Anaemia – Kokul Sriskandarajah

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Transcript Anaemia – Kokul Sriskandarajah

Kokul Sriskandarajah
Intercalating 5th Year, [email protected]
www.peermedics.com
 FBC – what’s included?
 What is anaemia?
 Classification
 Signs and Symptoms
 Causes
 Management
 Cases
 What's Included and normal values?
 HB: Men 130-180 g/L, women 115-160 g/L
 WCC: 4-11 x10^9/L
 Plts: 150-400 x 10^9/L
 MCV: 76-96 fL
 When would you order a FBC?
 Pretty much always!
Hb (g/dL)
8.9
MCV (fL)
81
Plts (x 10^9/L)
250
WCC (x10^9/L)
7
 Look at the Hb first and see if it is decreased beyond threshold
for sex = Anaemia
 Then look at MCV and note whether its high, normal or low
 WCC = assess if high or low.
 High suggests an infective and/or inflammatory process
 Low suggests a immunocompromised
 Platelets =assess if high or low
 High suggests an infective process, really high can mean DIC
 Low suggests thrombocytopenia; Do PT
• Anaemia is where you have a low Hb Concentration
• Typically described as:
• Males - Hb < 13.0 g/dl
• Females - Hb < 11.5 g/dl
• This can be caused by:
• A decrease in red blood cell mass (e.g. bleeding)
• Increased plasma volume (e.g. Pregnancy)
Mostly asymptomatic!
 Fatigue
 Dyspnoea (SOB)
 Faintness
 Palpitations
 Headaches
 Tinnitus
 Symptoms of underlying cause (e.g. malignancy etc.)
 Pallor
 Other signs of associated pathology
 Kollinchyia (spoon shaped nails)
 Atrophic glossitis (red swollen tongue)
 Angular stomatitis (fissuring at the corner of the mouth)
 If severe: Hyperdynamic circulation
 Ankle swelling with heart failure
 Yellow tinge to skin
Microcytic
<76
Haem defect
• IDA
• Sideroblastic
Globin
defect
Normocytic
76-96
Acute blood
loss
Anaemia of
chronic disease
Bone Marrow/
kidney failure
Aplastic Anaemia
(low reticulocytes)
• Thalassemia
Pregnancy
Macrocytic
>96
Megaloblastic
• B12/Folate deficiency
• Drugs Phenytoin/methotrexate
Nonmegaloblastic
• Reticulocytosis
• Alcohol/liver
disease
• Hypothyroidism
• Myelodysplasia
Microcytic – TAILS
Macrocytic – ABCDEF
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Alcohol excess
B12 deficiency
Compensatory Reticulocytosis
Drugs/Dyplasia (marrow)
Endocrine (Hypothyroidism)
Folate Deficiency
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Thalassemia
Anaemia of Chronic Disease
Iron Deficiency Anaemia
Lead poisoning
Sideroblastic Anaemia
Normocytic – AAA Help!
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Acute Blood loss
Anaemia of Chronic Disease
Aplastic Anaemia
Haemolytic
 Signs:
 Koilonychia
 Angular stomatitis
 Oesophageal web
 Causes:
 Loss: GI, menorrhagia, hookworms
 Reduced intake
 Malabsorption: Crohn’s and Coeliac
 Ix:
 TIBC, ferritin and transferrin saturation
 Blood film : Anisocytosis and ‘pencil’ Poikilocytosis
 Upper and lower scopes
 PathPhys:
 Point mutation (beta B/B) or deletion (alpha aa/aa)
 Leads to unbalanced or no globin chain production
 Precipitation of unmatched chains
 Haemolysis in BM and removal by spleen
 Epidemiology:
 Mediterranean and far east
 Investigations:
 Low MCV (<75) - too low for IDA
 High HBF and HbA2
 Target cells on blood film
BETA
 Point mutation of B-genes on
chromosome 11
ALPHA
 Deletions of alpha chain gene/s
on chromosome 16
 Trait/heterozygosity
 4 alleles
 B/B’ (reduced production)or B/b
(no production)
 Mild Anaemia (harmless)
 Major (Cooley’s)
 B’/B’ or B’/b or b/b
 Features from 3-6/12
 FTT, Anaemia and jaundice
 Extra-medullary erythropoiesis
 Rx: life-long transfusions
 Trait: 2 deletions
 --/aa or –a/-a
 Asymptomatic, carrier state
 HBH Disease: (--/-a)- 3 deletions
 Moderate anaemia/ jaundice
 Might need transfusion
 Hb Barts (--/--) – 4 deletions
 Hydrops fetalis
 Death in utero
ACQUIRED
Immune-mediated DAT+ve
• AIHA: warm, cold and PCH
HEREDITARY
• Enzymes:
• Pyruvate kinase deficiency
• G6PD deficiency
• Drugs: methyldopa
• Allo-immune: acute transfus.
Mechanical
• MAHA: HUS, TTP, DIC
• Heart valve
Infection : Malaria
Burns
• Membrane:
• Spherocytosis
• Elipsocytosis
• Hb-apathy
• SCD
• Thalassemia
Warm
Cold
 Ig-G mediated
 Ig-M mediated
 Binds at 37 C
 Binds at 4 C
 Direct Coombs test +ve
 Direct Coombs test +ve
 Idiopathic, SLE, RA
 Idiopathic, follow infections
 Extravascular
 Immunosuppressants/Steroids
+/- splenectomy
e.g. EBV
 Intravascular
 Avoid cold + rituximab
HUS
TTP
• Usually children
 Adult females
• E.coli 0157 from uncooked meat
 Genetic or acquired ADAMTS13 deficiency
• Triad (TAME)
 Pentad:
• Thrombocytopenia
 MAHA
• Acute Renal failure
 Fever
• MAHA
 CNS signs: confusion
• E.coli 0157
 Renal failure
• Schistocytes
• Resolves spontaneously
 Thrombocytopenia
 Schistocytes
 Plasmapheresis and splenectomy
B12
 Found in meat/fish
 absorbed in terminal ileum, aided by IF from gastric parietal cells
 Essential for DNA synthesis and myelin (along with folate)
Causes:
 Reduced intake: Vegans
 IF: Pernicious anemia/gastrectomy
 Terminal ileum: Crohn’s, bowel resection
Features:
 General: Anaemia, lemon tinge (J + P) and glossitis
 Neuro: paresthesia, peripheral neuropathy (mixed sensory and motor),
SACD (Spastic paraparesis)
 Investigations
 FBC: Low WCC and platelets
 IF antibodies
 Parietal cells antibodies especially in PA
 Blood smear: hypersegmented neutrophils
 Treatment: 1 mg IM injection of b12 (hydroxocobolamine)
 Pernicious Anaemia:
 Autoimmune gastritis, usually >40]
 Associated with other AA disease: Thyroid, Addison's .
 Folate deficiency:
 caused by reduced intake, pregnancy or AED/methotrexate/alcohol
 ALWAYS GIVE B12 first as folate alone might worsen SACD
Spherocytosis
G6PD Defeciency
 Commonest inherited anaemia
• In Mediterranean and far east,
 Spherocytes trapped in spleen
• Reduced NADPH >>>oxidative
in Europe, autosomal dominant
 Splenomegaly and gall stones
 Osmotic fragility and
spherocytes on film
 Folate and splenectomy
X-linked recessive disorder
damage to RBC
• Triggered by: infection, fava
beans and antimalarial
• Film: irregular cells, Heinz
bodies and Bite cells
• Avoid/treat triggers
• Transfusion might be needed
 68 y/o female is being investigated for altered bowel habit
 Has Hb 8.3g/dL (low), MCV 67 (low)
 Iron studies Show: low ferritin and raised TIBC
 Diagnosis?
 IDA
 ?Secondary to GI malignancy
 24 y/o female with active Crohn’s disease
 Hb 9.5 (low), MCV 101 (high) and normal Ferritin
 Peripheral blood film shows a number of hypersegmented
neutrophils
 Diagnosis?
 B12/Folate Deficiency
 55 y/o F
 Long Hx RA
 Hb 10.5 (low), MCV 80 (just normal), raised ferritin
 Her arthritis is treated only symptomatically w/ paracetamol
 Diagnosis?
 Anaemia of chronic disease secondary to RA
 Previously well 8/12 baby boy becomes generally unwell
 Hb: 4.0 g/dL (Low), MCV: 62 fL (low)
 Failure to Thrive
 Target cells seen on Blood film
 Diagnosis?
 Beta Thalassaemia Major
 56 y/o M comes to A&E feeling generally unwell and
complaining of palpitations and SoB
 Clearly jaundiced O/E
 Apart from HTN, there’s no significant PMHx
 FBC: Hb 7.4 (low), MCV 95 (normal), normal ferritin
 Peripheral blood film shows larger than expected number of
reticulocytes
 Coombs’ test is later shown to be positive
 Diagnosis?
 Autoimmune haemolytic anaemia
 IDA women: menorrhagia. Men/women: GI until proven otherwise
 Folate def in pregnancy and Methotrexate
 B12 def in Elderly, IBD and vegans
 Incidental very low MCV: thalassemia trait?
 Gall stones + anaemia : spherocytosis?
 Acute renal failure + anaemia/thrombocytopenia : HUS/TTP?
 ALWAYS treat the underlying cause
 Oxford handbook for Clinical Medicine 9th edition
 BMJ best practice approach to Anaemia
 Patient.co.uk