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Haematological Malignancy

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Presentation on theme: "Haematological Malignancy"— Presentation transcript:

1 Haematological Malignancy
Kim Gibson, F1 January 2015

2 Objectives Leukaemias Lymphomas Myeloma ALL, CLL, AML, CML.
Hodgkin’s vs Non-Hodgkin’s Myeloma

3 Blood Components

4 Blood Cell Components Lymphocytes form in marrow – T cells mature in the thymus. B and T cells are deposited in lymph organs (e.g. spleen) and circulate in lymphatic system. Myeloid cells include all other haematological cell types, which form in bone marrow before release into vascular space.

5 Leukaemia Leukaemic cells (cancerous WBC) infiltrate bone marrow, affecting normal function, i.e. There is inadequate haematopoeisis. Aetiology mostly unknown, but viruses/chemicals/drugs/radiation all implicated

6 Leukaemia

7 Leukaemia: Signs & Symptoms
Mostly seen in acute leukaemia, late chronic. Due to bone marrow failure – red marrow in central skeleton and proximal long bones

8 Leukaemia: Investigations
FBC – Hb low, WBC usually high Blood film – shows precursor lymphoblast cells Bone Marrow aspirate – increased cellularity, reduced erythropoeisis, reduced megakaryocytes - replaced by blasts CXR – why? LP for CSF examination in ALL

9 Leukaemia: Treatment Without treatment, acute leukaemia is invariably fatal within months. ALL in the young has best chance of cure, AML good chance of cure but secondary AML poorest prognosis. Curative treatment carries both morbidity and mortality – Risk vs Benefit

10 Leukaemia: Treatment All need supportive treatment: Curative Intent:
Transfusions of red cells and platelets and necessary, prevention of infection, treatment of infection with antibiotic/antifungal agents as soon as fever develops Curative Intent: Try to return marrow to normal – complete remission (CR). ‘Induction chemo’ not specific to cancerous cells – major infection risk. Followed by specific ‘consolidation’ regimen – ESSENTIAL or remission will certainly occur.

11 Acute Myeloid Leukaemia
Treatment with curative intent if <60 Risk and prognosis based on bone marrow result Bone marrow transplant in some higher risk patients, or if fail 2 cycles chemo 75% achieve CR, 50% of those are ‘cured’ (1/3 overall)

12 Acute Lymphocytic Leukaemia
ALL commonest childhood malignancy, but can be any age CR and consolitation achieved with different agents than in AML Early transplant if high risk (older pts, high WCC, >3-4 weeks to achieve CR) Major difference is need to treat CNS as soon as CR achieved – intrathecal chemo Very high risk may need cranial radiotherapy Maintenance Tx for at least 2 years

13 Acute Leukaemias ALL in childhood good prognosis – 80% alive at 5 years Overall cure rate for ALL 30% In both AML and ALL recurrence usually within 3 years – poor prognosis. Transplant usually considered, despite the risks.

14 Chronic Myeloid Leukaemia
Peak age years. Slow, progressive disease. If untreated  acute phase (blast crisis) leads to death rapidly. Often no symptoms in chronic phase. Symptoms often suggest blast crisis is occurring. Philadelphia chromosome associated with 95% cases Fluoroscein-in-situ-Hybridisation and reverse-transcriptase PCR helps diagnosis and monitoring response to Tx

15 CML: Treatment Supportive treatment as necessary
Imatinib is therapy of choice in chronic phase – complete response in 95%, well tolerated, can continue indefinitely Acute phase treated in similar way to acute leukaemias to try and return to second chronic phase. Stem cell transplant cures 75% where imatinib fails

16 Chronic Lymphocytic Leukaemia
Commonest leukaemia, presents mostly >65 years Mostly asymptomatic incidental finding Median survival approx 10 years Diagnosis depends on lymphocyte count >5x 109/L Clinical course is variable, difficult to determine prognosis

17 CLL: Management Major decision is when to treat – 1/3 never need intervention Rai and Binet systems use presence of symptoms and results of investigations to stage – helps determine when to treat Treatment (intermittent chemo) can help the symptoms and signs, effect on life expectancy uncertain. Supportive treatments as above are important Stem cell transplant benefits under Ix in younger patients CLL can undergo lymphomatous (Richter’s) transformation (7%) – survival is short.


19 Lymphomas Commoner than leukaemias
Abnormal proliferation of lymphoid tissue – can occur anywhere lymph tissue found (LNs, spleen, thymus, GI tract...) Classifed as Hodgkin’s or Non-Hodgkin’s depending on histology

20 Hodgkin’s Lymphoma Rare, M:F = 1.3:1, 90% in over 16s. Peak incidence in 20s Mostly involves LNs Definition of HL is presence of Reed-Sternberg cells in lymph tissue on biopsy.

21 Hodgkin’s Lymphoma: Classification
Classical HL: Nodular sclerosing (70%): fibrotic bands and nodules, usually affects young adults Lymphocyte-rich HL (5%): infiltration of many small lymphocytes and R-S cells, peripheral nodes, older age. Mixed Cellularity HL (25%): M>F, associated with B symptoms Lymphocyte-depleted HL: rare. Numerous R-S cells, advanced B symptoms, associated with HIV.

22 HL: Signs & Symptoms Lymph node enlargement (usually cervical) – painless and rubbery. Enlarged spleen/liver Others include pruritis, fatigue, anorexia, alcohol-induced pain at affected LNs.

23 HL: Investigations & Treatment
LN biopsy. FBC – Hb can be normal or low. High lymphocytes. ESR raised, abnormal LFTs. CXR and CT, ? PET scan Staging by Ann Arbor classification based on number/spread affected nodes and presence of B symptoms which carry a worse prognosis. Fever, drenching night sweats, >10% weight loss in 6/12 Treatment – curative intent. Chemo and site-specific radiation. Recurrence uncommon, but 2nd/3rd remissions achievable

24 Non-Hodgkin’s Lymphoma
70% B cell, 30% T cell Viruses including HBV, herpesvirus 8, HIV all implicated Malignant proliferation of lymphocytes Signs and Symptoms: painless superficial LN enlargement, systemic B symptoms. Extra-nodal involvement inc GIT, lung, brain, testes, thyroid, skin... Ix – FBC to look for bone marrow infiltration, ESR raised, U&Es (big nodes obstruct ureters), LFTs, CXR, CT, Bone Marrow Bx, trephine Bx, LN Bx

25 NHL: Types Follicular Lymphoma: painless lymphadenopathy. Remitting/relapsing over ~10 years. Treatment: chemo-immunotherapy, radiotherapy and occasioanlly stem cell transplant. High risk transformation to diffuse large B cell lymphoma Lymphoplasmacytic lymphoma: uncommon. Extensive marrow infiltration. Management can be expectant, supportive or chemotx. Survival ~4 years Diffuse large B cell lymphoma: commonest. Without Tx fatal in months. >50% younger pts cured. Rapid infiltration of other organs, treated with chemoimmunotherapy. Burkitt’s lymphoma – endemic to Africa. Rapid progression. 30% have meningitis at presentation. 60% cure with chemo.


27 Multiple Myeloma Malignancy of plasma (B!) cells within bone marrow
Clonal expansion of abnormal proliferative plasma cells  paraproteinaemia and excretion of light chains in urine (Bence Jones protein) Disease of elderly, commoner in men

28 Multiple Myeloma Features
C alcium R enal failure A naemia B one problems If you see back pain and renal failure, think MYELOMA Bone destruction (vertebral or long bones, can  cord compression). Pain and/or pathological fractures Hypercalcaemia Bone marrow infiltration (anaemia, neutropenia, thrombocytopenia) Renal impairment from light chain deposition in tubules Recurrent infections (healthy immunoglobulins reduced)

29 Multiple Myeloma: Investigations
FBC – suggestive of marrow infiltration ESR and CRP possibly raised U&Es (renal failure) and uric acid Blood film Serum calcium Serum free light chain assay Urinary Bence-Jones protein Bone Marrow aspirate Skeletal Survey AKA: MYELOMA SCREEN


31 Multiple Myeloma: Treatment
Survival is 5-10 years if treatment of complications is successful. Chemo and steroids There is no ‘cure’ for multiple myeloma Supportive therapy as before Orthopaedic involvement in pathological fractures

32 Conclusion Haematological malignancies are COMPLICATED Lots of overlap
Symptoms are non-specific and often insidious Easier to learn if you break it down into different classifications

33 Any questions?

34 Thank you!

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