTE MYELOID LEUKEMIA PROF S SHIVAKUMAR P SENTHIL KUMAR MD PG P SENTHIL KUMAR MD PG.
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Transcript of A CASE OF ACUTE MYELOID LEUKEMIA PROF S SHIVAKUMAR P SENTHIL KUMAR MD PG P SENTHIL KUMAR MD PG.
A CASE OF ACUTE A CASE OF ACUTE MYELOID MYELOID LEUKEMIALEUKEMIA
PROF S SHIVAKUMARPROF S SHIVAKUMAR
P SENTHIL KUMAR MD PGP SENTHIL KUMAR MD PG
Rajendran Rajendran 42 yrs/ M42 yrs/ M c/oc/o
Multiple swellings in the neck and Multiple swellings in the neck and submandibular regionsubmandibular region
Fever Fever Breathlessness Breathlessness
3months3months Pain abdomenPain abdomen Loss of appetite/ Loss of wt.Loss of appetite/ Loss of wt. No cough with sputumNo cough with sputum No dysuria, rashes, jaundiceNo dysuria, rashes, jaundice Bowel and bladder - N Bowel and bladder - N
Past H/oPast H/o No DM/ HTN/ IHD/ PT/ BA/ STROKENo DM/ HTN/ IHD/ PT/ BA/ STROKE
Personal H/oPersonal H/o Smoker/ alcoholicSmoker/ alcoholic Agricultural workerAgricultural worker
Family H/oFamily H/o Nil Nil
Treatment H/oTreatment H/o Pt on ATT for PL effusion past 3 monthsPt on ATT for PL effusion past 3 months
O/EO/E ConsciousConscious OrientedOriented FebrileFebrile Pallor +Pallor + Clubbing+Clubbing+ Subconjunctival hemorrhageSubconjunctival hemorrhage Submandibular/ cervical / supraclavicular Submandibular/ cervical / supraclavicular
LN +LN + No icterus/ cyanosis/ PE/ JVP.No icterus/ cyanosis/ PE/ JVP.
RSRS BS BS Lt mamary/ infra Lt mamary/ infra
axillary/ axillary/ infrascapular areasinfrascapular areas
Stony dullnessStony dullness Chest wall movements Lt sideChest wall movements Lt side
CVSCVS S1 S2 +S1 S2 + No murmurNo murmur
ABDABD No organomegalyNo organomegaly No FFNo FF
LABLAB HemogramHemogram
HbHb - - 5.65.6 g/dl g/dl TLCTLC -- 30,10030,100/cu mm/cu mm DCDC -- blast 60%blast 60% P24 L 04 P24 L 04
Myelocyte 12Myelocyte 12 Platlets Platlets - - 20.00020.000/ cu mm/ cu mm RBC RBC - - 1.6 million/ cu mm1.6 million/ cu mm PCVPCV - - 15 %15 %
PERIPHERAL SMEARPERIPHERAL SMEAR RBC RBC -- Normochromic Normocytes Normochromic Normocytes
and few hypochromic microcytes.and few hypochromic microcytes.
-- No hemoparasites seen No hemoparasites seen
WBCWBC -- Count increased with Count increased with BLASTS BLASTS showing occasional showing occasional Auer RodsAuer Rods..
PLATLETS-PLATLETS- DiminishedDiminished
BONE MARROWBONE MARROW HypercellularHypercellular Marrow replaced by Marrow replaced by
Leukemic cells with Leukemic cells with BLAST BLAST showing showing MYELOID MONOCYTICMYELOID MONOCYTIC differentiation up to differentiation up to 70 %70 %
ERYTHROPOIESIS ERYTHROPOIESIS MEGAKARYOPOIESISMEGAKARYOPOIESIS CYTOCHEMISTRYCYTOCHEMISTRY
MPOMPO -- InconclusiveInconclusive PASPAS -- NegNeg
RFTRFT UreaUrea -- 32 mg/dl32 mg/dl CreatinineCreatinine -- 0.8 mg/dl0.8 mg/dl
Bl sugarBl sugar -- 105 mg/dl105 mg/dl LFTLFT
TBTB -- 0.7 mg/dl0.7 mg/dl DBDB -- 0.3 mg/ dl0.3 mg/ dl SGOTSGOT -- 24 Iu/L24 Iu/L SGPTSGPT -- 22 Iu/L22 Iu/L SAPSAP -- 1O5 Iu/L1O5 Iu/L
CXRCXR -- Lt pleural effusionLt pleural effusion ECGECG -- WNLWNL USG ABDUSG ABD -- Lt pleural effusionLt pleural effusion
- - LIVER/ SPLEEN LIVER/ SPLEEN normalnormal
SPUTUMSPUTUM AFBAFB -- NegNeg Grams stainGrams stain -- NegNeg Malig cellsMalig cells -- NegNeg
Pleural FluidPleural Fluid ProteinProtein -- 4.6 g/dl4.6 g/dl SugarSugar - - 84 mg/dl84 mg/dl WBCWBC -- 26002600/ cu mm / cu mm
( P 11 L 39 )( P 11 L 39 ) RBCRBC -- 30,00030,000/cu mm/cu mm SmearSmear -- +ve for MALIGNANT +ve for MALIGNANT
CELLSCELLS Grams stainGrams stain -- No organismsNo organisms AFBAFB -- NegNeg
Problems Problems
FeverFever LymphadenopathyLymphadenopathy Pleural effusionPleural effusion Subconjunctival hemorrhageSubconjunctival hemorrhage Loss of wtLoss of wt
DIAGNOSISDIAGNOSIS
ACUTE MYELOID LEUKEMIA ACUTE MYELOID LEUKEMIA – M4 WITH MALIGNANT – M4 WITH MALIGNANT PLEURAL EFFUSION PLEURAL EFFUSION
ACUTE ACUTE MYELOID MYELOID LEUKEMIALEUKEMIA
ETIIOLOGYETIIOLOGY HEREDITYHEREDITY - - Downs , Klinefelters, Downs , Klinefelters,
patau, patau, Chromosomal breakage Chromosomal breakage syndromessyndromes
RADIATIONRADIATION CHEMICALCHEMICAL - - Benzene, smoking, paint, Benzene, smoking, paint,
petroleum, pesticides.petroleum, pesticides. DRUGSDRUGS - - Alkylating agents, Topo Alkylating agents, Topo
isomerase II inhibitors, isomerase II inhibitors, Chloramphenicol and phenyl butazoneChloramphenicol and phenyl butazone. .
CLINICAL FEATURESCLINICAL FEATURES Age of onset - AdultAge of onset - Adultonset and incidence onset and incidence
increases increases with agewith age M/F M/F - 4.4 : 3- 4.4 : 3 FatigueFatigue LOW/ LOALOW/ LOA Fever with or without infectionFever with or without infection Bleeding symtomsBleeding symtoms Bone painBone pain LymphadenapathyLymphadenapathy HepatosplenomegalyHepatosplenomegaly GI, PUL, Intra cranial, Retinal hhage (APL – GI, PUL, Intra cranial, Retinal hhage (APL –
M3)M3) Chloroma ( M1 M2)Chloroma ( M1 M2) Gum hypertrophy (M4 M5 )Gum hypertrophy (M4 M5 )
Anemia with dec Retic count
WBC – mean 15,000 <5000 in 25 – 40% > 100,000 in 20% No malig cells in 5% Dysfunction +
Platlets - < 100,000 in 75%< 25000 in 25%Dysfunction +
BLOOD PARAMETERS
CLASSIFICATIONCLASSIFICATIONSS
FABFAB Based on Morphological charecteristicsBased on Morphological charecteristics BLAST cells > 30 %BLAST cells > 30 %
WHOWHO BLAST cells > 20 %BLAST cells > 20 % Based on Morphology, Molecular and CF.Based on Morphology, Molecular and CF.
FABFAB M0M0 - Minimal diff- Minimal diff M1M1 - Myeloblastic without maturation- Myeloblastic without maturation M2M2 - Myeloblastic with maturation- Myeloblastic with maturation M3M3 - Promyelocytic- Promyelocytic M4M4 - Myelomonocytic- Myelomonocytic M4E0M4E0 - Marrow eosinophilia- Marrow eosinophilia M5M5 - Monocytic- Monocytic M6M6 - Erythroleukemia- Erythroleukemia M7M7 - Megakaryoblastic- Megakaryoblastic
WHOWHO AML IAML I – – GENETIC GENETIC abnormalitiesabnormalities
t ( 8/21), inv (16), t (16/16)t ( 8/21), inv (16), t (16/16) MLL abnormalitiesMLL abnormalities APL t ( 15/ 17)APL t ( 15/ 17)
AML II-AML II- Following Following myelodysplasiamyelodysplasia/ / myeloproliferative disordersmyeloproliferative disorders
AML III-AML III- Following Following DRUGDRUG induced induced myelodysplasiamyelodysplasia
AML IVAML IV – M0 to M7 of FAB , panmyelosis, – M0 to M7 of FAB , panmyelosis, basophilic and myeloid sarcomabasophilic and myeloid sarcoma
PROGNOSISPROGNOSIS GOODGOOD
Young ageYoung age Chromosomal defects Chromosomal defects
(t8/21, inv 16, (t8/21, inv 16, t15/17)t15/17)
Rapid induction of CRRapid induction of CR Long duration of CRLong duration of CR
POORPOOR Advanced age ( > 60)Advanced age ( > 60) Chrom defects ( inv Chrom defects ( inv
3, -7 )3, -7 ) Asso med illnessAsso med illness Prolonged Prolonged
cytopeniascytopenias Prev myelodysplasiaPrev myelodysplasia Drug induced AMLDrug induced AML Delayed and short Delayed and short
duration of CRduration of CR
MANAGEMENMANAGEMENTT
REMISSION INDUCTIONREMISSION INDUCTION
POST REMISSION MANAGEMENTPOST REMISSION MANAGEMENT
REMISSION INDUCTIONREMISSION INDUCTION(7&3 REGIME)(7&3 REGIME)
CYTARABINECYTARABINE 100 – 200 mg / sq m/ day IV infusion 7 100 – 200 mg / sq m/ day IV infusion 7
daysdays
ANTHRACYCLINEANTHRACYCLINE Daunorubicin – 45- 60mg/sq m/day IV 3 Daunorubicin – 45- 60mg/sq m/day IV 3
days days IdarubicinIdarubicin - 12 mg/sq m/ day IV - 12 mg/sq m/ day IV
With or without With or without ETOPOSIDEETOPOSIDE
COMPLETE COMPLETE REMISSIONREMISSION
BloodBlood WBC >1500WBC >1500 Platlet > 1 LacPlatlet > 1 Lac RBC count not consideredRBC count not considered No BLAST cellsNo BLAST cells
BMBM Trilineage cellularity > 20%Trilineage cellularity > 20% Blast cellBlast cell < 5% < 5% NO Auer rodsNO Auer rods RT PCR/ FISH to r/o residual leukemic RT PCR/ FISH to r/o residual leukemic
cellscells
If 7 & 3 RegimeIf 7 & 3 Regime 50% - CR50% - CR
start 5 & 2 Regimestart 5 & 2 Regime 25% - CR25% - CR 25% - NO CR25% - NO CR
NO CRNO CR 50% - Drug Resistance50% - Drug Resistance 50% - Fatal complications of Drugs50% - Fatal complications of Drugs
POST REMISSION POST REMISSION MANAGEMENTMANAGEMENT
HIGH DOSE HIGH DOSE CYTARABINECYTARABINE 3-4 cycles3-4 cycles 3 g/sq m / day bd 3 g/sq m / day bd
on 1,3 & 5 th dayon 1,3 & 5 th day
STEM CELL TxSTEM CELL Tx AutologousAutologous allogenicallogenic
LESS INTENSIVE LESS INTENSIVE CHEMOTHERAPYCHEMOTHERAPY
STEM CELL TxSTEM CELL Tx Non myeloablative Non myeloablative
AllogenicAllogenic
AGE < 65 yrs AGE > 65 YRS
TREATMENT OF APL TREATMENT OF APL ( M3)( M3)
TRETINOINTRETINOIN( Maturation of blast cells,No ( Maturation of blast cells,No DIC)DIC) 45 mg/ sqm/day Untill 45 mg/ sqm/day Untill
remissionremission ANTHRACYCLINESANTHRACYCLINES
Maintanance therapyMaintanance therapy Tretnoin or chemotherapyTretnoin or chemotherapy
RELAPSERELAPSE
STEM CELL TX