9. Myeloid Malignancies Flashcards

1
Q

What are the two subtypes of acute leukaemia ?

A

acute lymphoblastic leukaemia- arise from lymphoid progeitor cells
acute myeloid leukaemia- arise from myeloid progenitor cells

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2
Q

What is acute myeloid leukaemias symptoms?

A

Bone marrow failure

Leads to anaemia
Thrombocytopenic bleeding (purpura and mucosal membrane bleeding)

Infection due to neutropenia (predominantly bacterial and fungal)

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3
Q

What are the histological findings of normal bone marrow vs acute myeloid

A

Normal- no blast cells

Leaukemia- replacing the bone marrow with blast cells

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4
Q

What sort of problems are associated with leukaemia?

A

Echimosis-Spontaneous bleeding or bleeding with minor trauma

Bacterial infection at the end of a catheter

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5
Q

What are the progressions of acute leukaemia?

A

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

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6
Q

What are the characteristics of chronic leukaemia?

A

Leukemic cells retain the ability to differentiate

Proliferation without bone marrow failure

survival for a few years

hard to cure

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7
Q

Where do B cells proliferate?

A

The germinal centre of the lymph node

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8
Q

What are the differentials for a lymphadenopathy?

A

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

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9
Q

What are the two forms of chronic leukaemia

A

Chronic myeloid leukaemia (CML)- near mature WBC and RBC and platelets (myeloid cells)

Chronic lymphocytic leukaemia- b lymphocyte damage

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10
Q

What are the two types of lymphomas?

A

Non-hogdkin lymphoma (NHL)
Hodgkin lymphoma

both arise from B and T lymphocytes

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11
Q

What essential investigations should be undertaken when investigating suspected leukaemia?

A

Blood count and blood films

Bone marrow aspirate

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12
Q

What are the different options for treatment in AML?

A

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

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13
Q

Discuss the features of AML?

A

BONE MARROW FAILURE

  • Anaemia
  • Thrombocytopenic bleeding (Purpura and mucosal membrane bleeding)
  • Infection because of neutropenia
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14
Q

Explain the presentation of CML?

A

Anaemia

Splenomegaly, often massive

Weight loss

Hyperleukostasis - Fundal haemorrhage and venous congestion, altered consciousness, respiratory failure.

Gout

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15
Q

What findings of CML would you see on a investigations?

A

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)

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16
Q

How do you treat CML?

A

TYROSINE KINASE INHIBITORS!

e.g. imatinib, dasatinib

If that fails give bone marrow transplant

17
Q

What is polycyteamia Vera?

A

Precursor to acute myeloid leukaemia

Causes excess of red blood cells

18
Q

Symptoms of polycythemia Vera?

A
Headache
Itch
Vascular occlusion
Thrombosis
TIA,stroke
Splenomegaly
19
Q

What are the laboratory findings of PV?

A

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

20
Q

How do you treat PV?

A

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

21
Q

What will happen to PV in the long run?

A

Stroke and other arterial or venous thromboses if poorly controlled

Bone marrow failure from the development of secondary myelofibrosis

Transformation to AML

22
Q

What is essential thrombocytopenia?

A

Myeloproliferative disease with predominant feature of raised platelet count

Precursor to AML

23
Q

How does essential thrombocytopenia present

A

Symptoms of arterial and venous thromboses

digital ischemia

gout

headache

Mild splenomegaly

24
Q

How is essential thrombocytopenia treated

A

Treated with aspirin and hydroxycarbamide