Haem Flashcards
what is polycythaemia vera
myeoloproliferative disorder causing excessive cell production
what gene mutation is associated with polycythaemia vera
JAK2 mutation
3 features of polycythaemia vera
- pruritus after hot bath
- splenomegaly
- hyperviscosity
bloods for polycythaemia vera
- FBC (high haematocrit)
- ESR
- U&Es & LFTs
- JAK2 mutation
how do you investigate polycythaemia if JAK2 negative
red cell mass
serum erythropoietin
how do you manage polycythaemia vera
venesection + aspirin
what are the three types of anaemia and how are they classified
- microcytic (low MCV)
- normocytic (normal MCV)
- macrocytic (high MCV)
based on MCV
list two causes of microcytic anaemia
- iron deficiency (most common)
- thalassaemia
what are features of a mixed anaemia
- iron deficiency features (low ferritin, high TIBC)
- normocytic MCV
list four causes of normocytic anaemia
- anaemia of chronic disease (hospital)
- aplastic anaemia
- haemolytic anaemia
- CKD (low erythropoietin)
what do you see in FBC in aplastic anaemia and what virus is associated with it
- pancytopenia
- low reticulocytes
- parvovirus
list two causes of megaloblastic anaemia
B12 and folate deficiency
what are the causes of non-megaloblastic macrocytic anaemia
AMHLF (alcoholics may have liver failure)
- alcohol
- myelodysplasia
- hypothyroidism
- liver disease
- folate/B12
what are the symptoms of anaemia (Asya’s acronym)
PALE
- pallor
- absent minded
- lethargy (tired)
- exertional dyspnoea
two signs of severe anaemia
tachycardia and tachypnoea
what are the three causes of iron deficiency anaemia
- reduced uptake
- malnutrition, vegetarian
- coeliac, IBD
- increased loss
- bleed (colon cancer, ulcer NSAIDs)
- menstruation
- increased requirements
- pregnancy
3 signs of iron deficiency anaemia
- conjuctival pallor
- angular stomatitis
- koilonychia
what do you see in the blood film in iron deficiency anaemia
- hypochromic cells
- anisopoikilocytosis
- pencil cells
what do you see in the bloods in iron deficiency anaemia
- low ferritin
- high TIBC
what do you want to do for males and post-menopausal females with unexplained iron deficiency anaemia
- endoscopy to investigate GI bleed
- 2ww referral to gastroenterologist
3 ways to manage iron deficiency anaemia
- treat the cause
- oral ferrous sulphate for 3 months
- iron rich diet (dark leafy veg, meat)
what causes anaemia of chronic disease
- autoimmune, infection
- chronic inflammation decreases ferroportin so less iron uptake and more iron storage
what do you see in the bloods in anaemia of chronic disease
- high/normal ferritin
- low TIBC
- MCV can be low or normal
what is the pathophysiology of haemolytic anaemia
- increased red cell breakdown
- increased red cell production
how do you classify haemolytic anaemia
based on the site of haemolysis
- intra-vascular (transfusion, G6PD, microangiopathic)
- extra-vascular (haemoglobinopathies, sickle cell)
three causes of hereditary haemolytic anaemia
- enzyme defects (G6PD deficiency)
- membrane defects (hereditary spherocytosis)
- haemoglobin issues (sickle cell, thalassaemia)
what do you see on the blood film of G6PD deficiency
Heinz bodies
what is the pathophysiology and associations of G6PD deficiency
- oxidant damage caused by Fava beans (Heinz beans)
- males only
what do you see on the blood film for hereditary spherocytosis
spherocytes
what is the pathophysiology of hereditary spherocytosis
round cells get trapped in the spleen
what are acquired causes of haemolytic anaemia
- Coombs positive:
- autoimmune
- transfusion reaction
- Coombs negative
- microangiopathic (HUS, DIC, TTP)
antibodies associated with warm and cold autoimmune haemolytic anaemia
- warm: IgM
- cold: IgG
what are four features of HUS
- thrombocytopenia
- AKI
- haemolysis
- abdominal pain
what is the epidemiology and cause of HUS
- children
- E Coli
what are the three features of TTP
- HUS
- fever
- CNS issues
two specific signs of haemolytic anaemia
- jaundice (dark urine, yellow sclera)
- splenomegaly
what are three signs of haemolysis seen in bloods
- high reticulocytes
- high LDH
- high bilirubin
what is a sign of intravascular haemolysis seen in bloods
low haptoglobin
pathophysiology behind B12 and folate deficiency anaemia
- B12 and folate needed for DNA synthesis
- delayed nuclear maturation compared to the cytoplasm so megaloblastic cells
where is folate found and how long does it last in the blood
- green leafy vegetables
- lasts for 4 months
4 causes of folate deficiency
- increased requirement (pregnancy)
- malabsorption (coeliac, IBD)
- drugs (methotrexate)
- alcohol
where is B12 found and how long does it last in the blood
- meat
- lasts for 4 years
2 causes of B12 deficiency
- pernicious anaemia
- vegan
what is pernicious anaemia and what is its epidemiology
- autoimmune atrophic gastritis
- most common B12 deficiency
- young females
what is a characteristic sign of pernicious anaemia
Lemon tinge skin
what are 2 symptoms of B12 deficiency
- glove and stocking paraesthesia
- hyporeflexia
what do you see on the blood film of megaloblastic anaemia
hypersegmented neutrophils
antibodies for pernicious anaemia
- intrinsic factor (most specific)
- anti-parietal cell
how to manage folate and B12 deficiency
- hydroxocobalamin (B12) + folic acid tablets
- prophylactic folate in pregnancy
- always remember to treat B12 before folate
what is the treatment for pernicious anaemia
- IM B12 injections
- 3 per week for 2 weeks then every 3 months
complication of pernicious anaemia
gastric cancer
what is the epidemiology of Hodgkin’s lymphoma
bimodal (30 and over 70 yrs)
what are 2 associations with Hodgkin’s lymphoma
- HIV
- EBV
what are the symptoms of Hodgkin’s lymphoma
- supraclavicular painless neck lump that’s painful after alcohol
- B symptoms: fever, weight loss, night sweats
what is the significance of B symptoms in Hodgkin’s lymphoma
- they appear later so indicate poor prognosis
what are two features seen in the bloods in Hodgkin’s lymphoma
- eosinophilia
- high LDH
what is the diagnostic investigation and finding for Hodgkin’s lymphoma
- lymph node biopsy
- Reed-Stenberg cells (mirror nuclei)
what is the management for Hodgkin’s lymphoma
chemotherapy
what is tumour lysis syndrome and how do you prevent it
- complication of chemotherapy for lymphoma
- high potassium, low calcium
- allopurinol prophylaxis
what is the epidemiology for non-Hodgkin’s lymphoma
old and white
what are 3 associations with non-Hodgkin’s lymphoma
- HIV
- EBV
- Sjogren’s
what are 2 features of non-hodgkin’s lymphoma
- painless lump in the neck, axilla
- organ involvement (hepatomegaly)
what is seen in the bloods for non-Hodgkin’s lymphoma
neutropenia
what is a characteristic feature of Burkitt lymphoma
lump in the jaw
what is seen in the lymph node biopsy for Burkitt lymphoma
starry sky appearance
what is sickle cell disease and what is its epidemiology
- abnormal haemoglobin HbS is fragile so it breaks and gets stuck, blocking vessels
- African, chromosome 11
how does sickle cell disease present
- usually asymptomatic
- acute painful crises such as dactylitis from the cold
- sequestration crisis causing splenomegaly
- gallstones due to high bilirubin
what do you see in the FBC and blood film for sickle cell disease
- FBC: high reticulocytes
- blood film: Howell Jolly bodies
how do you investigate an acute crisis of sickle cell
clinical no investigation needed
what is the diagnostic investigation for sickle cell disease
haemoglobin electrophoresis
how do you manage an acute painful crisis of sickle cell
- sats: oxygen
- antibiotics
- pain: analgesia
- cannula: fluids
what is a long term way to prevent recurrent sickle cell crises
hydroxycarbamide
