haem other Flashcards

1
Q

what chromosome has a defect in beta thalassemia

A

11

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2
Q

what chromosome has a defect in alpha thalassemia

A

16

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3
Q

how do Red Blood Cells protect themselves from oxidative stress

A

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.

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4
Q

bite and blister cells on blood film

A

GP6D deficiency

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5
Q

Haemophillia A inheritence
what is the deficiency

A

X linked recessive
VIII deficiency

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6
Q

Xa inhibitors end in

A

Ban
Eg. Apixaban

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7
Q

Mechanism of action heparin

A

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

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8
Q

Mechanism of action warfarin

A

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

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9
Q

Aspirin MOA

A

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

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10
Q

Adp receptor antagonists

A

Clopidogrel. Prasugrel

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11
Q

Main diff between venous and arterial thrmobosis

A

Arterial- rich in platelets

Venous- rich in fibrin- little platelets involved

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12
Q

Primary haemostats problems manifestations

A

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

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13
Q

What factor starts off secondary haemostasis

A

Factor VII activated by damaged surface contact

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14
Q

Vit k required for what factors

A

II VII IX X

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15
Q

How do u get vitamin k
Most at risk

A

Diet- green veg
Gut bacteria

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

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16
Q

What do u need for vitamin k to absorb

A

Fat soluble
Responsible for bile salt absorption

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17
Q

ttp presents with

A

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

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18
Q

High grade histology for acute leukemia

A

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

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19
Q

Viral vs bacterial lymphadenopathy on examination

A

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

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20
Q

Which B cell NHL is curable low grade or high grade

A

High grade- progresses quicker but more treatable/curable

Low grade- non curable

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21
Q

TTP isa result of what deficiency

A

ADAMTS13 (function is to cleaves VWF)

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22
Q

Thrombocytosis vs thrombophillia

A

Thrombophillia- too much clotting
Thrombocytosis- too many platelets

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23
Q

Where is folate absorbed

A

Duodenum and jejenum

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24
Q

where is iron absorbed

A

duodenum and jejenum

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25
Q

What must be checked in folate deficiency

A

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

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26
Q

Where is b12 absorbed

A

Terminal ileum

27
Q

What is haptoglobin

A

Serum glycoprotein which binds free haemoglobin released on damage of red cells

(Depleted from serum in states of intravascular haemolysis)

28
Q

What is urobolinogen

A

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

29
Q

What factors does vit k synthesise

A

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

30
Q

What factors does protein s breakdown

A

Va & VIIa

31
Q

What factors does anti thrombin breakdown

A

XIa, Xa and thrombin (factor II)

32
Q

Stable clot formed from

A

Platelets, fibrin and factor XIIIa

33
Q

What factor is tissue factor

A

Factor III

34
Q

Auer rods seen in

A

Acute myeloid leukaemia
Includes AMPL

35
Q

Smudge cells seen in

A

Chronic lymphocytic leukaemia

36
Q

What cells differentiate from the myeloid stem cell

A

RBCs
Platelets
Mast cells

granulocytesdifferentiate from myeloblast which comes from myeloid stem cell
Eosinohils
Basophils
Neutrophils
monocyte> macrophage

37
Q

What cells differentiate from the lymphoid stem cell

A

B cell
T cell
NK cells

38
Q

Acute vs chronic leukemia

A

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

Chronic- mature, maturation maintained

39
Q

Translocation mutation on philadelphia chromosome, what leukemia?

A

Chronic myeloid leukaemia

40
Q

What are eosinohils and basophils high in

A

Myeloproliferative disorder

41
Q

Translocation 15:17- what leukemua

A

Acyte promyelocytic leukemia

42
Q

CRAB symptoms for what haematological cancer

A

multiple myeloma

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

43
Q

what is reduced in pancytopenia

A

u have- anaemia, wbc and thrombocytopenia

44
Q

What factors in common pathway

A

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.

45
Q

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

A

philadelphia chromsome - t(9;22)

46
Q

what lymph nodes do the ovaries drain into

A

para-aortic

47
Q

warm Autoimmune Haemolytic Anaemia - what antibody

A

IgG

48
Q

Cold autoimmun haemolytic anaemia- what antibody

A

IgM

49
Q

CML is what type of myeloproliferative disorder

A

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

50
Q

what is the name of a tyrosine kinase inhibitor

A

imatinib

51
Q

causes of leucoerythroblastic film

A

sepsis
marrow infiltrate
myelofibrosis

52
Q

what is myelodysplastic syndrome

A

form of cancer from mutation in myeloid cells causing ineffective hematopoiesis: causes anaemia, neutropenia, thrombocytopenia

may be asymptomatic

can progress to acute myeloid leukaemia

53
Q

How to tell if cml or aml

A
  1. Low lymphocytes> aml or cml
  2. Wbc> 100> chronic
  3. Presence if band cells> confirms CML
    (Blasts indicates aml)
54
Q

What can chronic lymphocytic leukaemia transform into

A

Non hodgkins
(Richters transformation)

55
Q

TTP classic pentad of signs / symptoms

A

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

56
Q

most common blood cancer for ages 2-5 years

assoc with?

A

Acute Lymphoblastic leukaemia- most common cancer in children

associated with down syndrome

57
Q

most common blood cancer in teens

A

Chronic myelogenous leukemia

58
Q

when do most cases of acute myelogenous leukaemia occur

A

under 2 years

59
Q

Blood results for hodgkins

A

Normocytuc anaemia, eosinphillia
Inc LDH

60
Q

red cell lifespan
neutrophil lifespan
platelets lifespan

A

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

61
Q

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

A
62
Q

sickle cell inheritance pattern

A

autosomnal recessive

63
Q

rolauex formations are a sign of…

A

high plasma protein eg. multiple myeloma or waldenstroms etc