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Summary Haematology overview

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Overview of the key conditions within haematology that medical students need to know for finals.

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  • May 22, 2021
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  • 2020/2021
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charlottetomlinson
Haematology
Pre-clinical................................................................................................................................................2
Haemostasis..........................................................................................................................................................2
Haematopoiesis.....................................................................................................................................................2
Coagulation screen and blood film interpretation.....................................................................................3
Sickle cell disease......................................................................................................................................5
Acute intermittent porphyria....................................................................................................................7
Anaemia...................................................................................................................................................7
Microcytic anaemia................................................................................................................................................8
Iron deficiency anaemia................................................................................................................................................................8
Thalassaemia.................................................................................................................................................................................9
Sideroblastic anaemia.................................................................................................................................................................10
Normocytic anaemia............................................................................................................................................10
Anaemia of chronic disease........................................................................................................................................................10
Aplastic anaemia.........................................................................................................................................................................10
Macrocytic anaemia.............................................................................................................................................11
Folate deficiency.........................................................................................................................................................................11
B12 deficiency.............................................................................................................................................................................11
Haemolytic anaemia............................................................................................................................................12
Blood presentations................................................................................................................................14
Neutropenia.........................................................................................................................................................14
Neutrophilia.........................................................................................................................................................15
Pancytopenia.......................................................................................................................................................16
Thrombocytopenia...............................................................................................................................................16
Thrombophilias.......................................................................................................................................17
Inherited thrombophilias.....................................................................................................................................18
Antithrombin III deficiency.........................................................................................................................................................18
Protein C and S deficiency..........................................................................................................................................................18
Factor V Leiden mutation...........................................................................................................................................................18
Prothrombin 202010 A mutation...............................................................................................................................................19
Acquired thrombophilias.....................................................................................................................................19
Paroxysmal nocturnal haemoglobulinuria..................................................................................................................................19
Antiphospholipid syndrome.......................................................................................................................................................19
Myeloproliferative disorders......................................................................................................................................................20

Thrombosis.............................................................................................................................................23
Venous thromboembolism..................................................................................................................................23
Arterial thrombosis..............................................................................................................................................24
Paraproteinaemias.................................................................................................................................24
Congenital bleeding disorders.................................................................................................................25
vWF deficiency.....................................................................................................................................................25
Platelet deficiency................................................................................................................................................26
Haemophilia........................................................................................................................................................26
Acquired bleeding disorders....................................................................................................................26

, Warfarin..............................................................................................................................................................26
Antiplatelet therapy.............................................................................................................................................27
Hereditary haemochromatosis.............................................................................................................................28

Pre-clinical
Haemostasis
Haemostasis – natural process in which blood flow
slows and a clot forms to prevent blood loss during
an injury.
1. Platelet plug formation
2. Coagulation
Platelet plug formation:
 Endothelial injury – nerves and SM cells
detect injury. This  contraction of blood
vessel. Secretion of NO and prostaglandins
stop and secretion of endothelin begins 
further contraction.
 Damage to endothelial cells exposes
collagen, and the damaged cells release Von
Willebrand factor which binds to the
collagen.
 GP1B surface proteins on platelets bind to
VWF
 Platelets change shape, release more VWF,
serotonin, calcium, ADP, thromboxane A2.
ADP and thromboxane A2  GPIIB/IIIA
expression.
 GPIIB/IIIA binds to fibrinogen which links
the platelets  platelet plug
Coagulation:
 Extrinsic pathway – trauma damages blood
vessel, exposes cells under endothelial
layer. Factor III (tissue factor) is embedded
in membrane. Factor VII in the blood binds
to tissue factor  VIIa-TF complex
o Measured by PT
 Intrinsic pathway – circulating factor XII
contacts negatively charged phosphates on
platelets/subendothelial collagen  Factor
XIIa. Factor XIIa cleaves factor XI  factor
XIa. Factor XIa (+calcium) cleaves factor IX
 IXa. Factor IXa + factor VIIIa (which is
bound to VWF) = prothrombinase complex,
which then enters the common pathway
o Measured by APTT
Vitamin K regulates blood coagulation – converts
coagulation factors into mature forms. Factors 2, 7,
9, 10 require vitamin K.



Haematopoiesis
Haematopoiesis = process of haematopoietic stem cells in BM producing mature blood cells.
 In embryos, haematopoiesis occurs in blood islands in the yolk sac, and in spleen, liver and LNs. At around 4-5
months gestation the BM takes over.
Haematopoietic stem cells can form 2 cell lineages.

, Coagulation screen and
blood film
interpretation
Activated partial thromboplastin time
(APTT) – measure of time taken for intrinsic
pathway to occur. Usually 35-40 seconds.

Prothrombin time (PT) – measure of the
time taken for the extrinsic pathways to
occur. INR is a direct measure of PT
(internationally standardised, therefore
used for warfarin checks). Usually 12-13
seconds. INR usually 0.8-1.2.

Thrombin time – test of how fast fibrinogen
is converted  fibrin by thrombin. This can
be due to DIC, liver failure, malnutrition,
abnormal fibrinolysis.

Blood clotting results:
 Haemophilia -  APTT, normal PT, normal bleeding time APTT = intrinsic and
 Von willebrand’s disease -  APTT, normal PT,  bleeding time common pathways
 Vitamin K deficiency -  APTT,  PT, normal bleeding time PT = extrinsic and common
 DIC -  APTT, PT,  bleeding time,  platelets pathways
 ITP/TTP/HUS - bleeding time and  platelets, normal APTT and PT

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