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Haem Flashcards

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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
Describe the 2 types of autoimmune hepatitis
Type 1 Females aged 40/50s presents: fatigue, features of liver disease Type 2 female young children/adolescents acute high transaminases & jaundice
26
Give 5 findings in autoimmune hepatitis type 1
high transaminases (ALT & AST) Autoantibodies: * Anti-nuclear antibodies (ANa) * Anti-smooth muscle antibodies (anti-actin) * Anti-soluble liver antigen (ani-SLA/LP
27
what is Richters transformation?
when CLL transforms to high-grade lymphoma ( non-hodgkins)
28
around 75% of which haematological cancer is found in those <6oyo
ALL
29
give 4 complications of CLL
anaemia hypogammaglobulinaememia ( which causes recurrent infections ) warm autoimmune haemolytic anaemia transformation to hihg-grade lymphoma ( Richter's transformation
30
how does Richter's transformation present?
pt with CLL with acute ( 1 of these symptoms suffices) lymph node swelling fever without infection weight loss night sweats nausea abdo pain
31
difference in leukaemia lymphoma myeloma
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)
32
which leukaemia is associated with down syndrome
ALL ALL is most associated with children & down syndrome
33
which Leukaemia shows smear/smudge cells on blood film?
CLL is associated with * richters transformation * smudge cells ( fragile cells rupture while preparing blood film) * warm haemolytic anaemia
34
Which Leukaemia is associaed with philadeplhia chromosomes
CML CML has 3 phases, and is associated with the Philadelphia chromosome ( translocation from chromosome 9 to 22, affecting chromosome 22)
35
The leukaemia which may from from a myeloproliferative disorder is associated with which finding on blood film
Auer rods AML may develop from a myeloproliferative disorder it is associated with Auer rods
36
what cells are formed from the myeloid line?
WBCS RBCs Thrombocytes
37
describe the Sx in the3 3 phase of CML
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
what cells are formed from the lymphoid line ( accumulate in ALL)?
lymphocytes ( T&B0
37
leukaemia is typically treated with Chemo & targeted therapy (@ radiotherapy, bone marrow transplant and surgery are other options). give 2 examples for targeted theraapies
ibrutinib , Rituximab * Tyrosine kinase inhibitors (e.g., ibrutinib/ imatinib) * Monoclonal antibodies (e.g., rituximab, which targets B-cells)
37
what proliferative disorders may precede AML
Primary myelofibrosis Polycythaemia vera Essential thrombocythaemia
38
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)
high: uric acid, potassium, phosphate) low: calcium all increase as cell lysis releases them into the body calcium decreases due to high phosphate
38
what drugs can be used to supress the high uric acid levels caused by tumour lysis syndrome?
Allopurinol/ Rasburicase otherwise good hydration & urine output is required prechemo as prophylaxis
38
what is the name of the genes affected in the translocation which is found in chronic myeloid leukaemia
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
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?
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
what type of cells would largely be found in AML ( maturity)
blasts - immature cells (think acute, so rushing, doesn't wait for differentiation --> precursors proliferate)
40
what type of cells would largely be found in ALL ( maturity)
blasts - immature cells (think acute, so rushing, doesn't wait for differentiation --> precursors proliferate)
41
a normal thrombocyte range is (140-400 x 10*9/l) this may be raised e.g. to 420 x 10*9/l in CML from which point is essential thrombocytosis considered?
typically >450 * 109/l (much higher)
42
Platelet counts below 10 x 10*9/L are at high risk of what types of bleeds
spontaneous bleeding. Particularly: * Intracranial haemorrhage * Gastrointestinal bleeding <50 is where e.g. bleeding gums, haematuria,
43
what is the 1st line Tx in ITP
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
what is the cut off for tx in ITP
plts <30x10*9/L or slightly higher if poroloned bleeding/ reduced QOL/ injury prone profession
45
what is the most suitable investigation in suspected autoimmune haemolytic anaemia
Direct coombs' test if positive: positive direct antiglobulin test
46
do reticulocytes increase, decrease or stay the same in autoimmune haemolytic anaemia
increase (bone marrow responds by increasing reticulocyte synthesis)
47
rituximab is one of the medications used in ITP. how does it work
Abs produced by B cells Rituximab ( monoclonal antibody) targets CD20 proteins on surface of B cells so reduction of Bcells = reduction of Aba
48
describe TTP
thrombotic thrombocytopenic purpura - thrombi develop in small vessels causes: thrombocytopenia, .purpura, ischaemia & end organ damage
49
deficiency in what protein leads to TTP
ADAMTS13 normally - inactivates vWF - reduces platelet adhesion to vessel walls - reduces clot formation Mx of TTP - haematologists
50
what causes Heparin-induced thrombocytopenia ( HIT)
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
in HIT, heparin is stoppped, what alternatives can be used
fondaparinux/ argatroban
52
give the 5 causes of microcytic anaemia
TAILS * T – Thalassaemia * A – Anaemia of chronic disease * I – Iron deficiency anaemia * L – Lead poisoning * S – Sideroblastic anaemia
53
Give 5 causes of normocytic anaemia
3As,2Hs * A – Acute blood loss * A – Anaemia of chronic disease * A – Aplastic anaemia * H – Haemolytic anaemia * H – Hypothyroidism
54
what is the management in anaemia of chronic disease caused by CKD
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
what are the 7 causes of macrocytic anaemia
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
give 2 sx of anaemia specific to IDA
pica hair loss
57
which forms of anaemia are specific to these signs & symptoms * Koilonychia * Angular cheilitis * Atrophic glossitis * Brittle hair and nails * Jaundice * Bone deformities * Oedema
* 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
what antibodies found in the blood indicte pernicious anaemia
intrinsic factor
59
following bloods, what tests should be done for unexplained anaemia
Exclude GI cancer s source of bleeding in unexplained IDA: colonoscopy/ oesophagogastroduodenoscopy Unexplained anaemia/ possible malignancy - bone marrow biopsy
60
which form of anaemia presents as a microcytic hypochromic anaemia
IDA (hypochromic as its pale due to reduced haemoglobin concentration)
61
most common cause of iron deficiency anaemia in - children - adults
children - dietary insufficiency adults - bleed (inc. IBD, angiodysplasia)
62
what parts of the bowel is iron mainly absorbed in
duodenum and jejunum (acid from stomach maintains stable Fe2+ , less acid --> unstable Fe3+ so PPIs may reduce absorption)
63
why do coeliac and crohns disease reduce iron absorption
cause inflammation of the duodenum +/ jejunum
64
what protein is bound to iron and transports it
transferrin
65
what does low ferritin normal ferritin raised ferritin indicate
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
what does the marker Total iron-binding capacity indicate?
the amount of transferrin in the blood TIBC and transferrin increase with iron deficiency and decrease with iron overload
67
what does transferrin saturation indicate
total iron in body less iron - less saturated fasting sample is the most accurate as transferrin can temporarily increase post meal/ supplement
68
give 3 causes of iron overload
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
what is the Ix in IDA without clear underlying cause ( e.g. menstruation)
colonoscopy & oesophagogastroduodenscopy check for malignancy
70
what are the 3 options for tx IDA
Oral iron ( ferrous sulphate/ ferrous fumarate) - slow increase iron infusion ( IV) - fast increase blodd transfusion ( severe)
71
what are the risks/ side effects in Mx IDA with oral iron
common: constipation black stools
72
what are the risks/ side effects in Mx IDA with IV CosmoFer ( iron infusion)
small risk: allergic reaction/anaphylaxis do not use in infection - bacteria can "feed" on it
73
Give 3 causes of low B12
Pernicious anaemia low intake (vegan diet) meds that reduce absorption ( PPIs metformin
74
75

Its the final countdown (23 decks)

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