Acute leukemia

seminar on internal medicine

1. Acute Leukemia Anil Regmi Intern NGMC, Banke

2. Definition Classification of leukemia Etiology Clinical feature Investigation Treatment

3. Leukemia are the Group of malignant disorders of the hematopoietic tissues characteristically associated with increased numbers of white cells in the bone marrow and / or peripheral blood

4. Classified based on cell type involved and the clinical course 1. Acute : ALL AML 2. Chronic : CLL CML

5. Acute Lymphoblastic Leukemia(ALL) Neoplastic disease which results from a mutation in a single lymphoid progenitor cell at one of several discrete stages of development B Cell or T Cell Most common childhood acute leukemia, ~80% Incidence in adults ~20% Bimodal distribution of occurrence: Peak at age 2-5 Second increased incidence after age 50

6. Classification of ALL French American British Classification L1: small uniform blasts (pediatric ALL) L2: larger, more variable sized blasts (adult ALL) L3: uniform cells with basophilic and sometimes vacuolated cytoplasm (mature B cell ALL)

7. Acute myeloid leukemia (AML) Acute myeloid leukemia (AML) is a neoplastic disease characterized by infiltration of the blood, bone marrow, and other tissues by proliferative, clonal undifferentiated cells of the hematopoietic system usually occurs at a very early stage of myeloid development Rare in childhood & incidence increases with age

8. Classification of AML WHO classification AML with minimal differentiation AML without maturation AML with maturation Acute myelomonocytic leukemia Acute monoblastic and monocytic leukemia Acute erythroid leukemia Acute megakaryoblastic leukemia Acute basophilic leukemia Acute panmyelosis with myelofibrosis

9. ETlOL0GY Heredity: Down syndrome, Fanconi anemia, Bloom syndrome, ataxia telangiectasia . Radiation: High-dose radiation increases the risk of myeloid leukemias that peaks 5-7 years after exposure. Chemical and Other Exposures: benzene, Smoking and exposure to petroleum products， paint， embalming fluids， ethylene oxide， herbicides， and pesticides Drugs : Alkylating agent, Chloramphenicol ， phenylbutazone , chloroquine and methoxypsoralen

10. Clinical features: General : Onset is abrupt & stormy (usually present within 3 months) Bone marrow failure (anemia, infection ,bleeding) Nonspecific Symptoms Fatigue/decreased energy Fever Easy bruising Bleeding Dyspnea Dizziness Infection

11. Physical Examination Performance status (prognostic factor) Ecchymosis and oozing from IV sites Fever and tachycardia (signs of infection) Papilledema ， retinal infiltrates， cranial nerve abnormalities (CNS leukemia) Poor dentition， dental abscess Gum hypertrophy ( Ieukemic infiltration) Skin infiltration or nodules ( Ieukemia infiltration) Lymphadenopathy ， splenomegaly ， hepatomegaly Back pain

12. Hematological findings: Anemia; normocytic , normochromic Reduced reticulocyte WBC usually elevated (50,000- 1,00,000 / cumm ) Platelet counts <100,000/ cumm Bone marrow aspirate: Hypercellular , blast cells ( > 20%), presence of Auer rods - AML type Other Inv: Immunophenotyping Molecular genetic Cytogenetics : Chromosomal abnormalities Coagulation screen, RFT, LFT LDH, Uric acid Urine CXR ECG, ECHO

13. Treatment

14. Supportive care : Anemia – red cell transfusion Thrombocytopenia – platelet concentrates Infection – broad spectrum IV antibiotics Barrier nursing Indwelling central venous catheter Metabolic problems : Monitoring hepatic / renal / hematologic function; Fluid & electrolyte balance, nutrition Hyperuricemia - hydration, Allopurinol Psychological support

15. SPECIFIC THERAPHY: Chemotherapy

16. CNS Prophylaxis Given during induction and intensification Intrathecal : MTX, Cytarabine , corticosteroids Systemic: high dose mtx , cytarabine , L- asparaginase +/- Cranial Irradiation PALLIATIVE THERAPHY Chemo: Hydroxycarbamide , mercaptopurine RT Blood product support

17. Prognosis: Median survival without treatment is 5 weeks 30% 5-yr survival in younger patients with chemotherapy Disease which relapses during treatment or soon after the end of treatment has a poor prognosis Poor prognostic factors: Increasing age Male sex High WBC count at diagnosis CNS involvement at diagnosis Cytogenetic abnormalities No complete remission

18. Thank you!!!