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Acute leukemia Mohammed Al-matrafi. Leukemia Malignancy of leucocytes precursors Appearance of abnormal cells in BM, peripheral blood, infiltration in.

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Presentation on theme: "Acute leukemia Mohammed Al-matrafi. Leukemia Malignancy of leucocytes precursors Appearance of abnormal cells in BM, peripheral blood, infiltration in."— Presentation transcript:

1 Acute leukemia Mohammed Al-matrafi

2 Leukemia Malignancy of leucocytes precursors Appearance of abnormal cells in BM, peripheral blood, infiltration in LN, Liver spleen etc.

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5 Childhood Leukemia Types: Based on clinical presentation Acute leukemia 95% Chronic leukemia 5% Based on type of predominant leukemic cells Acute leukemia: Acute lymphoblastic leukemia- 85% Acute myeloid leukemia- 15%

6 Childhood leukemia Prevalence: Most common malignancy in children 30% of all pediatric malignancies Average incidence 4/100,000 children Peak age: ALL : 4 years AML: Same from birth –10 years

7 Etiology Unknown: Genetic predisposition Viral infection Cong. immune deficiency Ionizing radiation Certain toxic chemicals

8 At risk patients

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13 Genetics At risk: Trisomy 21 {15 times} Fanconi aplastic anemia Ataxia telengectasia Siblings of patient with ALL {2-3 times} Identical twins{ concordance of ALL}

14 Clinical presentation of ALL

15 SYMPTOMS: Usually < 4 weeks history at diagnosis Fatigue/malaise Fever/infection Extremity, joint or bone pain Bleeding manifestations CNS symptoms (Increased ICP) Weight loss Others: DIC, Chloroma {AML}

16 Clinical Presentation of ALL SIGNS: Pallor Hepatomegaly Spleenomegaly Lymphadenopathy Petechie Bony tenderness

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18 Diagnosis

19 Peripheral blood : CBC : Normal, increased, decreased > 100,000 bad prognosis Anemia Neutropenia Blast cells Thrombocytopenia

20 Normal blood film

21 L1 L2 L3

22 : Diagnosis Bone marrow (BM) Morphology: >25% blast cells in marrow (normal <5%)

23 Other investigations Uric acid high LDH high

24 Bone marrow aspirate Morphological classification: Cytochemical analysis: Immune phenotyping: Cytogenetic: Molecular studies:

25 Morphology FAB classification: { depending on size,cytoplasm,nucleus} L1 L2 L3 L1 : commonest and has good prognosis

26 Immune phenotype T cell leukemia B cell leukemia Non T cell non B cell leukemia

27 Cytogenetic studies Higher ploidy { >50 chromosomes}: good prognosis Diploidy or hyperdiploidy: { chromosomes} Intermediate prognosis Haploid cell: worst prognosis

28 Differential diagnosis Non malignant conditions like: Juvenile Rheumatoid Arthritis / other connective disorders Infectious Mononucleosis Aplastic Anemia Idiopathic Thrombocytopenic Purpura {ITP}

29 Treatment of ALL BASED ON: Lineage (B or T) Cytogenetic abnormalities Patient’s age and other risk factors White blood cell count (WBC)

30 Supportive measures Hydration Treatment of infection Correction of electrolyte disturbances Blood product transfusion Psychological support Treatment of tumor lysis syndrome

31 Treatment of ALL REQUIRES: Intensive systemic multi agent chemotherapy Repetitive intrathecal chemotherapy Cranial irradiation when necessary in older children Bone marrow transplant in special circumstances Treatment continued for  3 years

32 Treatment Induction phase: 4 weeks {3-4 drugs} vincristine, prednisilone, L-asparaginase etc CNS prophylaxis: Intrathecal methotrexate Cranial irradiation Consolidation phase:2-4 weeks {For prevention of relapse} Maintenance phase:{2-5 years}

33 Sanctuaries areas Relatively impermeable to the medications: Sites of relapse: 2 sites: CNS Testis

34 Prognostic factors {contd.} Morphology, histochemistry, cytogenetic L1; good prognosis Response to induction therapy Rapid- good prognosis Slow- poor prognosis B cell leukemia: worst prognosis

35 Prognostic factors Demographic Age: 10year poor prognosis Race: Black poor prognosis Sex: Male poor prognosis Leukemic burden WBC: >50,000 poor prognosis Mediastinal LN: poor prognosis CNS involvement. at diagnosis: poor prognosis

36 Outcome Relapse sites: Bone marrow CNS Testis in males Disease free for 5 years after diagnosis: overall 60-70%, in standard risk group 80% Relapse: Allogenic bone marrow transplant

37 Bone marrow transplant Very high risk cases Following relapse

38 Bone marrow transplant Blood stem cell transplant Autologous Allogeinic

39 In a blood stem cell transplant, the patient is first given a pre-transplant treatment of chemotherapy and/or radiation therapy to destroy the patient's leukemia cells and immune system. Blood stem cells are then put into the patient's blood to restore the patient's immune system and blood production.

40 Acute myelogenous leukemia FAB classification: M1,M2,M3,M4,M5,M6,M7 M3 { promyelocytic} may present with DIC Disease free survival with chemotherapy 30 % BMT %

41 Questions Commonest childhood malignancy Types of Ac leukemia Peak age Etiology At risk Patients Symptoms Signs Diagnosis: PBS and bone marrow changes D/D Sanctuary areas Prognostic factors: eg: age 100,000, mediatinal mass, CNS invol L1 type, Rapid response to induction therapy Relapse site BMT indication


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