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4th year everything > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the process of blood formation called?

A

Haemopoiesis

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2
Q

The haemopoietic system includes what five components?

A

Bone marrow, liver, spleen, lymph nodes and thymus

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3
Q

What is the average lifespan of red cells?

A

120 days

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4
Q

What is the average lifespan of platelets?

A

7 days

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5
Q

What is the average lifespan of granulocytes?

A

7 hours

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6
Q

What are reticulocytes?

A

Young red cells

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7
Q

Reticulocytes represents what percentage of total circulating red blood cells?

A

0.5-2.5%

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8
Q

What makes up a haem group?

A

A single molecule of protoporphyrin IX bound to a single ferrous ion (Fe2+)

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9
Q

What symptoms may arise from anaemia?

A

Fatigue, faintness and breathlessness

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10
Q

In patients with concurrent atheromatous disease, what more serious symptoms can anaemia precipitate?

A

Angina pectoris and intermittent claudication

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11
Q

What signs might be observable in the anaemic patient? (4)

A

Skin and mucous membranes may be pale
Tachycardia
Systolic flow murmur
Cardiac failure (in elderly people/ those with compromused cardaicfunction)

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12
Q

What does microcytosis usually represent about the structure of a red blood cell?

A

Decreased haemoglobin content

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13
Q

What is the most common cause of anaemia worldwide?

A

Iron deficiency anaemia

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14
Q

What are the causes of iron deficiency anaemia? (4)

A

Blood loss
Increased demands (such as growth and pregnancy)
Decreased absorption
Poor dietary intake

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15
Q

What signs does iron deficiency produce? (3)

A

Brittle hair and nails
Atrophic glossitis
Angular stomatitis

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16
Q

Ansiocytosis and poikilocytosis are both features seen on a blood film of an iron-deficient patient; what do these two terms mean?

A

Ansiocytosis - variation in size

Poikilocytosis - variation in shape

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17
Q

What investigations are indicated in a suspected case of iron deficiency anaemia?

A

Blood count and blood film
Serum ferritin (reflecting iron stores) are low
Serum iron is low

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18
Q

How is iron deficiency managed?

A

Find and treat underlying cause

Oral iron supplementation e.g. ferrous sulphate/gluconate

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19
Q

What is sideroblastic anaemia?

A

A rare disorder of haem synthesis characterised by refractory anaemia in the peripheral circulation and ring sideroblasts in the bone marrow

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20
Q

What are ring sideroblasts?

A

Erythroblasts with iron deposits in the mitochondria and reflect impaired utilisation of iron within the red cell

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21
Q

What causes sideroblastic anaemia?

A

It may be inherited or acquired (secondary to myelodysplasia, alcohol excess, lead toxicity)

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22
Q

What is anaemia of chronic disease?

A

Anaemia occuring in patients with either:

  • Chronic inflammatory diseases (such as Crohn’s disease or rheumatoid arthrtitis)
  • Chronic infections (e.g. tuberculosis)
  • Malignancy
  • Chronic kidney disease
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23
Q

As seen on a blood film; what type of anaemia is anaemia of chronic disease?

A

Normochromic, normocytic anaemia

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24
Q

How is anaemia of chronic disease managed?

A

Treatment of the underlying chronic disease and sometimes erythropoietin

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25
Q

What is the most important investigation of a macrocytic anemia?

A

Measurement of serum B12 and red cell folate

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26
Q

What is megaloblastic anaemia?

A

A macrocytic anaemia charactersed by the presence of megaloblasts in the bone marrow (immature red cells with delayed nucelar maturation relative to that of the cytoplasm)

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27
Q

What is the most common cause of megaloblastic anaemia?

A

Vitamin B12/folate deficiency

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28
Q

What is pernicious anaemia?

A

An autoimmune condition in which there is atrophic gastritis with loss of function of the parietal cells and hence a failure to produce intrinsic factor (essential for uptake of vitamin B12)

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29
Q

What are the clinical features of pernicious anaemia?

A 
Insidious onset 
Glossitits (sore, red tongue)
Angular stomatitis 
Mild jaundice 
Neurological features (due to B12 deficiency)
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30
Q

What neurological features may be present in B12 deficiency?

A

Symmetrical damage of the spinal cord leading to progressive weakness, ataxia, and eventual paraplegia

Dementia and visual distrubances may also occur

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31
Q

What is the main difference in the symptom profile between B12 deficiency and folate deficiency?

A

In folate deficiency there are no neurological features

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32
Q

What might cause folate deficiency? (4) (Non exhsautive list)

A

Pregnancy, coeliac disease, acohol excess, trimethoprim

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33
Q

What is aplastic anaemia?

A

Defined as a pancytopenia (deficiency of all blood cells) due to bone marrow failure

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34
Q

What are the clinical features of aplastic anaemia? Why do they occur?

A

Deficiency of red cells, white cells and platelets

Causing anaemia, susceptibility to infection and bleeding

Physcial findings include bruising, bleeding gums, epistaxis and mouth infections

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35
Q

How is aplastic anaemia investigated and confirmed?

A

Trephine bone marrow biopsy for assessment of bone marrow cellularity

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36
Q

What causes aplastic anaemia?

A

Most commonly encountered due to chemotoxic drugs and radiation

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37
Q

What is the management strategy for aplastic anaemia?

A

Withdrawal of the offending agent (chemotoxic drug for example) followed by definitive therapy such as blood and platelet transfusions

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38
Q

What medical emergancy may occur in apalstic anaemia?

A

Neutropenic sepsis

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39
Q

How might neutropenic sepsis present?

A

Neutropenic patient (neutrophil count <1 x10^9/L) who is pyrexial, or has new-onset confusion, tachycardia, hypotension, dyspnoea or hypothermia

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40
Q

List some blood investigations which are necessary in the case of neutropenic sepsis

A 
FBC
Differential white cell count
CRP
U/Es
Liver biochemistry
Clotting screen
Blood cultures
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41
Q

What radiological investigations might you consider in the neutropenic patient?

A
  • Chest X-ray

- Consider further imaging if there are localising signs e.g. CT abdomen/pelvis

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42
Q

What empirical antibiotics are indicated in the case of suspected neutropenic sepsis?

A

Piperacillin and aminoglycoside (to cover Gram-negatives and Pseudomonas)

Consider adding vancomycin if clincal deterioration, fever persists or suspected MRSA infection

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43
Q

What is haemolytic anaemia?

A

Anaemia resulting from increased destruction of red cells with a reduction in lifespan and therefore a compensatory rise in premature reticulocytes

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44
Q

Haemolysis may be either intravascular or extravascualar - which is more common?

A

Extravascular (within the reticuloendothelial system, mainly the spleen)

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45
Q

What investigations may indicate a intravascular haemolysis?

A

Raised level of plasma haemoglobin

Positive Schumm’s test (methaemalbumin in plasma)

Low/absent haptoglobins (complex formed from free haemoglobin - removed rapidly by the liver)

Haemosiderinuria - haemosiderin in the urine

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46
Q

Inherited haemolytic anaemias are due to defects in one of three components - what are they?

A

Defects in either:

  • Cell membrane
  • Haemoglobin structure
  • Metabolic procresses of the red cell
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47
Q

Hereditary spherocytosis is an example of an inherited haemolytic anaemia caused by a defect in which red cell component?

A

Cell membrane

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48
Q

What inheritance does hereditary spherocytosis show?

A

Autosomal dominant

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49
Q

What is the pathophysiology of hereditary spherocytosis?

A

Deficiency in spectrin (most commonly) leading to increase permeability to sodium ions, causing rigid and spherical red cells which are prematurely removed by the spleen (extravascular)

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50
Q

What symptoms may arise in hereditary spherocytosis?

A

Ranges from asymptomatic to severe haemolysis (anaemia, jaundice, splenomegaly)

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51
Q

What types of crises may occur in any haemolytic anaemia?

A

Megaloplastic
Aplastic
Haemolytic

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52
Q

What does aplastic crisis usually occur as a result of in the course of normal haemolytic disease?

A

After infection (particularly erythro(paro)virus)

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53
Q

What does megaloblastic crisis usually occur as a result of in the course of normal haemolytic disease?

A

Folate deficiency caused by hyperactivity of the bone marrow

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54
Q

What biliary pathology may occur as a result of chronic haemolytic disease?

A

Pigment gallstones

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55
Q

What investigations may confirm hereditary spherocytosis?

A

Blood count demonstrates reticulocytosis and anaemia
Blood film shows spherocytes
Evidence of haemolysis (raised serum bilirubin and urinary urobilinogen)

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56
Q

How is hereditary spherocytosis managed?

A

Splenectomy indicated in adulthood to relieve symtptoms

not in childhood due to risk of fulminant infections

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57
Q

What are thalassaemias?

A

A group of genetic disorders causing reduced rate of production of one or more globin chains used in the synthesis of haemoglobin (ineffective erythropoiesis)

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58
Q

There are three main forms of beta-thalassaemia; what are they?

A
  1. beta-thalassaemia minor (trait) - carrier state due to heterozygous genetic profile
  2. beta-thalassaemia intermedia - moderate penetrance causing mild anaemia and other thalassaemic features
  3. beta-thalassaemia major - presents in first year of life with significant thalassaemic features, severe anaemia and hepatosplenomegaly
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59
Q

What are the talassaemic features? Why do they occur?

A

Hypertrophy of inept bone marrow causes:

  • Bony abnormalities (enlarged maxilla, prominent frontal and parietal bones)
  • Recurrent leg ulcers
  • Gallstones
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60
Q

How is thalassaemia diagnosed?

A

Haemoglobin electrophoresis

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61
Q

How is thalassaemia treated?

A

Regular blood transfusions (keeping Hb >100g/L) - suppressing ineffective erythropoiesis and bony abnormalities

Aduvant chelation therapy required to prevent iron overload and folate supplementation.

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62
Q

Describe the clincal spectrum of alpha-thalassaemia

A

Ranging from mild anaemia with microcytosis to severe condition incompatible with life resulting in stillbirht (hydrops fetalis)

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63
Q

What are sickle syndromes?

A

A family of haemoglobin disorders in which the sickle beta-globin gene is inherited.

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64
Q

Why does sickle cell anaemia not present and features until around 6 months of age?

A

Production of foetal haemoglobin (HbF) is unaffected and so sickle disease doesnt manifest until HbF decreases to adult levels (at 6 months of age)

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65
Q

Sickle cell anaemia has marked phenotypic variation. Outline some of the clinical features which may present.

A

Vaso-occlusion - acute dactylitis in children is more likely. In adults, long bone, rib, spinal and pelvic pain more likely.

Other vaso-occulsive complications: avascular necrosis of bones, retinal iscahemia, cerebral infarction, priapism

Anaemia - usually exists in a steady state Hb 60-80g/L with a high reticulocyte count (10-20%)

Other complications: Chronic kidney disease, leg ulcers, osteomyelitis, pulmonary hypertension, acute chest syndrome

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66
Q

What events may precipitate a rapid destabilisation of haemoglobin levels in a patient with sickle cell disease? (2)

A

Splenic sequestration - spleen becomes engorged with red cells leading to rapid enlargement of spleen (can also occur in the liver)

Aplatic anaemia - commonly due to eythrovirus B19 infection

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67
Q

What is acute chest syndrome?

A

Medical emergancy characterised by fever, cough, dyspnoea and pulmonary infiltrates on chest X-ray

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68
Q

How are painful crises managed?

A

Morphine 0.1mg/kg IV or SC every 20 mins with aduvants

Also prescribe: laxatives (lactulose 10ml BD), anti-pruritics, antiemetics, anxiolytics, antibiotics

Oxygen, rehydration

Check for acute chest or liver/splenic sequestration

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69
Q

In patients with recurrent painful crises, what drug is indicated to help prevent them?

A

Hydroxycarbamide (raises concentration of foetal haemoglobin)

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70
Q

What is the most common red cell enzyme deficiency causing haemolytic anaemia?

A

Glucose-6-phosphate dehydrogenase deficiency (G6PD)

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71
Q

What is the inheritance pattern of G6PD?

A

X-linked

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72
Q

What are the clinical features of G6PD?

A

Neonatal jaundice, chronic haemolytic anaemia, acute haemolysis (precipitated by fava beans, nitrofurantoin and other drugs)

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73
Q

How are autoimmune haemolyses categorised?

A

Depending on whether the antibody reacts best at body temperature (warm antibodies) or at lower temperatures (cold)

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74
Q

What is mechanical haemolytic anaemia?

A

Red cells are injured by physical trauma in the circulation. Can be due to a number of things:

  • Leaking prosthetic valves
  • March haemoglobinuria ( damage to red cells from prolonged marching)
  • Microangiopathic haemolysis (e.g. in DIC)
75
Q

What are myeloproliferative disorders?

A

Disorders of uncontrolled clonal proliferation of one or more cell lines in the bone marrow.

76
Q

Name the myeloproliferative disorders?

A

Polycythaemia vera
Essential thrombocytopaenia
Myelofibrosis
Chronic myeloid leukaemia

77
Q

What are the symptoms of polycythaemia vera?

A

All as a result of both hypervolaemia and hyperviscocity

Headache, vertigo, tinnitus, visual disturbance, angina pectoris, intermittent claudication, pruritis, venous thrombosis

78
Q

What signs may be found in polycythaema vera?

A

Plethoric complexion, hepatosplenomegaly (distinguishes primary from secondary polycythaemia)

79
Q

What other conditions are people with polycythaemia vera at risk of?

A

Gout - due to increased uric acid production

Haemorrhage - due to friable haemostatic plugs

80
Q

How is polycythaemia vera treated?

A

Venesection (maintain PCV <0.45L/L) - may be sufficient
Chemotherapy:
1. Hydroxycarbamide to reduce platelets
2. Low dose aspirin - reduce recurrence of thromboses
3. Anagrelide - useful for thrombolysis
4. Radioactive phosphorus - to combat risk of leukaemic conversion
5. Allopurinol - decrease uric acid levels

81
Q

What is essential thombocythaemia?

A

Conditon characterised by normal haemoglobin levels and white cell counts but elevated platelets

82
Q

How may essential thombocythaemia present?

A

Either with bleeding or thromboses

83
Q

How is essential thrombocythaemia diagnosed?

A

An otherwise well with a platelet count of over 1000 x10^9/L is generally considered to be sufficient for diagnosis

84
Q

How is essential thrombocythaemia treated?

A

Hydroxycarabamide and/or anagrelide if required

85
Q

List some differnetial diagnoses for essential thrombocythaemia

A 
Reactive thrombocytosis 
Autoimmune rheumatic disorders
Malignancy 
Polycythaemia vera
Myelodysplasia
86
Q

What is myelofibrosis?

A

A condition characterised by haemopoietic stem cell proliferation associated with marrow fibrosis

87
Q

How does myelofibrosis present?

A

Insidious onset of weakness, weight loss and lethargy

Bleeding may occur in thrombocytopaenic patient

Hepatomegaly and massive splenomegaly

88
Q

What are the four most common causes of death in myelofibrosis?

A

Transformation into acute myeloid leukaeia
Progression of myelofibrosis
Cardiovascular disease
Infection

89
Q

What investigations might support your diagnosis of myelofibrosis?

A
  1. Blood count (showing anaemia)
  2. Trephine bone marrow biospy (increased fibrosis)
  3. Absent philidelphia chromosome (distiinguishes myelofibrosis from chronic myeloid leukaemia)
  4. JAK2 mutation (present in 50% of cases)
90
Q

How is myelofibrosis managed?

A

Transfusions (correct anaemia)
Allopurinol (prevent gout)
JAK2 inhibitors (e.g. rutixumab)
Allogenic stem cell transplant in young patients (offers only hope to cure)

91
Q

What is myelodysplasia?

A

A group of acquired marrow disorders caused by defects in stem cells; resulting in progressive bone marrow failure and potential evolution into acute myeloid leukaemia

92
Q

How is myelodysplasia diagnosed?

A

Paradoxical appearance of peripheral pancytopenia on blood film and hypercellular bone marrow (due to prematuer loss of cells by apoptosis)

93
Q

What blood constituents are present in fresh frozen plasma?

A

All coagulation factors

94
Q

What blood constituents are present in cryoprecipitate?

3

A

Factor VIII
Von Willebrand factor
Fibrinogen

95
Q

What complications may occur after a transfusion (excluding massive transfusion)?

(5)

A
  1. ABO mismatch (most severe)
  2. Febrile reactions (due to activation of anti-leucocyte antibodies in the recipient)
  3. Anaphylactic reactions (in IgA deficient patients)
  4. Infection
  5. Heart failure (particularly in elderly)
96
Q

What complications may occur during/after massive transfusion?

A

Hypocalcaemia
Hyperkalaemia
Hypothermia

97
Q

What are the five types of white cells found in the peripheral blood?

A

Neutrophils, eosinophils, basophils (all called granulocytes) and lymphocytes and monocytes

98
Q

What are monocytes?

A

Precursors to tissue macrophages

99
Q

What pathological processes do eosinophils play a part in?

A 
Allergic reactions
Helminth infections and protozoa 
Asthma
Malignancy 
Hyper-eosinophilic syndrome
100
Q

What is hyper-eosinophilic syndrome?

A

Restrictive cardiomyopathy, hepatosplenomegaly and very high eosinophils (>0.4 x10^9/L)

101
Q

What is immune thrombocytopenic purpura (ITP)?

A

Immune destruction of platelets

102
Q

Describe the different occurrences of immune thrombocytopenic purpura in adults and children

A

In children - usually follows viral infection rapidly and is self-limiting

In adults - less acutely and often seen concurrently with other immune conditions e.g. SLE

103
Q

How is immune thrombocytopenic purpura diagnosed?

A

Diagnosis of exclusion

Platelet antibodies not essenstial for diagnosis but may be seen in 60-70% of patients

104
Q

How is immune thrombocytopenic purpura managed?

A

First-line therapy - oral corticosteroids or IV IgG where rapid rise in platelets is optimal (pre-surgery)

Second-line therapy - splenectomy and thrombopoietin receptor agonists (e.g. romiplostim)

105
Q

What is thrombotic thrombocytopenic purpura?

A

Widespead adhesion and agglutination of platelets leading to microvascular thrombosis and profound thombocytopenia (<150 x10^9/L)

106
Q

Deficiency of what protease causes thrombotic thrombocytopenic purpura

A

ADAMTS-13

107
Q

What can cause thrombotic thrombocytopenic purpura?

A

Congenital or sporadic or autoantibody-associated (pregnancy, infection etc.)

108
Q

What are the symptoms of thrombotic thrombocytopenic purpura?

A

Florid purpura, renal failure, fluctuant cerebral dysfunction and haemolytic anaemia

109
Q

How is thrombotic thrombocytopenic purpura treated? What is counter-intuitively contraindicated?

A

Plasmapheresis
High dose steroids

Platelet concentrates are contraindicated

110
Q

What is Haemophilia A caused by? What is the inheritance pattern?

A

X-linked recessive disorder resulting in a deficiency of factor VIII

111
Q

Haemophiliac is categorised into mild, moderate and severe entities. Describe each.

A
  1. Mild (>5U/dL) - bleeding only with trauma
  2. Moderate (1-5U/dL) - severe bleeding following injury and occaisional spontaneous bleeding
  3. Severe (<1U/dL) - severe spontaneous bleeding into muscles/joints causing crippling arthropathy
112
Q

What investigations would help confirm the diagnosis of Haemophilia A?

A

Prolonged APTT

Normal PT and vWF

113
Q

How is haemophilia A treated?

A
  • IV infusion of recombinant factor VIII (with supplies at home too)
  • Synthetic vasopressin (used to increase production in patients with mild haemophilia)
  • Advice about lifestyle e.g. avoiding contact sports
114
Q

What is the difference between haemophilia A and B? What treatment options differ in haemophilia B?

A

Haemophilia B is a deficiency of factor IX

Vasopressin is ineffective and therefore not used in treatment of haemophilia B

115
Q

What is von Willebrand’s disease?

A

hereditary deficiency of von Willebrand Factor (vWF)

116
Q

What is the function of von Willebrand factor?

A

Contributes to platelet adhesion to damaged subendothelium and stabilisaton of factor VIII

117
Q

Outline the three different types of von Willebrand’s disease

A 
Type 1 (quantitative) - decreased vWF 
Type 2 (qualitative) - poor functioning vWF
Type 3 (absolute deficiency) - absence of vWF
118
Q

Outline the inheritance pattern of the types of von Willebrand’s disease

A

Type 1 and 2 are autosomal dominant

Type 3 is autosomal recessive

119
Q

What investigations might help support a diagnosis of von Willebrand’s disease?

A
  1. Prolonged bleeding time (indicating poor platelet adhesion)
  2. Prolonged APTT
  3. Normal PT
120
Q

Vitamin K is reuqired for the formation of which coagulation factors?

A

Factors II, VII, IX and X

121
Q

What may cause vitamin K deficiency?

A

Malnutrition, malabsorption or warfarin use

122
Q

How is vitamin K deficiency treated?

A

IM Vitamin K supplementation

123
Q

What is disseminated intravascaulr coagulation (DIC)?

A

Widespread generation of fibrin within blood vessels. There is massive consumption of platelets and coagulation factors

124
Q

What are the most comon causes of disseminated intravascaulr coagulation (DIC)? (4)

A
  • Sepsis
  • Major trauma (surgery, burn etc.)
  • Advanced cancer
  • Obstetric complications (amniotic embolism, abruptio placentae)
125
Q

What is the clinical presentation of disseminated intravascaulr coagulation (DIC)?

A

Varies from no bleeding at all to complete haemostatic failure

Thrombotic events may occur too

126
Q

What investigations are indicated in the susepcted case of disseminated intravascaulr coagulation (DIC)?

A

Blood count - showing severe thrombocytopenia
Prolonged APTT, PT and bleeding time
Blood film - fragmented red cells

127
Q

How is disseminated intravascaulr coagulation (DIC) treated?

A 
Treat the underlyig cause
Platelet concentrates (maintain platelets above 50 x10^9/L

Cryoprecipitate and red cells are also indicated in bleeding patients

128
Q

Aterial thrombi are formed of what blood constituent and arise due to what underlying chronic pathology?

A

Platelets adhere to atheroma (atherosclerosis)

129
Q

Where do arterial thrombi occur?

A

Areas of turbulent blood flow e.g. bifurcating arteries

130
Q

What are venous thrombi formed of?

A

Fibrin and red cells

131
Q

Where do venous thrombi occur?

A

Originate around the valves of veins; partiularly in the deep veins of the leg

132
Q

What sort of drugs target venous thombi?

A

Heparin and warfairn (anti-coagulants)

133
Q

What sort of drugs target arterial thrombi?

A

Aspirin and clopidegrel (anti-platelets)

134
Q

Fondapariunux is an anti-coagulant; what coagulation factor does it inhibit?

A

Factor X (similar in action to herapin)

135
Q

What is dabigatran and what is it prescribed for?

A

Direct thrombi inhibitor; given as prophylaxis for venous thromboembolism following hip or knee replacement surgery

136
Q

What is the target INR of a paitent to prevent thromboembolism?

A

2.5 (3.5 if recurrent venous thromboembolism despite monotherapy with warfarin)

137
Q

What are the two most common side effects of iron supplementation?

A

Constipation and diarrhoea

138
Q

What are common side effects of B12 supplementation? What is the potential severe side effect on resolution of severe B12 deficiency anaemia?

A

Itching, fever, nausea and diziness

Potential for severe hypokalaemia due to intracellular shift on anaemia resolution

139
Q

What does NOAC stand for?

A

Novel Oral Anti-Coagulants

140
Q

What is alpha-fetoprotein?

A

Serum tumour biomarker for hepatocellular carcinoma and non-seminomatous germ cell tumour of the testes

141
Q

What is beta-human chorionic gonadotropin (beta-HCG) ?

A

A serum tumour biomarker for choriocarcinoma, germ cell tumours of the testes and lung cancer

142
Q

What is the tumour biomarker for prostate cancer?

A

Prostate specific antigen (PSA)

143
Q

Chorioembryonic antigen (CEA) may be raised in what cancers?

A

Colorectal cancer

Some gastrointestinal malignancies

144
Q

What is the tumour biomarker for ovarian cancer?

A

CA-125

145
Q

What is CA19-9?

A

Serum tumour biomarker for upper gastrointestinal malignancies

146
Q

What is CA15-3?

A

Serum tumour biomarker for breast cancer

147
Q

What is osteopontin?

A

Serum tumour biomarker for mesothelioma

148
Q

Outline the four sources of haemopoietic stem cell transplant

A

Allogenic - bone marrow from another individual
Autologous - bone marrow harvested from self
Syngenic - bone marrow from identical twin
Umbilical cord

149
Q

What is superior vena cava syndrome?

A

Arising from any upper mediastinal mass (most commonly lung cancer or lymphoma) - characterised by compression of the superior vena cava

150
Q

What is the presentation of superior vena cava syndrome?

A

Difficulty breathing or swallowing
Odematous face
Venous congestion in the neck (dilated veins)

151
Q

How is superior vena cava syndrome treated?

A

Immediate steroids and vascular stenting

Potentially radiotherapy/chemotherapy to shrink mass

152
Q

What is acute tumour lysis syndrome?

A

A condition characterised by rapid breakdown of tumour following commencement of treatment.

153
Q

What biochemical abnormalities arise in acute tumour lysis syndrome?

A

Hyperphosphataemia (and secondary hypercalcaemia)
Hyperkalaemia
Hyperuraemia

154
Q

What is the main complication of acute tumour lysis syndrome?

A

Acute kidney injury due to urate and calcium phosphate deposition in renal tubules

155
Q

Acute Lymphoblastic Leukaemia has a propensity to involve the CNS. For this reason, what extra treatment measure is taken?

A

Intrathecal chemotherapy (prophylaxis)

Sometimes cranial irratiation is used in high risk patients

156
Q

What are the clinical features of acute leuakemia?

A

Bone marrow failure (anaemia, thormbocytopenia and prepensity to infection)

Sometimes there is lymphadenopathy and heaptosplenomegaly

157
Q

What genetic funding is characteristic of chronic myeloid leukaemia?

A

Philidelphia chromosome mutation

158
Q

What is the presentation of chornic myeloid leukaemia?

A

Insidious onset of fever, weight loss, sweating and symptoms of anaemia

Massive splenomegaly is characeristic

159
Q

Outline the phases of chronic myeloid leukaemia

A

Chronic-phase - lasts 3-4 years

Blastic transformtion - usually acute myeloid leukaemia (causing rapid death)

160
Q

What is the first-line treatment for chronic-phase chronic myeloid leukaemia?

A

Imatinib (tyrosine kinase inhibitors)

Not effective after blastic transformation

161
Q

What is chronic lymphocytic leukaemia?

A

Incurable disease of the elderly characterised by uncontrolled proliferation of mature B lymphocytes

162
Q

Outline the course of chronic lymphocytic leukaemia

A

Indolent course and generally asymptomatic

Later on, symptoms of bone marrow failure and hepatosplenomegaly

163
Q

What investigations would support a diagnosis of chronic lymphocytic leukaemia?

A

Blood count - lymphocytosis, anaemia, thrombocytopenia

Blood film - smudge cells (artefact of small mature lymphocytes)

Immunophenotyping - essential to exclude reactive lymphocytosis

Cytogenetics - identify specific mutation

164
Q

What is the standard first-line therapy for chronic lymphocytic leukaemia?

A

Combination of fludarabine, cyclophosphamide, rutixumab

165
Q

What is lymphoma?

A

B and T cell malignancies of the lymphoid system

166
Q

How are lymphomas classified?

A

Classified as either Hodgkin (specific entity) or Non-Hodgkin (over 50 subtypes)

167
Q

What virus is most strongly associated with Hodgkin lymphoma?

A

Epstein-Barr Virus

168
Q

What demographic is Hodgkin lymphoma most likely to present in?

A

It is typically a disease of young adults

169
Q

What are the clinical features of Hodgkin lymphoma?

A

Lymphadenopathy (often cervical) with rubbery consistency
Potentially hepatosplenomegaly
Systemic “B” symptoms
Constitutional symptoms

170
Q

What are systemic “B” symptoms often seen in Hodgkin lymphoma?

A

Fever
Drenching night sweats
Weight loss (>10% of body weight in 6 months)

171
Q

What constitutional symptoms may be featured in Hodgkin lymphoma?

A

Pruritus
Fatigue
Anorexia
Alcohol-induced pain at site of lymphadenopathy

172
Q

What investigations would help you confirm the diagnosis of Hodgkin lymphoma?

A

Blood count - anaemia (normochromic and normocytic)
ESR - raised (used as indicator of disease activity)
Serum lactate dehydrogenase (poor prognostic marker)
Chest X-ray - mediatinal widening

Diagnosis by lymph node biospy (seeing Reed-Steinberg cells)

Staging by CT and PET scans

173
Q

How is early-stage Hodgkin lymphoma treated?

A

Breif chemotherapy followed by field irradiation

174
Q

How is late-stage Hodgkin lymphoma treated?

A

Cyclical combination of chemotherapy and field irradiation

175
Q

Non-hodgkin lymphoma (NHL) is comprised of over 50 subtypes. The majority of NHLs are formed from which cell lineage?

A

80% are of B cell origin

176
Q

Some Non-Hodgkin Lymphomas are associated with specific infections; what lymphoma is associated with helicobacter pylori?

A

Gastric mucosal assocaited lymphoid tissue (MALT) lymphoma

177
Q

What is the classical presenation of Non-Hodgkin Lymphoma?

A

Presentation of a painless peripheral lymph node enlargement

Systemic featues and/or bone marrow infilatration may also occur

178
Q

What investigations are indicated in the case of Non-Hodgkin Lymphoma?

A

Blood count - anaemia, leucocytosis, thrombocytopenia
Abnormal LFTs
Serum lactate dehydrogenase (prognostic factor)
Bone marrow biospy (confirms marrow involvement)

Lymph node biopspy for definitive diagnosis

179
Q

What is multiple myeloma?

A

Malignant proliferation of plasma cells in the bone marrow producing paraproteins (immunoglobulins); most commonly IgA or IgG

180
Q

What are the clinical features of multiple myeloma?

A

Bone destruction - increased osteoclastic activity causing lytic lesions and pathological fractures

Bone marrow infiltration - causing anaemia, infections and bleeding

Acute kidney injury - due to light chain deposits in tubules, hypercalcaemia and hyperuraemia.

Paraprotein aggregates in blood - hyperviscocity causing blurred vision and gangrene

181
Q

What investigations allow the diagnosis of multiple myeloma?

A

Two of three of the following must be present:

  1. Paraproteinaemia/ urinary Bence-Jones protein
  2. Radiological evidence of bony features
  3. Plasma cell proliferation on bone marrow aspirate
182
Q

How is multiple myeloma managed?

A

Good supportive care - including transfusion/erythropoietin, prompt infection treatment, treatment of acute kidney injury, analgesia, plasmapheresis for hyperviscocity

Chemotherapy and autologous stem cell transplant

183
Q

A patient preents with paraproteinaemia but has no other signs of myeloma. What is this called?

A

Monoclonal Gammopathy of Unknown Significance

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