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A case study of a 42-year-old male presenting with multiple symptoms including neck swellings, fever, and weight loss, diagnosed with Acute Myeloid Leukemia (AML) M4 with malignant pleural effusion. The text details etiology, clinical features, blood parameters, classifications, prognosis, and management strategies in AML.
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A CASE OF ACUTE MYELOID LEUKEMIA PROF S SHIVAKUMAR P SENTHIL KUMAR MD PG
Rajendran • 42 yrs/ M • c/o • Multiple swellings in the neck and submandibular region • Fever • Breathlessness 3months • Pain abdomen • Loss of appetite/ Loss of wt. • No cough with sputum • No dysuria, rashes, jaundice • Bowel and bladder - N
Past H/o • No DM/ HTN/ IHD/ PT/ BA/ STROKE • Personal H/o • Smoker/ alcoholic • Agricultural worker • Family H/o • Nil • Treatment H/o • Pt on ATT for PL effusion past 3 months
O/E • Conscious • Oriented • Febrile • Pallor + • Clubbing+ • Subconjunctival hemorrhage • Submandibular/ cervical / supraclavicular LN + • No icterus/ cyanosis/ PE/ JVP.
RS • BS Lt mamary/ infra axillary/ infrascapular areas • Stony dullness • Chest wall movements Lt side • CVS • S1 S2 + • No murmur • ABD • No organomegaly • No FF
LAB • Hemogram • Hb - 5.6 g/dl • TLC - 30,100/cu mm • DC - blast 60% P24 L 04 Myelocyte 12 • Platlets - 20.000/ cu mm • RBC - 1.6 million/ cu mm • PCV - 15 %
PERIPHERAL SMEAR • RBC - Normochromic Normocytes and few hypochromic microcytes. - No hemoparasites seen • WBC - Count increased with BLASTS showing occasional Auer Rods. • PLATLETS- Diminished
BONE MARROW • Hypercellular Marrow replaced by Leukemic cells with BLAST showing MYELOID MONOCYTIC differentiation up to 70 % • ERYTHROPOIESIS • MEGAKARYOPOIESIS • CYTOCHEMISTRY • MPO - Inconclusive • PAS - Neg
RFT • Urea - 32 mg/dl • Creatinine - 0.8 mg/dl • Bl sugar - 105 mg/dl • LFT • TB - 0.7 mg/dl • DB - 0.3 mg/ dl • SGOT - 24 Iu/L • SGPT - 22 Iu/L • SAP - 1O5 Iu/L
CXR - Lt pleural effusion • ECG - WNL • USG ABD - Lt pleural effusion - LIVER/ SPLEEN normal • SPUTUM • AFB - Neg • Grams stain - Neg • Malig cells - Neg
Pleural Fluid • Protein - 4.6 g/dl • Sugar - 84 mg/dl • WBC - 2600/ cu mm ( P 11 L 39 ) • RBC - 30,000/cu mm • Smear - +ve for MALIGNANT CELLS • Grams stain - No organisms • AFB - Neg
Problems • Fever • Lymphadenopathy • Pleural effusion • Subconjunctival hemorrhage • Loss of wt
DIAGNOSIS ACUTE MYELOID LEUKEMIA – M4 WITH MALIGNANT PLEURAL EFFUSION
ETIIOLOGY • HEREDITY - Downs , Klinefelters, patau, Chromosomal breakage syndromes • RADIATION • CHEMICAL - Benzene, smoking, paint, petroleum, pesticides. • DRUGS - Alkylating agents, Topo isomerase II inhibitors, Chloramphenicol and phenyl butazone.
CLINICAL FEATURES • Age of onset - Adult onset and incidence increases with age • M/F - 4.4 : 3 • Fatigue • LOW/ LOA • Fever with or without infection • Bleeding symtoms • Bone pain • Lymphadenapathy • Hepatosplenomegaly • GI, PUL, Intra cranial, Retinal hhage (APL – M3) • Chloroma ( M1 M2) • Gum hypertrophy (M4 M5 )
BLOOD PARAMETERS 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 +
CLASSIFICATIONS • FAB • Based on Morphological charecteristics • BLAST cells > 30 % • WHO • BLAST cells > 20 % • Based on Morphology, Molecular and CF.
FAB • M0 - Minimal diff • M1 - Myeloblastic without maturation • M2 - Myeloblastic with maturation • M3 - Promyelocytic • M4 - Myelomonocytic • M4E0 - Marrow eosinophilia • M5 - Monocytic • M6 - Erythroleukemia • M7 - Megakaryoblastic
WHO • AML I – GENETIC abnormalities • t ( 8/21), inv (16), t (16/16) • MLL abnormalities • APL t ( 15/ 17) • AML II- Following myelodysplasia/ myeloproliferative disorders • AML III- Following DRUG induced myelodysplasia • AML IV – M0 to M7 of FAB , panmyelosis, basophilic and myeloid sarcoma
GOOD Young age Chromosomal defects (t8/21, inv 16, t15/17) Rapid induction of CR Long duration of CR POOR Advanced age ( > 60) Chrom defects ( inv 3, -7 ) Asso med illness Prolonged cytopenias Prev myelodysplasia Drug induced AML Delayed and short duration of CR PROGNOSIS
MANAGEMENT • REMISSION INDUCTION • POST REMISSION MANAGEMENT
REMISSION INDUCTION (7&3 REGIME) • CYTARABINE • 100 – 200 mg / sq m/ day IV infusion 7 days • ANTHRACYCLINE • Daunorubicin – 45- 60mg/sq m/day IV 3 days • Idarubicin - 12 mg/sq m/ day IV • With or without ETOPOSIDE
COMPLETE REMISSION • Blood • WBC >1500 • Platlet > 1 Lac • RBC count not considered • No BLAST cells • BM • Trilineage cellularity > 20% • Blast cell < 5% • NO Auer rods • RT PCR/ FISH to r/o residual leukemic cells
If 7 & 3 Regime • 50% - CR • start 5 & 2 Regime • 25% - CR • 25% - NO CR • NO CR • 50% - Drug Resistance • 50% - Fatal complications of Drugs
HIGH DOSE CYTARABINE 3-4 cycles 3 g/sq m / day bd on 1,3 & 5 th day STEM CELL Tx Autologous allogenic LESS INTENSIVE CHEMOTHERAPY STEM CELL Tx Non myeloablative Allogenic POST REMISSION MANAGEMENT AGE < 65 yrs AGE > 65 YRS
TREATMENT OF APL ( M3) • TRETINOIN( Maturation of blast cells,No DIC) • 45 mg/ sqm/day Untill remission • ANTHRACYCLINES • Maintanance therapy • Tretnoin or chemotherapy
RELAPSE STEM CELL TX