9. Myeloid Malignancies Flashcards
What are the two subtypes of acute leukaemia ?
acute lymphoblastic leukaemia- arise from lymphoid progeitor cells
acute myeloid leukaemia- arise from myeloid progenitor cells
What is acute myeloid leukaemias symptoms?
Bone marrow failure
Leads to anaemia Thrombocytopenic bleeding (purpura and mucosal membrane bleeding)
Infection due to neutropenia (predominantly bacterial and fungal)
What are the histological findings of normal bone marrow vs acute myeloid
Normal- no blast cells
Leaukemia- replacing the bone marrow with blast cells
What sort of problems are associated with leukaemia?
Echimosis-Spontaneous bleeding or bleeding with minor trauma
Bacterial infection at the end of a catheter
What are the progressions of acute leukaemia?
Leukemic cells do not differentiate
Bone marrow fails
It is rapidly fatal if left untreated
It can be potentially curable e.g. childhood acute lymphoblastic leukaemia
Good prognosis in acute myeloid leukaemia treated with chemotherapy
What are the characteristics of chronic leukaemia?
Leukemic cells retain the ability to differentiate
Proliferation without bone marrow failure
survival for a few years
hard to cure
Where do B cells proliferate?
The germinal centre of the lymph node
What are the differentials for a lymphadenopathy?
localised and painful- bacteria infection
localised and painless- rare infection (TB), metastatic carcinoma from draining site, lymphoma (rubbery),
Generalised and painful- viral
Generalised and painless- lymphoma, leukaemia, connective tissue disease, drugs
What are the two forms of chronic leukaemia
Chronic myeloid leukaemia (CML)- near mature WBC and RBC and platelets (myeloid cells)
Chronic lymphocytic leukaemia- b lymphocyte damage
What are the two types of lymphomas?
Non-hogdkin lymphoma (NHL)
Hodgkin lymphoma
both arise from B and T lymphocytes
What essential investigations should be undertaken when investigating suspected leukaemia?
Blood count and blood films
Bone marrow aspirate
What are the different options for treatment in AML?
Chemo- Daunorubicin & cytosine arabinoside (DA)
Bone marrow transplant
Chemo free- All-trans retinoic acid (ATRA) and arsenic trioxide (ATO) in low risk
Targeted treatment e.g. midostaurin
Discuss the features of AML?
BONE MARROW FAILURE
- Anaemia
- Thrombocytopenic bleeding (Purpura and mucosal membrane bleeding)
- Infection because of neutropenia
Explain the presentation of CML?
Anaemia
Splenomegaly, often massive
Weight loss
Hyperleukostasis - Fundal haemorrhage and venous congestion, altered consciousness, respiratory failure.
Gout
What findings of CML would you see on a investigations?
High WCC ( can be very high )
High platelet count
Anaemia
Blood film shows all stages of white cell differentiation with increased basophils
Bone marrow is hypercellular
nBone marrow and blood cells contain the Philadelphia chromosome - t(9;22)
How do you treat CML?
TYROSINE KINASE INHIBITORS!
e.g. imatinib, dasatinib
If that fails give bone marrow transplant
What is polycyteamia Vera?
Precursor to acute myeloid leukaemia
Causes excess of red blood cells
Symptoms of polycythemia Vera?
Headache Itch Vascular occlusion Thrombosis TIA,stroke Splenomegaly
What are the laboratory findings of PV?
raised haemoglobin concentration and haematocrit.
tendency to also have a raised white cell count and platelet count
raised uric acid
true increase in red cell mass when the blood volume is measured
How do you treat PV?
Blood letting (venesection) to keep the haematocrit below 0.45 - men and 0.43 - women
Aspirin
Hydroxcarbamide (HC)/Alpha Interferon
Ruxolitinib(JAK2 inhibitor) in HC failures with systemic symptoms
What will happen to PV in the long run?
Stroke and other arterial or venous thromboses if poorly controlled
Bone marrow failure from the development of secondary myelofibrosis
Transformation to AML
What is essential thrombocytopenia?
Myeloproliferative disease with predominant feature of raised platelet count
Precursor to AML
How does essential thrombocytopenia present
Symptoms of arterial and venous thromboses
digital ischemia
gout
headache
Mild splenomegaly
How is essential thrombocytopenia treated
Treated with aspirin and hydroxycarbamide
