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Its the final countdown > Haem > Flashcards

Haem Flashcards

1
Q

give the range
-normal neutrophils
- neutropenia

A

normal: 1.7 - 6.5 x109/L
neutropenia <1 x109/L
severe neutropenia <0.5 x10*9/L

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2
Q

causes of neutrophilia

A
  • acute bacterial infection.
  • conditions of severe stress (trauma, surgery, necrosis, burns, haemorrhage and seizures)
  • inflammation ( polyarteritis nodosa, myocardial infarction, and disseminated malignancy)
  • corticosteroid use ( - may also causes lymphopenia
  • myeloproliferative disease ( CML, polycythaemia vera, and essential thrombocythaemia)
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3
Q

A 63 yo alcoholic w/ acute upper GI bleed & shock is Adx onto ED.

Exam: Soft abdomen, liver not palpable, Spleen is moderately enlarged, no rashes.

FBC
- Hb of 68 g/L
- low grade leukocytosis
- thrombocytopenia.

What is the likely cause of this patient’s presentation?

A

Oesophageal varices

think varices in 1. Hx alcoholism, 2 splenomegaly w/ thrombocytopenia

plt count helps differentiate betwen variceal / non-variceal bleeding ( portal HTN –> splenomegally & hyperfunction –> plt sequestration

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4
Q

What is the MCV

A

The mean cell volume (MCV) - size of the red blood cell

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5
Q

Give the male and female normal ranges for
- Hb
- MCV

A

Hb male 140-180 g/L
Female 115-165 g/l
MCV (both ) 80-100 femolitres

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6
Q

Give the 5 causes of microcytic anaemia

A

TAILS

Thalassemia
Apanemia of chronic disease (mostly w/ CKD)
Iron deficiency
Lead Poisoning
Sideroblastic anaemia - bone marrow produced ringed sideroblasts, not healthy RBCs

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7
Q

Causes if normocyric anaemia

A

AAAHH
A – Acute blood loss
A – Anaemia of chronic disease
A – Aplastic anaemia
H – Haemolytic anaemia
H – Hypothyroidism

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8
Q

What are the types if macrocytic anaemia
What are the causes of both types

A

Megaloblastic anaemia : impaired DNA synthesis–>preventing normal cell division –> grow into large, abnormal cells.

Megaloblastic anaemia:
B12/folate deficiency

Normoblastic macrocytic anaemia:

Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs, such as azathioprine

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9
Q

1st line Mx in vWD

2nd line/ 1st line in type III von Willebrand disease

A

Desmopression - releases stored vWF and FVIII into blood –> homeostasis

VWF/FVIII concentrate

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10
Q

How would acute chest syndrome in Sickle cell Anaemia present

A

Pain, fever, resp Sx ( tachypnoea, Wheeze, cough)

CXR - pulmonary infiltrates ( the cause of Sx), involving whole lung segments

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11
Q

Acute chest syndrome management

A

Oxygen, analgesia, empirical Abx

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12
Q

How would sequestration crisis in Sickle cell Anaemia present

A

Shock (low BP, high HR)
Severe anaemia

Cause: blood pooling in spleen

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13
Q

Is the lifespan of neutrophils longer or shorter than that of platelets

A

Shoeter
Neutrophils: 4days
Platelets: 7-10 days

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14
Q

A pt with Hx of fatigues blood results are as follows:

WCC normal
Hb 103 g/L
MCV 119 fL
Plt low
Blood fill : megalobasts
Serum folate: normal

What is the most suitable tx and route

A

IV hydroxycobalamin (B12)

This is B12 deficiency anaemia - a megaloblastic macrocytic anaemia

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15
Q

How does vaso-occlusive crisis in Sickle cell Anaemia present

A

Sudden onset of pain
No abnormalities on exam
No sig drop in Hb

Hx common trigger: cold, dehydration, infection, hypoxia

Typical locations of pain
Toddlers: hands and feet
Others: anywhere

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16
Q

what is the genetic inheritance of Haemochromatosis

A

autosomal recessive

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17
Q

mutation in what gene causes haemochromatosis and on what chromosome is it located

A

HFE gene
Chromosome 6

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18
Q

at what age do the Sx of haemochormatosis appear

A

> 40
(post menopausal in women, as menstrual period heps rulate iron storage)

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19
Q

Give 8 Sx of haemochromatosis

A

chronic tiredness
joint pain
pigmentation ( bornze skin)
mles - testicular atrophy & erectile dysfunction

female - amenorrhoea ( absence of periods)

cognitive: memory & mood disturbance

hepatomegaly

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20
Q

what is the initial Ix in suspected haemochromatosis

A

serum ferritin

also raised in infections (acute phase reactant), chronic alcoholism, NAFLD, hepatitis, cancer

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21
Q

what marker can help distinguish between high ferritin caused by iron overload or other causes

A

transferrin saturation

  • high: iron overload
  • normal: other cause
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22
Q

what stain is used on a liver biopsy to establish the iron conc of the liver

A

Peri’s stain

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23
Q

Mx in haemochromatosis (x3)

A

venesection (2nd line iron chelation if regular venesection not tolerated

monitoring serum ferritin

monitoring and Tx complications

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24
Q

in anaemia of chronic disease,

is TIBC
ferritin

raised, normal or low

A

TIBC - reduced

ferritin normal/raised

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25
Q

Describe the 2 types of autoimmune hepatitis

A

Type 1
Females aged 40/50s
presents: fatigue, features of liver disease

Type 2
female young children/adolescents
acute
high transaminases & jaundice

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26
Q

Give 5 findings in autoimmune hepatitis type 1

A

high transaminases (ALT & AST)
Autoantibodies:
* Anti-nuclear antibodies (ANa)
* Anti-smooth muscle antibodies (anti-actin)
* Anti-soluble liver antigen (ani-SLA/LP

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27
Q

what is Richters transformation?

A

when CLL transforms to high-grade lymphoma ( non-hodgkins)

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28
Q

around 75% of which haematological cancer is found in those <6oyo

A

ALL

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29
Q

give 4 complications of CLL

A

anaemia
hypogammaglobulinaememia ( which causes recurrent infections )
warm autoimmune haemolytic anaemia
transformation to hihg-grade lymphoma ( Richter’s transformation

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30
Q

how does Richter’s transformation present?

A

pt with CLL with acute ( 1 of these symptoms suffices)

lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdo pain

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31
Q

difference in

leukaemia

lymphoma

myeloma

A

leukaemia - cancer of stem celll line in bone marrow

lymphoma - cancer of lymphocytes and in lymphatic system (causes lymphadenopathy )

Myeloma - cancer of plasma cells in bone marrow ( B lymphocytes)

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32
Q

which leukaemia is associated with down syndrome

A

ALL

ALL is most associated with children & down syndrome

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33
Q

which Leukaemia shows smear/smudge cells on blood film?

A

CLL is associated with
* richters transformation
* smudge cells ( fragile cells rupture while preparing blood film)
* warm haemolytic anaemia

34
Q

Which Leukaemia is associaed with philadeplhia chromosomes

A

CML

CML has 3 phases, and is associated with the Philadelphia chromosome ( translocation from chromosome 9 to 22, affecting chromosome 22)

35
Q

The leukaemia which may from from a myeloproliferative disorder is associated with which finding on blood film

A

Auer rods

AML may develop from a myeloproliferative disorder
it is associated with Auer rods

36
Q

what cells are formed from the myeloid line?

A

WBCS
RBCs
Thrombocytes

37
Q

describe the Sx in the3 3 phase of CML

A

Chronic - asymptomatic , dx incidentally. takes years to progress

accelerated - high proportion of abnormal blast cells (10-20%). More symptomatic, with anaemia, thrombocytopenia and immunodeficiency.

Blast - >20% blast cells. Sx severe, pancytopenia fatal

37
Q

what cells are formed from the lymphoid line ( accumulate in ALL)?

A

lymphocytes ( T&B0

37
Q

leukaemia is typically treated with Chemo & targeted therapy (@ radiotherapy, bone marrow transplant and surgery are other options). give 2 examples for targeted theraapies

A

ibrutinib , Rituximab

  • Tyrosine kinase inhibitors (e.g., ibrutinib/ imatinib)
  • Monoclonal antibodies (e.g., rituximab, which targets B-cells)
37
Q

what proliferative disorders may precede AML

A

Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia

38
Q

there are multiple chemo complications, one of which is tumour lysis syndrome.

what changes occur to teh blood concentrations of: uric acid, potassium, phosphate and calcium)

A

high: uric acid, potassium, phosphate)

low: calcium

all increase as cell lysis releases them into the body

calcium decreases due to high phosphate

38
Q

what drugs can be used to supress the high uric acid levels caused by tumour lysis syndrome?

A

Allopurinol/ Rasburicase

otherwise good hydration & urine output is required prechemo as prophylaxis

38
Q

what is the name of the genes affected in the translocation which is found in chronic myeloid leukaemia

A

BCR-ABL

translocation between the long arm of chromosome 9 and 22 - t(9:22)(q34; q11).

Part of the ABL proto-oncogene from chromosome 9 fuses with the BCR gene from chromosome 22. The resulting BCR-ABL gene

38
Q

an elderly pt presents with abdo discomfort. Splenomegaly is found on examination

the bloods show an increase in granulocytes at different stages of maturation +/- thrombocytosis. what leukaemia does this indicate?

A

chronic myeloid leukaemia

raised granulocytes at diff stages of maturation: e.g. monoblasts ( macrophage precursor) high, neutrophils high, plts high

so in increased granulocytes at different stages of maturation +/- thrombocytosis
= CML

(pts typically 60-70 yo)

39
Q

what type of cells would largely be found in AML ( maturity)

A

blasts - immature cells

(think acute, so rushing, doesn’t wait for differentiation –> precursors proliferate)

40
Q

what type of cells would largely be found in ALL ( maturity)

A

blasts - immature cells

(think acute, so rushing, doesn’t wait for differentiation –> precursors proliferate)

41
Q

a normal thrombocyte range is

(140-400 x 109/l)
this may be raised e.g. to 420 x 109/l in CML

from which point is essential thrombocytosis considered?

A

typically >450 * 109/l (much higher)

42
Q

Platelet counts below 10 x 10*9/L are at high risk of what types of bleeds

A

spontaneous bleeding.

Particularly:
* Intracranial haemorrhage
* Gastrointestinal bleeding

<50 is where e.g. bleeding gums, haematuria,

43
Q

what is the 1st line Tx in ITP

A

Oral prednisolone

(ITP = antiobodies against platelets)

others:
* Prednisolone (steroids)
* IV immunoglobulins - raises plt count quicker than steroids, so used in active bleed/ urgent procedure
* Thrombopoietin receptor agonists (e.g., avatrombopag)
* Rituximab (a monoclonal antibody that targets B cells)
* Splenectomy

44
Q

what is the cut off for tx in ITP

A

plts <30x10*9/L
or slightly higher if poroloned bleeding/ reduced QOL/ injury prone profession

45
Q

what is the most suitable investigation in suspected autoimmune haemolytic anaemia

A

Direct coombs’ test

if positive: positive direct antiglobulin test

46
Q

do reticulocytes increase, decrease or stay the same in autoimmune haemolytic anaemia

A

increase

(bone marrow responds by increasing reticulocyte synthesis)

47
Q

rituximab is one of the medications used in ITP. how does it work

A

Abs produced by B cells
Rituximab ( monoclonal antibody) targets CD20 proteins on surface of B cells

so reduction of Bcells = reduction of Aba

48
Q

describe TTP

A

thrombotic thrombocytopenic purpura - thrombi develop in small vessels

causes: thrombocytopenia, .purpura, ischaemia & end organ damage

49
Q

deficiency in what protein leads to TTP

A

ADAMTS13

normally
- inactivates vWF
- reduces platelet adhesion to vessel walls
- reduces clot formation

Mx of TTP - haematologists

50
Q

what causes Heparin-induced thrombocytopenia ( HIT)

A

platelet antibodies develop in response to heparin –> bind to plts –> activate clotting cascade –> hypercoagulable sgtate

typical pt: low plt count, abnormal blood clots, recently started heparin

5-10 days post starting heparin

51
Q

in HIT, heparin is stoppped, what alternatives can be used

A

fondaparinux/ argatroban

52
Q

give the 5 causes of microcytic anaemia

A

TAILS
* T – Thalassaemia
* A – Anaemia of chronic disease
* I – Iron deficiency anaemia
* L – Lead poisoning
* S – Sideroblastic anaemia

53
Q

Give 5 causes of normocytic anaemia

A

3As,2Hs

  • A – Acute blood loss
  • A – Anaemia of chronic disease
  • A – Aplastic anaemia
  • H – Haemolytic anaemia
  • H – Hypothyroidism
54
Q

what is the management in anaemia of chronic disease caused by CKD

A

erythropoietin

anaemia of chronic disease typically occurs with CKD –> lack of erythropoietin by kidneys

anaemia of chronic disease= the A in tAils ( microcytic anaemia)

55
Q

what are the 7 causes of macrocytic anaemia

A

megaloblastic macrocytic anaemia: B12/Folate deficiency

Normoblastic macrocytic anaemia ( HARD-L)

Hypothyroid (also causes normocytic anaemia)
Alcohol
Reticulocytosis ( high conc. retiiculocytes from haemolytic anaemia/blood loss)
Drugs ( azathioprine)
L liver disease

56
Q

give 2 sx of anaemia specific to IDA

A

pica
hair loss

57
Q

which forms of anaemia are specific to these signs & symptoms

  • Koilonychia
  • Angular cheilitis
  • Atrophic glossitis
  • Brittle hair and nails
  • Jaundice
  • Bone deformities
  • Oedema
A
  • Koilonychia (spoon-shaped nails) - IDA
  • Angular cheilitis - IDA
  • Atrophic glossitis (smooth tongue due to atrophy of the papillae) -IDA
  • Brittle hair and nails - IDA
  • Jaundice - haemolytic anaemia
  • Bone deformities - thalassaemia
  • Oedema, hypertension and excoriations on the skin - chronic kidney disease (anaemia of chronic disease )
58
Q

what antibodies found in the blood indicte pernicious anaemia

A

intrinsic factor

59
Q

following bloods, what tests should be done for unexplained anaemia

A

Exclude GI cancer s source of bleeding in unexplained IDA: colonoscopy/ oesophagogastroduodenoscopy

Unexplained anaemia/ possible malignancy - bone marrow biopsy

60
Q

which form of anaemia presents as a microcytic hypochromic anaemia

A

IDA (hypochromic as its pale due to reduced haemoglobin concentration)

61
Q

most common cause of iron deficiency anaemia in

  • children
  • adults
A

children - dietary insufficiency

adults - bleed (inc. IBD, angiodysplasia)

62
Q

what parts of the bowel is iron mainly absorbed in

A

duodenum and jejunum

(acid from stomach maintains stable Fe2+ , less acid –> unstable Fe3+ so PPIs may reduce absorption)

63
Q

why do coeliac and crohns disease reduce iron absorption

A

cause inflammation of the duodenum +/ jejunum

64
Q

what protein is bound to iron and transports it

A

transferrin

65
Q

what does

low ferritin

normal ferritin

raised ferritin

indicate

A

low - highly suggestive of IDA

normal - does not exclude iron deficiency

high could be raised in : inflammtaion, liver disease, iron supplements, haemochromatosis

ferritin is typically stored in cells, its an acute-phase reactant

66
Q

what does the marker Total iron-binding capacity indicate?

A

the amount of transferrin in the blood

TIBC and transferrin increase with iron deficiency and decrease with iron overload

67
Q

what does transferrin saturation indicate

A

total iron in body

less iron - less saturated

fasting sample is the most accurate as transferrin can temporarily increase post meal/ supplement

68
Q

give 3 causes of iron overload

A

haemochromatosis
iron supplements
acute liver damage ( liver contains lots of iron)

iron overload will show raised: serum iron ferritin and transferrin saturation ( normal should be 15-50%)

TIBC will not be raised

69
Q

what is the Ix in IDA without clear underlying cause ( e.g. menstruation)

A

colonoscopy & oesophagogastroduodenscopy
check for malignancy

70
Q

what are the 3 options for tx IDA

A

Oral iron ( ferrous sulphate/ ferrous fumarate) - slow increase

iron infusion ( IV) - fast increase

blodd transfusion ( severe)

71
Q

what are the risks/ side effects in Mx IDA with oral iron

A

common:

constipation
black stools

72
Q

what are the risks/ side effects in Mx IDA with IV CosmoFer ( iron infusion)

A

small risk: allergic reaction/anaphylaxis

do not use in infection - bacteria can “feed” on it

73
Q

Give 3 causes of low B12

A

Pernicious anaemia
low intake (vegan diet)
meds that reduce absorption ( PPIs metformin

74
Q
A
75
Q
A

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