Haematology Flashcards
what is the mode of inheritance for G6PD deficiency?
X-linked recessive
what is the role of G6PD enzyme?
protects red blood cells from oxidative stress
what is thrombocytopenia?
deficiency of platelets in blood
triad of haemolytic uraemic syndrome
AKI, anaemia and thrombocytopenia
commonest cause of haemolytic uraemic syndrome in children?
E.coli 0157
what is haemolytic anaemia?
premature destruction of red blood cells
hereditary haemolytic anaemia conditions
sickle cell, thalassaemias, hereditary spherocytosis
acquired haemolytic anaemia conditions
autoimmune disorders, infections e.g. malaria, medication reactions or trauma
Describe intravascular haemolysis and the pathophysiology of it
Haemolysis occurs in bloodstream
leads to release of free haemoglobin
-excess dealt with:
combo with haptoglobin/ albumin
lost in urine
stored in epithelial cells
Describe extravascular haemolysis and the pathophysiology of it
haemolysis taking place outside bloodstream
-mainly in spleen and liver -> RBCs= phagocytosed
splenomegaly and hepatomegaly are typical
what type of anaemia does anaemia of chronic disease present with
microcytic or normocytic
what are pencil cells associated with?
iron deficiency anaemia
(mainly when caused by coeliac disease)
what are Howell-Jolly bodies?
remnants of RBC nuclei
* they’re Jolly cause they have extra stuff to others *
what do Howell-Jolly bodies indicate?
functional hyposplenism - spleen not functioning or true - splenectomy
What is DIC? and what do blood results look like?
disseminated intravascular coagulation
consumption of clotting factors
blood results
- thrombocytopenia
- schistocytes
- raised d-dimer (fibrin degradation product)
- clotting profile = increased prothrombin time and APTT, decreased fibrinogen
what is ferritin?
acute phase protein- increases in response to infection
iron studies of anaemia of chronic disease
low serum iron - inhibition of iron absorption
high ferritin -
low transferrin - reduced ability
what is the blood universal donor?
O-
What blood can rhesus +ve patients receive?
Rh -ve AND +ve
fatigue, headache and itchy after hot bath?
polycythaemia rubra vera
what should be given to patient on warfarin experiencing significant bleed?
Prothrombin complex concentrate (PTCC)- reverses anticoagulation and contains factors 2, 7, 9 and 10
which test confirms the diagnosis of Haemophilia A?
factor VIII assay
NHS management of neutropenic sepsis?
IV piperacillin with tazobactam (broad spectrum asap)
diagnosis of chronic lymphocytic leukaemia
elevated white cell count with absolute lymphocytosis
-smudge cells on blood film
mot common hereditary thrombophilia
Factor V leiden
acquired thrombophilias
anti-phospholipid syndrome
what is myelofibrosis
myeloproliferative disorder
often presenting with pancytopenia
signs and symptoms of myelofibrosis
weight loss
night sweats
splenomegaly
marrow failure
signs: poikilocytes, dry tap on bone marrow aspiration
management of major bleed and INR>8
stop warfarin, give vit K and prothrombin complex concentrate
complication of essential thrombocytosis
ischaemic stroke
investigations for tumour lysis syndrome
ECG, U&E, calcium, uric aid
primary part of coagulation that heparin exerts action on
activation of antithrombin III - inactivation of thrombin -> inhibits formation of stable blood clots
type of reaction associated with immediate, severe reaction following blood transfusion
type II hypersensitivity - involves IgG/ IgM
what factor initiates the coagulation cascade?
factor XII
deficiency in what factor results in Haemophilia A
factor VIII
what feature may be present in patient with excessive loss of plasma proteins
increased susceptibility to infection
what blood type can receive donations from all blood types
AB positive