Haematological Malignancies

1. Most common malignancy in children
2. High remission rate in children >95%
3. Presents as recurrent infection / bleeding or easy bruising / constitutional symptoms / signs of organ infiltration
4. Lymphocytes rapidly divide in bone marrow and enter circulation to infiltrate other organs
5. Treatment: Induce remission: chemotherapy + steroids / intrathecal if CNS involved. Maintenance chemotherapy / stem cell
6. Blood smear and bone marrow shows high presence of blast cells

Annotations:

• ALL but in adults with a worse prognosis Also doesn't infiltrate other organs

1. >50's
2. Presents as fatigue / easy bruising / fever / infection (same as ALL)
3. Caused by chromosomal abnormalities / exposure to radiation / CML
4. Blood smear: Blast cells and Auer roads / Bone marrow >20% blasts
5. Treat with chemo and supportive treatment

1. Can progress to Acute Myeloid Leukaemia
2. Insidious onset - constitutional symptoms (Chronic phase)
3. Treatment - Imatinib
4. Due to philadelphia chromosome translocation (BCR-ABL)

1. Almost always B cell proliferation - builds up in bone marrow then spreads to lymphoid spleen & liver
2. Incidental finding on FBC / asymptomatic LNs / hepatosplenomegaly
3. Don't treat until symptomatic / then treat with chemotherapy + rituximab
4. Can develop autoimmune haemolytic anaemia and secondary skin cancer

1. Often disseminated at presentation
2. Presents with painless peripheral LNs / compression symptoms e.g. SVS / constitutional Sx / Hepatosplenomegaly
3. Worse prognosis than HL. Treat with chemotherapy + RT + rituximab
4. Proliferation of all lymphocytes. May be indolent or aggressive

1. Chemotherapy gives high cure rate
2. Painless cervical lymphadenopathy / constitutional symptoms / hepatosplenomegaly
3. Proliferation of B cells - Reed - Sternberg cells
4. Ass/ with: hx of chemo or RT / EBV / HIV / Immunosuppresion
5. Bimodal age - 25 and 50
6. Incidental on CXR / Node biopsy shows RS cells within normal field / Stage with CT or PET

1. Increased production of one of haematopoetic cell lines (usually RBCs)
2. Presentation - Thrombosis / Polycythaemia / Hyperviscocity / Extramedullary haematopoeisis
3. Treatment - Aspirin / Phelbotomy / Myelosuppresion - Hydroxyurea
4. Investigations - FBC (raised Hb) / Genetic testing (JAK2 mutation)
5. Progresses to 'spent phase' - myelofibrosis

1. Megakaryocyte dysplasia and increase in PLT production
2. Presents with vasomotor Sx / bleeding / thrombotic / viscocity
3. Treatment: Aspirin
4. Blood smear shows increased PLT count and Bone marrow shows megakaryocytes
5. Exclude secondary causes e.g. reactive thrombocythaemia

Annotations:

• 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

1. Bone marrow replaced by fibrous tissue
2. Presents with anaemia and hepatosplenomegaly
3. Tear drop poikilocyte RBCs and fibrous tissue in bone marrow
4. Treat symptoms e.g. anaemia / splenectomy / chemotherapy
5. Often secondary to other cancers e.g. Lymphoma

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