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A Case of Chronic Myeloid Leukemia

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A Case of Chronic Myeloid Leukemia

  1. 1. BY DR.ANIRUDH J SHETTY PROF.DR.G.ELANGOVAN’S UNIT
  2. 2. 28 yr male patient came with c/o abdominal pain-1 week in duration involving predominantly the left upper and centre of the abdomen vague dragging pain assoc with fullness of abd no h/o any aggravating or relieving factors
  3. 3.  h/o early satiety +  h/o loss of appetite +  No h/o loss of weight  No h/o jaundice  No h/o haematemesis  No h/o altered bowel habits  No h/o bleeding tendencies
  4. 4.  PAST h/o -> No h/o Tb/HTN/DM  PERSONAL h/o -> consumes alcohol occasionally not a smoker
  5. 5.  O/E – Pt conscious oriented afebrile anemic no Cyanosis,clubbing,icterus, lymphadenopathy B.P-110/70 mmHG P.R -80 min
  6. 6.  P/A –mild distention of abdomen+ no dialated viens,scars or sinuses massive spleenomegaly(+) crossing the umbilicus no hepatomegaly no FF+
  7. 7.  CVS – S1S2(+) NO murmurs RS – NVBS+ no added sounds CNS - NFND
  8. 8. CBC  Hb – 14  TC – 9300  P69,L31  ESR – 15  PLATLETS – 1,50,000 RFT  RBS -152  UREA – 32  CREAT – 1.0  SODIUM; 135  K+ - 6.0  CL – 105  HCO- 19
  9. 9.  ECG – WNL  CHEST X – RAY - WNL
  10. 10. CBC TC – 1,60,000 SHIFT TO LEFT BLAST 1% HB – 9.2 PLATLET -1,80,000 Chronic phase of CML  P/S Markedly increased TC, showsMYELOBLAST,PR OMYELOCYTE,METAMY ELOCYTE,BAND FORMS
  11. 11.  BONE MARROW FRAGMENT SHOWING A DENSLLY CELLULAR MARROW FRAGMENT WITH ABSENT FAT CELLS
  12. 12.  LOW POWER MICROSCOPE: MYELOID HYPERPLASIA WITH RELATIVELY REDUCED ERYTHROID PROGENITORS
  13. 13.  Patient was started on Imatinib mesylate 400mg 1-0-0
  14. 14.  CML accounts for 15% of all cases of leukemia.  More common in men  Peak incidence : fourth and fifth decade
  15. 15.  In CML the c-ABL gene is translocated from its normal abode on chromosone 9 to chromosone 22,where it fuses with bcr gene.  As a consequence of the fusion, c-ABL loses a region that controls tyrosine kinase activity. Thus the BCR-ABL protien,the product of the fusion gene has potent and constitutive tyrosine kinase activity.
  16. 16. 1. CHRONIC PHASE 2. ACCELARATED PHASE 3. BLAST CRISIS
  17. 17.  EASY FATIGABILITY  DECREASE TOLERANCE TO EXERTION  ABDOMINAL DISCOMFORT AND EARLY SATIETY( DUE TO SPLENIC ENLARGEMENT)  WIEGHT LOSS AND EXCESSIVE SWEATING The symptoms are vague, nonspecific and gradual in onset
  18. 18.  A physical examination may detect pallor and splenomegaly.The latter was present in 90% of patients. UNCOMMON PRESENTATIONS :  Dramatic hypermetabolism(night sweats,wt loss,heat intolerance)  Acute gouty arthritis(due to hyperurecemia)  Priapism,tinnitus  Lt upper quadrant n lt shoulder pain due to splenic infarction and perisplenitis.
  19. 19.  CML is often suspected on the basis on the complete blood count, which shows increased granulocytes of all types, typically including maturemyeloid cells.  Basophils and eosinophils are almost universally increased; this feature may help differentiate CML from a leukemoid reaction.  A bone marrow biopsy is often performed as part of the evaluation for CML, and CML is diagnosed by detecting the Philadelphia chromosome.
  20. 20.  10–19% myeloblasts in the blood or bone marrow.  >20% basophils in the blood or bone marrow  Platelet count <100,000, unrelated to therapy  Cytogenetic evolution with new abnormalities in addition to the Philadelphia chromosome  Increasing splenomegaly or white blood cell count, unresponsive to therapy
  21. 21.  Blast crisis is the final phase in the evolution of CML, and behaves like an acute leukemia, with rapid progression and short survival.  Blast crisis is diagnosed if any of the following are present in a patient with CML:  >20% myeloblasts or lymphoblasts in the blood or bone marrow.  Large clusters of blasts in the bone marrow on biopsy.  Development of a chloroma (solid focus of leukemia outside the bone marrow)
  22. 22.  IMATINIB MESYLATE  STEM CELL TRANSPLANTATION  IFN-ALPHA  HYDROXYUREA  SECOND GENERATION TK INHIBITOR- DASATINIB AND NILOTINIB
  23. 23.  IMTINIB NOW IS USED AS INITIAL THERAPY IN ALMOST ALL PATIENTS WITH CML.  IN CASES WHERE WBC COUNT IS MARKEDLY ELEVATED HYDROXYUREA CAN BE USED TO PREVENT HYPERLEUKOCYTIC SYNDROME.
  24. 24.  The bcr abl oncoprotien with a molecule of ATP in d kinase pocket.  Phosphorylation - > substrate .Activates downstream molecules  Imatinib occcupies d kinase pocket preventing phosphorylation of its substrates
  25. 25. HAEMATOLOGIC RESPONSE CYTOGENETIC RESPONSE MOLECULAR RESPONSE(BCR ABL TO CONTROL GENE RATIO ACCORDING TO IS SCALE) FREQUENCY .Every 2 weeks until a complete response has been achieved and confirmed. every 3 months unless othwise required every 6 months until a complete response has been achieved and confirmed. then every 12 months EVERY 3 MONTHS METHOD complete blood count (CBC) with differential Conventional cytogenetic examination .FISH (fluorescene in situ hybridisation) (only before treatment) .RQ-PCR (Reverse transcription quantitative polymerase chain reaction)
  26. 26. SMENT METHOD SUBOPTIMAL RESPONSE FAILURE tologic No Complete hematologic response(CHR) within 3 months No hematologic response within 3 months Loss of CHR at any time enetic No MCyR (Major cytogenetic response )within 6 months No CCyR(Complete cytogenetic response) within 18 months .No cytogenetic response within 6 months No MCyR within 12 mont No CCyR within 18 mont Loss of CCyR at any time Not applicable. CULAR No MMR(Major molecular response) within 8 months Loss of MMR at any time
  27. 27.  Second generation TKI like DASATINIB NILOTINIB  Allogenic human stem cell transplant
  28. 28.  Well tolerated compared to other treatment options in CML  Superficial edema  Nausea,muscle cramps  Rash,Diarrhea  Uncommon side effects:tumour lysis in accelerated phase,splenic rupture,cerebral edema,Varicella Zoster infections
  29. 29.  Imatinib is not recommended during pregnancy.  Hydroxyurea has the lowest mutagenic potential among the cytotoxic agents.  IFN can also be safely used during pregnancy.
  30. 30.  Patients who are younger than 65 yrs and who have a identical twin or a histocompatible sibling can be transplanted after intensive therapy usually with cyclophosphamide.  GVHD is common.
  31. 31. THANK YOU

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