Clinical Haematology Flashcards
What are the basic features of anaemia?
Pale conjunctivae
Fatigue
Increased RR and HR in severe forms
What is aniso-poikilocytosis?
Different shaped RBCs
What are the causes of IDA?
Reduced uptake
- Malnutrition
- Coeliac
- IBD
Increased Loss
- GI malignancy
- Peptic ulcer
- IBD
- Menstruation
Increased requirement
- Pregnancy
- Breast feeding
A triad of IDA, change in bowel habit and PR bleeding is suggestive of what?
Colon cancer
What is the triad of colon cancer?
IDA
Change in bowel habit
PR bleeding
What hormone is indicated in anaemia of chronic disease?
Hepcidin
What is the function of hepcidin?
Reduced iron uptake
Decreased iron transport
Increased storage of iron
-Reduced circulating iron
What is raised in anaemia of chronic disease?
Ferritin
Is TIBC raised or reduced in anaemia of chronic diseae?
Decreased
Is ferritin raised or decreased in IDA?
Reduced
What happens to the TIBC in anaemia of chronic disease?
Increased
What type of anaemia is typically associated with ACD?
Normocytic anaemia
What type of protein is ferritin?
Acute-phase protein - raised in infections.
What is the inheritance pattern of thalassaemia?
Autosomal recessive
What are the two types of thalassaemia?
Alpha defect
Beta defect - minor, intermediate and major
What are the three variants of alpha defect of thalassaemia?
Alpha +
Alpha*
HbH
Hb Barts
What type of anaemia is associated with thalasseamia?
Microcytic anaemia
Normal iron studies
Gel electrophoresis
What physiological advantage is occurred with thalassaemia minor?
Resistance to falciparum malaria
What is the long term management for beta-thalassaemia major?
Regular red cell transfusions every 2-4 weeks with iron chelation regime
Which thalassaemia Hb chains remain constant from birth?
Alpha chains
When does Hb Beta chains increase?
Increase from early infancy
What are the components of HbA1?
2-alpha, 2 beta
What are the components of HbA2?
2a and2 delta
What are the components of HbF?
2a and 2y
What is the hereditary pattern for sickle cell disease?
Autosomal recessive
What are the predisposing factors for sickling?
Hypoxia
Dehydration
Acidosis
Infection
What characteristic cell is associated from SCD?
Howell-Jolly body
And sickle cells
What occurs in a sickle cell crisis?
Acute painful crisis
Stroke
Sequestration crisis
Chronic cholecytisis
What is the management of an acute painful crisis?
Saturate with supportive oxygen Antibiotics (if needed) Pain relief Cannula (IV fluids) Crizanlizumab for prevention
What investigations are performed for the diagnosis of sickle cell anaemia?
Hb electrophoresis and blood film
What are the medical therapies available for the management of sickle cell anaemia?
Vaccinations
Hydroxyurea
Hydroxycarbamide
Prophylactic ABx
What is the surgical/curative management for SCA?
Bone marrow transplant (curative)
What are the features of SCA?
Haemolytic anaemia Dactylitis Acute chest syndrome Pripaism Aplastic crisis
What is the cause of megaloblastic anaemia?
Folate or B12 deficiency
What type of neutrophils are associated with folate and B12 deficiency?
Hypersegmented neutrophils
What are the causes of vitamin b12 deficiency?
Alcohol
IBD and coeliac disease
Pernicious anaemia
What are the causes of folate deficiency?
Alcohol
IBD & coeliac
Anti-folate drugs
Pregnancy
What are the neurological signs are associated with B12 deficiency?
Glove and stocking paraesthesia
Hyporeflexia
Romberg’s positive
Subacute degeneration of the spinal cord
What autoantibodies are associated with B12 deficiency?
Anti-parietal cells
Anti-intrinsic factor
What are the causes of non-megaloblastic macrocytic anaemia?
Myelodysplasia
Hypothyroidism
Liver disease
Alcohol
What are the common causes of microcytic anaemia (IDA)?
GI blood loss from hookwork infections
Reduced absorption (Small bowel disease)
Increased demands
Reduced intake
What hormone is elevated in anaemia of chronic disease?
Hepcidin - Suppresses iron absorption and transport
What is the presentation of IDA?
Tiredness Lethargy Malaise Dyspnoea Pallor Palpitations Exacerbations of ischaemic conditions
What is the presentation of lead poisoning (Haem)
Anorexia Nausea/vomiting Abdominal pain Constipation Peripheral nerve lesions
What are the main causes of microcytic anaemia?
Thalassemia Anaemia of chronic disease Iron deficiency anaemia Lead poisoning Sideroblastic anaemia
What examination signs are seen in iron deficiency anaemia?
Glossitis
Angular stomatitis
Pallor
Koilonychia
What are the examination signs for lead poisoning induced anaemia?
Blue gumline
Peripheral nerve lesions (causing wrist or foot drop)
Convulsions
Reduced consciousness
What is the serum ferritin in IDA?
Ferritin is low in IDA
Why is serum ferritin raised in anaemia of chronic disease?
Is an acute phase protein
What is the TIBC level in IDA?
High in IDA
What is the transferrin level in IDA?
Raised
What is the transferrin saturation level in IDA?
Low
What signs are seen in a blood film for IDA?
Microcytic anaemia
Hypochromic
Anisocytosis
Poikilocytosis
What blood film signs are seen in sideroblastic anaemia?
Dimorphic blood film
Hypochromic microcytic cells
Ring sideroblasts in the bone marrow
What blood film signs are see in lead poisoning?
Basophilic stippling
What is the first-line management for iron deficiency anaemia?
Ferrous sulphate
What is the management for sideroblastic anaemia?
- Treat underlying cause
- Pyridoxine used in inherited forms
- Blood transfusions and iron chelation can be considered if there is no response to other treatments.
What is the management for lead poisoning induced anaemia?
- Remove the source
- Dimercaprol
- D-penicillamine
What are the complications for IDA?
High-output cardiac failure
What are the causes of normocytic anaemia?
Acute blood loss - peptic ulcer, trauma, oesophageal varices
Failure of RBC production
Haemolysis
Uncompensated increase in plasma volume
Hypersplenism
What is macrocytic anaemia?
Anaemia is associated with an elevated MCV of erythrocytes (>100 fL in adults). Megaloblastic refers to a delay in the maturation of the nucleus while the cytoplasm continues to mature.
• Structurally abnormal, immature red cells (Oval macrocytes).
Which drugs are indicated in interfering with DNA synthesis resulting in macrocytic anaemia?
- Azathioprine
- Methotrexate (Dihydrofolate reductase inhibitor)
- Hydroxyurea
- Zidovudine
What are the causes of macrocytic anaemia?
• Vitamin B12/Folate deficiency
• Use of drugs interfering with DNA synthesis:
- Azathioprine
- Methotrexate (Dihydrofolate reductase inhibitor)
- Hydroxyurea
- Zidovudine
• Liver disease and ethanol toxicity
• Haemolytic anaemia (Reticulocytosis increased)
• Myelodysplasias
Which two vitamin deficiencies result in macrocytic anaemia?
Folic acid
B12 deficiency
What is the function of B12?
• B12 is required for DNA synthesis and the integrity of the nervous system.
Where is b12 ultimately absorbed?
Terminal ileum
Which gastric peptide facilitates the absorption of vitamin b12?
Intrinsic factor
What are the common causes of vitamin b12 deficiency?
Inadequate diet veganism
Gastrectomy -
Autoimmune
-Pernicious anaemia
Crohn’s disease - Ileal resection
Dietary: Poverty alcoholism
Malabsorption • Coeliac disease Jejunal resection Pregnancy Lactation
Increased cell turnover: Haemolysis
Poverty alcoholism
What is the definitive management for pernicious anaemia?
Hydroxocobalamin injections (IM)
What is the most common cause of B12 deficiency?
Pernicious anaemia
What are the signs of pernicious anaemia?
Mild jaundice
Glossitis (Beefy)
Angular stomatitis
Weight loss
Signs of anaemia
- Pallor
- Tachycardia
- Breathlessness
What are the neurological signs of B12 deficiency?
Peripheral neuropathy
Ataxia
Subacute combined degeneration of the spinal cord
Optic atrophy
Dementia
Positive Babinski’s sign - absent ankle reflex, upwards plantars
What blood test investigations are performed for macrocytic anaemia?
High MCV, low haematocrit
Serum B12 and folate
Red cell folate
Anti-parietal cell and anti-intrinsic factor antibodies
What autoantibodies are associated with pernicious anaemia?
• Anti-parietal cell and anti-intrinsic factor antibodies
What does a peripheral blood smear reveal in pernicious anaemia?
- Megaloblasts (Large with nucleocytoplasmic dissociation)
* Hypersegmented polymorphonucleated cells
Which dietary supplements are administered in b12 deficiency?
• Dietary supplements – PO cyanocobalamin
What is the management for folate deficiency?
Oral folic acid
Is there is co-existing B12 deficiency with folate acid deficiency, which should be treated first?
• If B12 deficiency is present – Treated prior to the folic acid deficiency as B12 is required for folate uptake.