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Year 2 > Aplastic Anaemia + Polycythaemia > Flashcards

Aplastic Anaemia + Polycythaemia Flashcards

1
Q

How common is Aplastic anaemia

A

Rare

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2
Q

Defining features of aplastic anaemia

A
  1. Pancocytopenia (reduction in all major cell lines, RBC, WBC and platelets)
  2. Hypocellularity (aplasia of bone marrow)
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3
Q

Main causes of aplastic

A
  1. Inherited
  2. Idiopathic acquired (67%)
  3. Benzene, toluene, glue sniffing
  4. Chemotherapeutic drugs
  5. Antibiotics
  6. Infections
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4
Q

Pathophysiology of Aplastic anaemia

A
  1. Reduction in pluripotent stem cells together with a fault in those remaining or an immune reaction against them so they are unable to repopulate bone marrow
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5
Q

Clinical presentation of aplastic anaemia

A
  1. Anaemia
  2. Increased susceptibility to infection
  3. Bleeding
  4. Bleeding gums, bruising with minimal trauma and blood blisters in the mouth
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6
Q

What can aplastic anaemia be mistaken for

A

NHL

Myeloma

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7
Q

Blood count in aplastic

A

Pancocytopenia + low reticulocyte count

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8
Q

Bone marrow examination aplastic

A

Hypocellular marrow + increased fat spaces

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9
Q

How is aplastic treated

A
  1. Antibiotics urgently to prevent infections
  2. Red cell and platelet transfusion
  3. Bone marrow transplant
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10
Q

Describe immunosuppressive therapy after bone marrow transplant for aplastic anaemia

A
  1. ANTHITHYMOCYTE GLOBULIN and CICLOSPORIN

in over 40 or below 40 with severe disease + no HLA identical donor

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11
Q

What is polycythaemia

A

Increase in Hb, haematocrit and red cell count (all conc.)

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12
Q

Why is PCV a more reliable indicator of polycthaemia than Hb

A

Disproportionally low in iron deficiency

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13
Q

What can Polycythaemia be divided into

A

ABSOLUTE (increase in RBC mass) +RELATIVE (decreased plasma vol + normal RBC mass)

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14
Q

What is PRIMARY ABSOLUTE POLYCYTHAEMIA

A
  1. P. VERA
    MUTATIONS IN ERYTHROPOIETIN RECEPTOR
    HIGH OXYGEN AFFINITY Hbs
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15
Q

What is SECONDARY ABSOLUTE POLYCYTHAEMIA

A
  1. Hypoxia

2. High erythropoietin secretion

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16
Q

What is APPARENT POLYCYTHAEMIA

A

Chronic form associated with obesity, hypertension + high alcohol and tobacco intake

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17
Q

How is Polycythaemia Vera caused

A

Acquired mutations of gene JAK2

18
Q

How is Polycythaemia vera caused genetically

A
  1. Point Mutation that causes the substitution of phenylalanine for valine at position 617
19
Q

Role of JAK2

A

Cytoplasmic tyrosine kinase that induces signals, especially those triggered by haematopoietic growth factors such as erythropoietin

20
Q

What age group does polycythaemia vera effect

A

Over 60

21
Q

Pathophysiology caused by mutation of JAK2

A
  1. A clonal stem disorder resulting in a malignant proliferation of a clone derived from one pluripotent marrow stem cell
22
Q

What is polycythemia vera

A

Bone marrow makes too many RBCs, WBCs and platelts

23
Q

How does PV effect the bloodviscocity

A

Thicker as RBC population is larger

24
Q

Do erythroid progenitor offspring need erythropoietin to avoid apoptosis

A

No

25
Q

Complications of PV

A
  1. Thrombosis

2. Haemorrhage

26
Q

If PV is asymptomatic, how is polycythemia diagnosed

A

Detected on FBC

27
Q

What aged people are effected by PV

A

Over 60

28
Q

Symptoms caused by hyperviscoity

A
  1. Headaches
  2. Itching
  3. Tiredness
  4. Dizziness
  5. Tinnitus
  6. Visual Disturbances
29
Q

Specific symptoms of PV

A
  1. Severe itching after a hot bath or when patient is warm
  2. Erythromelalgia
  3. Gout due to increased turnover may be a feature
  4. Angina
  5. Intermittent Claudication
  6. Plethoric complexion
  7. Hepatosplenomegaly
30
Q

What is erythromelalgia

A

Burning sensation in fingers and toes

31
Q

Why does PV result in gout

A

Increased turnover may be a feature

32
Q

What is plethoric complexion

A

Congested or swollen with blood in facial skin

33
Q

What causes hepatosplenomegaly

A

Extra medullary haemopoiesis DISTINGUISHES PV from secondary causes

34
Q

What would blood count show in PV

A
  1. Raised WBC + platelets (distinguishes PV from secondary causes)

Raised Hb

Serum erythropoietin low

35
Q

What would genetic screening in PV show

A

JAKE2 mutation

36
Q

What would bone biopsy show in PV

A

Prominent erythroid

Granulocytic

Megakaryocytic proliferation

37
Q

How can PV be treated

A
  1. No cure
  2. Treatment to maintain a normal blood count
  3. ASPIRIN with all treatments
38
Q

Surgical treatment PV

A
  1. Lowers PCV and normal count

Removes 400-500mL of blood weekly to relieve symptoms

39
Q

Chemotherapy treatment in PV

A
  1. HYDROXYCARBAMIDE
  2. BULSULFAN
  3. ALLOPURINOL
40
Q

When is chemotherapy for PV given

A

Do not tolerate venesection or poorly controlled features of the disease

41
Q

When is radioactive phosphorus given for PV

A

Only in those over 70 due to increased risk of acute leukaemia

42
Q

Why is allopurinol given for PV

A

Block uric acid production thereby reduce gout

Year 2 (155 decks)

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