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ACUTE LEUKEMIA ( asif rahman)
- 2. Case presentation DrASIF REHMAN PG TRAINEE MEDICAL UNIT 2 CIVIL HOSPITAL LALRKNA
- 3. 18 Yearsold female NOOR BANOO d/o MASHOOQ, resident of larkana, admitted in medical unit 2 civil hospital larkana on 28th February 2017 via emergency with chief complain of:
- 4. Chief complain Feverwith headache for 4 months Shortness of breath for 15days.
- 5. HOPC According tomy patient, She was in usual state of health 4 months back when she developed fever which was sudden in onset, high grade , continued in nature, associated with rigors & chills and with night sweats. it relieved temporarily by taking panadol. Fever was associated with generalized sever headache along with vomiting. During this illness, Patient has h/o loss of appetite and Approximately 4 to 5 kilogram loss of her body weight. She also complained of bony pains that involved ( backbone, ribs and sternum) for last 1 to 2 months.
- 6. HOPC Patient reported shortnessof breath for15 days which was gradual in onset and was progressive that worsened with lying down & walking, associated with chest pain. There is no history of jaundice, rashes, burning micturition, cough,PND, hematemesis and hemoptysis OR joint pain.
- 7. PAST HISTORY Nopast medical history of TB,IHD & hepatitis or hospitalization No h/o allergies Pt has h/o immunization.
- 8. DRUG HISTORY Tabpanadol syp lysovit CAP risek 20 mg There is history of transfusion of two pints of whole blood 2 months back.
- 9. PERSONAL HISTORY Sheis unmarried Non smoker, Sleep pattern is disturbed h/o weight loss Decreased appetite Bladder And bowel habits are normal
- 10. FAMILY HISTORY Herfather and mother are healthy and normal She has three sisters All are healthy and normal.
- 11. SOCIOECONOMIC HISTORY Patientbelongs low socioeconomic status Lives in cemented house Father is the only earner Hygienic conditions are satisfactory.
- 12. CASE SUMMURY 18years old NOOR BANO admitted via ER with complain of high grade, continued fever for 4 months, associated with chills & rigors, headache and vomiting, along with 4 to 5kg body weight loss. Patient also complain of progressive shortness of breath for15 days, associated with chest pain. Patient has bony pains but no h/o jaundice, rashes, burning micturation, cough, hematemesis , hemoptysis or joint pains. There is history of transfusion of two pints of whole blood 2 months back.
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- 15. GENERAL PHYSCIAL EXAMANITION Ayoung age female of average height and built , sitting comfortably on bed , cannulated in right arm , well oriented with time , place and person.
- 16. vitals BP: 110/70mmhg Pulse: 108 beats/minute R/R: 22breaths / minute Tem: 102F
- 17. SUB VITALS Pallor-present JVP-not raised Jaundice-Absent THYROID-not enlarged Cyanosis-absent LYMPH N- not palpable Clubbing-absent BONY TENTERNESS- PRESENT Edema-absent Koilonychias-absent Palmer Erythema-absent.
- 18. SYSTEMIC EXAMINATION Central NervousSystem Higher psychiatric function : she is conscious, well oriented with time, place and person, normal speech and intact recent and past memory Examination of cranial nerves : all cranial nerves are intact. Sensory system : normal Motor functions Bulk of muscles : normal Tone of the muscles: increased Power of the muscle: normal 5/5 in all limbs Deep reflexes ( ankle , knee, biceps and triceps) : +2 Plantar reflex : up going bilaterally signs of meningeal irritation: neck rigidity and kerning`s sign positive
- 19. Respiratory System Inspection: not allowed with out clothes, apparently shape of chest seems to be normal with reduced movements on right side of chest Palpation Trachea: Central. Apex beat: In the left 5th intercostal space in midclavicular line, 8 cm from midsternal line, normal in character Chest expansion: Reduced (1cm) Percussion: Area of liver dullness in 5th ICS in the right midclavicular line Percussion note: stony dull on right side of chest from 7th intercostal space to down ward posteriorly
- 20. Auscultation : breathsounds were diminished on right lower chest from 7th intercostal space to downward posteriorly Vocal resonance: reduced on right lower side of chest posteriorly
- 21. Gastrointestinal System Abdominalexamination was normal except pt had palpable spleen ,5 to 6 cm from left costal margin in anterior axillary line with regular surface ,soft to firm in consistency and non tender (Liver span 12cm).
- 22. Cardiovascular System Pulse rate : 108beats/min, With regular rhythm and normal volume pulse JVP not raised, Apex beat: In the left 5th intercostal space in midclavicular line, 8 cm from midsternal line, normal in character Area of cardiac dullness: Normal. Auscultation: . 1st and 2nd heart sounds are audible Added sounds are not present.
- 23. Case Summary 18years old NOOR BANO admitted via ER with complain of high grade, continued fever for 4 months, associated with chills & rigors, headache and vomiting, along with 4 to 5kg body weight loss. Patient also complain of progressive shortness of breath for15 days, associated with chest pain. Patient has bony pains but no h/o jaundice, rashes, burning micturition, cough, hematemesis , hemoptysis or joint pains. There is history of transfusion of two pints of whole blood 2 months back. Examination revealed anima and bony tenderness. Moderate splenomegaly, Increased muscle tone with up going planters & positive SOMI Right side of chest : Reduced chest expansion, stony dull percussion note, reduced breath sounds & reduced VR.
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- 26. COMPLETE BLOOD COUNT Hemoglobin- 8g/dl Haematocrit-25% M.C.V-98 Fl M.C.H-31 pg M.C.H.C-31.7 g/d Platelets- 200*10E/L W.B.S - 128*10E/L Neutrophills-3.1 % Lymphocytes -71.0% Eosinophil's -.01 % Monocytes- 25% Basophils – 0.0 %
- 27. PERIPHERAL FILM Macrocytic,normochromic red cells, platelets are normal in film 75% blast cells are seen.
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- 31. LFT , URINEDR S.UREA CREATININE NORMAL
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- 40. CSF ACID FASTBACILLI SMEAR
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- 44. BONE MARROW BIOPSY…. Conclusion…. BONE MARROW ASPIRATION : Hypercellular specimen shows diffuse infiltration with blast cells which constitute around 82% of total nucleated, nonerythroid cells population. These blast cells are medium in size having high nuclear to cytoplasmic ratio, agranular cytoplasmic, fine chromatin and inconspious nucleoli Normally hematopoiesis is markedly suppressed BONE MARROW TREHINE :Good length of specimen showing effected architecture with overall cellularity of 90 -95%. There is diffuse infiltration with blast cells
- 45. Conclusion…. IMMUNOPHENOTYPE OFBLAST CELLS: CD34 : Diffuse positive TdT : Diffuse positive CD79: Diffuse positive. CD3: NEGATIVE MPO : NEGATIVE FINDINGS ARE CONSISTENT WITH PRECURSOR- B CELLACUTE LYMPHOBLASTIC LEUKEMIA.
- 46. Conclusion… CYTOGENETICS REPORT: 45XX,(9;22)(q34;q11.2),-20(15) 15 cells were counted, all cells were positive for Philadelphia chromosome. Case comment: all cells showed translocation between chromosomes 9q34 and 22q11.2 and-20.
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- 54. The Acute Leukemias Acute lymphoblastic leukemia (ALL) is a malignant (clonal) disease of the bone marrow in which early lymphoid precursors proliferate and replace the normal hematopoietic cells of the marrow. The acute leukemias were usually malignancies of blast cells with few identifying characteristics acute lymphoid leukemias (ALLs) are pre- dominantly cancers of children and young adults. All lymphoid cells are derived from a common hematopoietic pro- genitor that gives rise to lymphoid, myeloid, erythroid, monocyte, and megakaryocyte lineage.
- 55. STAGES of differentiationof B CELLS AND THERE malignancies
- 57. CLASSIFICATION OF ACUTELYPHOID LEUKIMIA
- 60. PROGNOSIS Only 20-40%of adult with acute lymphoblast leukemia are cured with poor risk criteria with current treatment. Patients with ALL are divided in to GOOD RISK and POOR RISK CRITERIA.
- 61. Age youngerthan 30 years WBC Count less than 30,000/uL No adverse cytogenetics No CNS and testicular disease Female patient LDH not high HYPERDIPLOIDY Rapid response to induction therapy Age older than 60 years Precursors B-cell with WBC >100,000/uL Adverse cytogenetics- translocation t(9;22) ,t(4;11) CNS or TESTICULAR disease at presentation Male patient High LDH level HYPODIPLOIDY No Remission in GOOD RISK CRITERIA POOR RISK CRITERIA
- 64. Treatment THERE ARE TWOCOMPONENT OF TREATMENT SUPORTIVE TREATMENT SPECIFIC TREATMENT
- 65. SUPORTIVE TREATMENT Bloodtransfusion for anemia, platelets concentrates for thrombocytopenia and granulocytes for neutrophils. Antibiotics needed for control of infection, C0- TRIMOXALE as prophylaxis for pneumocystis carinii Allopurinol 10 mg/kg/day 3dd for 10 days) is given along with induction therapy to guard against with development of uric acid nephropathy Adequate fluids and nutrition support Analgesics prescribed as required .
- 66. Specific therapy Traditionallythere are four components of ALL treatment INDUCTION CHEMOTHERAPY CONSOLIDATION CHEMOTHERAPY MAINTENANCE CHEMOTHERAPY CNS PROPHYLAXIS ALL WITH CNS INVOLVEMENT ALL WITH PHILADILPHIA CHROMOSOMES.
- 70. Consolidation therapy (2-4 WEEKS) It is given after induction therapy it removes residual or resistant leukemic cells regimens using a standard 4- to 5-drug induction usually include consolidation therapy with CYTOSINE ARABINOSIDE(Ara-C) in combination with an anthracycline or epipodophyllotoxin.
- 71. Maintenance Therapy (2- 5YEARS) 6-mercaptopurine (50mg/d oral) Methotrexate 20mg?m2/wk oral .i.v With reinforcement : Vincristine 1.5mg/m2 (max 2mg) i/v every 4weeks Prednisone 40mg/m2/day *7 days every4 week.
- 72. CNS Prophylaxis patientswith acute lymphoblastic leukemia (ALL) frequently have meningeal leukemia at the time of relapse. A minority of patients have meningeal disease at the time of initial diagnosis high-dose systemic chemotherapy reduces CNS relapse early intrathecal chemotherapy is to achieve the lowest risk of CNS relapse
- 73. CNS relapse rates 31% for group 1 (standard chemotherapy, no CNS prophylaxis), 18% for group 2 (high-dose systemic chemotherapy, no CNS prophylaxis 17% for group 3 (high-dose systemic chemotherapy, intrathecal chemotherapy for high-risk subjects after achieving remission), 3% for group 4 hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD).
- 74. Treatment of Ph Chromosome–PositiveALL the tyrosine kinase inhibitor (inhibits the bcr-abl fusion protein of Ph+ ALL) = imatinib Newer tyrosine kinase inhibitors Nilotinib and dasatinib Ponatinib a kinase inhibitor, was approved by the US Food and Drug Administration (FDA) in December 2012 for patients with Ph+ ALL that is resistant or intolerant to prior tyrosine kinase inhibitor therapy.
- 75. Transplantation Hematopoietic stemcell transplantation (HSCT) seems to be a valuable option for ALL carrying poor prognostic factors.