Myeloid Malignancy Flashcards

1
Q

What is the origin of bone marrow malignancies that are myeloid?

A

Red blood cells
Platelets
Granulocytes
Monocytes

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2
Q

What are the main groups of myeloid malignancies?

A

Acute Myeloid Leukaemia (AML)
Chronic Myeloid Leukaemia (CML)
Myelodysplastic Syndromes (MDS)
Myeloproliferative Diseases (MPD)

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3
Q

What are the sub groups of acute leukaemia?

A

Acute myeloblastic leukaemia

Acute lymphoblastic leukaemia

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4
Q

What are the clinical features of AML?

A

BONE MARROW FAILURE
Anaemia
Thrombocytopenic bleeding (Purpura and mucosal membrane bleeding)
Infection because of neutropenia (predominantly bacterial and fungal)

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5
Q

What essential investigations should be done in AML?

A
Blood count and blood film
Bone marrow aspirate/ trephine
Cytogenetics of leukaemic blasts
Immunophenotyping of leukaemic blasts
CSF examination if symptoms
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6
Q

What is the treatment for AML?

A

Supportive care
Anti-Leukaemia chemotherapy
Stem cell transplantation
All-Trans Retinoic Acid in APL

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7
Q

What are the clinical features of CML?

A
Anaemia
Splenomegaly, often massive
Weight loss
Hyperleukostasis - Fundal haemorrhage and venous congestion, altered consciousness, respiratory failure
Gout
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8
Q

What are the laboratory features of CML?

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
Bone marrow and blood cells contain the Philadelphia chromosome - t(9;22)

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9
Q

What is the treatment for CML?

A

Tyrosine kinase inhibitors (Imatinib, Dasatinib, Nilotinib, Busitinib, Ponatinib)
Allogeneic Transplantation

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10
Q

What are myelodysplastic syndromes?

A

Acquired clonal disorders of the bone marrow
Commonly seen in old age
Pre-Leukaemic

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11
Q

How do myelodysplastic syndromes normally present?

A

Macrocytic anaemia

Pancytopenia

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12
Q

What is the treatment available for myelodysplastic syndrome?

A

Supportive

Stem cell transplantation in younger patients

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13
Q

What are the main myeloproliferative diseases?

A

Polycythaemia Vera
Essential Thrombocythaemia
Idiopathic Myelofibrosis

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14
Q

What are the clinical features of PRV?

A
Headaches 
Itch (particularly after hot bath/shower) 
Vascular occlusion 
Thrombosis 
TIA, stroke 
Splenomegaly
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15
Q

What are the laboratory features of PRV?

A

Raised haemoglobin conc and haematocrit
Raised WCC and platelet count
Raised uric acid
Increase in red cell mass

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16
Q

What is the treatment for PRV?

A

Venesection

Aspirin

17
Q

What is essential thrombocythaemia (ET)?

A

Myeloproliferative disease with predominant feature of raised platelet count

18
Q

What are the clinical features of ET?

A

Symptoms of arterial and venous thrombuses
Digital ischaemia
Gout
Mild splenomegaly

19
Q

What is the treatment for ET?

A

Aspirin

Hydroxyurea

20
Q

What are the mutations associated with myeloproliferative diseases?

A

JAK2V617F mutation

CALR mutation