Haematology Flashcards
Microcytic anaemia causes (5)
- Iron deficiency
- Thalassaemia
- Lead poisoning
- Anaemia of chronic disease (more commonly normocytic, normochromic picture)
- Congenital sideroblastic anaemia
TAILS
most common cause in pre-menopausal women
most common cause in men
Menorrhagia GI bleed (think colon cancer)
Ix iron deficiency anaemia
Findings on bloods:
TIBC low or high
Blood film - name two types
TIBC = high
Blood film = target cells, ‘pencil’ poikilocytes
Causes of normocytic anaemia
- Anaemia of chronic disease
- CKD
- Aplastic anaemia
- Acute blood loss
- Haemolytic anaemia
AAACH
Causes of macrocytic anaemia
Megaloblastic (2)
Normocytic (6)
Megalo 1. B12 deficiency 2. Folate deficiency Normo 3. Alcohol 4. Liver disease 5. Hypothyroid 6. Pregnancy 7. Reticulocytosis 8. Myleodysplasia
abdominal pain peripheral neuropathy (mainly motor) fatigue constipation blue lines on gum margin =
Lead poisoning
Lead poisoning
Mx (2)
- dimercaptosuccinic acid (DMSA)
2. D-penicillamine
Raised
- delta aminolaevulinic acid
- urinary coproporphyrin
=
Lead poisoning
target cells Howell-Jolly bodies Pappenheimer bodies siderotic granules acanthocytes
Hyposplenism e.g. post-splenectomy, coeliac disease
‘tear-drop’ poikilocytes
Myelofibrosis
schistocytes
Intravascular haemolysis
hypersegmented neutrophils
Megaloblastic anaemia
Blood film
anisopoikilocytosis (red blood cells of different sizes and shapes)
target cells
‘pencil’ poikilocytes
iron deficiency anaemia
Blood film:
basophilic stippling and clover-leaf morphology
lead poisoning
Blood film findings:
Myelofibrosis
‘tear-drop’ poikilocytes
Blood film findings:
Megaloblastic anaemia
hypersegmented neutrophils
Blood film findings:
Haemolysis
schistocytes
Blood film findings:
Hyposplenism (5)
target cells Howell-Jolly bodies Pappenheimer bodies siderotic granules acanthocytes
TAPSH
Spherocytes on blood film
Name two conditions
Hereditary spherocytosis
Autoimmune hemolytic anaemia
Basophilic stapling
Name four conditions
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
Heinz bodies
Name two conditions
G6PD deficiency
Alpha-thalassaemia
Schistocytes
Name three conditions
Haemolysis
Mechanical heart valve
Disseminated intravascular coagulation
Target cells
Name four conditions
Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease
positive direct antiglobulin test (Coombs’ test) =
Automimmune haemolytic anaemia
Warm autoimmune haemolytic anaemia
What causes haemolysis
Where does haemolysis tend to occur?
Mx
IgG
haemolysis tends to occur in extravascular sites, for example the spleen
steroids, immunosuppression and splenectomy
Cold autoimmune haemolytic anaemia What causes haemolysis Where does haemolysis tend to occur? Features (2) Mx
IgM
Haemolysis is mediated by complement and is more commonly intravascular.
Features may include symptoms of Raynaud’s and acrocynaosis. Patients respond less well to steroids
Hereditary spherocytosis
AD/AR
Features (4)
AD
- failure to thrive
- jaundice, gallstones
- splenomegaly
- aplastic crisis precipitated by parvovirus infection
Hereditary spherocytosis
AD/AR
Features (5)
AD
- failure to thrive
- jaundice, gallstones
- splenomegaly
- aplastic crisis precipitated by parvovirus infection
- MCHC elevated
Hereditary spherocytosis
Dx
Clinical - if equivocal
cryohaemolysis test and EMA binding test
for atypical presentations for electrophoresis
Spherocytosis
Mx
acute haemolytic crisis: 1. supportive 2. transfusion if necessary longer term treatment: 1. folate replacement 2. splenectomy
Difference between G6PD deficiency and spherocytosis
Country
Blood film
Genetics
Haemoylsis location
G6PD African + Mediterranean, Heinz bodies, X linked, intravascular heamolysis
Spherocytosis Northern european, AD, extravascular haemolysis
Drugs that can cause haemolysis (3)
- anti-malarials: primaquine
- ciprofloxacin
- sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
Medications that increase VTE risk (4)
- Antipsychotics
- COCP
- HRT
- Tamoxifen
Direct thrombin inhibitor medication (1) –> reversal (1)
Direct factor Xa inhibitor (2) –> reversal (1)
Dabigatran –> Idarucizumab
Apixaban + rivaroxaban –> adnexanet
Examples of myeloproliferative disorders RBC WBC Platelets Fibroblasts
Polycythaemia rubra vera (PRV)
CML
Essential thrombocythaemia
Myelofibrosis
Auer rods
DIC or thrombocytopenia often at presentation
ApML
Philadelphia chromosome =
Due to a translocation between the long arm of chromosome 9 and 22
Mx (4)
CML
- imatinib is now considered first-line treatment
- hydroxyurea
- interferon-alpha
- allogenic bone marrow transplant
blood film: smudge cells (also known as smear cells) =
CLL
CLL
Ix:
immunophenotyping
CLL
Complications (4)
anaemia
hypogammaglobulinaemia leading to recurrent infections (low IgG)
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)
What is Richter’s transformation?
leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma
Features of Richter’s transformation?
lymph node swelling fever without infection weight loss night sweats nausea abdominal pain
Massive splenomegaly causes (5)
- myelofibrosis
- leukaemia
- leishmaniasis
- malaria
- Gaucher’s syndrome
leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma occurs in which leukemia
CLL
What is ITP?
an immune-mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.
ITP Mx
Management
- oral prednisolone
- pooled normal human immunoglobulin (IVIG)
What is thrombocytosis?
Mx (3)
High platelets
- hydroxyurea
- interferon-α
- low-dose aspirin to reduce the thrombotic risk
Essential thrombocytosis
Features (4)
- platelet count > 600
- both thrombosis and haemorrhage can be seen
- burning sensation in the hands
- JAK2 mutation is found in around 50% of patients =
Well score interpretation
=>2 –> DVT likely
If likely then for USS within 4 hours if +ve for rx
If negative then for d-dimer, if negative scan and negative d dimer –> unlikely, and consider alt dx
If scan cannot be done within 4 hours, then for d dimer and interim anticoag with DOAC, USS to be done within 24 hours
If scan negative but d-dimer +ve, stop DOAC and repeat scan in 1 week
If DVT unlikely 1 or less, for d-dimer within 4 hours, if not for interim DOAC. If +ve dimer then for USS within 4 hours. If unable to do within 24 hours then for interim DOAC
When to not use DOAC
CKD eGFR<15
Antiphospholipid syndrome
TO use LMWH or unfractionated heparin
platelet count > 600 * 109/l
both thrombosis and haemorrhage can be seen
burning sensation in the hands
JAK2 mutation is found in around 50% of patients =
Essential thrombocytosis
Types of Burkitt’s lymphoma and locations
Which virus is associated with the first type?
- Endemic –> maxilla + mandible –> assoc with EBV
2. Sporadic form –> abdominal (e.g. ileo-caecal) tumours, common in pt’s with HIV
Burkitt’s lymphoma miscroscopy findings?
Mx
Name of medication given before mx to prevent which condition?
‘starry sky’ appearance
Chemo
Rasburicase given prior to chemo to prevent tumour lysis syndrome
Which condition?
bone disease: bone pain, osteoporosis + pathological fractures, osteolytic lesions
lethargy
infection
hypercalcaemia
renal failure
other features: amyloidosis e.g. Macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity
Multiple myeloma
What is multiple myeloma?
Neoplasm of increased plasma cells
Multiple myeloma Ix
Bloods (2)
Scan (1)
Xray finding?
- monoclonal proteins (usually IgG or IgA) in the serum and urine (Bence Jones proteins)
- Whole body MRI
- ‘rain-drop skull’
Diagnostic criteria for multiple myeloma
one major and one minor criteria OR
three minor criteria
Major criteria
Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine
Minor criteria
10% to 30% plasma cells in a bone marrow sample.
Minor elevations in the level of M protein in the blood or urine.
Osteolytic lesions (as demonstrated on imaging studies).
Low levels of antibodies (not produced by the cancer cells) in the blood.
What is Factor V Leiden?
Most common thrombophillia secondary to activated protein C resistance
Increased risk of VTE
What is Von Willebrand’s disease?
AD/AR?
Sx
Most common inherited bleeding disorder
AD
epistaxis and menorrhagia
Von Willebrand
Ix (4)
prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin
Von Willebrand
Mx
tranexamic acid for mild bleeding
desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
factor VIII concentrate
HbA2 raised (> 3.5%) =
Beta thalassemia
Hepatitis C is associated with?
cryoglobulinaemia
Type 1 cryoglobulinaemia examples
monoclonal - IgG or IgM
multiple myeloma, Waldenstrom macroglobulinaemia
Type 2 cryoglobulinaemia examples
mixed monoclonal and polyclonal: usually with rheumatoid factor
hepatitis C, rheumatoid arthritis, Sjogren’s, lymphoma
Raynaud’s only seen in which type of cryoglobulinaemia?
Type 1
Haemophilia genetics
Haemophilia A and B which factors?
X linked recessive disorder of coagulation
VIII and IX
prolonged APTT only =
Haemophillia
usually asymptomatic
lower level of paraproteinaemia than myeloma
Monoclonal gammopathy of undetermined significance
C1 1NH deficiency =
Hereditory angioedema
attacks may be proceeded by painful macular rash
painless, non-pruritic swelling of subcutaneous/submucosal tissues
may affect upper airways, skin or abdominal organs (can occasionally present as abdominal pain due to visceral oedema)
urticaria is not usually a feature
=
Hereditory angioedema
What is Waldenstrom macroglobulinemia?
type of non-Hodgkin lymphoma
What is Paroxysmal nocturnal haemoglobinuria
acquired disorder leading to haemolysis caused by a lack of glycoprotein glycosyl-phosphatidylinositol (GPI)
Increased risk of VTE
Dark-coloured urine, haemolytic anaemia, thrombosis =
Paroxysmal nocturnal haemoglobinuria
Paroxysmal nocturnal haemoglobinuria
Ix
Mx (2)
flow cytometry of blood to detect low levels of CD59 and CD55
blood product replacement
anticoagulation
Secondary causes of polycythaemia (4)
COPD
altitude
obstructive sleep apnoea
excessive EPO: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids*
JAK2 mutation hyperviscosity pruritus, typically after a hot bath splenomegaly haemorrhage (secondary to abnormal platelet function) plethoric appearance hypertension in a third of patients low ESR =
polycythaemia
low ESR and a raised leukocyte alkaline phosphatase =
polycythaemia
Polycythaemia
Mx (3)
aspirin
venesection - first line treatment
hydroxyurea -slight increased risk of secondary leukaemia
definitive Ix of sickle cell disease =
haemoglobin electrophoresis
Name the crises sickle cell
sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
Sequestration
Name the crises sickle cell
dyspnoea, chest pain, pulmonary infiltrates, low pO2
the most common cause of death after childhood
Acute chest syndrome
Name the crises sickle cell
caused by infection with parvovirus
sudden fall in haemoglobin
Aplastic
Name the crises sickle cell
fall in haemoglobin due an increased rate of haemolysis
Haemolytic
Name the crises sickle cell
also known as painful crises or vaso-occlusive crises
precipitated by infection, dehydration, deoxygenation
Thrombotic
Most common cause of thrombophillia =
Second most common cause
factor V Leiden
prothrombin gene mutation
vit B12 deficiency is most commonly caused by?
pernicious anaemia
Causes of vit b12 deficiency (5)
pernicious anaemia post gastrectomy vegan/ poor diet disorders/surgery of terminal ileum (site of absorption) e.g Crohn's metformin (rare)
What does B12 use to be absorbed?
Where is this secreted from?
Where is it absorbed?
intrinsic factor (secreted from parietal cells in the stomach) and is actively absorbed in the terminal ileum.
Mx B12
if no neuro 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then every 3 months
if also deficient in folic acid then to treat B12 deficiency first to avoid precipitating subacute combined degeneration of the cord
Sx B12 deficiency (4)
type of anaemia
- macrocytic anaemia
- sore tongue and mouth
- neurological symptoms
the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia - neuropsychiatric symptoms: e.g. mood disturbances
Which haemophillia accounts for 90% of cases?
A
What is the antidote for heparin
protamine sulfate
HbA2 raised (> 3.5%) =
beta thalassemia trait
