Anaemia Flashcards

1
Q

What is Anaemia?

A

Decreased Hb in the blood such that there is inadequate oxygen delivery to tissues

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2
Q

What are the Hb levels indicating anaemia?

A

Hb <135g/L in men

Hb <115g/L in women

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3
Q

What are the Sx of anaemia?

A
Can be asymptomatic
Fatigue
Weakness
Headaches
Dyspnoea on exertion
Angina
Intermittent claudication
Palpitations
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4
Q

What are the signs of anaemia?

A

Pallor
Tachycardia
Systolic flow mumur
Cardiac failure

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5
Q

What peripheral signs are indicative of different anaemia types?

A

Koilonychia (IDA)
Jaundice (haemolytic anaemia)
Leg ulcers (sickle cell disease)
Bone marrow expansion (thalassaemia)

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6
Q

What are the three main types of anaemia?

A

Microcytic (<80fl)
Normocytic (80-96fl)
Macrocytic (>96fl)

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7
Q

What are the common causes of microcytic anaemia?

A

Iron deficiency anaemia (commonly blood loss)
Thalassemia
Lead poisoning
Sideroblastic anaemia

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8
Q

What are the common causes of normocytic anaemia?

A
Acute blood loss
Anaemia of chronic disease
Renal anaemia
Haemolytic anaemia (can be macro)
Marrow failure
Pregnancy
CT diseases
Diamorphic blood film
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9
Q

What are the common causes of macrocytic anaemia?

A
B12 deficiency
Folate deficiency
Alcohol excess
Myelodysplastic syndromes
Severe hypothyroidism
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10
Q

What are the clinical signs of IDA?

A

Koilonychia
Angular stomatitis
Brittle nails/hair

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11
Q

What are the common causes of IDA?

A
Blood loss (hookworm, heavy menstruation, GI bleed)
Dec absorption (coeliacs, antacids, post gastrectomy)
Inc demand (growth, pregnancy)
Inad intake (premature infants/prolonged breastfed)
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12
Q

What are the appropriate investigations to confirm IDA?

A

Serum Fe, ferritin, total iron binding capacity
Serum soluble transferrin receptors (increased)
Blood film

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13
Q

What signs of IDA would be present on a blood film?

A

Typically hypochromic
Sideroblasts
Signs of thalassaemia

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14
Q

What further investigations are necessary after confirming IDA?

A

Coeliac serology
GI investigation (OGD, colonoscopy)
Stool microscopy

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15
Q

How is IDA managed?

A

Address underlying cause
Oral ferrous sulphate 200mg t.d.s
Increase dietary Fe intake

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16
Q

What are the common side effects of Ferrous Sulphate?

A

Cramping, bloating
N/V
Constipation
Black stools

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17
Q

What investigations are appropriate in suspected anaemia of chronic disease?

A

Serum Fe (dec)
TIBC (dec)
STR (normal)
Ferritin (inc)

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18
Q

How long should treatment for IDA be continued?

A

Monitor after 1mo
-improvement in sx
-increase of Hb 20g/L
3mo after bloods return to normal

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19
Q

What is the Rule of 10’s?

A

Max rise in Hb in 1wk is 10g/L
If >10g/L decline in 1wk then blood is being lost
When transfusing 1 bag will raise Hb 10g/L

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20
Q

What is Paterson-Brown Kelley (Plummer Vinson) Syndrome?

A
Rare disease occurring in post-menopausal women
Characterised by
   -dysphagia
   -odynophagia
   -IDA
   -glossitis
   -chelitis
   -oesophageal webs
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21
Q

How is Paterson-Brown Kelley (Plummer Vinson) Syndrome managed?

A

Iron supplementation & mechanical widening of oesophagus

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22
Q

What is Thalassemia?

A

Genetic disorders of Hb synthesis

-deficient a/b chain synthesis

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23
Q

What are the two main types of B-thalassemia?

A

B-thalassemia minor (trait)

B-thalassemia major (Cooley’s anaemia)

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24
Q

Describe B-thalassemia minor

A

Carrier state
Usually asymptomatic, mild microcytic anaemia worsening in pregnancy
-HbA2/HbF raised

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25
Describe B-thalassemia major
Abnormality in both b-globin genes | Survival possible due to HbF
26
How does B-thalassemia major present?
``` In first year with -severe anaemia -hepatosplenomegaly -failure to thrive Facial deformities (due to extramedullary haematopoiesis) ```
27
What are the abnormalities on a blood film in B-thalassemia major?
Hypochromic microcytic cells Target cells Nucleated RBCs
28
How is B-thalassemia major managed?
Lifelong blood transfusions
29
What are the main types of a-thalassemia?
Major/hydrops fetalis/Bart's hydrops HbH disease Minor Carrier
30
Describe a-thalassemia major
Deletion of all 4 globin genes | -death in utero
31
Describe HbH disease
Deletion of 3 globin genes | -moderate microcytic anaemia w/ haemolysis
32
Describe a-thalassemia minor
Deletion of 2 globin genes | -asymptomatic carrier w/ reduced MCV
33
Describe a-thalassemia carrier
Deletion of 1 globin gene | -clinically normal
34
What is sideroblastic anaemia?
Bone marrow produces sideroblasts instead of erythrocytes
35
What causes sideroblastic anaemia?
Congenital disorder OR | Acquired in myelodysplastic syndrome
36
What are the key investigations in a macrocytic anaemia?
``` Blood film LFTs/TFTs Serum B12/folate -if B12 low --> anti-parietal Ab, anti-IF Ab, Schilling test Bone marrow biopsy ```
37
What is the Schilling test?
Assesses B12 metabolism w/ & w/o IF | Distinguishes b/w pernicious anaemia & small bowel disease
38
What is the common mechanism by which B12/folate deficiency lead to a megaloblastic anaemia?
B12 co-enzyme for activation of folate Activated folate needed for DNA synth Deficiency leads to inc erythrocyte development -large cells -trapped/destroyed in reticuloendothelial system
39
What are the dietary sources of B12?
Meat Fish Eggs Milk
40
How is B12 absorbed/stored?
Binds w/ IF (secreted by gastric parietal cells) Absorbed in terminal ileum (brush border) Stored in liver (3yr store) Excreted in bile (70% reabsorbed)
41
What are the common causes of B12 deficiency?
``` Chronic low dietary intake (vegans) Impaired binding (pernicious, congenital IF absence, gastrectomy) Small bowel disease (resection, Chron's, UC, bacterial overgrowth) ```
42
What is Pernicious anaemia?
Autoimmune disease resulting in severe B12 deficiency
43
What are the three autoantibodies that may be present in pernicious anaemia?
``` Autoantibodies against parietal cells Blocking antibodies (stop IF-B12 binding, most common) Binding antibodies (prevent IF binding to ileum) ```
44
What is subacute combined degeneration of the cord?
Simultaneous post column (LMN) & CST (UMN) loss due to B12 deficiency
45
How does subacute combined degeneration of the cord present?
``` Peripheral neuropathy Extensor plantars Brisk knee jerk but absent ankle jerk Tone/power normal Gait ataxic ```
46
What are the major dietary sources of folate?
Leafy green vegetables/offal (as DHF/THF) | -90% lost in cooking
47
How is folate absorbed & stored?
DHF/THF converted to folate in upper GI Absorbed in jejunum Low stores (3mo)
48
What are the common causes of folate deficiency?
Poor nutritional intake (diet/alcohol/anorexia) Malabsorption (Coeliac) Anti-folate drugs (trimethoprim, methotrexate, anti-convulsants) Excess physiological use (preg, lact, prematurity) Excess pathological use (haemolysis, malig, inflam)
49
How should folate deficiency be managed?
Folic acid 5mg/day PO for 4mo | -always w/ combined B12
50
How should pernicious anaemia be managed?
IM B12 initially then oral B12 for maintenance
51
What is anaemia of chronic disease?
Normochromic/hypochromic -low Fe, high ferritin, low TIBC, normal STR Rarely severe Seen in chronic infection, malignancy, CKD, rheumatoid
52
How does bone marrow failure appear on blood tests?
Hb, reticulocytes, WCs, platelets all low | Alterations on blood film
53
What are the potential causes of bone marrow failure?
``` Aplastic anaemia (idiopathic/drugs) Malignancies Metastatic disease Meylofibrosis Meylodysplasia Parviovirus infection ```
54
What is Haemolysis?
Breakdown of RBCs before 120days - intravascular or extravascular - asymptomatic --> haemolytic anaemia
55
What are the intrinsic causes of a haemolytic anaemia?
Haemaglobinopathies (sickle cell/thalassemia) Membranopathies (spherocytosis/eliptocytosis) Enzymeopathies (G6DP deficiency)
56
What are the extrinsic causes of a haemolytic anaemia?
Autoimmune disease (warm/cold) Alloimmune disease (transfusion/transplant, rhesus) Drug induced (penicillins) Paraistes (plasmodium) Microangiopathic haemolytic anaemia (DIC)
57
What are the investigation results that would indicate increased RBC breakdown?
``` Anaemia w/ raised MCV Raised bilirubin (unconjugated, pre-hepatic) Raised serum LDH ```
58
What are the investigation results that would indicate increased RBC production?
Reticulocyte count >2% OR 100*10^9/L | -will give a raised MCV
59
How can a blood film identify the cause of a haemolytic anaemia?
``` Hypochromic, microcytic = thalassemia Sickle cells = SCA Spherocytes = hereditary spherocytosis/AI haemolysis Eliptocytes = hereditary eliptocytosis Heinz bodies = G6PD deficiency ```
60
What further investigations are indicated in haemolytic anaemia?
``` Coomb's test (?immune cause) Hb electrophoresis (haemaglobinopathies) Enzyme assays Plasma haptoglobin/urinary haemosiderin -indicate intravascular haemolysis ```
61
What is Sickle Cell Anaemia?
Autosomal recessive disorder producing abnormal B-globulin chains - HbS rather than HbA - more common in african pts
62
What are the two genotypes of SCA?
HbSS - sickle cell anaemia phenotype HbAS - sickle cell trait -protective against falciparum malaria -rarely symptomatic, vaso-occlusive crises can occur in hypoxia
63
What is the underlying pathophysiology of SCA?
HbS polymerises when deoxygenated --> produces sickle cells | -fragile, haemolyse, block small vessels
64
How is SCA diagnosed?
Guthrie card Sickle cells on blood film Hb electrophoresis to confirm/distinguish variants
65
What are the sx of SCA?
Anaemia in first few months of life Acute haemolytic crises (bone infarcts/painful dactylitis) Repeated splenic infarction (CKD, CVA) Chronic haemolytic anaemia (60-90g/L)
66
What are the potential complications of SCA?
``` Hyposplenism CKD Bone necrosis Chronic leg ulcers Fe overload Long term pulmonary damage ```
67
What is the long-term management of SCA?
Folate supplementation Pneumococcal vaccination & prophylactic penicillin (hyposplenism) Hydrocycarbamide (if frequent crises) Transfusions (2-4/wk) w/ Fe chelators
68
What is the curative treatment for SCA?
Bone marrow transplantation
69
What are vaso-occlusive crises?
Occur due to micro-vascular occlusion - affect bone marrow, cause severe pain - caused by cold, infec, dehydration, hypoxia
70
How do vaso-occlusive crises present?
``` Usually severe pain Can be -mesenteric ischaemia (acute abdomen) -cerebral infarcts -priapism ```
71
What are aplastic crises?
Sudden reduction in marrow production due to parvovirus B19 | -self limiting (<2wks), may need transfusion
72
What are sequestration crises?
Pooling of blood in spleen +/- liver - presents w/ organomegaly, severe anaemia, shock - typically in children - urgent transfusion required
73
How should a sickle cell crisis be managed?
``` A-E resus, O2, IV fluids Analgesiaa FBC, reticulocytes, cross-match Screen for infec Prophylactic enoxaparin Give blood transfusion ```
74
What is G6PD deficiency?
X-linked deficiency of G6PD - most common in Africa/mediterranean males - females have mild sx
75
How does G6PD deficiency present?
Asymptomatic Oxidative crises (reduced glutathione production) -rapid anaemia & jaundice -bite & blister cells on blood film
76
What are the triggers for oxidative crises in G6PD deficiency?
Drugs (aspirin, primaquine, sulphonamides) Broad bean consumption Illness
77
How is G6PD deficiency diagnosed?
Enzyme assay 3mo after crisis
78
How is G6PD deficiency managed?
Precipitant avoidance +/- transfusion
79
What is Pyruvate Kinase deficiency?
AR condition of reduced ATP production
80
How does PK deficiency present?
Neonatal jaundice | Chronic jaundice w/ hepatosplenomegaly
81
How is PK deficiency diagnosed?
Enzyme assay
82
How is PK deficiency managed?
Well tolerated, no specific therapy needed | -splenectomy may help
83
What is Hereditary Spherocytosis?
AD membran defect --> spherical RBCs | -trapped in spleen
84
How does HS present?
Haemolysis Splenomegaly Jaundice
85
What is Hereditary Eliptocytosis?
AD defect | -asymptomatic
86
How are HS/HE managed?
Folate Splenectomy -curative, saved for severe disease
87
What is the underlying pathophysiology of an autoimmune haemolytic anaemia?
Autoantibodies --> extravascular haemolysis & spherocytosis
88
What are the common causes of AHA?
Idiopathic | 2o to lymphoproliferative disease/other AI conditions
89
What are the two types of AHA?
Warm | Cold
90
Describe warm AHA
``` IgG mediated (binding at 37o) Treat w/ steroids/immunosuppressants +/- splenectomy ```
91
Describe cold AHA
``` IgM mediated (binding at <4o) Treat w/ cold avoidance +/- chlorambucil ```
92
What is a microangiopathic anaemia?
Mechanical haemolysis caused by physical trauma in the circulation -shistocytes on blood film
93
What are the common causes of a microangiopathic anaemia?
``` Malignant HTN/Pre-eclampsia HUS Thrombotic thrombocytopenic purpura Vasculitis DIC Mechanical heart valves ```
94
What are allommune reactions?
Immune mediated BUT coomb's negative reactions linked to transplantation/transfusion/rhesus
95
What screening for anaemia takes place before surgery?
Most common abnormality pre-op - <60g/L = transfusion - <100g/L = transfusion if cardiac risk/high anticipated blood loss