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Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What does myeloproliferative mean?

A
  • Myelo = bone marrow lineage (granulocytes, red cells and platelets)
  • Proliferative = rapidly multiplying
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2
Q

What are chronic myeloproliferative disorders?

A
  • Clonal haemopoietic stem cell disorders with an increased production of one or more types of haemopoietic cells
  • In contrast to acute leukaemia, maturation is relatively preserved
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3
Q

What does this blood film show?

A

Normal blood film

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4
Q

What does this blood film show?

A

Acute leukaemia

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5
Q

What does this blood film show?

A

Myeloproliferative disorder

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6
Q

How are myeloproliferative disorders divided into sub-types?

A

Presence of BCR-ABL1

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7
Q

What are examples of each of the sub-types of myeloproliferative disorders?

A
  • BCR-ABL1 positive = chronic myeloid leukaemia
  • BCR-ABL1 negative = polycythaemia rubra vera, essential thrombocythaemia, idiopathic myelofibrosis
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8
Q

In basic terms what is chronic myeloid leukaemia?

A

Over production of myeloid cells - granulocytes and their precursors +/- other lineages e.g. platelets

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9
Q

In basic terms what is polycythaemia rubra vera?

A

Over-production of red cells

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10
Q

In basic terms what is essential thrombocythaemia?

A

Over-production of platelets

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11
Q

What blood results and signs would make you suspect in a MPD?

A
  • High granulocyte count +/-
  • High RBC count/Hb +/-
  • High platelet count +/-
  • Eosinophilia/basophilia
  • Splenomegaly
  • Thrombosis in an unusual place
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12
Q

Describe the chronic phase of CML

A
  • Previously, chronic phase with intact maturation for 3-5 years, followed by blast crisis reminiscent of acute leukaemia with maturation defect
  • Fatal without stem cell/bone marrow transplantation in the chronic phase
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13
Q

What are the 3 stages of CML?

A
  1. Chronic phase
  2. Accelerated phase
  3. Blast crisis
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14
Q

What are the clinical features of CML?

A

Asymptomatic, splenomegaly, hypermetabolic symptoms, gout, priapism

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15
Q

What are some of the expected lab features of CML?

A
  • Blood count: Normal/decreased Hb
  • Leucocytosis with neutrophilia and myeloid precursors, eosinophilia, basophilia
  • Thrombocytosis
  • Bone marrow
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16
Q

What is the hallmark of CML?

A

Philadelphia chromosome

17
Q

What happens as a result of the Philadelphia chromosome?

A
  • Results in a new gene BCR-ABL1
  • The gene product is a tyrosine kinase which causes abnormal phosphorylation leading to the haematological changes in CML
18
Q

What drug is used to target the gene product of CML?

A
  • Imatinib - tyrosine kinase inhibitor
  • Usually good response to this
19
Q

What are some clinical features common to all MPDs?

A
  • Increased cell turnover - gout, fatigue, weight loss, sweats
  • Symptoms/signs due to splenomegaly
  • Marrow failure - fibrosis or leukaemic transformation
  • Thrombosis - arterial or venous including TIA, MI, abdominal vessel thrombosis, claudication
20
Q

What is the disease mechanism involved in polycythaemia rubra vera (PRV)?

A
  • High Hb/haematocrit accompanied by erythrocytosis but can have excessive production of other lineages
  • Important to distinguish from secondary polycythaemia (chronic hypoxia, smoking, epo-secreting tumour) and pseudopolycythaemia (dehydration, diuretic therapy. obesity)
21
Q

What are the clinical features of PRV?

A
  • Clinical features common to MPD
  • Headache, fatigue - remember blood viscosity is raised not plasma viscosity
  • Aquagenic puritis (itch)
22
Q

How is PRV investigated?

A
  • History + examination
  • FBC, blood film
  • JAK2 mutation status
  • Investigation for secondary/pseudo causes e.g. CXR, ABGs, drug history
  • Can do epo levels and bone marrow biopsy
23
Q

What is JAK2 mutation?

A
  • JAK2 = kinase
  • JAK2 mutations are present in over 95% of these patients
  • The mutation results in loss of auto-inhibition
  • Activation of erythropoiesis in the absence of ligand
  • Mutational analysis forms part of initial screening and has replaced a number of other tests in routine practice
24
Q

How is PRV treated?

A
  • Vensect to haematocrit <0.45
  • Aspirin
  • Cytotoxic oral chemotherapy e.g. hydroxycarbamide
25
Q

What is the underlying disease mechanism in essential thrombocythaemia (ET)?

A
  • Uncontrolled production of abnormal platelets
  • Platelet function is abnormal; thrombosis, at high levels can also cause bleeding due to acquired vWD
26
Q

What are the clinical features of ET?

A
  • Clinical features common to MPD
  • Bleeding - unpredictable risk especially at surgery
27
Q

How is ET diagnosed?

A
  • Exclude reactive thrombocytosis - blood loss, inflammation, malignancy, iron deficiency
  • Exclude CML
  • Genetics; JAK2, CALR, MPL mutations
  • Characteristic bone marrow appearances
28
Q

How is ET treated?

A
  • Anti-platelet - aspirin
  • Cytoreductive therapy to control proliferation - hydroxycarbamide, anagrelide, interferon alpha
29
Q

What are the two types of myelofibrosis?

A
  • Idiopathic
  • Post-polycythaemia or ET
30
Q

What are the lab features of idiopathic myelofibrosis?

A
  • Marrow failure
  • Bone marrow fibrosis
  • Extramedullary haematopoiesis (liver + spleen)
  • Leukoerytroblastic film appearance
  • Teardrop-shaped RBCs in peripheral blood
31
Q

What are the clinical features of myelofibrosis?

A
  • Marrow failure = anaemia, bleeding, infection
  • Splenomegaly = LUQ pain, PHT
  • Hypercatabolism
    • clinical features common to MPD
32
Q

How is myelofibrosis diagnosed?

A
  • Typical blood film (tear-drop shaped RBC and leukoerythroblastic)
  • Dry aspirate
  • Fibrosis on trephine biopsy
  • JAK2 or CALR mutation in a proportion
33
Q

What are some of the potential causes of a leukoerythroblastic film?

A
  • Reactive (sepsis)
  • Marrow infiltration
  • Myelofibrosis
34
Q

How is myelofibrosis treated?

A
  • Supportive care - blood transfusion, platelets, antibiotics
  • Allogenic stem cell transplantation in a select few
  • Splenectomy
  • JAK2 inhibitors
35
Q

Is it more common to have high blood counts due to reactive changes or MPD?

A
  • Much more common to have a reactive cause!
  • Granulocytes - infection, post-surgery, steroids
  • Platelets - infection, iron deficiency, malignancy, blood loss
  • Red cells - dehydration (pseudopolycythaemia), secondary polycythaemia

Haematology (22 decks)

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