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Haematology Flashcards

1
Q

What are the symptoms of anaemia?

A 
Fatigue
Dyspnoea
Dizziness
Palpitations
Headache
Tinnitus
Anorexia
Angina (if pre-existing coronary disease)
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2
Q

Signs of anaemia?

A

Pallor (+ of conjunctiva)
Tachycardia
Flow murmurs (ejection systolic over apex)
Rare: retinal haemorrhage, cardiac enlargement, heart failure

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3
Q

Causes of microcytic anaemia?

A 
Iron deficiency
Thalassaemia
Sideroblastic
Anaemia of chronic disease (usually normocytic)
Lead poisoning (rare)
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4
Q

Causes of normocytic anaemia?

A 
Chronic disease
Acute blood loss
Bone marrow failure
Renal failure (erythropoietin)
Hypothyroid
Haemolysis
Pregnancy
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5
Q

In a patient with normocytic anaemia who also has low WCC and low platelets, what diagnosis would you suspect?

A

Marrow failure

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6
Q

Causes of macrocytic anaemia?

A 
B12/folate deficiency
Alcohol excess/liver disease
Reticulocytosis (e.g. with haemolysis)
Cytotoxics, e.g. hydroxycarbamide
Myeloma
Marrow infiltration
Hypothyroid
Antifolate drugs, e.g. phenytoin
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7
Q

Causes of iron deficiency anaemia?

A

Blood loss (menorrhagia, GI)
Poor diet
Malabsorption (coeliac)
Hookworm

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8
Q

Signs of iron deficiency anaemia?

A

Koilonychia (spoon-shaped nails)
Atrophic glossitis
Angular stomatitis

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9
Q

Tests in iron deficiency?

A 
FBC
Haematinics
Blood film (target cells, poikilocytes)
Coeliac serology
Gastroscopy + colonoscopy
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10
Q

Treatment for iron deficiency anaemia?

A

Treat the cause
Ferrous sulphate - continue until Hb normal + another 3 months to replenish stores
(then IV iron)

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11
Q

Side effects of ferrous sulphate?

A

Nausea
Abdominal discomfort
Constipation/diarrhoea
Black stools

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12
Q

Pathophysiology behind anaemia of chronic disease?

A

Poor use of iron in erythropoiesis
Cytokines shorten RBC survival
Reduced EPO production + response to it

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13
Q

List some examples of causes of anaemia of chronic disease

A 
Malignancy
RA
Chronic infection
Vasculitis
Renal failure
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14
Q

Management of anaemia of chronic disease?

A

Treat underlying cause

EPO

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15
Q

What is sideroblastic anaemia?

A

Ineffective erythropoiesis and iron loading in the marrow

*think of sideroblastic anaemia whenever microcytic anaemia does not respond to iron

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16
Q

What is haemosiderosis in sideroblastic anaemia?

A

Endocrine, liver and heart damage due to iron deposition

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17
Q

Causes of sideroblastic anaemia?

A 
Congenital (delta-aminolevulinate synthase 2 deficiency)
Idiopathic
Chemo
Anti-TB drugs
Irradiation
Alcohol excess
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18
Q

What test results would be seen in sideroblastic anaemia?

A 
Low Hb
Low MCV
High ferritin
Hypochromic blood film
Sideroblasts in marrow biopsy
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19
Q

Treatment of sideroblastic anaemia?

A

Remove cause
Transfusion
Pyridoxine

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20
Q

Tests in macrocytic anaemia?

A

FBC, haematinics, LFT, TFT
Blood film
Bone marrow biopsy

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21
Q

In what foods is folate found?

A

Liver
Green vegetables
Nuts
Yeast

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22
Q

Maternal folate deficiency causes what problems in the foetus?

A

Neural tube defects

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23
Q

List some causes of folate deficiency

A

Poor diet (elderly, poverty, alcoholics)

Increased demand, e.g. pregnant, increased cell turnover (CA, haemolysis, inflammatory disease, renal dialysis)

Malabsorption (coeliac)

Alcohol

Drugs (anti epileptics, methotrexate, trimethoprim)

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24
Q

Management of folate deficiency?

A

Assess for poor diet
Coeliac serology

Folic acid + B12

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25
Q

Causes of glossitis?

A 
B12 deficiency
Iron deficiency
Contact dermatitis/ food allergy
Crohns, coeliac
Drugs
Alcoholism
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26
Q

Causes of B12 deficiency?

A

Diet (vegan, elderly)
Malabsorption (PA, crohns, resection, pancreatic insufficiency)
Chronic biguanide (metformin) use

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27
Q

Food sources of B12?

A

Meat
Fish
Dairy products

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28
Q

How does absorption of B12 occur?

A

Intrinsic factor from parietal cells in the stomach bind to B12, allowing it to be absorbed in the terminal ileum

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29
Q

What is the alternative name for B12?

A

Cobalamin

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30
Q

What are the clinical features of B12 deficiency?

A

Lemon tinged skin (pallor + jaundice)
Glossitis
Anaemia Sx
Psych: irritable, dementia, depression, psychosis
Parasthesia, peripheral neuropathy
Subacute combined degeneration of the spinal cord

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31
Q

What is the classical triad of subacute combined degeneration of the spinal cord?

A

Extensor plantars
Absent knee jerks
Absent ankle jerks

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32
Q

What is the pathology behind pernicious anaemia?

A

Autoimmune atrophic gastritis
Leading to reduced secretion of intrinsic factor from parietal cells
Unbound B12 therefore not absorbed

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33
Q

What other diseases may be associated with pernicious anaemia?

A

Autoimmune: thyroid, addison’s, vitiligo, hypoparathyroidism

Gastric carcinoma

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34
Q

Investigations in pernicious anaemia?

A 
FBC (Hb, MCV, wcc, platelets)
Haematinics
Parietal cell autoantibodies
Intrinsic factor antibodies
Blood film (hypersegmented neutrophils)
Marrow
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35
Q

Management of B12 deficiency?

A

Treat cause

B12 IM/ PO

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36
Q

Why is there a marked continuing high MCV after initiating B12 treatment for deficiency?

A

Reticulocytosis

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37
Q

Normal lifespan of a RBC?

A

120 days

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38
Q

Haemolysis can occur intravascularly or extravascularly. Explain extravascular haemolysis.

A

Macrophages in liver, spleen, bone marrow (reticuloendothelial system)

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39
Q

Give some investigation findings that would support increased red cell breakdown

A

Raised bilirubin
Low Hb, normal/high MCV
High urinary urobilinogen
High serum LDH (released from red cells)

40
Q

What blood test finding would suggest increased red cell production?

A

High MCV (reticulocytosis)

41
Q

What examination finding might suggest extravascular haemolysis?

A

Splenomegaly

42
Q

Give some important aspects of the history in suspected haemolytic anaemia?

A 
FHx
Previous anaemia
Ethnicity
Dark urine
Drugs
Travel
43
Q

Important aspects of the examination in suspected haemolytic anaemia?

A

Jaundice
Hepatosplenomegaly
Gallstones (^bili)
Leg ulcers

44
Q

Investigations in haemolytic anaemia?

A 
FBC
LFT (bili)
LDH
Urinary urobilinogen
Haptoglobin
Blood film

If travel Hx - malaria thick + thin films

Extras:
osmotic fragility testing
direct coombs test
Hb electrophoresis

45
Q

What does coombs test identify?

A

Immune causes of haemolytic anaemia

Identifies RBCs coated with antibody

46
Q

Acquired causes of haemolytic anaemia?

A 
Infection
Autoimmune
Malignancy CLL, lymphoma
TTP, HUS
Drugs
DIC
Transfusion
Hep B+C
Vaccinations
Pre-eclampsia
47
Q

Hereditary causes of haemolytic anaemia?

A

Enzyme defect: G6PD deficiency, PKD

Membrane defect: hereditary spherocytosis

Haemoglobinopathy: sickle cell, thalassaemia

48
Q

How is sickle cell inherited?

A

Autosomal recessive

49
Q

Pathophysiology of sickle cell?

A

Abnormal Hb results in deformed RBCs (sickle cells)

These haemolyse readily -> Vaso-occlusive crisis

50
Q

Investigation findings in sickle cell?

A

FBC: low Hb, ^reticulocytes/MCV
Blood film: sickle cells + target cells
LFT: ^bili

51
Q

Triggers for vaso-occlusive ‘painful’ crisis in sickle cell?

A

Cold
Hypoxia
Dehydration
Infection

52
Q

Presentation of vaso-occlusive ‘painful’ crisis in sickle cell?

A 
Severe pain
Dactylitis in <3 yr olds
Acute abdomen (mesenteric ischaemia)
Stroke, seizure, cognitive defects
Avascular necrosis (eg femoral head)
Leg ulcers
Priapism
53
Q

What is a sequestration crisis in sickle cell?

Management?

A

Pooling of blood in spleen + liver > organomegaly, shock, severe anaemia

Transfusion

54
Q

What is an aplastic crisis in sickle cell?

A

Parvovirus B19 infection leads to sudden reduction in marrow production of RBCs

55
Q

Complications of sickle cell?

A 
Splenic infarction
Poor growth
CKD
Gallstones
Retinal disease
Iron overload
Lung hypoxia -> Fibrosis -> Pulm HTN
56
Q

Management of chronic sickle cell?

A 
Analgesia for crises
Hydroxycarbamide
Abx prophylaxis + immunisations (splenic infarct)
Transfusions
Bone marrow transplant
57
Q

Management of sickle cell crisis?

A 
Analgesia
Septic screen
Fluids
O2
Abx if pyrexial
Transfusion/ exchange transfusion
58
Q

Sx of acute chest syndrome in sickle cell?

A 
Wheeze
Dyspnoea
Pain
Fever
Cough
59
Q

What causes the infiltrates in acute chest syndrome in sickle cell?

A

Infection

Fat embolism from bone marrow

60
Q

Management of acute chest syndrome in sickle cell?

A 
O2
Analgesia
Abx (cephalosporin + macrolide)
Bronchodilators in wheeze
Transfusion/ exchange
61
Q

Examples of macrolide antibiotics?

A

Azithromycin
Clarithromycin
Erythromycin

62
Q

Examples of cephalosporin antibiotics?

A

Cefuroxime

Ceftriaxone

63
Q

What is thalassaemia?

A

Reduced production of one globin chain

Leads to haemolysis

64
Q

Where in the world is thalassaemia common?

A

Med to far East

65
Q

Ix in beta thalassaemia?

A 
FBC
Haematinics
Film
Hb electrophoresis
MRI (for myocardial siderosis)
66
Q

Clinical features of patient with beta thalassaemia?

A 
Presents in 1st year of life
Severe anaemia
Failure to thrive
Hepatosplenomegaly
Skull deformity
Osteopenia
67
Q

Management of beta thalassaemia?

A

Life-long regular transfusions
Folate
Iron chelators (deferipone + desferrioxamine)
Ascorbic acid -> iron excretion
Splenectomy
Management of endocrine complications (DM, thyroid)
Marrow transplant

68
Q

Side effect / risk of regular transfusions in beta thalassaemia, and the consequences of this?

A

Iron overload
Endocrine: pituitary, thyroid, pancreatic failure (DM)
Liver disease
Cardiac toxicity

69
Q

What are the 3 processes that halt bleeding after injury? (+ therefore the 3 types of bleeding disorder)

A

Vasoconstriction
Platelet plugging
Coagulation cascade

70
Q

Bleeding disorders. Platelet/Vascular vs Coagulation disorder. What is the pattern of bleeding in platelet/vascular disorders?

A
  1. Prolonged bleeding from cuts
  2. Bleeding into skin (bruising, purpura)
  3. Mucous membranes (epistaxis, gums, menorrhagia)
71
Q

What is the bioactive form of folate?

A

THF (tetrahydrofolate)

72
Q

Why does a B12 deficiency cause a folate deficiency?

A

Methionine synthase (B12-dependent enzyme) is required for the formation of the bioactive form of folate, tetrahydrofolate (THF)

73
Q

Which tends to be more severe: extravascular, or intravascular haemolysis?

A

Intravascular

74
Q

What is the difference between intravascular and extravascular haemolysis?

A

Extravascular: ^RBC breakdown, e.g. by macrophages

Intravascular: destruction of RBCs in circulatory system

75
Q

What is multiple myeloma?

A

Cancer of bone marrow plasma cells (differentiated B lymphocytes)

76
Q

What is the peak incidence for multiple myeloma?

A

60-70 years

77
Q

What are the clinical features of multiple myeloma?

A 
CRAB
HyperCalcaemia
Renal impairment
Anaemia
Bone lesions/disease

Others: bone disease, lethargy, infection + progressive bone marrow failure, paraprotein production, amyloidosis, macroglossia, CTS, neuropathy, hyperviscosity

78
Q

What are the SYMPTOMS of myeloma that patients present with?

A

Bone disease:
Backache, pathological fractures (bone ribs), vertebral collapse

Hypercalcaemia:
Moans, groans, bones etc

Frequent infections

Symptoms of anaemia (fatigue, pale, SOB)

79
Q

What investigation should be carried out for all patients over 50 with back pain?

A

Serum protein electrophoresis and ESR

80
Q

What Ix should be done for multiple myeloma?

A

FBC (anaemia)

Serum monoclonal proteins (usually IgG/IgA)

Urine proteins (Bence Jones proteins)

BMBx (bone marrow biopsy - increased plasma cells

Blood film (roleaux formation - stacked RBCs)

Imaging (XR - lytic lesions - rain-drop skull)

81
Q

What is the term given to the lytic lesions seen on X-ray in multiple myeloma?

A

‘Rain-drop skull’

82
Q

In what condition is ‘pepper-pot skull’ seen?

A

Hyperparathyroidism

83
Q

What conditions are urinary Bence Jones proteins seen in?

A

Multiple myeloma

Waldenstrom’s macroglobulinaemia

84
Q

What are the diagnostic criteria for multiple myeloma?

A

One major and one minor OR 3 minor criteria

85
Q

What are the major criteria for diagnosis of multiple myeloma?

A

Plasmacytoma

30% plasma cells in bone marrow sample

^M protein in blood/urine

86
Q

What are the minor criteria for multiple myeloma diagnosis?

A

10-30% plasma cells in bone marrow sample

Minor ^M protein in blood/urine

Osteolytic lesions

Low levels of antibodies (not from cancer cells) in blood

87
Q

Give 4 poor prognostic features of multiple myeloma

A

> 2 osteolytic lesions

Beta-2 macroglobulin >5.5mg/L

Hb <11g/L

Albumin <30g/L

88
Q

What are the principles of management of myeloma?

A

Only treat symptomatic patients

For asymptotic MM and MGUS - regular monitoring

89
Q

How is symptomatic myeloma managed?

A

If candidate for stem cell transplant - induction treatment followed by stem cell transplant

If not - intensive drug treatment

90
Q

What drug treatments may be used in the management of multiple myeloma?

A

Thalidomide or bortezomib based chemo- regimes, alongside dexamethasone

Supportive treatment alongside chemo: analgesia, bisphosphonates, blood transfusions/EPO to treat anaemia

91
Q

What is monoclonal gammopathy of undetermined significance?

A

Asymptomatic premalignant plasma cell proliferative disorder (M protein <3/dL)

92
Q

Give 2 risk factors for MGUS

A

Male

Agent Orange exposure

93
Q

What will be seen on bone marrow biopsy in a patient with MGUS?

A

Plasma cells <10%

94
Q

What is lymphoma?

A

Malignant proliferation of lymphocytes

These accumulate in lymph nodes and cause lymphadenopathy (also found in peripheral blood/other organs, e.g. liver, spleen, bone marrow)

95
Q

What are the causes of lymphoma?

A

Most cases unknown

Infection (EBV, HTLV-1, H pylori)

Primary immunodeficiency (ataxia telangiectasia, Wiscott-Aldrich syndrome)

Secondary immunodeficiency (HIV, transplant patients)

Autoimmune disorders

96
Q

What are the symptoms of Hodgkin’s lymphoma?

A

Enlarged, painless, non-tender, ‘rubbery’ superficial lymph nodes. Asymmetrical.

Constitutional ‘B’ symptoms

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