Anaemias Flashcards

1
Q

What is Aplastic Anaemia?

A

A pancytopenia due to destruction of the HSC

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2
Q

Name three causes of Aplastic Anaemia

A

Genetic - Fanconi Anaemia
Radiation
Infection - HIV/EBV

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3
Q

Name two other features of Fanconi Anaemia

A

Predisposition to malignancy
Short Stature

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4
Q

Name two investigations for Aplastic Anaemia

A

Bloods (inc EPO)
BM Biopsy (dry tap)

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5
Q

How is Aplastic Anaemia managed?

A

<50 - SCT
>50 - Immunosupression +/- GCSF +/- Transfusions

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6
Q

What characterises autoimmune haemolytic anaemia?

A

Anaemia
Increased Reticulocytes
Reduced Haptoglobin
+ve Coombs
Sphero and Reticulocytes

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7
Q

What is Warm AHA?

A

IgG causes haemolysis at extravascular sites at body temperature

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8
Q

Name two associations of Warm AHA

A

CLL
SLE

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9
Q

What is Cold AHA?

A

IgM causes haemolysis at 4 degrees intravascularly generally causing raynaud symptoms alongside typical haemolytic anaemia picture

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10
Q

How is AHA managed?

A

Steroids +/- Rituximab

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11
Q

What is Beta Thalassaemia major?

A

Comlete absence of beta globulin chains presenting in first year of life with failure to thrive

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12
Q

How is Beta Thalassaemia physiologically compensated?

A

Increase in HBA2 and HBF

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13
Q

How is Beta Thalassaemia Major managed?

A

Repeated Transfusion
Desferrioxamine

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14
Q

How does Beta Thalassaemia trait present?

A

Typically asymptomatic as only reduced production

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15
Q

Name three hereditary haemolytic anaemias

A

Sickle Cell
G6PDH
Hereditary Spherocytosis

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16
Q

Name two immune haemolytic anaemias

A

AHA
Transfusion related

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17
Q

Name two non immune haemolytic anaemias

A

DIC
TTP/HUS

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18
Q

What is Hereditary Spherocytosis?

A

Most common hereditary HA

Autosomal abnormality in cytoskeleton - then destroyed by spleen

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19
Q

Name three features of H.Spherocytosis

A

Failure to thrive
Jaundice
Splenomegaly

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20
Q

How is H.Spherocytosis diagnosed?

A

Family History +Suggestive bloods + Symptoms

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21
Q

Describe the long and short term managements of H.Spherocytosis

A

Acute - Supportive +/- Transfusion
Long term = folate and splenectomy

22
Q

What age group has the highest incidence of iron deficiency anaemia?

A

PreSchool

23
Q

What is characteristic of iron deficiency anaemia on blood film?

A

Anisopoikilocytes

24
Q

Name four causes of macrocytic anaemia

A

Megaloblastic - B12 deficiency, Folate Deficiency
Non Megaloblastic - Alcohol, Liver Disease

25
Q

What is Methaemaglobinaemia?

A

Haemoglobin becomes oxidised so cannot carry oxygen

Associated - congenital, nitrates, dapsone

26
Q

How does Methaemaglobinaemia present?

A

CHocolate blood
Dyspnoea
Headaches

27
Q

How is Methaemaglobinaemia investigated?

A

Normal pO2 and reduced sats

28
Q

How is Methaemaglobinaemia managed?

A

Ascorbic Acid +/- Methylene Blue

29
Q

What are Myelodysplastic Syndromes?

A

Essentially Carcinoma in Situs

Can be Primary or Secondary (radiation or chemo)

30
Q

How do Myelodysplastic Syndromes present?

A

Reduction in various blood counts

Can either remain stable or progress to AML

31
Q

What is Myelofibrosis?

A

Overgrowth of megakaryocytes causing release of platelet growth factors and fibrosis

32
Q

How does myelofibrosis present?

A

Anaemia
Extramedullary Hameatopoiesis
Hypermetabolic sx (weight loss, night sweats)

33
Q

Name two characteristic investigations for myelofibrosis

A

Tear drop poikilocytes on blood film
Dry Tap from BM

34
Q

Give three possible managements for Myelofibrosis

A

JAK2 inhib
Thalidomide
Periodic Infusions

35
Q

What levels define Polycythamia

A

Red cells >35ml in males and 32ml in women

36
Q

Name two causes of relative polycythaemia

A

Dehydration
Stress

37
Q

Name two causes of secondary polycythaemia

A

COPD
ALtitude

38
Q

What is Polycythaemia Vera?

A

95% due to JAK2 mutation causing clonal proliferation

39
Q

Name three presenting features of PV

A

Itchiness (worse afterbath)
Plethora
Splenomegaly

40
Q

How is PV managed?

A

Hydroxyurea+ Aspirin +Therapeutic Venesection

41
Q

Describe the different phenotypes of Hb in Sickle Cell

A

HbAA
HBAS - Trait
HBSS - Sick Cell

42
Q

Why does sickle cell not develop until 4-6m?

A

As that is when foetal hB reduces

43
Q

How is Sickle Cell Diagnosed?

A

Hb Electrophoresis

44
Q

What is the long term management of sickle cell?

A

Hydroxyurea - increases HbF and reduces crises
Pneumococcal Vax

45
Q

Name the five types of sickle cell crises

A

THrombotic
Acute Chest
Aplastic
Sequestration
Haemolytic

46
Q

In general - how are sickle cell crises managed?

A

Supportive
Oxygen
Exchange Transfusions
Opiates

47
Q

What is Sideroblastic Anaemia?

A

Red cells fail to form haem properly

Can be congenital or acquired (lead, alcohol)

48
Q

What is characteristic of sideroblastic anaemia?

A

basophillic stippling

49
Q

How is sideroblastic anaemia managed?

A

Supportviely

50
Q

How is Vit B12 deficiency managed?

A

Ig IM hydroxycoalbumin 3 times a week for 2 weeks, then once every 3m

Treat B12 deficiency before folate (SCDC)