haem other Flashcards
what chromosome has a defect in beta thalassemia
11
what chromosome has a defect in alpha thalassemia
16
how do Red Blood Cells protect themselves from oxidative stress
glucose - 6- dehydrogenase enzyme- needed for pentose phosphate pathway aka HMP shunt. this shunt = NADPH
NADPH allows for glutathione to be reduced.
reduced glutatione> detoxifys free radicals eg. turnd hydrogen peroxide into water.
bite and blister cells on blood film
GP6D deficiency
Haemophillia A inheritence
what is the deficiency
X linked recessive
VIII deficiency
Xa inhibitors end in
Ban
Eg. Apixaban
Mechanism of action heparin
activating antithrombin , which accelerates the inactivation of coagulation enzymes thrombin (factor IIA), factor Xa and factor IXA.
Mechanism of action warfarin
competitively inhibits the vitamin K epoxide reductase complex 1 (VKORC1), an essential enzyme for activating the vitamin K available in the body
vit k required for factors 2,7,9,10
Aspirin MOA
Inhibits cycloixygenase-1 = reduced thromboxane A2 and prostoglandins>reduced platelets
Adp receptor antagonists
Clopidogrel. Prasugrel
Main diff between venous and arterial thrmobosis
Arterial- rich in platelets
Venous- rich in fibrin- little platelets involved
Primary haemostats problems manifestations
Mucosal linings bleeding eg mouth, back of eye, bruising of lower limbs (purpuric rash)
What factor starts off secondary haemostasis
Factor VII activated by damaged surface contact
Vit k required for what factors
II VII IX X
How do u get vitamin k
Most at risk
Diet- green veg
Gut bacteria
Neonates - haemorrhagic disease of the newborn (not hus!)
What do u need for vitamin k to absorb
Fat soluble
Responsible for bile salt absorption
ttp presents with
fever, neuro signs, thrombocytopenia, haemolytic anaemia and renal failure
High grade histology for acute leukemia
Large cells with high nuclear-cytoplasmic ratio, prominent nuclei, rapid proliferation
Viral vs bacterial lymphadenopathy on examination
Viral- no skin inflammation/thethered skin
Bac- yes skin inflamed, maybe skin tethered (due to fibrosis if chronic)
(both tender)
Which B cell NHL is curable low grade or high grade
High grade- progresses quicker but more treatable/curable
Low grade- non curable
TTP isa result of what deficiency
ADAMTS13 (function is to cleaves VWF)
Thrombocytosis vs thrombophillia
Thrombophillia- too much clotting
Thrombocytosis- too many platelets
Where is folate absorbed
Duodenum and jejenum
where is iron absorbed
duodenum and jejenum
What must be checked in folate deficiency
B12
Why? Because if u treat folate and b12 is low= subacute degeneration of the cord
Where is b12 absorbed
Terminal ileum
What is haptoglobin
Serum glycoprotein which binds free haemoglobin released on damage of red cells
(Depleted from serum in states of intravascular haemolysis)
What is urobolinogen
Product of bilirubin metabolism- some excreted in feces and some reabsorbed from gut and excreted in urine
What factors does vit k synthesise
II, VII, IX, X
And
Protein c & s (which are natural anticoags hence LMWH heparin required too initially)
What factors does protein s breakdown
Va & VIIa
What factors does anti thrombin breakdown
XIa, Xa and thrombin (factor II)
Stable clot formed from
Platelets, fibrin and factor XIIIa
What factor is tissue factor
Factor III
Auer rods seen in
Acute myeloid leukaemia
Includes AMPL
Smudge cells seen in
Chronic lymphocytic leukaemia
What cells differentiate from the myeloid stem cell
RBCs
Platelets
Mast cells
granulocytesdifferentiate from myeloblast which comes from myeloid stem cell
Eosinohils
Basophils
Neutrophils
monocyte> macrophage
What cells differentiate from the lymphoid stem cell
B cell
T cell
NK cells
Acute vs chronic leukemia
Acute- immature cells (so blasts would be seen), failure of maturation
Chronic- mature, maturation maintained
Translocation mutation on philadelphia chromosome, what leukemia?
Chronic myeloid leukaemia
What are eosinohils and basophils high in
Myeloproliferative disorder
Translocation 15:17- what leukemua
Acyte promyelocytic leukemia
CRAB symptoms for what haematological cancer
multiple myeloma
(Calcaemia, Renal, Anaemia (and thrombocytopenia), Bone pain)
what is reduced in pancytopenia
u have- anaemia, wbc and thrombocytopenia
What factors in common pathway
1 +13
2
5
10(a)
(2x5=10)
factor 10’ and 5 allow for the conversion of prothombin into thrombin (factor 2). this then allos fibrinogen > fibrin (1). factor x111>a helps stabilise fibrin mesh.
what mutation is seen in 95% of chronic myeloid luekaemia patients
philadelphia chromsome - t(9;22)
what lymph nodes do the ovaries drain into
para-aortic
warm Autoimmune Haemolytic Anaemia - what antibody
IgG
Cold autoimmun haemolytic anaemia- what antibody
IgM
CML is what type of myeloproliferative disorder
BCR-ABL1 positive- this gene encodes for tyrosine kinase. (target of therapy)
what is the name of a tyrosine kinase inhibitor
imatinib
causes of leucoerythroblastic film
sepsis
marrow infiltrate
myelofibrosis
what is myelodysplastic syndrome
form of cancer from mutation in myeloid cells causing ineffective hematopoiesis: causes anaemia, neutropenia, thrombocytopenia
may be asymptomatic
can progress to acute myeloid leukaemia
How to tell if cml or aml
- Low lymphocytes> aml or cml
- Wbc> 100> chronic
- Presence if band cells> confirms CML
(Blasts indicates aml)
What can chronic lymphocytic leukaemia transform into
Non hodgkins
(Richters transformation)
TTP classic pentad of signs / symptoms
F- ever
A- Anaemia (microangiopathic haemolytic anaemia)
T- hrombocytopenia
R- enal disease
N- eurological abnormalities
most common blood cancer for ages 2-5 years
assoc with?
Acute Lymphoblastic leukaemia- most common cancer in children
associated with down syndrome
most common blood cancer in teens
Chronic myelogenous leukemia
when do most cases of acute myelogenous leukaemia occur
under 2 years
Blood results for hodgkins
Normocytuc anaemia, eosinphillia
Inc LDH
red cell lifespan
neutrophil lifespan
platelets lifespan
RBC- 120days
neutrophils- 7-8 hours
platelets- 7-10 days
Draw the hematopoietic tree thing with all the different cells that are made
sickle cell inheritance pattern
autosomnal recessive
rolauex formations are a sign of…
high plasma protein eg. multiple myeloma or waldenstroms etc
