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CP - JL > Haematology - Part 1 > Flashcards

Haematology - Part 1 Flashcards

1
Q

Describe what happens on each day of the origins of haemopoiesis

A

Day 27 - starts in the aorta-gonado-mesonephros region
Day 35 - expands rapidly
Day 40 - migrates to the liver which becomes the main site of haemopoiesis

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2
Q

Name Myeloid cells

A

Granulocytes, erythrocytes, platelets

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3
Q

Name lymphoid cells

A

lymphocytes

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4
Q

RBC life span

A

120 days

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5
Q

Most common cell type in blood

A

Neutrophils

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6
Q

Increased in bacterial infections

A

neutrophils

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7
Q

Increased in parasites and allergies e.g. aspirin

A

eosinophils

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8
Q

Basophilia indicates

A

chronic myeloid leukeamia

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9
Q

life span of neutrophils

A

6-8 hours

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10
Q

Name of monocytes in the liver and skin

A

Kuppfer cells in liver, langerhans cells in skin

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11
Q

What does monocytosis indicate

A

TB

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12
Q

Causes of lymphocytosis

A

atypical lymphocytes of glandular fever (usually self limiting) or chronic lymphocytic leukaemia

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13
Q

Causes of lymphopenia

A

3 months post bone marrow transplant

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14
Q

What is myeloma

A

the malignant overproduction of plasma cells

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15
Q

What do we collect blood into

A

into EDTA anti coagulated tubes

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16
Q

Where do we take a bone marrow aspirate from

A

liquid bone marrow aspirate from the posterior iliac crest. Treptine core biopsy is also taken

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17
Q

What is sensitivity?

A

Proportion of abnormal results correctly identified by the test,

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18
Q

What is specificity?

A

The proportion of normal results correctly classified by the test

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19
Q

What could cause a false result of thrombocytopenia?

A

Platelets clotting in the tube

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20
Q

What happens to lymphocyte count post splenectomy

A

Mild lymphocytosis

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21
Q

Causes of microcytic anaemia

A

Iron defeciency, chronic disease, thalassaemia, lead poisoning, sideroblastic anaemia

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22
Q

Normocytic anaemia causes

A

Blood loss, haemolytic, chronic disease, bone marrow failure (post chemo or due to infiltration by carcinoma)

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23
Q

Macrocytic anaemia causes

A

Megaloblastic –> B12 or folate defeciency –> get hypersegmented neutrophils and oval microcytes
Non-megaloblastic –> alcohol (most common cause), liver disease, myelodysplasia and aplastic anaemia).

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24
Q

Ranges for anaemia types

A

Microcytic: MCV 27pg
Macrocytic: MV: >95

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25
Q

What does leucodepletion do?

A

Separates RBC from the whole blood

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26
Q

What can plasma be split into?

A

Fresh frozen plasma (FFP), cyroprecipitate or fractionation into factor concentrates and immunoglobulins

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27
Q

What do we replace plasma with in RBC storage

A

glycose, electrolyes and adenine to keep RBC healthy

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28
Q

What is the transfusion threshold trigger

A

The lowest haemoglobin concentration not associated with anaemia symptoms

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29
Q

How do we adapt to anaemia

A

Increase cardiac output, increase cardiac artery blood flow, increase Epo, Increase 2,3 DPG

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30
Q

Thresholds for transfusion

A
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31
Q

What do we do for patients on regular transfusion due to inherited anaemias?

A

Suppress endogenous Epo

Beware of Iron overload can cause cardiomyopathy and liver failures

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32
Q

When do we give FFP?

A

Massive haemorrhages, coagulopathy with bleeding/surgery, thrombotic thrombocytopenia purport

DO NOT GIVE to reverse warfarin or to replace single factor

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33
Q

What is crossmatching

A

mixing the donor RBC with the patients plasma to see if a reaction occurs

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34
Q

Acute Transfusion Reactions

A

Immune: Acute haemolytic reaction, allergic reaction, TRALI

Non-immune –> bacteria, contamination, TACO, febrilenon-haemolytic transfuion reaction

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35
Q

Delayed transfuion reaction

A

Immune: TA GVHD, Post transfusion Purpura

Non-immune: TTI

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36
Q

Acute haemolytic Reaction summary

A

Release of free Hb –> renal failure, microvascular thrombosis, cytokine storm, vasoconstriction

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37
Q

What may be the first sign of acute haemolytic reaction in anaesthetised patients?

A

Haemoglobinuria

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38
Q

Signs of acute haemolytic reactions

A

fever/chills, back pina, infusion pain, haemoglobinuria, bleeding, chest pain

Fatal in 15-20% of people

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39
Q

What causes delayed haemolytic reactions

A

Develop antibodies to RBC antigens other than ABO Post transfusion

Get fatigue, jaundice, increased bilirubin/LDH, decreased Hb, fever

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40
Q

What is TRALI?

A

Where the donors plasma has antibodies against the recipients leucocytes (anti-HLA, anti-HNA).
Activated WBC lodge in the pulmonary capillaries –> release substances causing endothelial damage

Fever, hypotension, DO NOT GIVE DIURETICS OR STEROIDS, fluid loading improces

Fatal in 5-10% of people

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41
Q

What is TACO?

A

Get sudden dyspnoea, orthopnea, tachycardia, hypertension, hyperaemia, increased JVP

Risk factors are the elderly, small children, reduced LV function, fast transfusion or high volume

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42
Q

What is urticarial rash

A

May occur with a wheeze, occurs due to hypersensitivity to a random plasma protein

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43
Q

What is anaphylaxis

A

Severe life threatening reaction soon after transfusion starts, wheeze, tachcardic, hypotension, laryngeal/facial oedema

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44
Q

What causes febrile non-haemolytic anaemia

A

Due to the accumulation of cytokines during blood storage

Unpleasant but not fatal

Occurs during or soon after reaction. Get a fever and go tachycardic

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45
Q

What do we give to treat thrombotic stokes?

A

Anti-platelets

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46
Q

How does a haemostatic plug form

A

Damage to the vessel wall, platelets adhere via vWF –> platelets activate and clump together to form clot

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47
Q

What is thromboxane

A

Produced by platelets (using COX enzyme) –> causes the constriction of vessel walls

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48
Q

What blocks the COX enzyme

A

Aspirin inhibits COX irreversibly (whereas the other NSAIDS are reversible) and inhibits COX1 more than COX2

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49
Q

What receptor binds fibrin for aggregation

A

Glycoprotein alpha2-beta3

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50
Q

What does clopidogrel black

A

The ADP receptor as ADP activates platelets

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51
Q

What pathway is the intrinsic pathway

A

12,11,9,7 - APTT

Factor 12 activates when in contact with a foreign surface (i.e. silica)

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52
Q

Extrinsic pathway

A

Prothrombin time –> F7 and TF. Tissue factor is expressed on the surface of damaged cells

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53
Q

What factor does thrombin activate?

A

Factor 11. Hence Factor 12 deficiency is asymptomatic, factor 11 deficiency has varying symptoms

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54
Q

Name the anticoagulatns

A

Protein C ( and its cofactor protein S), antithrombin III, fibrinolytic syndrome

55
Q

What does protein C cleave?

A

Factor V

56
Q

What enzyme mops up plasmin

A

alpha2-antiplasmin

57
Q

What bleeds do platelet/vessel wall defects cause?

A 
Mucosal and skin bleeding
Superficial bruising
Petechiae (not palpable and don't blanch)
Spontaneous
Prolonged and non-recurrent
58
Q

What types of bleed do coagulation defects cause?

A 
Joint bleeds
Deep spreading haematomas
Retroperitoneal bleeds
Deep muscular bleeds
Prolonged and recurrent
59
Q

Causes of vessel wall//platelet defects

A

thrombocytopenia, abnormalities in platelets, abrnormal vessel wall (Ehlers Danlos and Scurvy), Abnormal reaction between platelets and vessel wall (e.g. vWF)

60
Q

What is vWF

A

A large multimeric protein with lots of subunits

vWF disease is the most commonly inherited bleeding disorder

61
Q

Which part of vWF is good for adhesion

A

the high molecular weight part

62
Q

Which blood group have lower levels of vWF

A

blood group O

63
Q

What are the types of vWF

A

Type 1 - reduced amount of normal vWF
Type 2 - abnormal vWF but normal amount (only have low molecular weight part)
Type 3 - severe

64
Q

type of inheritance for vWF

A

type 1 & 2 are autosomal dominant

Type 3 is autosomal recessive!!

65
Q

What does vWF carry?

A

Factor VIII, hence defeciencies cause coagulation problems too. In types 1 & 2 it may also cause low FVIII especially in women

66
Q

Symptoms of vWF disease?

A

mucocutaneous bleeding including menorrhagia, post op+ post partum bleeding

67
Q

Treatment of vWF

A 
Antifibrinolytics (tranaexmic acid)
DDAVP --> releases vWF from endothelial cells --> can be tolerant after 3rd time
Factor concentrates of vWF
COCP for menorrhagia
Recombinant vWF in trials
68
Q

Who do we not give DDAVP too?

A

Under 2’s as it acts as a vasoconstrictor like vasopressin

69
Q

What do we do before giving factor concentrates?

A

Ensure they are vaccinated against hepatitis

70
Q

What inheritance are the haemophililas

A

X-linked recessive

71
Q

What is haemophilia A

A

Factor VIII defeciency 1in 5000 males

72
Q

What is haemophilia B

A

Factor IX defeciency 1 in 30000 males

73
Q

What types of bleed are the haemophilia?

A

muscle haemorraghes (can cause limb deformities), haemarthrosis, risk of intracranial bleed during pregnancy, spontaneous and severe,

74
Q

Treatment of Haemophilia

A

Replace protein
Factor concentrates
Antifibrinolytics
Vaccine against hepatitis

75
Q

What do we give only to treat haemophilia A

A

DDAVP as it releases vWF which carries factor VIII

76
Q

What can stop factor concentrates working?

A

Inhibitor development in 25% of haemophilia A (more common than in B). Mostly occurs in early treatment (first 10 days) and there is a genetic predisposition.
Give very high levels of Factor 8 so the body doesn’t see it as foreign

77
Q

How do you distinguish between inhibitor or deficiency?

A

repeat test with 50:50 normal to patients blood. If inhibitor APTT remains the same, if defeciency APTT improves

78
Q

What pathway does APTT measure?

A

Intrinsic

79
Q

What pathway does prothrombin time measure?

A

Extrinsic

80
Q

Where are the coagulation factors made?

A

All in the liver hepatocytes except Factor VIII made in the Kuppfer cells

81
Q

Reasons for Vitamin K Defeciency

A

Nutritional defeciency
Obstructive jaundice
Broad spectrum antibiotics (kill off the bacteria in the gut that synthesise vitamin K)
Neonates for 1-7 days –> give vitamin K injections to prevent this

82
Q

What factors are vitamin K dependent?

A

2, 7, 9, 10

83
Q

Describe changes in coagulation in liver disease

A

Thrombocytopenia –> due to portal hypertension and splenic congestion
Plateley dysfunction –> abnormal platelets as plasmin cleaves surface glycoproteins
Excess plasmin activity
Reduced factor concentration except for factor VIII
Delayed fibrin monomerisation due to altered finbrin glycosylation (as a result of excess silica acid)

84
Q

What defines a massive transfsion

A

Transfusion patients total body blood volume in 24 hours or 50% in 3 hours

85
Q

Complications of massive transfusions

A

DIC is common
Dilutional depletion of platelets and coagulation factors
- mainly factors V, VIII and fibrinogen
- need at least 7-8 litres of transfusino to cause thrombocytopenia in adults
Citrate toxicity (rare but if hypothermic or neonate risk increased)
Hypocalcaemia –> but no clinical significance

86
Q

Describe DIC

A

inappropraite activation of clotting and secondary fibrinolytic syndrome
Bleeding and small vessel thrombosis occurs, microvascular thrombosis causes end organ damage and microangiopathic haemolysis

87
Q

Acute causes of DIC

A

Sepsis, obstetric complications, trauma/tissue necrosis, acute intravascular haemolysis, fulminant liver disease

88
Q

Chronic causes of DIC

A

Obstetrics (retained dead foetus), malignancy, end stage liver disease, severe localised intravascular coagulation

89
Q

Diagnosis of DIC

A

Prolonged APTT & PT, raised D-dimer levels

No one diagnostic test

90
Q

Treatment of DIC

A

Treat underlying cause, supportive measures

91
Q

How do we control doses of oral anticoagulants

A

INR = (prothrombin time)^ISI

Prothrombin time = patients prothrombin time/normal
ISI = correction factor for sensitivity of thromboplastic to the international reference

92
Q

What drugs potentiate warfarin

A 
Erythromycin
NSAIDS
Cephalosporins
Sulphanylureas
Corticosteroids
Ampicillin
Amiodarone
93
Q

What drugs potentiate warfarin

A

Cholestyramine, spirinalactone, Rifampicin, Carbamazepine, Vitamin K

94
Q

How do we measure effects of heparin

A

Mainly use APTT (But doesn’t measure LMWH)

  • measure is effects on anti-thrombin and Xa
  • can use calcium prothrombin time or anti Xa Assays (does measure LMWH hepatic time)
95
Q

How to treat over coagulation with heparin

A

consider protamine

96
Q

LMWH acts most on which factorq

A

Higher ration of Xa than IIa activity. Higher bioavailability, longer half life and also more predicatable anti-coagulant response

97
Q

What do we give as supportive if long term DIC

A

Folic acid and vitamin K

98
Q

When is Epo produced

A

produced by the kidneys in response to hypoxia

99
Q

What does the globin chain do in haemoglobin

A

keeps it soluble, protects it from oxidation, allows for varying oxygen affinities

100
Q

What chromosome is alpha for Hb on

A

Chromosome 16

101
Q

What chromosome are the other Hb chains on

A

all the other Hb chains are on chromosome 11

102
Q

Describe normal adult Hb

A

Hb-A 95% (alpha, alpha) Hb-A2 3.5% (alpha, delta) HbF 1% (alpha, F)

103
Q

What are haemoglobinopathies

A

changes in globin genes or their expression that causes disease.
Either structural or thalassaemia

104
Q

What is a thalassaemia

A

changes in gene expression: imbalance of normal alpha and beta chain production
FREE GLOBIN DAMAGES THE RBC CELL MEMBRANE

105
Q

How to diagnose haemoglobinopathis

A

Hb Electrophoresis (but doesn’t pick up thalassaemias)
Isolectric Focusing
High Performance Liquid Chromatography

106
Q

Describe the sickle cell mutation

A

Valine to Glutamine substitution at position 6 of the beta globin gene on chromosome 11

107
Q

What happens in sickle cell disease

A

HbS polymersises at low oxygen to form long fibrils, distort the cells membrane causing a sickle cell shape
Reduces RBC life span to 20 days –> haemolytic anaemia

108
Q

What can reduce the effect of haemoglobinopathies

A

If there are other haemoglobin types in the blood i.e. HbF

HbF persists for 6 months after birth and can hide the effects of sickle cell

109
Q

Where do sickle cells occlude

A

Post capillary vessels as they cause an inflammatory response

110
Q

Symptoms of sickle cell trait

A

no clinical problems except with dehydration/severe hypoxia

111
Q

Where is sickle cell disease most common

A

West Africa

112
Q

Acute complications of sickle cell disease

A

Vado-Occlusive crises: bone, brain, dactyitis, priapism, chest syndrome
Septicaemia
Aplastic Crisis
Sequestration crisis (enlargement of spleen and liver)

113
Q

What do we give to prevent septicaemia in sickle cell

A

prophylactic penicllin

114
Q

Chronic complications of sickle cell

A 
Hyposplenism
Renal disease (medullary infarct, papillary necrosis, tubule damage --> inability to concentrate urine)
Avascular necrosis
Leg ulcers
Respiratory and cardiac complications
Gall stones
Osteomylitis
Retinopathies
115
Q

How do we treat sickle cell

A

Penicillin, analgesia, ECHO screen, transfusion (reduces risk of stroke), hydroxycarbamide

Bone marrow transplant is the only cure

116
Q

What does hydroxycarbamide do?

A

Treats sickle cell anaemia –> reduces the adhesion to the endothelium and enhances NO (a vasodilator)

117
Q

Name the types of alpha thalassaemia

A 
Hb Barts (alpha0) --> hydrops fetalis
HbH --> Tetramer of beta globin, soluble but not very functional
118
Q

What is beta thalassaemia

A

reduced rate of production of beta globin genes pathologically caused by the excessive production of alpha genes

119
Q

Describe thalassaemia minor

A

blood resembles iron deficiency

no clinical implciations

120
Q

Describe problems in thalassaemia intermedia

A

Pulmonary hypertension –> do an ECHO scan
Leg Ulcers
Hormone/endocrine problems (diabetes due to Fe deposits in endocrine organs and hypothyroidism)
Bone changes –> do a DEXA scan
Extramedullary haematopoiesis

No requirement for transfusion in first 3-5 years of life unless not meeting development foals

121
Q

Describe thalassaemia major problems

A

Presents at 1-2 years
Short stature and distorted limbs due to premature closing of the growth epiphysis
Blood transfusios in life saving
Abnormal lots of nucleated RBC –> as the bone marrow is pumping reticulocytes

122
Q

Describe the pathology of thalassaemia major

A

Excess alpha chains (due to ineffecetive erythropoieisis and reduced life spain)
Increased bone marrow activity (causes skeletal deformity, increases iron absoprption and end organ damage, protein malnutrition (transfusion can exacerbate organ damage)
Enlarged and overactive spleen due to pooling of RBC

123
Q

Describe thalassaemia facies features

A

Maxillary hypertrophy
Abnormal dentition
Hair on end skull as diplopic cavities widened by expanding bone marrow
Frontol bossing due to expanding bone marrow

124
Q

Treatment of thalassaemia major

A

Transfusion every 3 to 4 weeks: Problem is no system to excrete iron from the body causing iron overload

125
Q

Problems of iron overload

A

Dilated cardiomyopathy/heart failure, liver cirrhosis, failure of puberty/growth, diabetes

126
Q

Common causes of death in thalassaemia major

A

cardiac failure, arrhythmia and infections

127
Q

How to Treat Iron Overload

A

Iron chelation therapy from 2nd year of life –> promotes the excretion of iron in faeces and urine

Hormone replacement of hypogonadism

Good iron chelation makes you fertile but the stress of pregnany on the weak heart from iron overload is bad

128
Q

Examples of iron chelators

A

Desforrioxamine

New oral iron chelators are deferipone and deferasirox

129
Q

Describe changes physiological anaemia in pregnancy

A

Plasma volume expands 50%, RBC mass expands 25% –> hence haemodilution max at 32 weeks.

causes physiological anaemia

130
Q

Describe what happens to leucocytes in pregancy

A

Get leucocytosis as the placenta secretes GCSF causing the bone marrow to produce more WBCS

Mainly neutrophilic from the 2nd month to peak in 2nd/3rd trimester

131
Q

When does neutrophilic peak in pregnancny

A

increases from 2nd month to a peak in the 2nd/3rd trimester

132
Q

What happens to platelets in pregnancy

A

Gestational thrombocytopenia –> platelets fall after 20 weeks, most marked in late pregnancy

Occure due to folate defeciecny and pre eclampsia

133
Q

What happens to coagulation in pregnancy

A

Pregnancy is pro-thrombotic state. Persists for 6-8 weeks after giving birth

134
Q

Describe the changes that makes pregnancy prothrombotic

A

Increase in fibrinogen, factors V, VII, VII, X, XII, Minimal increase in IX, XIII increases initially then falls to 50% of pre pregnancy levels

Increase in procoagulant factors and platelet activation and thrombin generation markers

Decrease in natural anticoagulants and fibrinolysis

Increase in vWF (2fold more than increase in FVIII)

Small decrease in factor XI

CP - JL (14 decks)

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