Hematological Malignancies-leukemias.pptx

This presentation include a clear and informative knowledge regarding hematological maliganancies

1. Hematological Malignancies

2. Blood Cells

3. Hematopoiesis

4. Overview Leukemias-Too many WBCs Lymphomas- Too many lymphocytes Multiple Myeloma- Too many B Cells Essential Thrombocytosis- too many platelets Polycythemia Rubra Vera- Too many RBCs Primary Myelofibrosis-Fibrosis of BM- Affects everything

5. Leukemias Vs Lymphomas Leukemia Arises from bone marrow Affects immature WBCs Lymphomas Arise from lymph nodes Affects mature lymphocytes

6. Leukemias Unregulated proliferation of immature/non – functional leukocytes in bone marrow ( Undifferentiated ) Build up of these immature leukocytes crowd out the normal cells, resulting in decreased amounts of functional cells- known as crowding. More often seen in acute leukemias. Acute Leukemias-more aggressive but often curable. Chronic Leukemia-often asymptomatic, rarely curable

7. Clinical Manifestation Anemia- Syncope, Angina,Dyspnea , fatigue (SADF) Thrombocytopenia- increase bleeding Leukopenia- increased infections Systemic- Weight loss, fever, night sweats, loss of appetite Organs- hepatosplenomegaly, bone pain, lymphadenopathy https://www.youtube.com/watch?v=s7ObC6uw3h8

8. Acute myeloid leukaemia (AML) Neoplastic proliferation of blast cells(>20%) derived from marrow myeloid elements. Commonest acute leukaemia of adults (1/10,000/year) Associated with radiation and Downs Syndrome. Symptoms – marrow failure, infiltration (gum hypertrophy, CNS involvement at presentation rare.) Investigation – FBC and film, bone marrow aspirate with immunophenotyping.( Leukopenia, thrombocytopenia, anaemia) Treatment – supportive (walking exercises can relieve fatigue), chemotherapy (intensive, long periods of immunosuppression) bone marrow transplant. Prognosis – Death in ~ 2 months if left untreated. - After treatment ~ 20% 3 yr. survival.

9. Acute myeloid leukaemia (AML)

10. Typical Labs of AML Leukocytosis Blastemia Leukemic hiatus Auer rods Thrombocytopenia Anemia >20% blasts in BM

11. Chronic myeloid leukaemia (CML) Uncontrolled clonal proliferation of myeloid cells. Associated with Philadelphia chromosome ( present in >80%) Symptoms/Signs – chronic and insiduous – weight loss, fever, fatigue, sweats , gout, bleeding (platelet dysfunction), abdominal discomfort (splenomegaly >75%, often massive) Around 30% detected by chance on routine FBC. Investigations – WBC ↑, Hb low or normal. Bone marrow diagnosis. Treatment – Chemotherapy (imatinib- >90% response rate), allogenic transplant only hope of long term survival. Prognosis – Good prognosis with treatment.

12. Acute lymphoblastic leukaemia (ALL) Malignancy of lymphoid cells with uncontrolled proliferation of blast cells. Genetic susceptibility and environmental trigger. Associated with Downs Syndrome. Commonest cancer of childhood, rare in adults. Signs/symptoms – Marrow failure (pancytopenia) leading to anaemia, infection and bleeding. Hepato/splenomegaly. CNS infiltration (meningism, nerve palsies) Investigation – FBC, blood film, CXR/CT, LP. Treatment – Supportive (transfusion,antibiotics), chemotherapy (often intrathecal), allogenic marrow transplant. Prognosis – cure rates for children 70-90%, adults ~ 40%.

13. Chronic lymphoid leukaemia (CML) Accumulation of mature B cells. Commonest leukaemia (~4/100000/year) Symptoms/signs – often none, found on routine FBC. May have enlarged rubbery non tender lymphadenopathy, hepato / spleomegaly , systemic symptoms. Investigations – increased lymphocytes, later autoimmune haemolysis and pancytopenia. Treatment – monitoring, chemotherapy only indicated if symptomatic/specific genetic mutations, radiotherapy to enlarged nodes. Prognosis – 1/3 never progress, 1/3 progress in time and 1/3 are actively progressing.

14. Chronic lymphocytic leukaemia (CLL)

15. CHRONIC LYMPHOCYTIC LEUKEMIA Blood smear Nodular marrow infiltration Packed marrow Smudge cell

16. Lymphomas Non Functional Lymphocytes accumulating in the lymph nodes. 2 types 1. Hodgkin’s Lymphoma-Contain Reed- sternberg cells ( Owl eyes). Has better prognosis 2. Non- Hodgikin Lymphoma- Doesn’t Contain reed-Sternberg cells

17. Myeloid Diseases Polycythemia Rubra Vera Essential Thrombocytosis Primary Myelofibrosis Multiple Myeloma Myelodysplastic Syndrome

18. Essential Thrombocytosis Over production of platelets Clinical Features Thrombotic Events Spontaneous Abortion Headaches Treatment- Low dose aspirin And Hydroxyurea

19. Polycythemia Rubra Vera Too Many RBC Production 95% association with a JAK 2 mutation

20. Signs and symptoms Fatigue Headaches Dizziness Episosic Blurred Vision Peripheral Burning or tingling sensation Pruritus after a hot shower

21. Investigations Increase RBCs,HB , HCt Bone Marrow Biopsy EPO levels Management Venesection Aspirin for prophylaxis Cytoreductive therapy (Hydroxy urea)

22. Myelofibrosis Fibrosis of bone marrow as a result of myeloproliferative neoplasm Will present with Hepatosplenomegaly Blood Film- Teardrop Cells Treatment- Stem Cell transplantation

23. Multiple Myeloma Malignant clonal proliferation of B lymphocyte derived plasma cells. Incidence 5/100,000, peak age 70 years. Symptoms /signs : C- Hypercalcaemia R - Renal impairment (light chain deposition) A - Anaemia, thrombocytopenia, neutopenia B - osteolytic bone lesions – backache, pathological fractures. - Recurrent bacterial infections - Can present acutely with SCC, hyperviscocity , ARF. Investigation – check serum electrophoresis and ESR in all patients over 50 with back pain, xrays (punched out lesions, pepperpot skull), bone marow biopsy. Treatment – supportive (analgesia, bisphosphonates, vertebroplasty, fluids, dialysis), chemotherapy. Prognosis – median survival 3-4 years, death usually due to infection or renal failure.

24. MYELODYSPLASTIC DISORDERS Myelodysplastic disorders are primary stem cell disorders characterized by: One or more peripheral blood cytopenias resulting from ineffective hematopoiesis Prominent maturation abnormalities in the bone marrow ( dyspoiesis or dysplasia) Usually occurs in the elderly Commonly progress to an acute leukemia Are refractory to treatment