Haem Flashcards
What is a PT blood test?
Measure of the time for a blood clot to form via the extrinsic pathway.
What is an APTT blood test?
Measure of the time for blood to clot via the intrinsic pathway
Associated with factors 8, 9 and 11
What diseases have a prolonged thrombin time?
DIC, liver failure malnutrition, abnormal fibrinolysis
Causes of microcytic anaemia?
Anaemia of chronic disease, thalassaemia, iron deficiency and sickle cell
Causes of normocytic anaemia?
Acute blood loss, chronic disease, pregnancy, renal failure,
Causes of Macrocytic anaemia?
B12 deficiency, excess alcohol/liver disease, hypothyroidism, bone marrow failure.
Symptoms of anaemia?
Fatigue, headache, irritability, difficulty concentrating, dyspnoea and breathlessness, anorexia, intermittent claudication, palpitations.
Signs of anaemia?
Pallor, tachycardia, systolic flow murmur, cardiac failure, spoon shaped nails, angular stomatitis.
Investigations for anaemia?
FBC, reticulocyte count, serum iron and ferritin test, peripheral blood smear (to look at shape of RBC’s, Haemoglobin electrophoresis.
Treatment for iron deficiency anaemia?
oral iron ferrous sulphate, IV iron in emergencies.
Side effects of ferrous sulphate?
Nausea, abdo discomfort, diarrhoea/constipation, black stools,
How to treat normocytic anaemia?
Treat underlying cause, improve diet with plenty of vitamins, erythropoietin injections.
How to treat folic acid deficiency?
folic acid tablets daily for 4 months
How to treat pernicious anaemia?
B12 injections, IM hydroxocobalamin
What is G6PD deficiency?
The deficiency of G6PD makes red cells vulnerable to oxidative damage.
Epidemiology of G6PD def?
More common in Africa, middle east and SE asia, more common in males as its x-linked
Signs and symptoms of G6PD?
Pallor, fatigue, palpiatations, SOB, jaundice, exacerbated by ingesting fava beans
Investigations for G6PD?
Bite cells (membrane indentation), irregularly contracted cells, reticulocytosis.
Treatment for G6PD?
Blood transfusion, Stop exacerbating drugs such as: Primaquine Nitrofurantoin Sulphonamides
What is alpha thalassemia?
Gene deletion of one or both alpha chains.
How do people with 3 gene deletion in alpha thalassemia present?
Moderate anaemia, splenomegaly, not transfusion dependant.
How is alpha thalassaemia diagnosed?
Diagnosed with FBC showing microcytic anaemia, elevated serum iron and hamegobin electrophoresis.
How to treat alpha thalassemia?
Treat with transfusions during crises
Desferrioxamine to aid iron excretion
and folic acid
What is beta thalassaemia?
Excess alpha chains, point mutations in the gene
Minor beta thalassemia presentation?
Mild anaemia, pale red blood cells, often asymptomatic
How does intermedia beta thalassemia present?
Infections, gallstones, recurrent leg ulcers, bone deformities
How does Major beta thalassaemia present?
Failure to thrive, severe anaemia from 3-6months, thalassaemic faces, bone abnormalities.
How to diagnose beta thalassemia?
Blood film: reticulocytosis, microcytic anaemia, nucleated RBC’s in circulation.
How to treat beta thalassaemia>
Treat with regular life-long transfusions, iron-chelating agents, ascorbic acid and folic acid.
What is sickle cell anaemia?
Autosomal recessive disorder, production of abnormal beta-globin chains via a single base mutation of A to T
WHat are the benefits of being a carrier of sickle cell?
Protection against falciparum malaria and symptom free.
Symptoms of sickle cell crisis?
Acute pain in hands and feet, long bone pain, cognitive defects in children, pulmonary hypertension and chronic lung disease, splenic sequestration
Diagnosis of sickle cell ?
Blood film (sickled erythrocytes),
Hb 60-80
Positive sickle solubility test
Haem electrophoresis confirms diagnosis
How to treat sickle cell?
Precipitating factors avoided e.g. cold
folic acid
Acute attacks: IV fluids, analgesia, oxygen.
Oral hydroxycarbamide to reduce frequency of crises.
What is acute myeloid leukaemia?
Build up of myeloblasts in the bone marrow spilling out into the blood stream
Who does AML affect?
Adults between 50-60
Risk factors for AML?
Downs’s syndrome, raditation
Symptoms for AML?
Anaemia, infection, hepatosplenomegaly, peripheral lymphadenopathy, gum hypertrophy, bone marrow failure and bone pain
Why do you get anaemia with AML?
Myeloid cells crowd out the normal cells, leading to fewer RBC’s, anaemia and fatigue
WHat would you see on bone marrow biopsy for AML?
AUER RODS
Investigations for AML?
FBC: anaemia, thrombocytopaenia and neutropoenia.
Blood film: leukaemic blast cells
Bone marrow biopsy
Treatment of AML?
Blood and platelet infusions IV fluids Allopurinol chemo steroids infection control
What is ALL?
Malignancy of the lymphoid cells, affecting the B or T lymphocytes.
Uncontrolled proliferation of immature blast cells
What are risk factors for ALL?
Radiation during pregnancy, Down’s syndrome
Signs and symptoms of ALL?
Anaemia, infection, hepatosplenomegaly, peripheral lymphadenopathy, headache and crainial nerve palsy,
Investigations for ALL?
FBC: anaemia, thrombocytopaenia, neutropoenia.
Blood film: leukaemic blast cells
LP
CXR and CT: lymphadenopathy
How to treat ALL?
Steroids, allopurinol, methotrexate, antibiotics, chemo, blood and platelet transfusion
Which chromosome is associated with CML?
Philadelphia chromosome.
Which cells does CML affect?
Neutrophils, basophils, eosinophils and macrophages
What ages does CML most commonly affect?
40-60 rare in childhood
Signs and symptoms of CML?
Loss of weight, fever, sweats, fatigue, gout, bleeding, abdo pain
HEPATOSPLENOMEGALY
What do you see on FBC in CML?
High white cells and anaemia.
Eosinophillia, basophillia, neutrophillia
Increased B12
What do you see on blood film in CML?
Left shirt, basophillia
Treatment for CML?
Chemo and Tyrosine kinase inhibitors
IMANTINIB
What is chronic lymphocytic leukaemia?
Accumulation of mature B cells that have escaped apoptosis.
What ages is CLL seen in?
70+
What can trigger CLL?
pneumonia
Signs and symptom of CLL?
Often asymptomatic:
Enlarged, rubbery non-tender nodes
Weight loss, sweats and anorexia.
What do you see on FBC with CLL?
High WCC, with high lymphocytes
What do you see on blood film with CLL?
Small mature lymphocytes, smudge cells.
How to treat CLL?
Watch and wait
Chemo
Rituximab
What is multiple myeloma?
Affects plasma cells, excess IgG or IgA
WHo is most commonly affected by multiple myeloma?
75+ afro-caribbean’s
What is the acronym for the signs of multiple myeloma?
OLD CRAB
What does old crab stand for?
Old - over 75 C - Calcium over 2.75 R - Renal impairment A - Anaemia B - Bone lesions (pepperpot skull and back pain)
What would you see on blood film for multiple myeloma?
Rouleaux
What protein is found in the urine in multiple myeloma?
Bence Jones
What would you see on x-ray in multiple myeloma?
Pepper pot skull and fractures
What would you see on CT in multiple myeloma?>
Bone lesions and cord compression
WHat would you see in an FBC in multiple myeloma?
Anaemia and raised ESR
Treatment for multiple myeloma?
Bisphosphonates for bones Analgesia Radiotherapy Blood transfusions Infection control Chemo: CTD or VAD
Which cells are affected in lymphoma?
T and B cells in the lymphnodes
Who is commonly affected by hodgkins?
Young adults and elderly
Male predominance
Associated with EBV, SLE and siblings
What are the signs and symptoms of hodgkins?
Fever and sweating
Enlarged rubbery, tender nodes
B symptoms
Painful nodes on drinking alcohol
What cells do you see in Hodgkins lymphoma and what do they look like?
Reed Sternberg cells
Owls eyes
Investigations for hodgkins?
FBC: anaemia and high ESR
CXR - wide mediastinum
PET scan
Reed sternberg
WHat is the chemo regime for hodgkins?
ABVD
What does ABVD stand for?
Adriamycin, bleomycin, vinblastine, Dacarbazine
Which people get non-Hodgkin?
Adults over 40, EBV and Burkitt’s, FH increases risk
What are the symptoms of non-hodgkins?
Fever and sweating, enlarged rubbery non-tender nodes, systemic B symptoms, GI and skin involvement.
What is raised in non-hodkin?
Lactose dehydrogenase
What investigations would you do for non-hodgkins?
Lymphnode biopsy, bone marrow biopsy, FBC (anaemia and high ESR)
How would you treat Non-hodgkin?
Steroids, Rituximab, CHOP regimen
How do you tage lymphoma?
Ann-arbour staging
What are stages 1 to 4 in the ann-arbour staging system?
- Single lymphnode region
- 2 or more nodal areas on the same side of diaphragm
- 2 or more nodal areas on both sides of the diaphragm
- spread beyond the lymphnodes to the bone marrow or liver
WHat stimulates the production of platelets?
TPO (thrombopoietin)
Which cells are platelets produced by?
megakaryocytes
What is the lifespan of a platelt?
7-10days
Thromboxane A2 receptor?
Synthesised in platelets via COX 1
Induces platelet aggregation and vasoconstriction
What is a P2Y12 receptor?
Receptor on platelets, amplifies platelet activation
Important for clopidogrel
What is the GP2b/3a receptor?
Receptor for vWF factor which is essential for clotting and platelet adherence to vessels
What is ITP?
Autoimmune destruction of platelets
Who is ITP seen in?
mainly seen in children ages 2-6
Signs and symptoms of ITP?
Asymptomatic, purpura (bruising and purple/red rash), nosebleeds, menorrhagia, prolonged bleeding from the gums, severe headache, vomiting, fatigue.
Investigations for ITP?
FBC: thrombocytopenia, bone marrrow biopsy, platelet autoantibodies present.
How to treat ITP?
Prednisolone, IV immunoglobulins, platelet transfusions, immunosuppression (oral azathioprine)
What is TTP?
Caused by a deficiency of VWF cleaving protein
Signs and symptoms of TTP?
Flu like symptms, purpura, nosebleeds, easy bruising, menorrhagia, headache, haemoptysis, abdo pain, GI bleeding,
Investigations for TTP?
Normal coag screen
Blood smear - FRAGMENTED ERYTHROCYTES,
Raised creatinine, raised bilirubin, raised reticulocytes, proteinuria and haematuria.
Treatment for TTP?
IV plasma exchange, methylprednisolone, rituximab, folic acid
What is DIC?
Systemic activation of blood coagulation, resulting in thrombosis of small/medium size vessels and organ disfunction.
As the clotting factors are used up this may cause bleeding to occur.
What are the signs and symptoms of DIC?
Large bruises, spontaneous bleeding after venepuncture, nosebleeds, GI bleeds, confusion and shock, ARDS, purpura, gangrene.
What would you find on investigations for DIC?
Prolonged PT time, prolonged APTT, low fibrinogen and severe thrombocytopenia.
Treatment of DIC?
Treat underlying cause, platelets in those who present with bleeding, red cell transfusion, FFP to replace clotting factors
What is polycythaemia?
An increase in RBC count and haematocrit
What is absolute polycythaemia due to?
Increase in RBC mass (polycythaemia vera)
What is secondary polycythemia due to?
Hypoxia (high altitude) and high EPO secretion
Which mutation do most patients have in polycythaemia?
JAK2 mutation
Symptoms of polycythemia?
Headaches and dizziness, itching, fatigue, tinnitus, erythromelalgia (burning sensation in fingers and toes), hypertension, angina.
Investigations for polycythemia?
RAISED HAEMOGLOBIN
Raised WCC and platelets, JAK2 mutation on genetic screening
Low serum EPO
Treatment of polycythemia?
Aim is to maintain normal blood count
Venesection relieves symptoms
Low dose aspirin
Haemaphillia A?
Factor 8 deficiency
Haemaphillia B?
Factor 9 deficiency
Who gets haem A?
Males as x-linked much more common
Symptoms of moderate hemophilia A/
Bleeding following venepunctire, bleeding following trauma
Symptoms of severe hemophilia A?
Neonatal bleeding, spontaneous bleeding, GI bleeds, haematuria.
Investigations for haemophilia A?
Low Hb Normal PT and bleeding time Prolonged APTT reduced factor 8 CT head to look for hemorrhages
Treatment fo haem A?
Prophylactic factor 8, FFP containing factor 8 to be given for acute bleeds.
Desmopressin to boost factor 8 activity
Which is more common haem A or Haem B?
Haem A is much more common
Symptoms of Ham B?
Bruising, epitaxis, pallor, haemoptysis, heavy bleeding from minor trauma., bleeding from tooth loss in childhood.
Investigations for haem B?
PROLONGED APTT
low factor 9
Treatment for heam B?
Treatment must be starred before confirmed diagnosis
Vacc against hep A and B
Recombinant factor 9
Emergency bracelet
What is VW disease?
deficiency of VWF which is used in forming platelet plugs.
Symptoms of VW disease?
Bleeding from mucosa, menorrhagia, spontaneous bleeding.
Investigations for VWD?
FBC: Fibrinogen level platelet count Clotting screen plasma vWF decreased factor 8 levels can be decreased.
Treatment for VWD
Educate on bleeding risk
Stop antiplatelet drugs and NSAIDs
Tranexamic acid for minor bleeds
Desmopressin to ncrease vWF leves
What is a DVT?
When a blood clot forms in a vein, usually in the legs
Causes of DVT?
Surgery, immobilisation, contraceptive pill, longhaul flights, leg fractures, malignancy
Signs and symptoms of DVT?
Pain in calf, swelling, redness and warmth over area, unilateral and ankle oedema.
What risk score is used for DVT?
Wells score
Is a D-dimer diagnostic?
If negative it excludes DVT but if positive it isnt diagnostic
What to do if d-dimer is positive?
Doppler ultrasound
Treatment of DVT?
LMWH, Warfarin, DOAC (apixaban)
Compression stockings
What is the most common parasite to infect for malaria?
P.falciparum
Is malaria a notifiable disease?
Yes
Which is the least common parasite infection in malaria>
P.malariae
When do symptoms occur in malaria?
6 days post infection
When do p.vivax and p.ovale most commonly present?
6 months post infection
Symptoms in severe disease of p.falciparum?
SOB, fits and hypovolaemia, AKI and nephrotic syndrome.
What is the specific investigation for malaria?
Thick and thin blood smears
Treatment of nonfalciparum malaria?
Chloroquine
How to treat falciparum malaria?
Oral quinine sulphate
Angular stomatitis, lemon-yellow skin, ataxia and delusions are hallmarks of which type of anaemia?
Pernicious
You are a junior doctor on your haematology rotation. You have just seen a 50 year old woman who presented with anaemia, gum hypertrophy and bone pain. You do a full blood count which shows thrombocytopaenia and neutropoenia. A bone marrow biopsy shows Auer rods.
AML
You are an F2 on a tropic medicine rotation. You are seeing a patient who has recently been on holiday to Asia and is now complaining of nausea, loss of appetite, abdominal pain and some weight loss. Her symptoms started 6 weeks after returning to the UK. You perform some tests and diagnose tapeworm.
Eosinoplilia is raised in parasitic disease
You are a junior doctor working on a paediatric ward. Your patient is aged 3 and has anaemia, hepatosplenomegaly, headaches and bone pain. You perform a number of tests and start treating them for cancer.
ALL is the most common paeds cancer
You are an F1 who sees a 5 year-old patient with diagnosed ITP. His mother tells you that he has recently had a lot of nosebleeds and feels tired all the time.
How would you treat this patient?
Prednisalone
Mr. Jones has been in hospital for a week recovering from a knee replacement. He is 75, has hypertension and is a smoker. He hasn’t been able to move very much since his surgery and refuses to wear compression stockings. Suddenly, Mr Jones becomes very short of breath and sits up to breath. His heart rate increases to 108 and he is breathing much faster than usual.
What investigation do you want to order?
CTPA
Camilla is a 25 year old lady who has just returned home from visiting Ghana. She presents with very generic flu-like symptoms (fever, headache, cough) and is concerned that something is wrong. Whilst taking her history, you learn that she suffered from many mosquito bites during her trip.
What investigation would you organise to confirm your diagnosis?
Thick and thin blood smears
Last week you saw a patient in GP who was complaining of tiredness and recurrent infection. You ordered a full blood count and the results have shown neutropenia.
Name 2 causes of neutropenia.
When would you want to repeat the patient’s bloods?
Name 2 causes of neutropenia. Infection Drugs (phenytoin, antipsychotics) Malignancy Excess alcohol and liver disease
When would you want to repeat the patient’s bloods?
4 weeks’ time