Haematology Flashcards
When does haemopoiesis begin in the embryo?
Where does it start?
Day 9
Aorto-gonado mesonephros
What is the most common WBC in adult blood?
Neutrophils
What are eosinophils important for?
Parasitic infections and allergies
What WBCs are involved in CML?
Basophils
Kuppfer cells and Lagerhans cells are examples of
APCs (monocytes)
What is the NR for platelets in adult blood?
150-400
What is the NR for WBCs in adult blood?
4-11
Microcytic hypochromic anaemia can be caused by
iron deficiency
thalassemia
Macrocytic anaemia can be caused by
Vit B12 deficiency
alcohol
Leucodepletion is
where RBCs are removed from whole blood
What does FFP contain?
Clotting factors
What does Prothrombin complex concentrate contain?
Vit K dependent factors
What does cryoprecipitate contain?
fibrinogen, vWF, FVIII and FXIII
What is giving transfusions of platelets contraindicated in?
TTP
ITP
Heparin induced thrombocytopenia&thrombosis
What can be used to reverse warfarin?
Prothrombin complex concentrate
What is the most common reaction to transfusion?
Febrile non-haemolytic transfusion reactions
it is due to cytokines and other active molecules accumulating during storage
What are the pulmonary complications of transfusion?
TACO- acute LV failure with pulmonary oedema
TRALI- immune mediated reaction causing leaky pulmonary capillaries
TAD- dyspnoea without signs of LV failure/abnormalities
What is measured in thrombin time?
Fibrinogen
What factors are measured in prothrombin time?
Fibrinogen
Factor II, V, VII and X
What factors are measure in aPTT?
Factors II, V, X, VIII, IX, XI and fibrinogen
What are examples of anticoagulants?
Heparin
Warfarin
DOACs
Fondaparinux
when treating someone with unfractioned heparin, what must be checked?
The patient hasn’t had a vasectomy or allergy to fish
What would you give to reverse the effect of heparin?
Protamine sulphate
What DOACs work to inhibit FXa?
Rivaroxaban
Apixaban
Edoxaban
What DOAC inhibits thrombin?
Dabigatran
What is virchow’s triad?
stasis of blood flow
endothelial injury
hypercoagulability
What type of thrombi are predominantly composed of fibrin?
Venous thrombi
What can be used a pharmacological prophylaxis for VTE?
LMW heparin (Tinzaparin)
Fondaparinux
Rivaroxaban
Dabigatran
Is the D-dimer test positive or negative in VTE?
Positive
Factor V Leiden is
where Arg 506 is replaced by Gln and so FV is more resistant to cleavage
Prothrombin 20210A is caused by
a point mutation in the 3’ untranslated region of the prothrombin gene
Anti-phospholipid syndrome can cause
thrombophilia
What type of inheritance pattern does vWD have?
Autosomal dominant
Haemophilia B and Haemophilia A are caused by deficiencies in
FIX and FVIII respectively
When does postnatal screening occur?
Day 5
When in thrombocytopenia most marked in pregnancy?
After 20 weeks
When does someone with the HbA/S trait struggle?
In extreme hypoxia and dehydration
What are examples of chronic complications of sickle cell disease?
Hyposplenism
Renal disease with tubular damage (bed wetting)
Avascular necrosis @ femoral/humeral heads
Leg ulcers
How is sickle cell disease treated?
From 6 months: penicillin
In an acute crisis: analgesia and hydration, hydroxycarbamide (to increase HbF)
Hb Barts can cause what
hydrops fetalis
beta-thalassaemia major can cause
anaemia
short stature and distorted limb growth
enlarged and overactive spleen
X-rays that show ‘hair on end’ skulls are caused by
Beta-thalassaemia major
What is a complication of beta-thalassemia major?
Severe iron overload and toxicity
It can be treated with iron chelation therapy (desferrioxamine) to prevent death
What are examples of chronic myeloproliferative disorders?
Polycythaemia vera
Essential thrombocytosis
Idiopathic myelofibrosis
What are causes of some secondary polycythaemia?
chronic lung diseases
high altitude
CO poisoning
Epo producing tumours
JAK2 mutations are common in what
various myeloproliferative disorders
In the JAK2 V617F mutation, what occurs?
Phenylalanine is changed to valine at position 617 in the protein
How is polycythaemia vera treated?
Aspirin 75mg
Reactive thrombocytosis can be caused by
surgery
Infection
Inflammation
Hyposplenism
In thrombocytosis and myelofibrosis, what mutations can be found?
JAK2
CALR (calreticulin mutation on exon 9)
Massive splenomegaly and pancytopenia can be found in
myelofibrosis
What are causes of splenomegaly?
C Cancer H Haematological (CML,CLL) I Infection (malaria, EBV) C Congestion A Autoimmune G Glycogen storage disorders O Other (sarcoidosis)
Gout, splenomegaly and fatigue are symptoms of
CML
Where is iron absorbed?
Duodenum
What group is at risk of megaloblastic anaemia due to a lack of vit B12?
Vegans (it is only found in animal sources)
what is also associated with a vit B12 deficiency?
subacute degeneration of the cord and pernicious anaemia
What can cause haemolysis?
Haemoglobinopathies
G6PD deficiency
Hereditary spherocytosis
Drugs
In anaemia of chronic disease what will the MCV be like?
Normal
What should ITP be treated with?
Steroids
IV immunoglobulin
A deficiency in ADAMTS-13 can lead to
TTP
TTP should be suspected where there is evidence of thrombocytopenia and
fever
neurological symptoms
haemolysis
How does AML present?
Bone marrow failure:
- Anaemia
- Infections
- Easy bruising and haemorrhage
FLT3 ITD mutation points to a
‘bad risk’ AML
NPM1 mutation points to a
‘good risk’ AML
What are hypomethylating agents that can be used in AML?
Azacytidine
Decitibine
Where is FLT3 found?
Chromosome 13q12
it is a proto-oncogene
How can ALL present?
Fatigue
Bruising/bleeding
Night sweats
Lymphadenopathy
How does Blinatumomab work?
combines the binding site of CD3 and CD19 to engage T cells in the lysis of CD19 B cells
CD22 is the most attractive target of
Immunotoxins and immunoconjugates
Myelodysplasia can be described as
clonal bone marrow stem cell disorders that result in ineffective haematopoiesis
Where is haemoglobin produced initially?
Yolk sac, liver and then bone marrow
In the embryonic stage, what type of Hb is present?
Hb Gower 1
Hb Gower 2
Hb Portland
In the fetal stage, what type of Hb is present?
HbF (2 alpha, 2 gamma)
In the adult, what type of Hb is present?
HbA (2 alpha, 2 beta)
HbA2 (2 alpha, 2 delta)
Why does Hb switching occur?
to take 02 from the mother
What type of Ig can cross the placenta?
IgG
What type of factors are normal at birth?
5, 8, 13 and fibrinogen
What factors are Vit K dependent?
1972, Protein S and C
What is myeloma preceded by?
MGUS
monoclonal gammopathy of undetermined significance
How can myeloma be diagoned?
C hypercalcaemia
R renal insufficiency
A anaemia
B bone lesions (osteolytic)
How can myeloma be treated?
Dexamethasone
Patients with myeloma will often have
renal insufficiency
AKI with suspected myleoma is
a medical emergency that should be treated with steroids
What is AL amyloidosis?
Where amyloid light chain fragments are aggregated and form deposits in organs
Lymphoma with Reed Sternberg cells are known as
Hodgkin’s
What type of lymphoma has a bimodal age distribution?
Hodgkin’s lymphoma
Follicular lymphoma can be characterised by
slowly enlarging lymph nodes
What is used for prognosis of follicular lymphoma?
FLIPI
>60 Ann Arbor stage III or IV LDH above limit at diagnosis Hb <120g/L 4 nodal sites of disease
Diffuse large B-cell lymphoma and Burkitt’s lymphoma are
aggressive non-Hodgkin lymphomas
CLL is
a malignant disorder of mature B-cells
staged using the Binet system
What can be used to treat CLL?
Rituximab
Bendamustine
17p deletion in CLL is associated with
a worse prognosis
Neutropenic sepsis can’t be judged by
temperature
there is an abnormal neutrophil count
it should be treated with Abx promptly
Secondary ITP can be caused by
infection
drugs
herbal remedies
How should ITP be treated?
Glucocorticoids (prednisolone)
IV immunoglobulin
Pneumonia in immunocompromised patients is usually caused by
normally ‘harmless’ organisms
CMV, RSV, Streptococcus pneumoniae, pneumocystitis
