Haematological Malignancies

benryan95's version from 2016-01-17 22:56

Section 1

Question Answer
What do patients with acute leukaemias present with?Pancytopenia --> anaemia, infections, bleeding
What is the time between diagnosis and death in acute leukaemias if untreated?6-12 weeks
What is first line treatment for acute leukaemias?Chemotherapy, as it is chemosensitive
What is the best initial test in acute leukaemia?Blood smear
What is present on blood smear in acute leukaemia?Blast cells
What is more likely to present with cytopenia, acute or chronic leukaemia?Acute
What is the time between diagnosis and death in chronic leukaemias if untreated?6-12 years
What is the best initial test in chronic leukaemias?Full blood count
What would you see in full blood count in chronic leukaemia?WCC always high, always look at white cell differential
What is meant by leukaemia?Proliferation of progenitor cells in the bone marrow, resulting in replacement of normal haematopoetic cells and bone marrow failure

Section 2

Question Answer
What are 2 acquired risk factors for acute leukaemia?Cytotoxic chemotherapy, haematological malignancies (myelodysplasia)
What are 3 inherited risk factors for acute leukaemia?DNA repair defects, immune defects, or other (e.g. Down Syndrome)
What is the commonest type of childhood acute leukaemia?ALL
What % of ALL are derived from B cell precursors?85%
What are 3 signs found in ALL?Splenomegaly (10-20%), lymphadenopathy, and CNS signs
What could you see on full blood count of ALL?Anaemia, WBCs usually low,
What is the commonest type of adult acute leukaemia?AML (90%)
Name a significant class 1 mutation in AML (and CML)?BCR/ABL
What is the name of the red marks found on skin in AML?Leukaemia cutis
Is lymphadenopathy common in AML?No
What can be given in tumour lysis syndrome?Allopurinol
How long should induction phase of chemotherapy last in acute leukaemia?4-6 weeks

Section 3

Question Answer
What are 3 acquired risk factors for acute leukaemia?Radiation (CML), immunodeficiency, pesticides
What % of CLL come from B cells?98%
How does CLL usually present?It is usually asymptomatic
What is found on examination in CLL?Lymphadenopathy/splenomegaly
What is found on full blood count in CLL?WBCs high
What is Richter syndrome?Transformation of CLL to small lymphocytic lymphoma
Is CML more or less common than AML and CLL?Less common
What genetic risk factor is strongly associated with CML?Philadelphia chromosome (translocation of 9:22 - fusion of BCR/ABL and tyrosine kinase)
How does CML usually present?It is usually asymptomatic
What is found on examination and full blood count in CMLSplenomegaly late on, anaemia, high WBCs
How is CLL treated?Watchful waiting, there are indications for chemotherapy (imatinib)
What is imatinib?Tyrosine kinase inhibitor, targets BCR/ABL1

Section 4

Question Answer
What are the 4 myeloproliferative disorders?Polycythaemic vera, essential thrombocytosis, myelofibrosis, CML
What mutation are myeloproliferative disorders associated with?JAK2
Name 4 clinical features of polycytaemic veraNight sweats, splenomegaly, plethoric face, hyperviscosity symptoms
What are 3 investigation findings in polycythaemic vera?High Hb, low EPO, JAK2
How can polycythaemic vera be treated?Venesection, cytotoxic myelosuppresion

Section 5

Question Answer
Where does Hodgkin's lymphoma originate from?B cells in the germinal centre of lymphoid tissue
What is Hodgkin's lymphoma characterised by?Orderly spread from one lymph node to the other
What % of lymphomas are Hodgkin's?30%
Describe the distribution of Hodgkin's lymphoma?Bimodal, peaking between 15-30, and other 50
Name 3 acquired risk factors for Hodgkin's lymphoma?HIV, previous Non-Hodgkin's, autoimmune conditions
What cells can you see on blood film in Hodgkin's lymphoma?Reed-sternburg cells
What is found on presentation on Hodgkin's lymphoma?Painless, non-tender lymph node (most likely cervical, also axillary and inguinal) alcohol induced pain at lymph nodes, fever, pruritus, splenomegaly
What staging is used for Hodgkin's lymphoma?Ann-arbour staging
What can be used for treatment of Hodgkin's lymphoma?Radiotherapy and chemotherapy

Section 6

Question Answer
Where do most Non-Hodgkin's lymphoma originate from?Lymphoid B cells
What are Non-Hodgkin's lymphoma characterised by?Irregular pattern of spread and common extranodal disease
What are the 2 most common types of non-Hodgkin's lymphoma?Diffuse Large B cell and follicular
Name 4 acquired risk factors for Non-Hodgkin's lymphomaInfection (EBV, H. pylori, Hep C), previous chemotherapy, autoimmune disorders, immunodeficiency
Name 7 findings in Non-Hodgkin's lymphoma?Enlarged lymph nodes, systemic symptoms, rash, abdominal pain, mass (testicular/GI), respiratory problems, neurological problems
What can be used for treatment of Non-Hodgkin's lymphoma/Chemotherapy, Radiotherapy, surgery

Section 7

Question Answer
Name 3 paraproteinaemiasMultiple Myeloma, MGUS and Waldenstrom's macroglobulinaemia
Describe the presentation of myelomaCRAB HAI - HyperCalcaemia, Renal impairment, Anaemia, Bone disease, Hyperviscosity, Amyloidosis, Infection
What are the 3 components of myeloma diagnosis?Production of a single monoclonal antibody, increased clonal plasma cells in bone marrow, evidence of organ damage
Describe the MM management?Incurable, supportive, mean survival 3-4 years, cycles of chemotherapy
How is MGUS often found?Incidentally in the elderly, often benign
What is MGUS characterised by?Low levels of paraprotein, normal Ig, low plasma cells in bone marrow, absence of bone lytic lesions and end organ damage, 1% per year develop multiple myeloma
What is Waldenstrom's macroglobunlinaemia characterised by?Lymphoplasmacytoid proliferation, IgM paraprotein, organomegaly and lymphadenopathy, no end organ damage