- malignancy - chronic infection (TB) - CTDs (RA)

- intercurrent illness/infection - fava beans - henna - meds: primaquine, sulfa-drugs, nitrofurantoin, dapasone, NSAIDs/aspirin

Anaemia Flashcards by Mia Shand

What are the 6 types of anaemia?

anaemia of chronic disease (ACD)
aplastic anaemia (ApA)
haemolytic anaemia (HmA)
iron deficiency anaemia (IDA)
pernicious anaemia (PA)
sideroblastic anaemia (SdbA)

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What causes ACD?

malignancy
chronic infection (TB)
CTDs (RA)

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How do chronic diseases cause anaemia?

IL-1, IL-6
- hepcidin release from liver inhibits Fe absorption (ferroportin activity)

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What is ferroportin?

Fe export channel
- gut enterocytes
- reticuloendothelial cells (macrophages)

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What cells are seen on a blood film in ACD?

Initially: normochromic, normocytic

Develops to: hypochromic, microcytic
(low TIBC, high ferritin)

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What is aplastic anaemia?

Pancytopenia + hypocellular marrow

(no abnormal cells)

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What bloods are diagnostic of an aplastic anaemia?

anaemia (Hb<10)
thrombocytopenia (platelets<50)
neutropenia (absolute<1.5)

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Name the common acquired causes of aplastic anaemia?

drugs (NSAIDs, chloramphenicol)
infection (hepatitis)
paroxysmal nocturnal haemoglobinuria (PNH)

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Name the common inherited causes of aplastic anaemia?

Fanconi’s anaemia (AR)
Dyskeratosis congenita (XL)

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How does aplastic anaemia typically present?

Pancytopenic features:
- fatigue, pallor
- infection
- easy bruising/bleeding

Risk:
- drug exposure (6/12)
- occ exposure
- recent infection
- comorbidities

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What is Fanconi’s anaemia?

Aplastic anaemia +

pigmentation abnormalities
hearing defects
renal/ genital abnormalities
solid tumours
short stature

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How do patients with dyskeratosis congenita present?

Aplastic anaemia +

nail malformations
oral leukoplakia
reticulated skin rash

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How is haemolytic anaemia classified?

Intravascular
Extravascular
AI (Coombs+)
Non-AI (Coombs -)

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How does haemolytic anaemia present on biochemistry?

low Hb
high UCB, LDH, urinary UBG
reticulocytosis

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Intravascular haemolysis releases Hb into the circulation. How does the body deal with this excess Hb?

Combination
- haptoglobin
- albumin (methaemalbuminaemia)

Excretion
- urine (haemoglobinuria, haemosiderinuria)

Storage
- haemosiderin (tubular epithelial cells)

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What are the causes of intravascular haemolytic anaemia?

intrinsic cellular injury (G6PD)
compliment-mediated lysis (AI, PNH, acute transfusion reactions)
mechanical injury (microangiopathy & cardiac valves)
autoimmune

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What are the features of a Glucose-6-phosphate deficiency anaemia? (G6PD)

XLR
presents neonatally with jaundice
presents later with episodic intravascular haemolysis (exposure to oxidative stressors)

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What triggers G6PD?

intercurrent illness/infection
fava beans
henna
meds: primaquine, sulfa-drugs, nitrofurantoin, dapasone, NSAIDs/aspirin

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How is a G6PD investigated?

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Blood film
- Heinz bodies
- bite cells

Diagnostic test
- G6PD enzyme assay

How is G6PD managed?

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avoid precipitants
transfusions (rare)

How does extravascular haemolytic anaemia present?

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hepatomegaly
splenomegaly
(Reticuloendothelial system)

What are the causes of extravascular haemolytic anaemia?

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abnormal RBCs (SCA, hereditary spherocytosis)
cells marked by abds for splenic phagocytosis

What is hereditary spherocytosis?

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AD
- mutations in RBC membrane proteins
- membrane sections removed by spleen
- reduced SA:vol
= spherical distortion > haemolysis

What is the classic presentation of hereditary spherocytosis?

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neonatal jaundice/anaemia
splenomegaly

Management of hereditary spherocytosis?

Conservative - folate supplements Severe disease - splenectomy (<5) curative

What is the test for AIHA ?

Direct Coombs test - Abd against autoabd - cross-link & agglutinate

What are the two types of Coombs+ AIHA?

Warm & cold AIHA

What is warm AIHA?

IgG-mediated - spleen tags cells for splenic phagocytosis

What is cold AIHA?

IgM-mediated - IgM fixes complements, causing direct INTRAvascular haemolysis (cold agglutinins)

What are the causes of warm AIHA?

- idiopathic - lymphoproliferative neoplasms (CLL, lymphoma) - drugs (methyldopa) - SLE

What are the causes of cold AIHA?

- idiopathic - infection post-2/3w (EBV, mycoplasma)

What disorders present as Coombs- haemolytic anaemias?

- microangiopathy - PNH - physical lysis of RBCs (malaria) - haemolytic uraemic syndrome (E.coli) - infection causing DIC (fulminant meningococcemia)

What is microangiopathic haemolytic anaemia?

A type of intravascular Coombs- haemolytic anaemia - physical lysis of RBCs by deposited fibrin strands - schistocytes on film Cause: - isolated - associated with thrombotic microangiopathy syndromes (DIC, haemolytic uraemic syndrome, thrombotic thrombocytopenia purpura)

What is PNH?

Paroxysmal nocturnal haemoglobinuria - abnormal surface glycoprotein expressed on subclone of RBC marks for complement-mediated lysis

How does PNH present?

- young adult - nocturnal intravascular haemolysis - other stem cell defects - increased clotting risk

What test is diagnostic of PNH?

Flow cytometry

In what circumstances would physical lysis of RBCs occur?

- falciparum malaria - babesiosis - prosthetic valves

What is pernicious anaemia?

Vitamin B12 deficiency causing anaemia (2ry to AI impairment of IF production)

What causes a Vitamin B12 deficiency?

Gastric - pernicious anaemia - chronic severe atrophic gastritis Pancreatic insufficiency Small bowel - bacterial overgrowth - resection of terminal ileum - Crohn's TB Metformin ZES

What does pernicious anaemia present as on biochemistry?

- macrocytic anaemia - high MCH (normal conc) - low serum B12, folate - low reticulocyte count - large, oval-shaped RBCs - IF abd/parietal cell abd (Hyperchlorhydria - gastric body atrophy)

How is pernicious anaemia managed?

Lifelong replacement (cobalamin) - monitor for early diagnosis of IDA - advise about GI complications (gastric cancer, carcinoids)

What is sideroblastic anaemia?

Microcytic anaemia due to ineffective erythropoiesis (increased Fe absorption in marrow)

How does sideroblastic anaemia appear on film?

- sideroblastic inclusions in RC cytoplasm - haemosiderosis of liver, heart, endocrine organs

How does sideroblastic anaemia present clinically?

- microcytic anaemia refractory to intensive Fe Tx - high serum ferritin & Fe

What are the causes of sideroblastic anaemia?

Congenital (XL, AR, AD) Acquired - drugs/toxins (alcohol, Pb, isoniazid) - myelodysplastic syndromes

Anaemia Flashcards

(45 cards)