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Anaemia/iron/haemochromatosis Flashcards

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1
Q

When do you look for iron deficiency

A

Microcytic anaemia
Symptoms of
At risk of/prev IDA- heavy menstrual, veggie
Pre surgery - avoid transfusion
Standard pregnancy management - booking and 28 weeks

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2
Q

Iron deficiency symtpoms

A

Anaemia
Fatigue, poor conc, low mood
Hair thinning
Itch
Restless legs
Koilonychia, glossitis, pica

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3
Q

causes IDA

A

Diet - veggie
blood loss
malabsorption
Increased requirement for iron eg pregnancy

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4
Q

Blood loss causing IDA

A

Menstrual
GI bleed - ulcers, malignancy, inflammation, parasites
Blood donation

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5
Q

Who qualifies for an endoscopy/colonsocopy from IDA

A

All men and post menopausal women considered unless clearly non GI bleed

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6
Q

Investigation of IDA

A

Colonsocopy, endoscopy
Malabsorption - coeliac screen, helicobacter pylori
Urinalysis
Blood tests

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7
Q

What see on FBC in IDA

A

Hb - low or nomal
MCV<80 (some may be normal)
MCH <27
RBC - low/normal, RDW - normal/high

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8
Q

Blood film IDA

A

Hypochromia, microcytosis, pencil cells

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9
Q

Why does a normal or high ferritin not necessarily exclude IDA

A

High with inflammation and liver disease

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10
Q

What ferritin evels suggest IDA

A

<15 = no stores - pathogonomic
<30 - IDA
30-50 borderline/probable
<100 - = inflammation = possible IDA

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11
Q

Iron studies

A

Serum iron
Total binding iron capacity
Transferrin saturaiton
Not very useful IRL

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12
Q

Secondary tests for iron deficiency

A

Iron studies
Reticulocyte Hb <28
% hypochromic cells >6%
Zinc protoporphyrin >80
Oral trial of iron - improves by 20g/L in 3 weeks/Hb increases

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13
Q

Low MCV causes

A

IDA
Thalassemia/Hbinopathy
Anaemia of chronic disease
Lead poisonning
Inherited sideroblastic anaemia

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14
Q

Treating IDA

A

Diet
100-200mg elemental iron per day, 3 months after normal Hb
Aim for ferritin >50

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15
Q

What dietary iron is best absorbed

A

Haem iron from meat or fish

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16
Q

How is iron absorbed better

A

Prior to food - acid stomach
With vit C - fresh orange juice
Avoid antacids and tannins

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17
Q

What to do if oral iron not tolerated

A

Reduce dose to alternate days
Lower elemental iron/change formulation
Take w vit C, with food
Treat inflam conditions
Laxatives if constipated
Ensure compliance and no malabsorption, no haematinic deficiency

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18
Q

How does parenteral difffer to oral iron in terms of Hb

A

May initially work faster
4-6 weeks similar levels of Hb to oral iron

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19
Q

When is parenteral iron useful

A

Inflammation
Dialysis
Not tolerated oral despite trying
High levels of blood loss eg HereditaryHT elagniectasia

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20
Q

Complications pareneteral iron

A

Rare anyphylaxis, Low PO4, extravasation

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21
Q

What is hyperferritinaemia

A

Ferritin levels are high, doesnt necessarily mean iron is high

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22
Q

Causes of hyperferritinaemia

A

Inflammation incl AI disease, infection, malignancy
Liver disease, metabolic syndrome
Genetic haemohc=chromatosis
Renal failure
Myedodysplasia
THalassemia intermedia/major + other rare anaemias
Chronic blood transfusion
Porpphyria cutanea tarda
Other rare syndromes eg hereditary hyperferritenemia cataract syndrome, gauchers, acaeruloplasminaemia, friedrichs ataxia

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23
Q

What is high ferritin in men vs women

A

> 300 men
200 women

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24
Q

What do next when raised ferritin

A

Check FBC, U+Es, LFs, inflam markers, transferrin saturation

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25
What does a high ferritin and abnormal FBC warrant investigatio for
Iron loaing apaemia Haemolysis Myedoplasia Thalassemia
26
When test for genetic haemochromatosis when high ferritin?
When trasnferrin saturations also high eg >50% in men >40% in women Check HFE genotyping
27
What can causes an isolated high ferritin
inflammation, malignancy, liver disease, metabolic syndrome etc
28
Genetic haemochromatosis why often not diagnosed
Often no clinically significnat iron overloading
29
Iron overlaoding symtpoms in haemochromatosis
Joint damage, osteoporosis Endocrine - diabetes, gonadal sailure Bronzed skin Liver failure/cirrhosis HCC Cardiac iron loading
30
Does high ferritin always cause iron overloading symptoms
No - only in people who have genetic haemochromatosis
31
Managment of haemochromatoss
Venesection 3-4 x a year aim for ferritin <50ug/L Check for complications, family Iron chelation if cant do venesection Avoid vit C, reduce alcohol
32
Four initial tests for anaemia
Hb MCV Reticulocyte count #Blood film/smear
33
If MCV <80 what differnetial anaemias suspect
IDA Thalassemia Anaemia of chronic disease Lead poisonning Sideroblastic
34
What does a normal MCV and low reticulocyte count suggest differentials
Bone marrow failure BM infiltration Organ failure/endocrinopathy
35
What does a normal MCV and high reticulocyte count suggest differentials
Bleeding Haemolysis Treated nutritionaldeficinecy
36
What differentials when MCV >100 eg macrocyticc
B12/folate deficinecy Liver disease Alcohol Drugs - methotraxtae, sulfasalazine, ART thryoid disease
37
What reticulocyte level would you expect in an anaemic patient with a healthy bone marrow response?
High - increased erythropoiesus
38
What are reticulocytes
Immature RBCs
39
Clinical features of haemolytic anaemias
Pallor Jaundice Gallstones pigmenet Splnomegaly, may have hepatomegaly
40
3 lab features common to all haemolytic anaemias
Reticulocytosis Elevated LDH Eleated indirect - conjugated - bilirubin
41
What ceclls see in warm AI haemolytic anaemia (WAIHA)
Spherocytes
42
What morpholoy see in cold AIHA
Agglutination
43
What cells see in microangiopathic haemolytic anaemia -MAHA
Shistocytes, some spherocytes
44
What cells see in oxidative haemolyiss
Bite cells
45
What does intravascular haemolysis occur in
ABO incompatability G6PD defiency PNH - Paroxysmal nocturnal hemoglobinuria (complement destorys RBC) PCH - AI after cold exposure, also complement Clostridial sepsis (c.difficile) Mechanical valve Severe burns
46
Characteristics of IV haemolysis
- plasma free haemoglobin (hemoglobinemia) - decreased haptoglobin (bound by free Hb) - increased methaemoglobin (heme with Fe3+) - haemoglobinuria and hemosiderinuria - chronically, may lead to iron deficiency
47
What conditions see extravascular haemolysis in
RES, most causes of haemolytic anaemia
48
Intrinsic RBC anaemia - congenital - where effected in cell by what disease
1) membrane – hereditary spherocytosis, elliptocytosis 2) haemoglobin – sickle cell disease, thalassaemia’s 3) enzymes – G6PD, PK deficiency
49
Secondary causes of WAIHA
Lymphoma Drugs Connective tissue disease Causes IgG AB to antigen on RBC
50
Treating WAIHA
Primarily extravascular haemolysis - Treatment: steroids, treat underlying cause - Azathioprine, cyclophosphamide, etc for steroid-sparing
51
Secondary causes Cold AIHA
lymphoma (adults), infections (children; EBV, Mycoplasma pneumoniae)
52
Antibodies CAIHA vs WAIHA
cold = IgM, antibody target I or i warm = IgG, many targets incl Rh
53
How does CAIHA work
- Primarily intravascular haemolysis; degree of haemolysis determined by thermal amplitude of cold agglutinin
54
Treatment CAIHA
NOT STEROIDS - dont work Supportive in children underlying cause adults Cyclophosphamide TRANSFUSE through blood warmer
55
Paroxysmal cold haemoglobinuria summary
– IgG Ab against p (globoside) antigen, binds RBC at 4C, activates complement when re-warmed to 37C: associated with viral infections, syphilis.
56
Alloimmune anaemias
haemolytic transfusion reactions (acute and delayed), haemolytic disease of the new born.
57
Summary of PNH
– acquired defect in PIG-A gene, unable to anchor proteins that require PIP, complement-mediated chronic intravascular haemolysis marked by acute attacks
58
Drug induced haemolysis causing drugs
Drug absorption (penicillin) ii. Membrane modification (cephalosporin) iii. Immune complex (quinine) iv. Autoantibody – levodopa, methyldopa
59
Non immune mediated anaemias
RBC fragmentation syndrome Infection Toxins Chemical agents eg copper, arsenic, hypophosphatemia Burns, downing, march heamoglobinuria
60
RBC fragmentation syndromes
Cardiac (valves, bypass, AVMs) ii. MAHA – TTP/HUS, malignant HTN, DIC, HELPP, pre-eclampsia
61
When do you suspect Beta thalassemia in Hb electrophoresis
Hb A2<3.5%
62
Electrophoresis and microcytic anaemia
* Suspect β thalassaemia when Hb A2 >3.5% Normal Hb electrophoresis does not exclude α thalassaemia or iron deficiency  In concomitant iron deficiency, Hb electrophoresis may be falsely normal; therefore, it should be repeated once iron stores are replete  If α-thalassaemia suspected, order DNA testing
63
What rule out in microytic anaemia
 GI investigations, rule out intravascular haemolysis, celiac disease, H .pylori, congenital
64
When expect Hb to respond to iron in IDA
4-6 weeks If doenst further investigations
65
What decereases iron absorption
 Inhibited by calcium, tea, coffee, phytic acid (grains, legumes) and  decreased gastric acidity (proton pump PPI)
66
Side effects of iron
nausea, vomiting, dyspepsia, constipation, diarrhoea, dark stools; generally dose-related and usually subside with continued therapy (except dark stools)
67
Oral preps of iron
 Fumarate 210mg = 65 mg elemental iron  Gluconate 300mg = 35 mg elemental iron  Sulphate 200mg = 65 mg elemental iron  Feredetate 190 mg/5ml = 27.5 mg/5ml elemental iron  Maltol 30mg = 30 mg elemental iron
68
What is HHT
Hereditary hemorrhagic telangiectasia, also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain. cause bleeding, known as arteriovenous malformations (AVMs).
69
Normal HbA1c
Men - 130g/L Women - 115g/L Varies with age
70
Lifespan of RBC
3 weeks for erythropoeisis 3 month life cycle in circulation
71
What stimulates erythropoietin release
Decreased oxygen
72
Causes of deveased RBC production
Reduced EPO response - kidney or chronic inflammation Raw material deficiencies eg chronic inflam - less use iron Reduced bone marrow production - infiltration (cancer, infection), aplastic anaemia, myelosupression (drug, infection)
73
INfections and drugs causing myelosupression
Chloramphenicol, alcohol Chemo DMARDs Propothyouracil, carbimaxole Tacrolimus Hydroxyurea Parvovirus
74
Causes increased RBC destruction
Haemolytic anaemias - AI, drug induced, hered spherocytosis Haemoglobinopathies Hypersplenism - RBCs out of circulation eg portal hypertenion
75
What can low folate indicate
Increased demand for RBC eg pregnancy, lactating
76
investigations in microcytic anaemia
Ferritin/iron studies Further depends on symptoms Consider haemoglobinopathy screen
77
investigations for normocytic anaemia
Blood film, reticulocytes (low - bone marrow) U+Es, LFTs CRP Ferritin/B12/folate HIV
78
Macrocytic anaemia investiations
Blood film Reticulocutes B12/folate LFTs, U+Es LDH, haptoglobin DAT - haemolysis Consider myeloma screen - serum Igs and serum free light chains HIV
79
Anaemia of chronic disease why and treatment
Inadequate usage of stored iron Inadequate eryhtropoietin response to anaemia RBC life span Treat - underlying cause, IV iron, EPO injections
80
Hypersegmented neutrophils ass with
Haemotinic deficiencies
81
Pernicious anaemia markers
Low B12 Intrinsic factor Parietal cell antibodies
82
Why need to do endoscopy in B12 deficiency anaemia
Ass w gastric cancer

Haemotology (19 decks)

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