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Belle Phase 2a > Haematology > Flashcards

Haematology Flashcards

1
Q

Describe the common pathway of the coagulation cascade

A

Intrinsic and extrinsic: Factor X > Factor Xa

Factor II (Prothrombin) > (Xa) > Factor IIa (Thrombin)

Fibrinogen > (IIa) > Fibrin

Factor XIII > Factor XIIIa

Fibrin > (XIIIa) > Fibrin clot

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2
Q

Name four causes of neutropenia

A

Infection e.g. EBV
Drugs e.g. phenytoin
Autoimmune
Myeloma

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3
Q

Name four causes of lymphocytopenia

A

Drugs e.g. steroids
Infection (post viral is common)
Renal impairment
Rheumatoid e.g. SLE, RA

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4
Q

Name four causes of neutrophilia

A

Infection e.g. bacterial
Drugs e.g. steroids
Leukaemia
Inflammation e.g. rheumatoid arthritis

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5
Q

Name four causes of lymphocytosis

A

Infection e.g. EBV
Stress
Vigourous exercise
Malignancy (CLL/ALL)

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6
Q

Name four causes of monocytosis

A

Malaria
Typhoid
TB
Myelodysplastic syndromes

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7
Q

Name four causes of eosinophilia

A

Asthma
Parasitic infections
Drugs e.g. peniciloin
Smoking

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8
Q

Name four causes of thrombocytopenia

A

Problems with production:
Viral infection e.g. EBV
Sepsis
B12/folate deficiency

Problems with destruction:
Drugs e.g. NSAIDs
Alcohol
ITP

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9
Q

Name four causes of thrombocytosis

A

Inflammation
Haemorrhage
Splenectomy
Iron deficiency

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10
Q

Name four things that cause PT/INR levels to change

A

Warfarin
Vitamin K deficiency
Liver disease
Disseminated intravascular coagulation

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11
Q

What does PT/INR measure?

A

The time taken for a blood clot to form via the EXTRINSIC pathway

Overall clotting factor synthesis/consumption

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12
Q

What does APTT measure?

A

(Activated partial thromboplastin time)

The time taken for blood to clot via the INTRINSIC pathway

Indicates issues with factors VIII, IX and XI:

  • Haemophilia A (VIII)
  • Haemophilia B (IX)
  • Haemophilia C (XI)
  • Von Willebrand’s disease (vWF pairs with factor VIII)
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13
Q

What does bleeding time measure?

A

Overall platelet function and levels
Measure of how long it takes a patient to stop bleeding from a wound

Platelet specific disorders increase bleeding time e.g. vWF disease, ITP, DIC, thrombocytopaenia

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14
Q

What does thrombin time measure?

A

How fast fibrinogen is converted to fibrin (by thrombin)

Prolonged by DIC, liver failure, malnutrition, and abnormal fibrinolysis

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15
Q

Name four causes of microcytic anaemia

A

TAILS:

Thalassaemia
Anaemia of chronic disease 
Iron deficiency anaemia (most common cause) 
Lead poisoning
Sideroblastic anaemia
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16
Q

Name four causes of normocytic anaemia

A

Acute blood loss
Anaemia of chronic disease

Haemolytic anaemia
Hypothyroidism

Pregnancy

Bone marrow failure
Renal failure

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17
Q

Two types of macrocytic anaemia

A

Megaloblastic

Normoblastic

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18
Q

Name two causes of megaloblastic macrocytic anaemia

A

B12 deficiency

Folate deficiency

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19
Q

Name four causes of normoblastic macrocytic anaemia

A 
Alcohol excess (affects bone marrow) 
Liver disease  
Iatrogenic e.g. azathioprine
Reticulocytosis
Hypothyroidism
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20
Q

Name four symptoms of anaemia

A 
Fatigue 
Shortness of breath (dyspnoea) 
Palpitations 
Anorexia 
Headache
Faintness
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21
Q

Name four signs of anaemia

A

Pallor e.g. conjunctival
Tachycardia
Hyperdynamic circulation e.g. systolic flow murmur
Raised respiratory rate

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22
Q

Name four causes of iron deficiency anaemia

A

Blood loss (most common cause in adults) e.g. GI bleed
Insufficient dietary iron (most common cause in children)
Increased requirements e.g. pregnancy
Inadequate iron absorption e.g. coeliac disease, IBD

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23
Q

Name three specific signs of iron deficiency anaemia

A

Koilonychia
Angular cheilosis
Atrophic glossitis

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24
Q

Blood film signs of megaloblastic macrocytic anaemia

A

Hypersegmented neutrophils and oval macrocytes

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25
Q

Mediastinal lymphadenopathy

A

Hodgkin lymphoma (85%)

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26
Q

Primary haemostasis

A

Initiation and formation of the platelet plug (platelet activation)

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27
Q

Secondary haemostasis

A

Formation of fibrin clot (coagulation cascades)

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28
Q

Dabigatran

A

Inhibits thrombin (DIRECT/DOAC)

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29
Q

Initial activation of platelets

A

Collagen binding to GPIIb/IIIa via vWF

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30
Q

Two hallmarks of AML

A

Gym hypertrophy

Auer rods

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31
Q

Most common leukaemia in children

A

ALL

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32
Q

Multiple myeloma features

A 
Old CRAB
Age (>65)
Calcium (hypercalcaemia)
Renal
Anaemia
Bone lesions
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33
Q

Multiple myeloma protein found in urine

A

Bence-Jones protein

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34
Q

Hodgkin lymphoma hallmark features

A

Lymphadenopathy painful after drinking alcohol
Reed-Sternberg cells
Bimodal distribution (early 20s and then 70s)

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35
Q

Ann Arbor staging for lymphoma

A

Stage 1: single lymph node
Stage 2: 2 or more (same side of diaphragm)
Stage 3: 2 or more (both sides of diaphragm)
Stage 4: extralymphatic

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36
Q

Blood test results for DIC

A 
Thrombocytopenia
Long PT 
Long APTT
Low fibrinogen 
Raised D-dimer
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37
Q

Mutation in polycythaemia vera

A

JAK2

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38
Q

Polycythaemia definition

A

High concentration of erythrocytes in the blood

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39
Q

Monoclonal gammopathy of undetermined significance (MGUS)

A

Close link to multiple myeloma (1% of cases develop into myeloma)

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40
Q

Philadelphia chromosome association

A

Chronic myeloid leukaemia

t (9;22)

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41
Q

Most common type of leukaemia in adults

A

AML

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42
Q

Blood film CLL

A

Smudge cells (abnormally fragile lymphocytes)

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43
Q

Bone marrow biopsy AML

A

Auer rods

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44
Q

ALL Px

A 
Anaemia 
Bleeding/bruising (thrombocytopenia)
Infections (neutropenia) 
Hepatosplenomegaly 
Lymphadenopathy 
CNS infiltration > headaches, CN palsies
Associated with Downs Syndrome
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45
Q

Leukaemia associated with warm haemolytic anaemia

A

CLL

46
Q

Richters transformation

A

Complication of CLL where the cancer transforms into Non-Hodgkin lymphoma

47
Q

Smudge cells on blood film

A

CLL

48
Q

Three phases including a 5 year asymptomatic chronic phase

A

Chronic myeloid leukaemia

49
Q

Multiple myeloma blood film

A

Rouleaux formation

50
Q

Relative polycythaemia

A

Normal number of erythrocytes but reduction in plasma

- obesity, dehydration, excessive alcohol consumption

51
Q

Absolute polycythaemia

A

Increased number of erythrocytes
Primary: bone barrow abnormality (polycythaemia vera)
Secondary: overstimulation of bone marrow (renal cancer, PKD, COPD)

52
Q

Myeloproliferative neoplasms (disorders)

A

Disorder of the bone marrow that causes abnormal growth of blood cells (platelets, WBCs, RBCs)

53
Q

2 types of myeloproliferative neoplasms

A

Chronic myeloid leukaemia

Polycythaemia vera

54
Q

Polycythaemia vera Px

A

Headaches, dizziness, fatigue, blurred vision
Erythema - hands, face, feet
Itching (especially after contact with warm water)
Hepatosplenomegaly

55
Q

Polycythaemia vera Tx

A

Venesection
Low dose aspirin daily
Hydroxycarbamide for those at risk of thrombus

56
Q

Leukaemia pathophysiology

A

Immature blast cells uncontrollably proliferate, taking up space within the bone marrow, and then infiltrating into other tissues

Lack of space within the bone marrow = fewer healthy cells can mature and be released into the blood (anaemia, thrombocytopenia)

57
Q

Thalassaemia

A

Autosomal recessive disorder causes less production of Hb

Alpha globin or Beta globin

58
Q

FBC: anaemia, thrombocytopenia, neutropenia

Type of leukaemia?

A

Acute lymphoblastic leukaemia

59
Q

Autosomal recessive (% inheritance)

A

Two carriers: 25%
One carrier, one disease: 50%
Two disease: 100%

60
Q

Sequestration crisis

A

Occurs in acute sickle cell crisis
Blood outflow from the spleen is blocked
Splenomegaly (due to blood accumulation)

61
Q

Aplastic anaemia

A

Pancytopenia

62
Q

General anaemia symptoms

A

Fatigue, headache, dizziness, dyspnoea

63
Q

General anaemia signs

A

Tachycardia, skin pallor, conjunctiva pallor, intermittent claudication

64
Q

Sign of iron deficiency anaemia

A

Koilonychia (spoon shaped nails)

Angular stomatitis

65
Q

Signs of B12 deficiency

A

Angular stomatitis

Lemon-yellow skin

66
Q

Signs of haemolytic anaemia

A 
Jaundice 
Dark urine (due to presence of Hb in urine)
67
Q 
FBC: low Hb, low MCV <95fl
Ferratin: low
Transferrin: raised 
Transferrin saturation: low 
Blood film: small, hypochromic cells
A

Iron deficiency anaemia

68
Q

Transferrin vs Transferrin saturation

A

Transferrin saturation is the ratio of serum iron to the TIBC (total iron binding capacity)

Transferrin is the main iron transport protein; its synthesis is inversely proportional to body iron stores. Hence, levels increase in iron deficiency to facilitate iron absorption. Transferrin levels are often low in inflammatory anaemia as transferrin expression is negatively affected by cytokines.

69
Q 
FBC: low Hb, low or normal MCV, high ESR
Ferratin: normal
Serum iron: low
Transferrin: low
Transferrin saturation: low
A

= AOCD
Chronic infection
Chronic inflammation (connective tissue diseases)
Neoplasia

70
Q

MCV: low
Serum iron: increased
Transferrin: increased
Ferratin: increased

A

Sideroblastic anaemia: Iron levels normal but the body can’t insert the iron into Hb
Blood film shows ringed sideroblasts

71
Q

Reticulocytes: raised
Bilirubin: raised
Urobilinogen: raised
Blood film: schistocytes

A

Haemolytic anaemia (normocytic)

72
Q

Causes of haemolytic anaemia

A

Sickle cell
Thalassaemia
Sepsis/DIC
Autoimmune

73
Q

Causes of B12 deficiency

A

Pernicious anaemia
Malabsorption (coeliac, IBD, ileostomy)
Decreased intake
Chronic nitrous oxide use

74
Q

Pernicious anaemia

A

Lack of intrinsic factor (usually produced by parietal cells in the stomach and allows B12 absorption in the terminal ileum) = causes B12 deficiency anaemia

75
Q

B12 foods

A

Meat, fish, eggs, milk

76
Q

Why does severe B12 deficiency cause neurological signs and symptoms

A

demyelination of the spinal cord

77
Q

Sickle cell genetics

A

Autosomal recessive, gene on cr11
Disease = Homozygous
Trait = Heterozygous

78
Q

Sickle cell pathophysiology

A

Glutamic acid substitution with valine = Beta globin polymerisation
= sickled cells, endothelial damage, reduced O2 carrying capacity

79
Q

Acute sickle cell presentation (crisis)

A 
MSK: bone pain, joint pain
Infection
Respiratory: dyspnoea, cough, hypoxia 
CNS: stroke 
Sequestration crisis
80
Q

Risk factors for sickle crisis

A

Low O2
Cold weather
Parvovirus B19
Exertion

81
Q

Chronic complications of sickle cell disease

A

Avacular necrosis of joints
Silent CNS infarcts
Retinopathy
Nephropathy

82
Q

Sickle cell Ix

A

FBC: low MCV, low Hb
Blood smear: sickled erythrocytes
Sickle solubility test (does not distinguish trait from disease)
Hb electrophoresis = HbS band

83
Q

Sickle cell crisis Tx

A

Morphine/O2/IV fluids/Transfusion exchange

84
Q

Chronic sickle cell disease Tx

A

Hydroxycarbamide (inhibits ribonucleotide reductase, decreases DNA synthesis, raises HbF levels)

85
Q

ADAM TS13 protein deficiency

A

Thrombotic thrombocytopenic purpura

86
Q

Thrombotic thrombocytopenic purpura pathophysiology

A

PRIMARY HAEMOSTASIS PROBLEM:
Deficiency in ADAM TS13 protein (vWF cleaving protease) = can’t breakdown clumps of vWF into useful monomers = microvascular clots form

87
Q

TTP Px

A

Adult female

Fatigue, fever, jaundice, petechiae, purpura, neurological deficit

88
Q

TTP Ix

A

FBC: raised WCC, low Hb, low platelets
Other: raised bilirubin (haemolytic anaemia), raised creatinine (renal damage from clots)
Blood smear: schistocytes
Clotting: normal PT and APTT (primary not secondary problem)

89
Q

TTP Tx

A

Plasma exchange
IV methylprednisolone
Monoclonal Abs
Contraindicated: platelet transfusion

90
Q

Immune thrombocytopenic purpura

A

Autoimmune
Primary haemostasis (GPIIb/IIIa)
Risk factors: paediatric, post-viral

91
Q

DIC Ix

A

Bloods: low platelets, low fibrinogen, high D-dimer, long PT, long APTT
Blood smear: schistocytes

92
Q

DIC Tx

A

Treat underlying cause (e.g. sepsis)
Low fibrinogen = cryoprecipitate
Low platelets = platelet transfusion

93
Q

Haemophilia A

A

Intrinsic pathway: Factor VIII deficiency
X-linked recessive
Male > Female

94
Q

Haemophilia Px

A

Soft tissue bleeding pattern: muscles, joints, haematoma formation

95
Q

Haemophilia A Ix

A

APTT: long
PT may be normal
Genetic testing + factor VIII testing

96
Q

Haemophilia A Tx

A

Recombinant factor VIII

97
Q

Type 1 Von Willebrands disease

A

Autosomal dominant
Defect in QUANTITY of vWF
- Primary haemostasis disorder

98
Q

Why can vWF disease result in factor VIII deficiency

A

vWF protects VIII from liver protein C destruction

99
Q

Presentation of vWF disease

A

Mucocutaneous bleeding (epistaxis, GI bleeds, menorrhagia, easy bruising)

100
Q

vWF disease Ix

A

Plasma vWF measurement

APTT can be prolonged if factor VIII low

101
Q

Type 1 vWF Tx

A

Desmopressin

Tranexamic acid can reduce acute bleeding

102
Q

ALL/AML treatment

A

Blood and platelet transfusions
Chemotherapy
Stem cell/bone marrow transplant
Antibiotics

103
Q

CLL Tx

A

Chemo (Rituximab)

104
Q

CML Tx

A

Chemo
Stem cell/bone marrow transplant
Tyrosine kinase inhibitors (Imatinib)

105
Q

ABVD Chemotherapy used in which cancer

A

Hodgkin lymphoma Tx

106
Q

RCHOP chemotherapy

A

Non-Hodgkin lymphoma

Rituximab, cyclophosphamide, prednisolone

107
Q

G6PD deficiency features

A

X-linked
West Africa, Middle East
Haemolytic anaemia + Splenomegaly
Heinz bodies/bite cells on blood smear

108
Q

Haemolytic uraemic syndrome

A

Triad:
Haemolytic anaemia
AKI (e.g. oliguria, haematuria)
Thrombocytopenia

Differentiate from TTP with ADAMTS13 testing

109
Q

A 50-year-old woman
weight loss
anaemia
splenomegaly

Haemoglobin: LOW
Platelets: VERY HIGH
White cell count: VERY HIGH
Blood film: Leucocytosis with all stages of granulocyte maturation

A

CML = increased turnover of MYELOBLAST cells (further differentiate into basophils, neutrophils and eosinophils)

110
Q

Reduced reflexes

Type of anaemia?

A

Macrocytic anaemia caused by hypothyroidism OR B12 deficiency

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