Haematological Malignancies

Haematological Malignancies

1. Leukaemias

   • Acute Lymphocytic Leukaemia
     • Most common malignancy in children
     • High remission rate in children >95%
     • Presents as recurrent infection / bleeding or easy bruising / constitutional symptoms / signs of organ infiltration
     • Lymphocytes rapidly divide in bone marrow and enter circulation to infiltrate other organs
     • Treatment: Induce remission: chemotherapy + steroids / intrathecal if CNS involved. Maintenance chemotherapy / stem cell
     • Blood smear and bone marrow shows high presence of blast cells
   • Acute Myeloid Leukaemia
     • ALL but in adults with a worse prognosis Also doesn't infiltrate other organs
     • >50's
     • Presents as fatigue / easy bruising / fever / infection (same as ALL)
     • Caused by chromosomal abnormalities / exposure to radiation / CML
     • Blood smear: Blast cells and Auer roads / Bone marrow >20% blasts
     • Treat with chemo and supportive treatment
   • Chronic Myeloid Leukaemia
     • Can progress to Acute Myeloid Leukaemia
     • Insidious onset - constitutional symptoms (Chronic phase)
     • Treatment - Imatinib
     • Due to philadelphia chromosome translocation (BCR-ABL)
   • Chronic Lymphocytic Leukaemia
     • Almost always B cell proliferation - builds up in bone marrow then spreads to lymphoid spleen & liver
     • Incidental finding on FBC / asymptomatic LNs / hepatosplenomegaly
     • Don't treat until symptomatic / then treat with chemotherapy + rituximab
     • Can develop autoimmune haemolytic anaemia and secondary skin cancer

2. Lymphomas

   • Non-Hodgkin Lymphoma
     • Often disseminated at presentation
     • Presents with painless peripheral LNs / compression symptoms e.g. SVS / constitutional Sx / Hepatosplenomegaly
     • Worse prognosis than HL. Treat with chemotherapy + RT + rituximab
     • Proliferation of all lymphocytes. May be indolent or aggressive
   • Hodgkin Lymphoma
     • Chemotherapy gives high cure rate
     • Painless cervical lymphadenopathy / constitutional symptoms / hepatosplenomegaly
     • Proliferation of B cells - Reed - Sternberg cells
     • Ass/ with: hx of chemo or RT / EBV / HIV / Immunosuppresion
     • Bimodal age - 25 and 50
     • Incidental on CXR / Node biopsy shows RS cells within normal field / Stage with CT or PET

3. Myeloproliferative disorders

   • Polycythaemia Vera
     • Increased production of one of haematopoetic cell lines (usually RBCs)
     • Presentation - Thrombosis / Polycythaemia / Hyperviscocity / Extramedullary haematopoeisis
     • Treatment - Aspirin / Phelbotomy / Myelosuppresion - Hydroxyurea
     • Investigations - FBC (raised Hb) / Genetic testing (JAK2 mutation)
     • Progresses to 'spent phase' - myelofibrosis
   • Essential Thrombocythaemia
     • Megakaryocyte dysplasia and increase in PLT production
     • Presents with vasomotor Sx / bleeding / thrombotic / viscocity
     • Treatment: Aspirin
     • Blood smear shows increased PLT count and Bone marrow shows megakaryocytes
     • Exclude secondary causes e.g. reactive thrombocythaemia
   • Primary Myelofibrosis
     • PATHOPHYS Replacement of bone marrow tissue with fibrous tissue from fibroblasts / Reduction in production of normal haematopoesis - leads to extramed haematopoeisis. CAUSE Primary or secondary to malignancyPRESENTATIONAnaemiaHepatosplenomegalyINVESTIGATIONTear drop RBC poikilocytes in circulation / bone marrow aspiration shows fibrous tissue (dry)TREATMENTTreat symptoms e.g. anaemia with EPO / transfusions Splenectomy Chemotherapy
     • Bone marrow replaced by fibrous tissue
     • Presents with anaemia and hepatosplenomegaly
     • Tear drop poikilocyte RBCs and fibrous tissue in bone marrow
     • Treat symptoms e.g. anaemia / splenectomy / chemotherapy
     • Often secondary to other cancers e.g. Lymphoma

4. Plasma Cell Malignancies

   • Multiple Myeloma
     • High protein levels in the blood and urine (Bence Jones Proteinuria) - nephrotic syndrome
     • X-ray: Lytic lesions in bones due to bone infiltration (leads to hypercalcaemia
     • Treatment: Chemo / Bisphosphonates / supportive
     • Persistent back pain and recurrent infections
     • Presentation (CRAB) Hypercalcaemia / Renal failure / Anaemia /Bone dysfunciton