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Haematology and Immunology > Myeloid Malignancy > Flashcards

Myeloid Malignancy Flashcards

1
Q

Myeloid Malignancy

A

Acute Myeloid Leukaemia (AML)

Chronic Myeloid Leukaemia (CML)

Myelodysplastic Syndromes (MDS)

Myeloproliferative Diseases (MPD)

All arise from mutational events (acquired) in the stem cell compartment. Different mutations lead to different phenotypes of disease.

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2
Q

Clinical features of AML

A

Bone marrow failure

  • anaemia
  • thrombocytopenic bleeding (purpura and mucosal membrane bleeding)
  • infection because of neutropenia which is predominantly bacteria and fungal
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3
Q

Investigations in AML

A

Blood count and blood film
Bone marrow aspirate/ trephine biopsy
Cytogenetics of leukaemic blasts (karyotyping)
Immunophenotyping of leukaemic blasts
CSF examination if symptoms
Molecular genetics for associated acquired gene mutations (FLT3, NPM1, IDH1 and 2)

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4
Q

Treatment of AML

A

Supportive Care

Anti-leukaemic Chemotherapy (Intensive chemotherapy, produces complete and absolute neutropenia for 2/3 weeks)

Stem Cell Transplantation
- Allogeneic

All-trans Retinoic Acid and Arsenic Trioxide which leads to differentiation of leukaemic blasts

Mylotarg

Midostaurin

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5
Q

Science of CML

A

translocation 9-22 (phildelphia chromosome) at the stem cell area. This leads to proliferation and differentiation. Marked accumulation of Neutrophils, Eosinophils, Basophils, Monocytes etc.

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6
Q

Presentation of CML

A 
Anaemia
Splenomegaly 
Weight loss due to hypercatabolic 
Hyperleukostasis - fundal haemorrhage and venous congestion resulting in altered consciousness and respiratory failure
Gout
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7
Q

Lab 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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8
Q

Treatment of CML

A 
Imatinib (Glivec)
Dasatinib (Sprycel)
Nilotinib  (Tasigna)
Busitinib
Ponatinib

These are all tyrosine kinase inhibitors (direct inhibitors of BCR-ABL)

Allogeneic Transplantation in TKI failure

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9
Q

Myelodysplastic Syndromes

A

Pre-leukaemic acquired clonal disorders of the bone marrow whoch are commonly seen in old age

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10
Q

Presentation of myelodysplastic syndromes

A

Macrocytic anaemia and pancytopenia (bone marrow failure)

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11
Q

Why are myelodysplastic syndromes fatal

A

Progression to bone marrow failure or AML

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12
Q

Treatment of myelodysplastic syndromes

A

Supportive or allogeneic stem cell transplantation for the few young patients.

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13
Q

Myeloproliferative Diseases

A

Polycythaemia Vera (overproduction of red cells)

Essential Thrombocythaemia (overproduction of platelets)

Idiopathic Myelofibrosis (proliferation of megalokaryocytes which leads to accumulation of chemicals which result in fibrosis of bone marrow)

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14
Q

Findings of Polycythaemic Vera

A

Increased haematocrit (70%), increased in red cell, white cell and megalocaryocytes. Slight bone marrow fibrosis.

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15
Q

Genetic abnormality in Myeloproliferative Neoplasms

A

JAK2 gene V617F Mutation

In the mutation, the proliferative pathways are switched on all the time, they are independent of regulator (erythropoetin)

Most commonly occurs in polycythaemia vera

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16
Q

Clinical features of PRV

A 
Headaches
Itch 
Vascular occlusion 
Thrombosis
TIA
Splenomegaly
17
Q

Lab features of PV

A

A raised haemoglobin concentration and haematocrit.
A tendency to also have a raised white cell count and platelet count
A raised uric acid
A true increase in red cell mass when the blood volume is measured

18
Q

Treatment of PRV

A

Venesection to keep the haematocrit below 0.45 - men and 0.43 - women

Aspirin

Hydorxcarbamide

19
Q

Natural history of PRV

A

Stroke and other arterial or venous thromboses if poorly controlled

Bone marrow failure from the development of secondary myelofibrosis

Transformation to AML

20
Q

Essential Thrombocythaemia

A

Myeloproliferative disease with predominant feature of raised platelet count
50% positive for JAK2V617F mutation
Symptoms of arterial and venous thromboses, digital ischaemia, gout
Mild splenomegaly
Treated with aspirin and hydroxyurea
Can progress to myelofibrosis or AML

Haematology and Immunology (20 decks)

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