HAEM - Normocytic Anaemia Flashcards

1
Q

How would blood results look in bone marrow failure?

A
Hb 
Reticulocytes 
WBC
Platelets 
All equally low
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2
Q

If there are no abnormal blasts in pancytopenic marrow - what is the diagnosis?

A

Aplastic anaemia

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3
Q

Which infecction can cause cessation of marrow erythropoesis

A

Parovirus

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4
Q

Def haemolysis

A

The breakdown of RBC before the end of their normal life span

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5
Q

Intrinsic causes of haemolysis (3)

A

Haemoglobinopathies - Sickle cell/thalassaemia
Membranopathies - spherocytosis, eliptocytosis
Enzymeopathies

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6
Q

Extrinsic causes haemolysis (5)

A
Autoimmune - warm/cold
Alloimmune - Rhesus, transfusion/transplant
Dx - pencillins 
Parasites - plasmodium 
Microangiopathic - DICq
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7
Q

Ix normocytic haemolysis

A
FBC 
Blood film 
Coomb's 
Hb electrophoresis 
Enzyme assays 
Plasma haptoglobin and urinary haemosiderin
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8
Q

Signs of haemolysis on blood count

A

Anaemia w/ incr MCV
Raised bilirubin
Raised se LDH
Reticulocyte count >2%

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9
Q

What are you looking for: haemolysis and Coomb’s test

A

RBC coated in ab/complement –> immune cause

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10
Q

Use of Hb electrophoresis in haemolysis

A

ID different haemoglobinopathies

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11
Q

Use of plasma haptoglobin + urinary haemosiderin in haemolysis

A

If plasma haptoglobin = reduced
And
Urinary Haemosiderin = present
== intravascular haemolysis

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12
Q

Genetic linkage Sickle cell anaemia

A

Autosomal recessive

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13
Q

What type of Hb is produced in Sickle cell

A

HbS

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14
Q

Who gets Sickle cell

A

people of African origin

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15
Q

What are the 2 Sickle cell genotypes

A

HBSS

HBAS

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16
Q

Which genotype –> Sickle Cell phenotype

A

HBSS

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17
Q

What positive is there to having the Sickle cell trait?

A

Protection from Malaria

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18
Q

Diagnosis Sickle Cell anaemia (3)

A

Guthrie
Sickle cells blood film
Hb electrophoresis (confirms)

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19
Q

Sx SIckle cell anaemia - first few m of life

A

Anaemia

Acute haemolytic crisis –> bone infarcts + painful dactylitis

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20
Q

What can happen if Acute haemolytic crisis is not treated in SickleCell disease

A

Splenic infarction –> hyposplenism

Repeated renal infarction –> CKD + CVA

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21
Q

Complications of having Sickle cell disease (6)

A
Hyposplenism 
CKD
Bone necrosis 
Chronic leg ulcers 
Fe overload 
LT pulmonary damage
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22
Q

Chronic treatment for Sickle Cell disease

A

Lifelong folate
Pneumococcal vaccine + prophylactic penicillin
Hydroxcarbamide
Life long transfusions + Fe chelate

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23
Q

Cure Sickle cell disease

A

BM transplant

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24
Q

Why do vasoocclusive crises occur in Sickle cell?

A

B/C microvascular occlusion

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25
Q

PS vasoocclusive crises occur in Sickle cell (4)

A
SEVERE PAIN 
Sometimes :
Mesenteric ischaemia 
Cerebral infarctions 
Priapism
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26
Q

Precipitants: vasoocclusive crises occur in Sickle cell (4)

A

Cold
Infection
Dehydration
Hypoxia

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27
Q

Cause aplastic crisis

A

Parovirus B19

28
Q

mx aplastic crisis

A

Usually self limiting

+/- transfusion

29
Q

Who gets sequestrian crises

A

Mainly children

30
Q

Sequestrian crises

A

Pooling of blood in spleeen/liver –> organomealy, anaemia + shock

31
Q

Mx sequestrian crises

A

Urgent transfusion

32
Q

2 examples of enzymopathies

A

G6PD deficiency

PK deficiency

33
Q

genetic linkage G6PD deficiency

A

X linked

34
Q

Which population suffers from G6PD deficiency?

A

African/med

35
Q

Precipitants - G6PD attacks (5)

A
Aspirin
Primaquine 
Sulphonamides 
Broad beans 
Illness
36
Q

Diagnosis G6PD deficiency

A

Enzyme assay 3 m after crises

37
Q

Tx G6PD defic

A

Ppt avoidance

Transfusion if severe

38
Q

Cells on film G6PD deficiency

A

Bite cells + blister cells

39
Q

Genetic linkage PK deficiency

A

Autosomal recessive

40
Q

PS PK deficiency

A

Neonatal jaundice

–> chronic jaundice + HS megaly

41
Q

Diagnosis PK deficiency

A

Enzyme assay

42
Q

Mx PK deficiency

A

Well tolerated

Hence not needed

43
Q

Genetic linkage Hereditary spherocytosis

A

Autosomal dominant

44
Q

RBC appearance Hereditary spherocytosis

A

Spherical

45
Q

Why do you get Sx in Hereditary spherocytosis

A

Spherical RBC = < deformable

Get trapped in spleen –> haemolysis, jaundice and splenomegaly

46
Q

Genetic linkage Hereditary Eliptocytosis

A

Autosomal dominant

47
Q

RBC appearance Hereditary Eliptocytosis

A

Ellipse/oval shaped

48
Q

Sx Hereditary Eliptocytosis

A

Mostly asymp

49
Q

Mx for membranopathies

A

Folate

Splenectomy - curative but only in severe cases

50
Q

e.g.s of Coomb’s +ve extrinsic causes of haemolysis (2)

A

Autoimmune haemolytic anaemia AHA

Drug induced haemolysis

51
Q

Cause AHA

A

Commonly idiopathic
Or 2’
to lymphoproliferative disorders

52
Q

How are AHA’s classified

A

On optimal Temp antibodies bind to RBC in vitro

53
Q

What are the 2 types of AHA

A

Warm AHA

Cold AHA

54
Q

Binding temperature warm AHA

A

37’

55
Q

Which immune Ig is warm AHA mediated by?

A

IgG

56
Q

Mx warm AHA

A

steroids
+/- immunosuppressants
+/- splenectomy

57
Q

Binding temperature cold AHA

A

4’

58
Q

What condition is cold AHA associated with?

A

Raynauds

59
Q

Which immune Ig is cold AHA mediated by?

A

IgM

60
Q

Mx cold AHA

A

Cold avoidance

Chlorambucil

61
Q

Drug induced haemolysis - pencillins

A

Formation RBC antibodies

62
Q

Drug induced haemolysis - quinine

A

Production of immune complexes

63
Q

Infection –> Coomb’s -ve haemolysis

A

Malaria/Parasites

64
Q

E.g.s of microangiopathic anaemia (6)

A
Malig HTN/pre-eclampsia 
HUS
TTP
DIC
SLE/vasculitis 
Mechanical heart valve
65
Q

Blood film appearance microangiopathic anaemia

A

Schistocytes

66
Q

Which category do alloimmune reactions haemolysis fall into?

A

Immune mediated
But
Coombs negative

67
Q

What anaemia levels before surgery req transfusion?

A

<60g/L