Flashcards in Haematology Deck (99)
What is the most common form of haemoglobin?
Hb A - made up of 2alpha chains and 2 beta chains. forms 90% of the haemaglobin
Hb A2 is made up of 2 alpha chains and 2 delta chains and makes up 1-3% of the haemoglobin. A raised level may indicate thallassaemia
what additional test should be ordered with iron studies?
What should you think of when patients have pre existing conditions in questions?
IS IT AUTOIMMUNE!!!
Sever complication of pernicious anaemia
subacute combined degeneration of the spinal cord
Name 4 inherited aplastic anaemias? what proportion of AA's do they make up?
10% the other 70% are idiopathic and should be treated with immunosuppression a nd supportive care - transfusion abx and iron chelation if persitant. marrow stimulation and recovery may be used also e.g g-csf.
(D)DFS - to buy them a sofa
Schwachman diamond syndrome
Diamond Blackfan syndrome
Triad of dyskeratosis congenita
chromasome instability and telomere shortening - BM failure
Symptoms of schwachman diamond syndrome?
primarily neutrophilia - endocrine and pancreatic dysfunction
What blood results do you see in DIC? causes?
low platelets and fibrinogen, high fibrinogen degredation products, high d dimer, prolonged prothrombin time
Causes = malignancy, trauma, burns, toxins, obstetric complications.
Treatment = FFP, platelets, cryoprecipitate
What joint is most commonly affected by pseudogout?
what is another name for pseudogout
what type of bifringence do crystals show?
rhomboid shape with positive bifringence? BLUE WITH the axis.
true gout is YELLOW WITH the axis
What counts as MASSIVE blood loss?
entire blood volume in 24 hours, >50% in 3 hours
What is the normal cutoff for platelet transfusion?
most people say <50 however some say 75 (x10^9)
What is the difference between red cell concentration and red cell mass
red cell concentration varies with hydration status and plasma volume. q
what drives anaemia of chronic disease
cytokines such as IFN, IL1 and TNF decrease EPO recepter expression
What are downs syndrome children at risk of (haem)
AML in first 3 years of life and then ALL
What is an acute leukaemia
Acute Neoplastic process of bone marrow and blood resulting in a rapidly progressing and fatal disease which has >20% blast cells on BM biopsy.
What is the best way to differentiate acute and chronic leukaemias clinically?
Chronic types are usually diagnosed incidentally, wheras acute have more severe presentations with BM failure and cytopenias - anaemia, petechiae, infections (neutropenia), and hepatosplenomegaly, cranial nerve palsies due to CNS involvement, gum hypertrophy (AMMoL often with hypoK) lymphadenopathy and bone bain.
However CML in blast phase can resemble an acute leukaemia.
ALL - displayed on pre b
Philadelphia chromasome positive
usually CML but can be ALL in adults. (30% positive)
Name 2 subtypes of acute myeloid leukaemia
Acute promyleocytic leukaemia characterised by a t(15,17) with hypergranular promyelocytes with auer rods. it causes early DIC and haemorrhage. (DIC may occur with infection as a result of any leukaemia)
Acute myelomonocytic leukaemia characterised by gum hypertrophy and
Are protein C and protein S, procoagulant or anticoagulant?
anticoagulant. along with TFPI, thrombomodulin, antithrombin (these are often expressed on the vessel wall)
how long does a flight need to be before it confers an increase thrombosis risk
Which has the largest thrombosis risk:
Factor 5 leiden
family history of thrombosis
Protein C/S deficnency
C/s and antithrombin deficiency are wrst followed by 5 leiden.
What cell lines should be seen on a CML Bone marrow
Hypercellular marrow with spectrum of immature myelocytes and mature granulocytic cells in the blood. neutrophils in blood are 50-500
what are ethe three phases of CML
Chronic <5% blasts
Accellerated >10% blasts - increasing splenomegaly
Blast >20% blasts resembling acute leukaemia
(ALL transformation can occur)
whats the difference between essential thrombocythemia and thrombocytopenic purpura
high and low
How do you differentiate hodgkins and non hodgkins lymphomas
hodgkins - pain on drinking alcohol in affected nodes, reed sternberg
what is nodular lymphocytic (predominant) pertain to?
non classical type of hodgkins lymphoma. accounts for small number of cases and is indolent. not EBV associated. rarely has B symptoms. mainly lymphadenotpathy. it has atypical reed sternberg cells.