haem other

Question 1

what chromosome has a defect in beta thalassemia

Answer 1

11

Question 2

what chromosome has a defect in alpha thalassemia

Answer 2

16

Question 3

how do Red Blood Cells protect themselves from oxidative stress

Answer 3

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.

Question 4

bite and blister cells on blood film

Answer 4

GP6D deficiency

Question 5

Haemophillia A inheritence
what is the deficiency

Answer 5

X linked recessive
VIII deficiency

Question 6

Xa inhibitors end in

Answer 6

Ban
Eg. Apixaban

Question 7

Mechanism of action heparin

Answer 7

activating antithrombin , which accelerates the inactivation of coagulation enzymes thrombin (factor IIA), factor Xa and factor IXA.

Question 8

Mechanism of action warfarin

Answer 8

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

Question 9

Aspirin MOA

Answer 9

Inhibits cycloixygenase-1 = reduced thromboxane A2 and prostoglandins>reduced platelets

Question 10

Adp receptor antagonists

Answer 10

Clopidogrel. Prasugrel

Question 11

Main diff between venous and arterial thrmobosis

Answer 11

Arterial- rich in platelets

Venous- rich in fibrin- little platelets involved

Question 12

Primary haemostats problems manifestations

Answer 12

Mucosal linings bleeding eg mouth, back of eye, bruising of lower limbs (purpuric rash)

Question 13

What factor starts off secondary haemostasis

Answer 13

Factor VII activated by damaged surface contact

Question 14

Vit k required for what factors

Answer 14

II VII IX X

Question 15

How do u get vitamin k
Most at risk

Answer 15

Diet- green veg
Gut bacteria

Neonates - haemorrhagic disease of the newborn (not hus!)

Question 16

What do u need for vitamin k to absorb

Answer 16

Fat soluble
Responsible for bile salt absorption

Question 17

ttp presents with

Answer 17

fever, neuro signs, thrombocytopenia, haemolytic anaemia and renal failure

Question 18

High grade histology for acute leukemia

Answer 18

Large cells with high nuclear-cytoplasmic ratio, prominent nuclei, rapid proliferation

Question 19

Viral vs bacterial lymphadenopathy on examination

Answer 19

Viral- no skin inflammation/thethered skin
Bac- yes skin inflamed, maybe skin tethered (due to fibrosis if chronic)
(both tender)

Question 20

Which B cell NHL is curable low grade or high grade

Answer 20

High grade- progresses quicker but more treatable/curable

Low grade- non curable

Question 21

TTP isa result of what deficiency

Answer 21

ADAMTS13 (function is to cleaves VWF)

Question 22

Thrombocytosis vs thrombophillia

Answer 22

Thrombophillia- too much clotting
Thrombocytosis- too many platelets

Question 23

Where is folate absorbed

Answer 23

Duodenum and jejenum

Question 24

where is iron absorbed

Answer 24

duodenum and jejenum

Question 25

What must be checked in folate deficiency

Answer 25

B12 Why? Because if u treat folate and b12 is low= subacute degeneration of the cord

Question 26

Where is b12 absorbed

Answer 26

Terminal ileum

Question 27

What is haptoglobin

Answer 27

Serum glycoprotein which binds free haemoglobin released on damage of red cells (Depleted from serum in states of intravascular haemolysis)

Question 28

What is urobolinogen

Answer 28

Product of bilirubin metabolism- some excreted in feces and some reabsorbed from gut and excreted in urine

Question 29

What factors does vit k synthesise

Answer 29

II, VII, IX, X And Protein c & s (which are natural anticoags hence LMWH heparin required too initially)

Question 30

What factors does protein s breakdown

Answer 30

Va & VIIa

Question 31

What factors does anti thrombin breakdown

Answer 31

XIa, Xa and thrombin (factor II)

Question 32

Stable clot formed from

Answer 32

Platelets, fibrin and factor XIIIa

Question 33

What factor is tissue factor

Answer 33

Factor III

Question 34

Auer rods seen in

Answer 34

Acute myeloid leukaemia Includes AMPL

Question 35

Smudge cells seen in

Answer 35

Chronic lymphocytic leukaemia

Question 36

What cells differentiate from the myeloid stem cell

Answer 36

RBCs Platelets Mast cells granulocytesdifferentiate from myeloblast which comes from myeloid stem cell Eosinohils Basophils Neutrophils monocyte> macrophage

Question 37

What cells differentiate from the lymphoid stem cell

Answer 37

B cell T cell NK cells

Question 38

Acute vs chronic leukemia

Answer 38

Acute- immature cells (so blasts would be seen), failure of maturation Chronic- mature, maturation maintained

Question 39

Translocation mutation on philadelphia chromosome, what leukemia?

Answer 39

Chronic myeloid leukaemia

Question 40

What are eosinohils and basophils high in

Answer 40

Myeloproliferative disorder

Question 41

Translocation 15:17- what leukemua

Answer 41

Acyte promyelocytic leukemia

Question 42

CRAB symptoms for what haematological cancer

Answer 42

multiple myeloma (Calcaemia, Renal, Anaemia (and thrombocytopenia), Bone pain)

Question 43

what is reduced in pancytopenia

Answer 43

u have- anaemia, wbc and thrombocytopenia

Question 44

What factors in common pathway

Answer 44

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.

Question 45

what mutation is seen in 95% of chronic myeloid luekaemia patients

Answer 45

philadelphia chromsome - t(9;22)

Question 46

what lymph nodes do the ovaries drain into

Answer 46

para-aortic

Question 47

warm Autoimmune Haemolytic Anaemia - what antibody

Answer 47

IgG

Question 48

Cold autoimmun haemolytic anaemia- what antibody

Answer 48

IgM

Question 49

CML is what type of myeloproliferative disorder

Answer 49

BCR-ABL1 positive- this gene encodes for tyrosine kinase. (target of therapy)

Question 50

what is the name of a tyrosine kinase inhibitor

Answer 50

imatinib

Question 51

causes of leucoerythroblastic film

Answer 51

sepsis marrow infiltrate myelofibrosis

Question 52

what is myelodysplastic syndrome

Answer 52

form of cancer from mutation in myeloid cells causing ineffective hematopoiesis: causes anaemia, neutropenia, thrombocytopenia may be asymptomatic can progress to acute myeloid leukaemia

Question 53

How to tell if cml or aml

Answer 53

1. Low lymphocytes> aml or cml 2. Wbc> 100> chronic 3. Presence if band cells> confirms CML (Blasts indicates aml)

Question 54

What can chronic lymphocytic leukaemia transform into

Answer 54

Non hodgkins (Richters transformation)

Question 55

TTP classic pentad of signs / symptoms

Answer 55

F- ever A- Anaemia (microangiopathic haemolytic anaemia) T- hrombocytopenia R- enal disease N- eurological abnormalities

Question 56

most common blood cancer for ages 2-5 years assoc with?

Answer 56

Acute Lymphoblastic leukaemia- most common cancer in children associated with down syndrome

Question 57

most common blood cancer in teens

Answer 57

Chronic myelogenous leukemia

Question 58

when do most cases of acute myelogenous leukaemia occur

Answer 58

under 2 years

Question 59

Blood results for hodgkins

Answer 59

Normocytuc anaemia, eosinphillia Inc LDH

Question 60

red cell lifespan neutrophil lifespan platelets lifespan

Answer 60

RBC- 120days neutrophils- 7-8 hours platelets- 7-10 days

Question 61

Draw the hematopoietic tree thing with all the different cells that are made

Answer 61

Question 62

sickle cell inheritance pattern

Answer 62

autosomnal recessive

Question 63

rolauex formations are a sign of...

Answer 63

high plasma protein eg. multiple myeloma or waldenstroms etc