Blood Cells and Disorders II Flashcards
What does thrombopoietin cause haemopoeitic stem cells to differentiate into?
Megakaryoblasts to produce megakarocytes
What are platelets?
Small anucleate cells for haemeostatis with 5-9 day lifespan
What 3 steps occur following an injury in haemostatis?
Vascular spasm (arteries/arterioles constrict to reduce blood flow) , Platelet plug formation, Blood clotting
what are the steps platelet plug formation?
adhesion, outside-in signalling - integrin activation, secretion, aggregation, thrombin production
What ECM proteins does platelet plug formation require?
vWF, fibrinogen and collagen
What 2 platelet disorders are there?
Bernard-soulier : abnormality in gene for glycoprotein Ib/V/XI
Glanzmann thrombasthenia: abnormality in gene for glycoprotein IIb/IIIa
What are symptoms for platelet disorders?
Easy bruising, nose bleeds, bleeding from gums, prolonged heavy menses, abnormal bleeding after surgery, rarely (vomit blood, pass blood)
What can storage pool deficiencies be caused by?
Lack of granules, or failure of platelets to empty the granules
What is delta storage pool deficiency?
A lack of dense granules or can be of other inherited conditions (hermansky pudlak and chediak-higashi syndrome)
What is Grey platelet syndrome?
Rare platelet disorder by lack of alpha granules and chemicals stored inside them so proteins cant stick to blood vessel wall or clump or repair injured site
What is extrinsic blood coagulation?
fewer and rapid, initiated by TF leaking into blood from cells outside the blood stream
FVII activated by thrombin and FXa, FVIIa activates FX
What is intrinsic blood coagulation?
More complex and slower - outside tissue damage is not needed e.g endothelial cell damage
FXII activates FXI releases bradykinin (vasodilator) from HMWK, activate FIX (serine protease) hydrolyses FX - leading to common pathway
What is common pathway?
FXa cleaves prothrombin on surface, FV activated by thrombin, Fibrinogen to fibrin by thrombin, thrombin activates FXIII crosslinks fibrin clot
What is clot retraction??
Tightening of fibrin clot
What is fibrinolysis?
dissolves small inappropiate clots and dissolved clots at sites of repair, inactive plasminogen incorporated into clot activated to plasmin by body tissue substances like thrombin, plasmin digests fibrin threads and inactivates fibrinogen
What is haemophilia A in terms of genetic disease?
X linked leading to FVIII deficiency
What is haemophilia B in genetics?
Christmas disease X linked leading to FIX deficiency normal pt time
What is warfarin?
Vitamin K antagonist to prevent clotting factor activation
What is EDTA?
Chelate calcium in donated blood
What does Aspirin do?
Inhibits TxA2 synthesis
What does Streptokinase do?
Activate t-PA
What are blood groups?
Surface of RBCs containing range of glycoproteins and glycolipids which act as antigens
What does Antigen A have?
Anti-b body
What does Antigen B have?
Anti-A body
What does Antigen a+b have?
Neither antibody
What does Neither A or B antigen have?
Both antibodies
What blood can Type A have?
A or O blood
CANT HAVE B or AB
What blood can Type B have?
B or O blood
CANT have A or AB
What blood can Type AB have?
Can have any type of blood - universal recipient
What blood can Type O have?
Only O blood
Universal donor
What is Rhesus?
fixed by your genes. If you’re rhesus positive (RhD positive), it means that a protein (D antigen) is found on the surface of your red blood cells. If you do not have the D antigen, you will be rhesus negative (RhD negative). Most people are rhesus positive .
Dont normally have Rh antibodies and develop after blood transfusion causes problems for later transfusions
What is haemolytic new born disease?
is an alloimmune condition that develops in a fetus, when the IgG molecules (one of the five main types of antibodies) produced by the mother pass through the placenta.
What is Anemia symptons?
Iron deficiency, Megaloblastic (Large rbc) Pernicious (lack of heamopoises) Haemorrhagic Haemolytic Thalassemias (haemoglobin problems)
What is sickle cell anemia?
Stiff angular RBC they stick to small capillaries, long inflexible chains, Autosomal recessive inheritance, Low potassium kills malaria
What is Haemophilia?
is a mostly inherited genetic disorder that impairs the body’s ability to make blood clots, a process needed to stop bleeding
What are Leukaemias?
Production of maligant WBC cells , supress production of ALL normal cells in RBM
What are Oncogenes?
Cause malignancy when mutated leading to their activation (growth factors, receptors, dna binding proteins)
What are Tumour supressor genes?
Cause malignancy when mutated leading to their suppress growth
What is acute lymphoblastic leukemia?
Short onset from lymphoid stem cells, most common in children
What is acute myelogenous leukemia?
Affects all stages
What is chronic myelogenous leukaemia?
Prolonged onset, from myeloid stem cells mainly in adults
What is Bone Marrow transplant for?
Replacement of cancerous or abnormal RBM with healthy one
Patient RBM destroyed by chemo and whole body radiation
healthy RBM from donor or patient in remission
Marrow migrates to RBM cavities and multiply, create graftvshost disease
need to stay on immunosuppressants
