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RACP Written Exam > Haematology > Flashcards

Haematology Flashcards

1
Q

Donation order of blood products

A

O can donate to A, B and AB
A and B can donate to AB
AB can only donate to AB

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2
Q

What are duffy antibodies and what pathology is associated

A

antibody which is negative on antibody testing but can cause haemolytic transfusion reactions
site of entry for plasmodium vivax into RBCs

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3
Q

What does FFP contain and what is used for

A

coagulation factors
used in coagulopathies

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4
Q

what does cryoprecipitate contain

A

Fibrinogen
factor 8, vWF, factor 13
used in critical bleeding, trauma, DIC, big procedures

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5
Q

Platelets orignate from

A

magakaryocytes

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6
Q

macrophages originate from

A

monocytes

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7
Q

What does hypersegmented neutrophils indicate

A

B12/folate deficiency

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8
Q

Receptors present on platelets

A

Gp1b for vWF receptor
Gp11b/111a receptor for fibriniogen

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9
Q

Platelet proliferation stimulated by

A

thrombopoeitin stimulates magakaryocyte proliferation

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10
Q

What are macrophages activated by

A

IFN gamma

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11
Q

Most important commponent of granuloma

A

macrophages fuse to form giant cells

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12
Q

Causes of eosinophilia

A

PACMAN Eats
Parasites
Asthma
Chronic adrenal insufficiency
Myeloproliferative disorders
Allergic processes
Neoplasm (hodgkins)
Eosinophilic granulomatosis with polyangitis

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13
Q

Causes of basophilia

A

myeloproliferative (esp CML)

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14
Q

Causes of IgE independent mast cell degranulation

A

Vancomycin, opioids and contrast

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15
Q

What is mastocytosis

A

rare proliferation of mast cells in skin and organs
associated with c-KIT mutations and raised serum tryptase
high histamine causes flushing, pruritis, hypotension, abdo pain, diarrhoea and peptic ulcer disease

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16
Q

NK cell surface receptors

A

CD56 and CD16

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17
Q

Nk cell function

A

induces apoptosis in those not espressing MHC1

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18
Q

B cell surface receptors

A

CD19, CD20, CD21

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19
Q

T cell surface receptors

A

both have CD3
CD8 (cytotoxic)
CD4 (helper)

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20
Q

DAT positive if

A

AIHA

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21
Q

Platelet plug formation process

A

injury -> vWF binds to exposed collagen -> platelets bind to vWF via Gp1b -> platelets release ADP binds to PY12 receptor and induces Gp11b/111a on surface of platelets -> more plalets bind to Gp11b/111a and link platelets

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22
Q

Mechanism of aspirin

A

irreversibly inhibits COX which inhibits TXA2 synthesis (pro-aggregation of platelets)

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23
Q

MoA for clopidogrel, ticagrelor and prasugrel

A

inhibit ADP binding to P2Y12 receptor, blocks expression of G11b/111a

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24
Q

Tirofiban and eptifibatide MoA

A

inhibit Gp11b/111a directly

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25
Q

Desmopressin effect on coagulation

A

promotes release of vWF and factor 8

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26
Q

What is in extrinsic pathway

A

VII to VII and tissue factor complex

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27
Q

Intrinsic pathway process

A

Tissue damage activates factor XII -> leads to downstream formation of IXa/VIIIa complex formation (to help process X to Xa)

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28
Q

How does C1 esterase inhibitor affect coagulation

A

inhibits XIIa and XIa in the intrinsic pathway

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29
Q

What is the function of protein C and S

A

inhibits IXa/VIIIa complex and inhibits prothrombinase (Va/Xa)
Stops coagulation cascade

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30
Q

Function of heparin, LMWH, dabigatran

A

direct thrombin (IIa) inhibitors (stop conversion of fibrinogen to fibrin)

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31
Q

Process how clots are broken down

A

cleaved by plasmin
plasminogen converted to plasmin by tPA (synthestic tPA tenectaplase, alteplase)
tPA blocked by transexamic acid

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32
Q

MoA transexamic acid

A

blocks tPA from processing plasminogen to plasmin

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33
Q

Vit K dependent factors

A

2, 7, 9, 10, protein C and S

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34
Q

Haemophilia A is def of

A

factor 8
x-linked recessive

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35
Q

Haemophilia B is def of

A

factor 9
x-linked recessive

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36
Q

haemophilia C is def of

A

factor 11
autosomal recessive

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37
Q

C1 esterase function

A

inhibits kallikrein which activates bradykinin
C1 esterase def -> hereditary angioedema

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38
Q

MoA antithrombin

A

mainly inhibits thrombin (IIa) and Xa
heparin enhances activity of antithrombin

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39
Q

Mechanism of factor V leiden mutation

A

produces factor V resistant to inhibition by activated protein C
increases clotting

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40
Q

Warfarin MoA

A

inhibits vit K epoxide reductase so Vit K cannot be activated

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41
Q

spur cells assoc

A

liver disease
vit E def
abetalipoproteinaemia

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42
Q

burr cells assoc

A

liver disease
ESRD
pyruvate kinase def

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43
Q

teardrop cells assoc

A

bone marrow infiltration

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44
Q

schistocytes association

A

MAHAs (DIP, TTP/HUS, HELLP) and mechanical haemolysis (valves)

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45
Q

bite cells assoc

A

G6PD deficiency

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46
Q

Spherocytes assoc

A

AIHA
hereditary spherocytosis

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47
Q

elliptocytes assoc

A

hereditary elliptocytosis - mutation in gene encoding for RBC membrane

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48
Q

target cells assoc

A

HbC disease
asplenia
liver disease
thalassemia

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49
Q

sickle cell crisis occurs when

A

low O2 conditions (high altitude, acidosis)
high HbS concentration (dehydration)

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50
Q

Howell-Jolly bodies assoc

A

functional hyposplenism (sickle)
asplenia

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51
Q

basophilic stippling assoc

A

sideroblastic anaemia
thalassemias

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52
Q

pappenheimer bodies assoc

A

siderblastic anaemia

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53
Q

heinz bodies assoc

A

G6pd deficiency

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54
Q

Causes of microcytic anaemia

A

TAIL
thalassemias
Anaemia of chronic disease
Irion def
Lead poisening

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55
Q

How to tell between intrinsic and extrinsic haemolytic anaemia

A

instrinsic - low hapto
extrinsic - +ve DAT

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56
Q

Causes of intrinsic haemolysis

A

Membrane defects - hereditary spherocytosis, PNH
Enzyme deficiencies - G6PD def, pyruvate kinase def
Haemoglobinopathies - sickle cell, HbC

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57
Q

Causes of extrinsic haemolysis

A

autoimmune
microangiopathic
macroangiopathic
infections

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58
Q

Difference between megaloblastic and non-megaloblastic anaemia

A

both MCV >100
Megaloblastic has DNA affected -> B12 def, orotic anaemia, fanconi anaemia
non - alcohol use, liver disease

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59
Q

Iron studies in iron def

A

low iron, low ferritin, low transferrin satn, high transferrin

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60
Q

iron studies in anaemia of chronic disease

A

low iron, high ferritin, low transferrin, low/normal transferrin satn

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61
Q

Iron studies in pregnancy/OCP use

A

high transferrin

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62
Q

How is iron absorbed

A

Iron is reduced to Fe2+ by DCYTB before it is absorbed by DMT1. Then bound to ferritin and moved out of cell by feroportin

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63
Q

Role of hepcidin

A

binds and internalises ferroprotin
decreases iron absorption

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64
Q

What regulates hepcidin

A

hepcidin decreased in iron deficiency, hypoxia, non-ferroportin mutations of haemochromotosis (HFE, TFR2, HAMP and HJV)
Hepcidin increased in inflammatory states, iron overload, feroportin mutated haemochromatosis

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65
Q

Signs of iron def

A

anaemia sx
koilonychia
pica
glossitis, cheilosis
Plummer-vinson sydrome

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66
Q

What is Plummer-vinson syndrome

A

iron def, esophageal webs and dysphagia

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67
Q

Population alpha thalassemia most prevalent

A

asian and african

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68
Q

alpha thalassemia what on peripheral smear

A

target cells

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69
Q

degrees of alpha thalassemia

A

Hb barts - 4 deletions, dead
HbH disease - 3 deletions, severe anaemia’
alpha thalassemia minor - 2 deletions, mild anaemia
alpha thalassemia minima - siletn carrier

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70
Q

population common in beta thalassemia

A

mediterraneans

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71
Q

Degree of beta thalassemia

A

beta thalassemia major - 2 absent Beta globin chains, severe anaemia, skeletal deformities, extramedullary haemotopoesis (heptosplenomegaly)
beta thalassemia intimedia - 1 absent or reduced beta globin chain with another reduced chain, variable anaemia
beta thalassemia minor - one chain reduced beta globin, mild anaemia, raised HbA2 >3.5%

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72
Q

What is luspatercept

A

for treatment of thalassemia, modified form of actin receptor IIb, binds TGF-beta ligands and increases differenciation and proliferation of erythroid precursors

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73
Q

how does lead poisening cause microcytic anaemia

A

inhibits ferrochelatase and ALA dehydrogrenase -> reduced heme synthesis

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74
Q

sx of lead poisening

A

lead lines on gingivae and metaphysis of bones
encephalopathy
erythrocyte basophilic stippling
abdo colic
sideroblastic anaemia
wrist and foot drops

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75
Q

treatment of lead poisening

A

chelation with succimer or EDTA

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76
Q

Causes of sideroblastic anaemia

A

genetic - x-linked defect in ALA synthase gene
acquired - myelodysplasic syndromes
reversible - alcohol, lead poisening, B6 def, copper def, drugs

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77
Q

where is B12 absorbed

A

terminal ileum

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78
Q

difference between folate and B12 def

A

folate doesn’t have neurological sx
folate has raised homocysteine but normal methylmalonic acic
B12 has cord degen and raised both homocysteine and methylmalonic acid

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79
Q

Associations of HLA DR5

A

pernicious anaemia and hashimoto’s

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80
Q

Lab indications of haemolysis

A

high LDH, reticulocytosis, unconjugated bili, pigmented gallstones, urobilinogen in the urine

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81
Q

Ix findings of intravascular haemolysis

A

low haptoglobin, schistocytes on smear
haemoglobin in urine
urinary haemosiderins
urobilinogen in urine

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82
Q

Causes of intravascular haemolysis

A

mechanical (prosthetic valve)
PNH
MAHAs

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83
Q

How does extravascular haemolysis occur

A

RBCs destroyed by macrophages in spleen -> unconjugated bili -> conjugated in liver -> urobilinogen to bowel, readbsorbed and process in liver to be excreted in urine

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84
Q

Ix findings in extravascular haemolysis

A

splenomegaly
spherocytes in smear
no Hb or haemosiderin in urine
can have urobilinogen in urine

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85
Q

Main mechanism of anaemia of chronic disease

A

inflammation raised IL6 which increases hepcidin which internalises ferroprotin and reduces iron absorption from gut

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86
Q

aplastic anaemia causes

A

radiation
viral
fanconi anaemia
idiopathic
drugs

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87
Q

which viruses cause aplastic anaemia

A

EBV, HIV, hepatitis

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88
Q

which drugs cause apastic anaemia

A

cholamphenicol
alkylating agents
antimetabolites

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89
Q

Cause of fanconi anaemia

A

Autosomal recessive DNA repair defect leading to bone marrow failure

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90
Q

Clinical features of fanconi anaemia

A

short stature
cafe au lait spots
thumb/radial defects
predisposition to malignancy

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91
Q

Ix findings of aplastic anaemia

A

low reticulocytes
elevated EPO

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92
Q

aplastic anaemia vs aplastic crisis

A

aplastic anaemia has pancytopenia whereas crisis has anaemia only

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93
Q

Sx of aplastic anaemia

A

those related to anaemia - fatigue, malaiase, pallor
thrombocytopenia - purpura, mucosal bleeding, petechiae
leukopenia - infection

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94
Q

Causes of intrinsic haemolysis

A

Hereditary spherocytosis
PNH
Pyruvate kinase deficiency
sickle cell
HbC disease
G6PD

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95
Q

Underlying pathology of hereditary spherocytosis

A

autosomal dominant
defect in proteins interacting with RBC skeleton
premature removal by spleen
EXTRAVASCULAR haemolysis

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96
Q

Sx of hereditary spheocytosis

A

splenomegaly
pigmented gallstones
aplastic crisis secondary to parvovirus

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97
Q

How to diagnose hereditary spherocytosis

A

decreased fluorescence of RBCs on EMA binding test
increased fragilty in osmotic fragility test

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98
Q

Treatment for hereditary spherocytosis

A

splenectomy

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99
Q

Pathology of PNH

A

haematopoetic stem cell mutation -> increased complement mediated intravascular haemolysis
loss of CD55 and CD59 which protect RBC from complement
occurs at night due to mild resp aciodosis when breathig slowly in sleep

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100
Q

Triad of PNH

A

DAT -ve (because intravascular haemolysis is -ve)
pancytopenia
venous thrombosis

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101
Q

sx of PNH

A

smooth muscle dystonias - dysphagia, abdo pain
pink/red morning urine
pancytopenia
venous thrombosis
Pulmonary HTN (decreased NO)

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102
Q

PNH associations

A

CML, aplastic anaemias

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103
Q

PNH dx

A

absence of CD55/59 on flow cytometry

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104
Q

PNH treatment

A

ravulizumab/eculizumab (targets C5 -> terminal complement)
- higher risk of meningitis from neisseria, needs abx prophylaxis (penicillin)
if bone marrow failure - allogenic stem cell transplant

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105
Q

G6PD pathology

A

x-linked recessive
G6PD defect -> reduced NADPH -> reduced glutathione -> increased RBC susceptibility to oxidative stress -> hameolysis to sulfa, antimalarials and fava beans
Intra and extravascular haemolysis

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106
Q

smear fings for G6PD def

A

heinz bodies and bite cells

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107
Q

pyruvate kinase deficiency pathology
smear findings

A

Autosomal recessive
pyruvate kinase defect -> reduced ATP -> rigid RBCs -> extravascular haemolysis
smear: burr cells

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108
Q

Sickle cell anaemia pathology

A

point mutation in Beta globin gene -> single AA substitution -> alters hydrophobic region of RBC, aggregation of Hb
intra and extravascular haemolysis
Low O2, high altitude or acidosis precipitates sickling -> vasoocclusive disease

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109
Q

XR findings in sickle cell anaemia

A

hair on end skull XR from increased erythropoeisis

110
Q

complications of sickle cell anaemia

A

aplastic crisis with parvovirus
autosplenectomy (howell jolly bodies)
splenic infarcts, sequestration
vaso-occlusive including acute chest syndrome
focal segmental glomerulosclerosis

111
Q

diagnosis of sickle cell anaemia

A

gel electrophoresis
low HbA
high HbS
high HbF

112
Q

Treatment of sickle cell

A

hydroxyurea - increases HbF
blood transfusions as required
asplenia tx
folic acid

113
Q

AIHI tests

A

DAT positive
usually normocytic
smear: spherocytes and agglutinated RBCs

114
Q

difference between cold and warm AIHA

A

warm -> IgG mediated
cold -> IgM and complement

115
Q

causes of warm AIHA

A

SLE
CLL
Beta lactams
methyldopa

116
Q

Causes of cold AIHA

A

CLL
mycoplasma pneumoniae
EBV

117
Q

treatment of warm AIHA

A

steriods
rituximab
splenectomy if refractory

118
Q

treatment of cold AIHA

A

avoid cold
rituximab

119
Q

causes of drug induced haemolytic anaemia

A

abxs (penicillins, cephalosporins)
NSAIDs
Immunotherapy
chemotherapy

120
Q

mechanism of MAHAs
types of MAHAs

A

RBCs damaged passing through obstructed or narrowed vessels
DIC
TTP/HUS
SLE
HELLP
hypertensive emergency

121
Q

findings which suggest MAHA

A

schistocytes on smear

122
Q

Lead poisening pathology and symptoms

A

blocks heme synthesis pathway leading to accumulated by-products (like porphyia)
sx: microcytic anaemia (basophilic stippling and ringed sideroblasts in bone), peripheral neuropathy, headahce, memory loss, GI and kidney disease

123
Q

Symptoms of acute intermittant porphyria

A

5 P’s
painful abdomen
port coloured urine
polyneuropathy
psych disturbance
perecepitated by CYP450 induces, EtOH and startvation

124
Q

diagnosis of Acute intermittant porphyria

A

measure porphobilinogen and total prophyrins as screening
elevated PBG during attack enough to diagnose
if PBG normal but porphyrins elevated -> a type of porphyria

125
Q

treatment of prophyria

A

hemin
if mild can do trial of carbohydrate loading

126
Q

What is prophyria cutanea tarda

A

type of porphyria causes blistering cutaenous photocensitivity in hands
can be triggered by hep C or hereditary
Tx: phlebotomy, sun avoidance, hydroxychloroquine

127
Q

Signs of acute iron poisening

A

abdo pain
vomitng
anion gap metabolic acidosis
multiorgan failure

128
Q

signs of chronic iron poisening

A

arthopathy, cirrhosis, cardiomyopathy, bronzing of skin, hypogonadism

129
Q

Prothrombin time is a measure of?

A

common and intrinsic pathway

130
Q

Partial thromboplastin time (PTT) is a measure of?

A

common and intrinsic pathway

131
Q

Thrombin time is a measure of?

A

rate of conversion of fibrinogen
prolonged with anticoagulants, DIC, liver disease, reduced fibrin

132
Q

what does mixing studies diagnose and how

A

Dx clotting factor deficiencies
add normal plasma to pt plasma, if corrects is clotting factor deficiency
If there is a factor inhibitor present it does not correct

133
Q

Symtpoms of haemophilia

A

normal PT, prolonged PTT
easy bleeding
haemarthrosis

134
Q

treatment for Haemophilia A B and C

A

A - if mild desmopressin (increases factor 8), factor 8 concentrate, emicizumab (bridges factor 9 and 10 to restore function of missing 8)
B - factor 9 concentrate
C - factor 11 concentrate

135
Q

Vitamin K deficiency coagulation studies

A

prolonged PT and PTT

136
Q

Indicated MAHA on MCQs

A

low Hb, schistocytes and raised LDH

137
Q

Most common cause of isolated thrombocytopenia

138
Q

Mechanism of ITP

A

anti-GpIIb/IIIa antibodies leads to destruction of platelets in spleen by macrophages

139
Q

Hypersensitivity type associated with ITP

A

T2 hypersensitivity

140
Q

Causes of ITP

A

idiopathic
autoimmune (SLE)
viral (HIV, Hep C)
Malignancy (CLL)
drug reactions
H pylori

141
Q

Ix in ITP

A

normal PT and PTT
higher megakaryocytes on bone marrow bx
low plts

142
Q

ITP treatment

A

critical bleeding -> glucocorticoids and IVIG
Severe bleeding - > glucocorticoids alone
rituximab
eltrombopag/romiplastin (TPO receptor agonist)
splenectomy for refectory ITP

143
Q

Difference between thrombotic microangiopathies (TMA) and DIC

A

TMAs have no lab findings of consumptive coagulopathy (normal PT, PTT and fibrinogen)
DIC has prloned PT, PTT and reduced fibrinogen

144
Q

Thrombotic thrombocytopenic purpura mechanism (TTP)

A

inhibition or deficiency of ADAMs 13 -> reduced degradation of vWF -> large vWF multimers -> increased platelet adhesion and aggregation (microthrombi formation)

145
Q

symptoms of TTP

A

thrombocytopenia, MAHA (low Hb, high LDH, schistocytes), fever
plus fever and neurological sx (confusion, headache)

146
Q

TPP bloods

A

normal PT and PTT, DAT -ve
Confirm Dx with ADAMS13 <10%

147
Q

TPP treatment

A

PLEX
glucocorticoids
when dx confirmed -> rituximab
if severe features -> caplacizumab (monoclonal against vWF)

148
Q

Haemolytic uraemic syndrome (HUS) mechanism

A

caused by shiga toxin producing E coli (STEC O157:H7) -> profound endothelial dysfunction

149
Q

Sx of HUS

A

thrombocytopenia, MAHA and AKI
plus blood diarrhoea
usually in children

150
Q

Tx of HUS

A

supportive care

151
Q

What does von willebrand bind to on platelets

152
Q

von willebrand disease mode of inhereitance

A

AD
most common inhereted bleeding disorder

153
Q

treatment for von willebrand disease

A

desmopressin (releases vWF stored in endothelium)
if severe bleeding von willebrand concentrate (if not available then cryoprecipitate)

154
Q

Pathology of DIC

A

widespread clotting factor activation -> thromboembolic state with excessive clotting factor consumption -> increased thrombosis and haemorrhage

155
Q

Causes of DIC

A

heat stroke
snake bites
gram -ve sepsis
trauma
obstetric complications
pancreatitis
malignancy (APML)
nephrotic synrome
transfusion

156
Q

DIC on bloods

A

prolonged PT and PTT
schistocytes
increased d-dimer (fibrin degredation products)
decreased fibrinogen
decreased factor 5 and 8

157
Q

treatment of DIC

A

treat underlying condition
Plt if < 20
VTE prophylaxis

158
Q

Antithrombin deficiency mechanism

A

can be acquired by renal failure (nephrotic syndrome)
antithrombin loss in urine -> reduced inhibition of factors 2a and 10a

159
Q

Factor V leiden inheritance and MoA

A

AD
mutant factor V production resistant to degredation by activated protein C

160
Q

Mechanism of protein C and S deficiency and manifestations

A

decreased ability to inactivate factors 5A and 8a
increased risk of warfarin induced skin necrosis

161
Q

Malignancy associated with DIC

162
Q

difference between leukaemia and lymphoma

A

lymphoma arising from LN, leukaemia widespread bone marrow involvement

163
Q

Cells indicating hodgkins lymphoma

A

Reed sternberg cells

164
Q

CD markers present and not present in hodgkins

A

CD15 and CD 30
CD 20 in lymphocyte rich (non-classic)
does not have CD45

165
Q

Histological subtypes of hodgkins lymphoma

A

nodular sclerosis
mixed cellularity
lymphocyte rich or poor

166
Q

Viral asscoiation with hodkins

167
Q

risk factors for hodgkins

A

age
mediastinal bulk
> 2 sites
B symptoms

168
Q

treatment of hodgkins

A

ABVD, BEACOPP, RTx
if relapses > pembrolizumab (PD1), Brentuximab (CD30) or autologous SCT

169
Q

Asscoiated conditions with hodgkins lymphoma

A

minimal change disease
paraneoplastic cerebellar degeneration
erythroderma

170
Q

Burkitt lymphoma translocation

A

t (8:14) [B kinda looks like at 8]
cMYC translocation

171
Q

CD Markers of burkitt lymphoma
lab findings of burkitts

A

CD19
very high LDH >1000
Ki67 95-100%

172
Q

association with burkitt lymphoma

173
Q

difference in endemic vs sporadic version of burkitt lymphoma

A

endemic version usually involves Jaw and occurs in Africa
Sporadic verison usually involve abdo/pelvis in 40s

174
Q

Burkitt’s treatment

A

low risk - EPOCH-R
high risk CODOXM, IVAC, hyperCVAD (CNS penetrating)

175
Q

Most common non-hodgkin lymphoma in adults?

A

diffuse B cell lymphoma

176
Q

mutations associated with diffuse B cell lymphoma

A

BCL2 and BCL6
germ centre B cells in 70%, BCL2 and CD10 (better prognosis)
activated B cell in 25%, BCL 6 and MUM 1

177
Q

Treatment for diffuse B cell lymphoma

A

1st line - r-chop
2nd - platinum or cytarabine therapy, autologous SCT
3rd - CART cells, polutuzumab (anti CD79), allogenic SCT

178
Q

Most common indolant lymphoma

A

follicular

179
Q

follicular lymphoma genetics and markers

A

t(14:18) BCL2 translocation
present CD 19, 20 and 10
CD 23 not present

180
Q

treatment for folicular lymphoma

A

limited stage - RTx
advanced - R-bendamustine (alkylating)

181
Q

Mantle cell lymphoma genetics and markers

A

t(11:14) translocation of cyclin D1 and Ig CD5+
CD19 positive
aberrant CD5 expression

182
Q

Mantle cell lymphoma treatment

A

if fit - R-CHOP, RDHAC and ASCT
if unfit - R bendamustine
if relapse - BKI (ibrutinib), venetoclax (BCL2 inhibitors), CART cells

183
Q

Marginal zone lymphoma genetics (MALT)

A

associated with t(11:18) and chronic inflammation
mature B cells, do not express CD5 or CD10

184
Q

MALT lymphoma treatment

A

localised - RTx
stage 3/4 if sx - R-bendamustine

185
Q

Primary CNS lymphoma associated with

A

EBV
HIV/AIDs

186
Q

Dx of CNS lymphoma

A

ring enhancing lesion on MRI, need tissue

187
Q

Treatment for primary CNS lymphoma

A

MTX, tituximab plus either tenozolanide or procarbazine and vincristine

188
Q

cause of mature T cell lymphoma

189
Q

CD markers for T cell lymphoma

A

CD5 positive
CD7 -ve

190
Q

Most common immunoglobulin produced in multiple myeloma

A

IgG > IgA > light chains

191
Q

diagnostic criteria of multiple myeloma

A

bone marrow plasma cells <10% or bx proven plasmacytoma plus >1 of
CRAB criteria (Ca>2.75, CrCl <40 or Creat >177, Hb <100)
biomarkers: BM plasma cells >60%, FLC ratio >100, > 1 focal lesion

192
Q

Most common cause of AKI in multiple myeloma

A

light chain cast nephropathy

193
Q

which type RTA associated with multiple myeloma

194
Q

high risk genetic mutations in MM

A

17p deletion (TP53 mutation)
t(14:16)
t(4:14)
high LDH, high beta2-microglobulin

195
Q

Multiple myeloma treatment

A

<70yo, induction with bortezomib, lenalidomide and dexamthasone then autologous SCT
if older, lenolidamide or bortezomib therapy

196
Q

Waldenstroms mutation and type of immunoglobulin

A

MYD88 mutation
IgM overproduction

197
Q

fundoscopy findings in waldenstroms

A

sasauge like veins

198
Q

BM Bx findings in waldenstroms

A

> 10% monoclonal B lymphocytes with plasma cell features and intranuclear inclusions

199
Q

When do you treat in waldenstroms and what with?

A

if symptomatic anaemia or hyperviscosity (>40)
DRC or R-bendamustine
BTK inhibitors

200
Q

MGUS risk of progressing to MM or waldenstroms

A

1-2% per year

201
Q

Most common type of MGUS

A

non-IgM
IgG>IgA>free light chains

202
Q

Difference between myelodysplastic syndrome and AML

A

bone marrow blasts <20% in MDS (>20% in AML)

203
Q

Difference between types of ALL presentations

A

T cell - ALL presents with mediastinal mass and SVC like syndrome
B-ALL usually in children

204
Q

Conditions assocaited with ALL

A

Down syndrome

205
Q

Markers of ALL

A

TdT (pre-T and B cell marker)
CD10 (pre B cell markers)

206
Q

Mutations in ALL and their associated prognosis

A

t(12:21) better prognosis
t(9:22) poor prognosis

207
Q

B-ALL markers and genetics

A

CD19 CD 79 CD 22
usually in children, 25% have T(9:22) BCR-ABL rearrangement

208
Q

ALL treatment

A

Philadelphia -ve -> vincristine, glucocorticoid and anthracycline
- younger adult with CD20 + -> add rituximab
Philidelphia +ve -> TKI

209
Q

Most common adult leukaemia

210
Q

Markers of CLL

A

CD20 CD5 B cell neoplasm
CD23

211
Q

Difference between CLL and small LL

A

CLL >5x109/L malignant cells in peripheral blood
< 5 is SLL

212
Q

CLL smear findings

A

smudge cells (autoimmune haemolytic anaemia)

213
Q

Most common CLL tranformation

A

Richter transformation into diffuse B cell lymphoma

214
Q

CLL associations

A

hypogammaglobulinaemia -> causing infections
immunodeficiency

215
Q

Indications for treatment in CLL

A

marrow failure/cytopenias
symptomatic splenomegaly/lymphadenopathy
lymphocyte doubling time <6 months
autoimmune complications
constitutional sx

216
Q

Best prognostic mutations in CLL

A

13q deletion

217
Q

adverse porgnosis factors/mutations in CLL

A

17p or 11q mutations on FISH
unmutated immunoglobulin heavy chain (IHGV)
CD38 or ZAP70 positive
elevated beta 2 microglobulin
age >65yo

218
Q

CLL teratment

A

17p mutations respond poorly to fludrabidine so give venetoclax (BCL2) and obinutuzumab (CD20)
- give same if unfit
If fit then give fludarabine regimine (2nd line venetoclax and rituximab
3rd line - BKI

219
Q

Pathology of hairy cell leukaemia

A

indolent disease of mature memory B cells due to BRAFV600E mutation
- causes marrow fibrosis which leads to dry tap on aspiration of BM

220
Q

Hairy cell leukaemia presentation

A

massive splenomegaly
pancytopenia
can have proximal myopathy secondary to polymyositis

221
Q

Sensitive and specific marker for hairy cell leukaemia

A

annexin A1

222
Q

treatment for hairy cell leukaemia

A

purine analogs - cladrabine and pentostatin
rituximab, ibrutinib
vemurafinib (inhibitor of BRAF V600E)

223
Q

What is the second most common leukaemia

224
Q

Risk factors for AML

A

down syndrome
previous chemo/RTx

225
Q

diagnosis of AML

A

> 20% blasts in BM or blood

226
Q

AMl presentation

A

cytopenias (neutropenic fevers)
bone pain
Leukostasis (capillaries blocked by malignanct cells -> organ damage)
primary tumour lysis
DIC in APML

227
Q

Favourable mutations in AML

A

t(15:17) - APML
t(8:21)
Inv(16)
mutated NPM1

228
Q

intermediate risk AML mutations

A

mutated NPM1 with high FLT3-ITD

229
Q

adverse mutations in AML

A

17p abberation
mutated TP53
wild type NPM1 and high FLT-ITD
complex karyotype

230
Q

blood findings of AMPL

A

auer rods (crystlaised MPO)

231
Q

APML mutation

A

t(15:17), PML-RARA

232
Q

treatment considerations for APML

A

PML-RARA blocks retinoic acid induced myeloid differenciation
ATRA and arsenic forces differenciation and can induce degredation of PML-RARA

233
Q

Complications of ATRA

A

ADR: headaches, hepatotoxicity, cytopenias
Differenciation syndrome
- fever, oedema, pulmonary infiltrates and serotsitis
prophylaxis with prednisolone
to treat with dexamethasone
only stop ATRA if severe

234
Q

Treatment for AML

A

< 70yo and fit - induction 7 days of cytarabine and 3 days of daunorubicin
consolidation high dose cytarabine
if FLT3 can give midostaurin
Allogenic SCT in second remisson (if no SCT fatal)

235
Q

What is considered remission in AML

A

<5% blasts in bone marrow

236
Q

CML mutation

A

t(9:22) BCR ABL1

237
Q

3 phases of CML

A

chronic - neurophilia and myelocytosis in blood, increased platelets
- BM blasts <10%, <20% basophils in blood
accelerated - increased blasts in BM, splenomegaly, blasts between chronic and blast phase
blast phase - acute leukaemia, blasts >20%

238
Q

Cluse to suggest CML

A

proliferation of all mature myeloid lines (neutrophilia, basophilia, eosinophilia, myelocyte peak)
median WCC at dx 100

239
Q

Treatment of CML

A

higher risk -> 2nd gen Tyrosine kinase inhibitors (nilotinib, dasatinib ->30% risk pleural effusion)
lower risk -> 1st gen TKI (imatinib)
if relapsed 3rd gen TKI ponatinib
goals of therapy 3 monthly BCR-ABL byt 1 year <0.1%

240
Q

Polycythemia vera mutation

241
Q

polycythemia vera sx

A

aquafenic pruritis, fatigue, microvascular sx (headache, lightheaded, visual disturbance, astypical chest pain)
erytheromelalgia (severe burning pain and red-blue discolouration)
mild/mod splenomegaly in some cases
associated with hyperviscosity and thromosis (splanchic thrombosis/budd-chiari

242
Q

major investigation finding in polycythemia vera

A

decreased EPO

243
Q

diagnostic criteria of polycytemia vera

A

Major - Hb >165 or HCT >0.49, BMbx showing hypercellularity, JAK2 V617F mutation (97%) or JAK2 exon 12 (3%)
minor - subnormal EPO

244
Q

major cause of mortality in polycythemia vera

A

thrombosis

245
Q

risk of leukaemic and myelofibrosis transofrmation in polycythemia vera

A

5-15% for both over 15 years
thrombosis is 5-15% in 3 years

246
Q

polycythemia vera threatment

A

all pt get venesection to aim for HCT <0.45
low risk - BD aspirin
high risk cytoreduction with hydroxyurea, IFN alfa, busulfan
only ruxolitinib (JAK1/2 inhibitor) if significant sx

247
Q

What is essntial thrombocytopenia

A

massive proliferation of megakaryocytes and platelets

248
Q

symptoms of essential thrombocytopneia

A

mostly asymptomatic
can have mucocutaenous bleeding due to acquired von willebrand disease

249
Q

essential thrombocytopenia diagnostic criteria

A

major - plt >450, BMbx megakaryocyte proliferation and no/small fibrosis, not meeting other criteria, JAK2 (55%), CALR (35%) ir MPL (<5%) mutation
minor - presence of clonal marker or absence of reactive thrombocytosis

250
Q

essential thrombocytopenia mutations

A

JAK2 (55%), CALR (35%) ir MPL (<5%) mutation
JAK2 and MPL most asscoiated with thrombosis

251
Q

essential thrombocytopenia risk of AML and myelofibrosis

A

AML <5% over 15 years
myelofibrosis 5-10%

252
Q

essential thrombocytopenia treatment

A

only affects thrombosis risk, not survival or transformation
low risk - observation, if risk factors aspirin
intermediate - BD aspirin, cytoreduction if CVD
high risk - cytoreduction and aspirin
anticoags if VTE

253
Q

what is primary myelofibrosis

A

megakaryocyte proliferation causing BM fibrosis and extramedullary haemotopoesis
atypical megakaryocyte hyperplasia -> increased TGF-beta secretions -> increased fibroblast activity -> obliteration of BM with fibrosis

254
Q

clinical findings in primary myelofibrosis

A

massive splenomegaly
teardrop RBCS
30% can be asymptomatic
associated with gout and renal stones (hyperuricaemia)

255
Q

diagnostic criteria of primary myelofibrosis

A

major - megakaryocyte proliferation and reticulin fibrosis, not meeting criteria for CML, PV, ET or MDS, presence of JAK2, CALR or MPL
minor - anaemia, slenomegaly, elevated LDH, leukoerythroblastic film

256
Q

treatment of primary myelofibrosis

A

intermediate 2 and high risk treated with allogenic SCT
low risk monitored
standard therapy is ruxolitinib
hydrocyurea for leukocytosis

257
Q

What is Myelodysplastic syndrome and subtypes

A

when blasts <20%, group of haematopeotic stem cell disorders
MDS with excess blasts -> 5-19% blasts
MDS with multilineage -> blasts <5%, 1-3 cytopenias
MDS with single lineage dysplasia -> blasts <5% and 1-2 cytopenias
MDS with isolated 5q deletion can be treated with lenolidamide

258
Q

treatment of myelodysplastic syndrome

A

azacitadine (inhibitrs DNA methyltransferease and induces cytotoxicity)
- delays time to death and progression to AML

259
Q

What is langerhan’s cell histiocytosis and what are sx

A

proliferative disorder of langerhans cells (APCs in skin), usually in children with lytic bone lesions, skin rash, recurrent otitis media or mass involving mastoid bone
cells do not stimulate T cells (functionally immature)
cells express S-100 and CD1a

260
Q

blood findings in tumour lysis syndrome

A

low Ca, high K, phosphate and uric acid

261
Q

treatment of tumour lysis syndrome

A

allopurinol or rasburcase

262
Q

MoA of heparin

A

activates antithrombin which decreases activity of 2a and 10a

263
Q

How to reverse heparin

A

protamine sulfate

264
Q

mechanism of heparin induced thrombocytopenia Type 2

A

IgG antibodies formed against heparin bound platelet factor 4 (PF4), binds and acitvates plt and is removed by spleen -> thrombocytopenia
5-10 days post heparin administration

265
Q

Warfrain MoA

A

inhibits vit K epoxide reductase

266
Q

Why is there initial hypercoagulation with warfarin

A

protein C has a shorter half life than factors 2 and 10. used up first, increased 2 and 10 without being inhibited -> hypercoagulation

267
Q

dabigatran reversal agent

A

idarucizumab

268
Q

dabigatran coags finding

A

raised aPTT

269
Q

apixaban/rivaroxaban reversal agent

A

andexanet alfa (recombinant factor Xa)

270
Q

apixaban/rivaroxaban coags finding

A

prolongs INR

271
Q

Multiple myeloma marker

RACP Written Exam (14 decks)

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