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Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What is meant by the term ‘myeloproliferative disorders’?

A

Myeloproliferative disorders are clonal blood disorders. where specific blood cell types are produced in excess

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2
Q

What is Polycythaemia?

A

Polycythaemia is an increase in RBC, haemoglobin and packed cell volume

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3
Q

What is the term for Polycythaemia caused by a myeloproliferative neoplasm?

A

Polycythaemia vera

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4
Q

Describe the pathophysiology of Polycythaemia vera?

A

Almost all patients have an identical acquired mutation in the Janus kinase 2 (JAK2) gene. This mutation allows the process of erythropoiesis to occur, even in the absence of EPO

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5
Q

What are the signs and symptoms of Polycythaemia vera?

A 
Fatigue
Dizziness
Increased sweating
Pruritis (especially after a hot bath/shower)
Redness in the face
Blurred vision
Headaches
Splenomegaly
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6
Q

Individuals with Polycythaemia vera are more prone to blood clots. Therefore, what complications can result from this?

A

Stroke
MI
DVT
Budd-Chiari syndrome

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7
Q

How can Polycythaemia vera result in gout and kidney stones?

A

The increased red cell mass in Polycythaemia vera means there is a high turnover of RBCs which can lead to a build-up of uric acid. This can cause gout and kidney stones

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8
Q

The diagnosis of Polycythaemia vera requires either (1) both major criteria and one minor criteria or (2) the first major. and two minor cirtiera. What are the two major criteria for the diagnosis of Polycythaemia vera?

A

Significantly raised haematocrit

Presence of JAK2 mutation

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9
Q

The diagnosis of Polycythaemia vera requires either (1) both major criteria and one minor criteria or (2) the first major and two minor cirtiera. What are the three minor criteria for the diagnosis of Polycythaemia vera?

A

Bone marrow trephine biopsy showing hypercellularity for age. and other features of myeloproliferation
Low serum EPO
Endogenous erythroid colony formation

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10
Q

How is Polycythaemia vera treated?

A

Aspirin (75 mg/day) unless contraindicated
Venesection
Ruxolitinib (JAK2 inhibitor)
Hydroxycarbamide, interferon alpha and busulphan

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11
Q

What is the likelihood of Polycythaemia vera transforming into myelofibrosis or acute leukaemia?

A

10% chance of transforming to myelofibrosis

5% chance of transforming to acute leukaemia

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12
Q

Polycythaemia can be a physiological response to hypoxia. Give some examples of causes of hypoxia which may lead to Polycythaemia?

A 
High altitude
Hypoxic lung disease
Cyanotic congenital heart disease
Smoking
Abnormal Hb with increased O2 affinity
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13
Q

Polycythaemia can be caused by an inappropriate secretion of EPO. Give examples of conditions which might cause this

A 
Renal disease (e.g. tumours, cysts, hydronephrosis)
Hepatoma
Cerebellar haemoglioblastoma
Phaeochromocytoma
Uterine fibroids
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14
Q

What is essential thrombocythaemia?

A

ET is a chronic myeloproliferative neoplasm characterised by a persistent increase in platelet count

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15
Q

Describe the pathophysiology of essential thrombocythaemia?

A

ET is a clonal stem cell disorder. Many patients have a JAK2 mutation which allows thrombopoiesis to occur in the absence of thrombopoietin. Patients may also develop mutations in the thrombopoietin receptor MPL or the chaperone protein calreticulin. Many of the platelets produced are misshapen and dysfunctional which can lead to bleeding. But the overall increase in the number of platelets can also increased the risk of thrombosis

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16
Q

Essential thrombocythaemia can be asymptomatic. T/F?

A

True

17
Q

What are the symptoms of essential thrombocythaemia?

A 
Fatigue
Headache
Dizziness
Nausea
Tinnitus
Numbness in the hands and feet
18
Q

What diagnostic tests can be run in suspected essential thrombocythaemia?

A

FBC
Bone marrow biopsy
JAK2 mutation testing

19
Q

How is essential thrombocythaemia?

A

Some patients may be observed only
Low dose aspirin can be given
Platelet count can be lowered using hydroxyurea, interferon alpha and anagrelide
Plateletpheresis can be used for those with very high platelet counts

20
Q

The prognosis of essential thrombocythaemia is generally good though there is a risk of progression to which conditions?

A

Polycythaemia vera
Myelofibrosis
Acute myeloid leukaemia

21
Q

What is myelofibrosis?

A

Myelofibrosis is a myeloproliferative disorder characterised by bone marrow fibrosis and splenomegaly

22
Q

Myelofibrosis can develop de novo or can develop from which other conditions?

A

Polycythaemia vera

Essential thrombocythaemia

23
Q

Describe the pathophysiology of myelofibrosis

A

Myelofibrosis is a neoplastic clonal disorder originating in a single pluripotent stem cell. Abnormal megakaryocytic are produced in increased numbers and these cells release cytokines which stimulate fibroblast proliferation and the build-up of collagen in the. bone marrow. The. scarred marrow is unable. to function properly and so haematopoietic stem cells move to the spleen and liver (extramedullary haematopoiesis). But this is often not bale to fully compensate for the loss fo bone marrow haematopoiesis and so can lead to pancytopenia

24
Q

In what age group does myelofibrosis usually present?

A

> 50 years

25
Q

Myelofibrosis is insidious in onset. T/F?

A

True

26
Q

What are the signs and symptoms of myelofibrosis?

A 
Bone pain
Fever
Itching
Fatigue
Weight loss
Recurrent infections
Symptoms of anaemia
Splenomegaly
Hepatomegaly
27
Q

What are the expected FBC results in myelofibrosis?

A

There is typically an initial increase in blood cells, particularly platelets
But eventually there will be pancytopenia

28
Q

In myelofibrosis, blood smear will show abnormal teardrop shaped cells. What is the name of these cells?

A

Poikilocytes

29
Q

What are the expected bone marrow biopsy results in myelofibrosis?

A

In early disease there can be an increase in haematopoietic cell numbers
In late disease there will be decreased haematopoietic cell numbers and fibrosis

30
Q

What is the only potentially curative treatment of myelofibrosis?

A

Allogeneic stem cell transplantation

31
Q

What can be used to treat anaemia in myelofibrosis?

A

EPO

32
Q

What medication can help to relieve symptoms and particularly reduced splenomegaly in myelofibrosis?

A

JAK 2 inhibitor - ruxolitinib

33
Q

What is the prognosis of myelofibrosis?

A

Average survival is 4-7 years

Leukaemia transformation occurs in 15% of cases

Haematology (10 decks)

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