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Haematology Path Year 5 > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What is polycythaemia?

A

Polycythaemia is described as a
Raised Hb [135-175]
Raised Haematocrit [0.41-0.53]

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2
Q

How do you differentiate between relative(pseudo) and true polycthaemia?

A
  1. Relative = same red cell mass, decreased plasma volume

2. True = Increased red cell mass, same plasma volume

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3
Q

What are causes of relative polycythaemia?

A
  • Alcohol
  • Obesity
  • Diuretics
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4
Q

What are causes of secondary true polycythaemia?

A

Secondary (i.e. Reactive with a raised EPO)

i) Appropriate
- > High Altitude
- > Hypoxic Lung Disease
- > Cyanotic Heart Disease
- > High Affinity Haemoglobin

ii) Inappropriate
- > Renal Disease i.e. Cysts, Tumours & Inflammation
- > Uterine Myoma
- > Other tumours i.e. liver&lung

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5
Q

What are causes of primary true polycythaemia?

A 
True Polycythaemia 
Primary (Low EPO)
i) Philadelphia Chromosome Negative
-> Polycythaemia Vera
-> Idiopathic Erythryocytosis
-> Essential Thrombocythaemia
-> Primary Myelofibrosis

ii) Philadelphia Chromosome Positive
- > Chronic Myeloid Leukaemia

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6
Q

How does tyrosine kinase (specifically JK) normally result in cellular proliferation?

A
  • > Growth Factor binds to receptor
  • > Phosphorylation of the JAK’s
  • > Activation of the STAT’s
  • > Translocation to the nucleus and stimulate cellular proliferation
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7
Q

What does a mutation in the tyrosine kinase (JK) result in?

A

-> The signalling cascade is continously activated

Erythyroblast Mutation - Polycythaemia
Megakaryocyte Mutation - Thrombocytopenia
Granulocyte Mutation - Chronic Myeloid Leukaemia.

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8
Q

Define Polycythaemia vera

A

An increase in the production of red blood cell and surrounding plasma, often accompanied by a variable increase in platelets and granulocytic cells

Peaks at 60 years of age

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9
Q

What are the clinical features of polycythaemia vera?

A 
-> Incidental diagnosis where there is a raised Hb and Hct. 
OR
Symptoms of Blood Hyperviscosity 
-> Headaches, light headedness, stroke 
-> Visual disturbances
-> Fatigue, dyspnoea
Increased Histamine Release
-> Aquagenic Pruritis
-> Peptic Ulceration
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10
Q

What are the clinical signs of polycythaemia?

A
  • > Splenomegaly 79%
  • > Plethora - reddish complexion
  • > Erythromelalgia i.e. red painful extremities
  • > Thrombosis
  • > Retinal vein engorgement
  • > Gout increased RBC turnover, therefore increased Uric Acid
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11
Q

What are the investigations for polycythaemia vera?

A

FBC

  • > Raised Hb
  • > Raised Hct
  • > Normal/Raised WCC
  • > Normal/Raised Platelets
  • > Low EPO

Film
-> No cirulating Immature cells

Genetic testing for JK mutation

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12
Q

What is the management of polycythaemia vera?

A 
Aim to reduce HCT ie. <45%
-> Venesection
-> Cytoreductive therapy with Hydroxycarbamide (Chemotherapy)
Aim to reduce thrombotic Risk
-> Aspirin
-> Keep Platelet count <400*10^9
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13
Q

Define Essential Thrombocythaemia

A

Chronic Myeloproliferative Neoplasm mainly involving the megakaryocytic lineage
-> Sustained Thrombocytosis i.e. platelets ( OVER 600*10^9)

Peaks at 55 years of age

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14
Q

What are the clinical features of Essential Thrombocythaemia?

A 
-> 50% of cases are incidental on FBC
OR
Pro-Thrombotic
-> Arterial ; CVA, Gangrene, TIA
-> Venous ; PE, DVT
OR
Pro-Bleeding (get you a platelet that can do both x x x but seriously idk why)
-> Mucous membranes cutaneous
AND
-> Headaches, Dizziness, Visual Field Disturbances
-> Modest Splenomegaly
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15
Q

What is the management of Essential Thrombocythaemia?

A

Aspirin - Reduce Thrombotic Risk
Anagrelide - Inhibits platelet formation, however can accelerate myelofibrosis
Hydroxycarbamide - can accelerate leukaemia

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16
Q

Define Primary Myelofibrosis

A
  • > A clonal myeloproliferative disease with proliferation mainly of megakaryocytes and granulocytes.
  • > ASSOCIATED WITH REACTIVE FIBROSIS and extramedullary haematopoiesis

7th decade of life

17
Q

What are the clinical features of primary myelofibrosis?

A

-> 30% present Incidentally
Polycythaemia Vera or Essential Thrombocythaemia can progress to Primary Myelofibrosis

OR
-> Cytopenia i.e. anaemia/thrombocytopenia
-> Thrombocytosis
-> Massive Splenomegaly – Extramedullary Haematopoesis
-> Hepatomegaly – Budd-Chiari Syndrome
Hypermetabolic State i.e. Weight Loss, Fatigue, Night Sweats and Hyperuricaemia

18
Q

What is Budd Chiari syndrome?

A

Budd-Chiari syndrome is a condition in which the hepatic veins (veins that drain the liver) are blocked or narrowed by a clot (mass of blood cells). This blockage causes blood to back up into the liver, and as a result, the liver grows larger.

19
Q

What are the investigations for primary myelofibrosis?

A

Blood Film

  • > Leucoerythroblastic Picture
  • > Tear Drop Poikilocytes o.e. Dacrocyte
  • > Giant Platelets
  • > Circulating Megakaryocytes

Bone Marrow Aspirate

  • > Dry Tap
  • > Increased reticulin or collagen fibrosis
  • > Prominent megakaryocyte hyperplasia
  • > New bone formation
20
Q

What is the management of primary myelofibrosis?

A

-> Supportive i.e. Transfusions of RBC and Platelets
these are often ineffective because of splenomegaly

-> Cytoreductive therapy i.e. Hydroxycarbamide
may help the thrombocytosis but worsen anaemia

-> Splenectomy
symptomatic relief, but may worsen condition in the long run

-> Ruxolotinib; JAK2 inhibitor
reserved for those with the worst prognosis

-> Allogenic Stem Cell Transplant
potentially curative

21
Q

Define Chronic Myeloid Leukaemia

A

-> The cancer of the granulocyte precursor (Myeloblast) which results in the overproduction of Basophils, Neutrophils, Eosinophils etc.

40-60 years of age

22
Q

What are the clinical features of Chronic Myeloid Leukaemia?

A
  • > Lethargy
  • > Hypermetabolism
  • > Thrombotic event i.e. blindness, CVA secondary to leucostasis blood
  • > May also present with bruising/bleeding
  • > Hepatosplenomegaly
23
Q

What are the investigations for Chronic Myeloid Leukaemia?

A

FBC

  • > Hb and Platelets preserved if not raised; in Acute Leukaemias, you would see the Platelets/Erythrocytes drop.
  • > MASSIVE leucocytosis

Blood Film

  • > Neutro/Eosino/Baso-philia
  • > No excess blast cells.

Genetic Testing
-> (9;22) Translocation produces the BCRABL fusion oncoprotein.

24
Q

What is the 1st line treatment for Chronic Myeloid Leukaemia?

A

First Line - Imatinib, 95% 5 year survival.

Orally Active ABL Tyrosine Kinase Inhibitor, as a result it is not perpetually activated and inducing proliferation

25
Q

What are the Phildelphia Positive and Phildelphia negative polycythaemia disorders?

A

Ph +ve

CML

Ph -ve

  • > Polycythaemia Vera
  • > Idiopathic Erythryocytosis
  • > Essential Thrombocythaemia
  • > Primary Myelofibrosis
26
Q

what are the ranges for the percentage of blasts found in:

  1. Accelerated phase CML
  2. Blast Crisis
A
  1. 10-19% blasts

2. >20% blasts

Haematology Path Year 5 (5 decks)

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