NEW HAEM 1 Flashcards
Microcytic anaemia
TAILS
Thalassaemia AoCD Iron deficiency Lead poisoning Sideroblastic
Normocytic anaemia
AoCD Acute bleed Pregnancy CKD Aplastic anaemia Bone marrow infiltration
Haemolytic anaemia
- G6PD
- Hereditary spherocytosis
- Sickle cell
Macrocytic anaemia
Megaloblastic
- Folate
- B12
Normoblastic
- Hypothyroid
- Alcohol
- Reticulocytosis
- Azathioprine
Iron deficiency anaemia aetiology
Uptake in the duodenum
Excessive blood loss
- Menorrhagia
- GI bleed - Colorectal cancer in men, peptic ulcers
Inadequate dietary intake
- Meat and leafy greens
- Veggie diet is RF
Poor intestinal absorption
- Coeliac / Crohn’s
- Gastrectomy
Increased iron requirements
- Children
- Pregnant ladies
Iron deficiency anaemia clinical features
Fatigue
SOBOE
Weakness
PICA
Restless leg syndrome
Kolionychia - Spoon nails
Glossitis
Angular stomatitis
Gastric difficulties
Hair loss
IDA investigations
FBC - Microcytic hypochromic + Low reticulocytes
Blood film - Anisopoikilocytosis - Red cells of different shapes and sizes
Iron studies
- MCH - Low
- Serum ferritin - Low
- TIBC - High
- Transferrin saturation - Low
Coeliac seriology
IDA history
- Change in diet
- Medication history
- Menstrual history
- Weight loss
- Change in bowel habit
IDA management
Treat cause
Ferrous sulphate
- Nausea
- Abdo pain
- Constipation
- Diarrhoea
- Black stools
Iron-rich diet
- Leafy greens
- Meat
- Iron-fortified bread
Pernicious anaemia pathophysiology
B12 deficiency
Pathology
- AI disorder
- Antibodies to IF and/or gastric parietal cells
- Antibodies bind to IF - Block B12 binding site
- Gastric parietal cell antibodies - Reduce IF production
B12 important for…
- Erythropoiesis - Megaloblastic anaemia
- Nerve myelination - Neuropathy
Pernicious anaemia RFs and clinical features
Females > Males
AI disorders - Thyroid, DM, Addison’s, RA, etc.
Symptomatic anaemia
Peripheral neuropathy - Paraesthesia
Subacute combined spinal cord degeneration
Neuropsychiatric features
- Memory loss
- Poor concentration
- Confusion
- Depression
- Irritability
Pernicious anaemia investigations
FBC - Macrocytic anaemia
- WCC - Low
- Platelets - Low
Blood film - Hypersegmented polymorphs
B12 < 200
Antibodies
- Anti-IF antibodies
- Anti-GP cell antibodies
Schilling test
Pernicious anaemia management and complications
B12 replacement therapy - IM Hydroxycobalamin
- 3 injections per week for 2 weeks
- Then 3 monthly
Folic acid supplementation
Complications - Increased risk of gastric cancer
Folate deficiency aetiology
Dietary deficiency Malabsorption - Coeliac/IBD Pregnancy and breast-feeding Prematurity Alcohol excess
Iatrogenic
- Phenytoin / Phenobarbitol
- Trimethoprim
- Methotrexate
- Sulfasalazine
- Pyrimethamine
- Anticonvulsants
Folate deficiency clinical features and investigations
Symptomatic anaemia
Exfoliative dermatitis
Weight loss / LOA
Headaches
Serum folate - Low
Blood film - Hypersegmented neutrophils
FBC - Macrocytic anaemia with LOW reticulocytes
- Hb - Low
- MCV ^
- MCH ^
Folate deficiency management
Folic acid - 5mg
Preventing NTD during pregnancy
- 400mcg folic acid until 12th week of pregnancy
- Higher risk of NTD - 5mg folic acid before conception to 12th week
Increased risk of NTD
- Either partner has NTD
- Previous pregnancy affected by NTD
- Family history of NTD
- Anti-epileptic drugs
- Coeliac disease
- DM
- Thalassaemia
- Obesity
G6PD deficiency
Haemolytic anaemia - X-linked
Clinical features - Haemolytic anaemia following a precipitant
- Abx - Nitro
- Anti-malarial - Chloroquine
- Infection
- Fava beans
Investigations - Normocytic anaemia with high reticulocytes
Blood film
- Heinz bodies
- Bite cells
- Hemi-ghosts
Management - Supportive
- Transfusion if severe
- Avoid precipitants
Hereditary spherocytosis
Haemolytic anaemia - AD
Defect in RBC membrane synthesis
Spherical RBCs removed by spleen
Reduced RBC lifespan
Patients from Northern Europe - Finland, etc.
Clinical features = Haemolytic anaemia + Splenomegaly
Investigations - Normocytic anaemia with high reticulocytes
- Blood film - Spherocytes
- EMA binding test
Management
- Acute crisis - Supportive + Transfusion
- Long-term - Folic acid + Splenectomy
Sickle cell pathophysiology
Haemolytic anaemia - Autosomal recessive
Glutamate to Valine
Abnormal HbS
HbS turns sickle shaped when deoxygenated
Results in haemolysis and capillary obstruction
Precipitants
- Hypoxia
- Cold
- Exercise
- Dehydration
- Infection
Sickle cell crises
Thrombotic - Painful / vaso-occlusive crises
- AVN femoral head
- Hand-foot syndrome in children
- Lungs
- Spleen
- Brain - CVA
- Mesenteric ischaemia
Sequestration - Pooling within spleen or lungs
Acute chest syndrome
- Dyspnoea
- Chest pain
Aplastic - Infection with parvovirus
Haemolytic
Sickle cell investigations and crisis management
Guthrie heel-prick test
FBC - Normocytic anaemia with high reticulocytes
Protein electrophoresis
- HbAS/HbSS
- HbF ^
Blood film - Howell jolly bodies
Crisis management - Treat cause
- O2
- IV fluids
- Analgesia
- Transfusion
Sickle cell long-term management and complications
Hydroxyurea - Stimulates HbF production Vaccinations - Influenza and pneumococcal Prophylactic abx - Penicillin Repeated transfusions Bone marrow transplant
Complications
- CVA
- Stunted growth
- Developmental delay
- Crises
- Osteomyelitis - Salmonella
Aplastic anaemia aetiology
Congenital - Fanconi or dyskeratosis congenita
Toxins - Benzene
Infection - Parvovirus and Hepatitis
Radiation
Iatrogenic
- Cytotoxics
- Chloramphenicol
- Sulphonamides
- Phenytoin
- Gold
Aplastic anaemia clinical features / investigations / management
Symptomatic anaemia
Neutropenia - Infections
Thrombocytopenia - Bleeding and bruising
Investigations
- Normocytic hypochromic anaemia
- Hypoplastic bone marrow
- Pancytopenia
- Reticulocytes - Low
Management - Monitoring
- Immunosuppressants
- Transfusions
- Prophylactic abx
AoCD
Inflammation mediated reduction in RBC production
Aetiology
- IBD
- RA
- JIA
- Malignancy
Clinical features - Symptomatic anaemia
Investigations - Normocytic anaemia with low reticulocytes
- Iron - Low
- TIBC - Low
- Transferrin saturation - Low
- Ferritin - High
Management - Treat cause
- Transfusion
- ESAs - Epoetin Alfa
- Iron supplementation - Sodium ferric gluconate complex
Sideroblastic anaemia aetiology
Microcytic hypochromic
Iron can’t bind haem
Free iron circulating
Leads to iron overload picture
X-linked or Acquired - Alcohol - Lead - Myelodysplasia - TB medication
Sideroblastic anaemia clinical features / investigations / management
Symptomatic anaemia + Iron overload
Investigations
- FBC - Hypochromic microcytic anaemia
- Ferritin ^
- TIBC - Low
- Iron ^
- Bone marrow biopsy - Sideroblasts
Management - Treat cause
- Desferoxamine
- Pyridoxine
Alpha thalassaemia
Hypochromic microcytic
SE Asia
Deficiency of alpha chains in Hb
2 separate alpha-globulin genes located on each Ch16
1 or 2 alleles affected - Normal Hb
3 alleles affected
- HbH disease
- Splenomegaly
All 4 alleles affected
- Death in utero
- Hydrops fetalis
- Bart’s hydrops
Thalassaemia clinical features / investigations / management
Haemolytic anaemia
Major - Hydrops fetalis
Present in first year of life
- FTT
- Hepatosplenomegaly
Skull bossing
Investigations - Unconjugated bilirubin ^
Hb electrophoresis
- HbA2 ^
- HbF ^
- HbA - Absent in major
Management
- Transfusion
- Desferoxamine
Polycythaemia aetiology
Congenital - JAK2 mutation
Acquired - Chronic hypoxia
- OSA
- COPD
- Altitude
Associated with Budd-Chiari syndrome
Polycythaemia clinical features
Ruddy appearance - Flushed Pruritus - Palms and soles - Worse after a bath Headaches Tinnitus Angina Dizziness Splenomegaly HTN - 1/3 patients VTE risk Haemorrhage - Secondary to abnormal platelet function
Polycythaemia investigations / management / prognosis
FBC
- Hb ^
- Crit ^
- Neutrophils ^
- Basophils ^
- Platelets ^
Mutation screen - JAK2
Management - Venesection
- Hydroxyurea
- Phosphorous-32 therapy
- VTE prophylaxis - Low-dose Aspirin
Prognosis
- VTE
- Myelofibrosis
- Acute leukaemia
