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Year 4 > Medicine - Haematology > Flashcards

Medicine - Haematology Flashcards

1
Q

Define anaemia

A

Low RBC’s / reduced O2 carrying capacity of RBC’s -> leads to not enough O2 in the blood to meet the body’s physiological demands

Hb <130g/L (M) or <120g/L (F)

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2
Q

Define the aetiologies of anaemia

A
  • increased cell loss (bleeding, hypersplenism, haemolysis)
  • reduced/ineffective production (malignancy, myelodysplasia, folate/B12/ferritin deficiencies, renal failure (low EPO), ACD (high hepcidin))
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3
Q

What is the role of hepcidin?

A

Produced by the liver

Controls movement of iron from the gut -> blood

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4
Q

What are the two storage forms of iron?

A

Old RBC’s are eaten by macrophages in the RES and then the iron is stored as

1) ferritin (soluble)
2) haemosiderin (insoluble)

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5
Q

What are signs and symptoms of anaemia?

A 
SOB
palpitations
chest pain
pale
cravings
faint/dizzy
pale mucous membranes
koilonychia
tachynoea
oedema
angular stomatitis/glossitis
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6
Q

What investigations should be carried out to investigate anaemia?

A
  • FBC (Hb, RBC, WCC)
  • MCV
  • Haematinics (B12, plasma ferritin, plasma folate)
  • Iron studies: serum iron, Tf saturation, TIBC
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7
Q

Describe iron deficiency anaemia and its causes

A 
Hypochromic microcytic anaemia
Tf saturation <15%
Causes:
- dietary (low iron)
- blood loss (menorrhagia, bleeding)
- malabsorption (coeliac disease)
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8
Q

What might be seen on the blood film of a patient with iron deficiency anaemia?

A

Poikilocytes (abnormally shaped RBC’s can be due to low vit B12)

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9
Q

In a patient with iron deficiency anaemia, what abnormalities in the following blood tests might be seen?

  1. Serum iron
  2. Serum ferritin
  3. TIBC
  4. MCV
  5. MCH
A
  1. low
  2. low
  3. high
  4. low
  5. low
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10
Q

Define anaemia of chronic disease and its causes:

A

Normochromic, normocytic anaemia
Failure of iron utilisation, as it is trapped in the RES
? thought to be due to increased hepcidin (as this protein controls iron movement from the gut into the blood)

Causes:

  • infection
  • renal failure
  • malignancy
  • inflammatory conditions e.g. arthritis, IBD (the severity of the anaemia correlates with the severity of the disease)
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11
Q

What would a blood film in ACD show?

A

Rouleux (stacks of RBC’s)

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12
Q

Describe the following blood results in a patient with ACD:

  1. serum iron
  2. serum ferritin
  3. TIBC
A
  1. low
  2. high/normal
  3. low (bound to ferritin)
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13
Q

Describe the treatment of ACD

A

Treat the underlying cause (anaemia will not respond to iron replacement)

  • ?EPO
  • ?hepcidin inhibitors
  • .?transfusion (beware of iron overload)
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14
Q

Define sideroblastic anaemia, its causes and how it may present (in addition to normal anaemia symptoms)

A
  • Ineffective erythropoiesis
  • Sideroblasts produced
  • Causes increased Fe absorption and haemosiderin accumulation -> think of sideroblastic anaemia when microcytic anaemia is not responding to iron
    Causes:
  • congenital
  • acquired (chemotherpay, MPD)

Will present with organomegaly (XS RBC destruction) and organ failure as iron accumulates in heart, kidneys, and liver

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15
Q

What would the blood film and bone marrow look like in a patient with sideroblastic anaemia

A

Film: HYPOCHROMIC micro/normo/macrocytic anaemia

Bone marrow: sideroblasts will be seen (nucleated erythrocytes with iron granules)

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16
Q

What would the following blood results be like in a patient with sideroblastic anaemia?

  1. Serum iron
  2. Serum ferritin
  3. TIBC
A
  1. high
  2. high
    3.
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17
Q

How is sideroblastic anaemia treated?

A

Treat the cause:

  • alcohol cessation
  • pyridoxine replacement (vit B6)
  • transfusions
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18
Q

Describe megaloblastic anaemia and its causes

A
  • megaloblastic anaemias are a group of conditions which are due to folate/B12 deficiencies leading to a reduced no. RBC’s which are very large
  • delayed maturation of RBC nucleus relative to cytoplasm
    Causes: low B12/ferritin due to ->
  • deficiencies
  • pernicious anaemia (antibodies against IF)
  • coeliac disease (malabsorption)
  • gastritis (malabsorption)
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19
Q

What would BR and LDH measurements be like in megaloblastic anaemia?

A

High BR, high LDH

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20
Q

What important questions should you ask a patient with megaloblastic anaemia?

A 
DIET!
vegetarian
vegan
coeliac 
dietary deficiencies
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21
Q

What would the following blood results be in megaloblastic anaemia?

  1. MCV
  2. haematocrit
  3. reticulocytes
  4. B12/folate
  5. anti-parietal cell antibody
A
  1. high
  2. low
  3. low
  4. low
  5. may be present if pernicious anaemia
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22
Q

What would a blood film/smear of megaloblastic anaemia show?

A

Hypersegmented neutrophils

Megaloblastic (big) RBC’s

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23
Q

When treating megaloblastic anaemia, why should B12 be replaced before folate?

A

Can cause subacute combined degeneration of the spinal cord

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24
Q

Compare intra/extravascular haemolysis and their features

A

Intravascular -> in the circulation, raised free Hb in the plasma, may present with red/brown urine (haemoglobinuria)
Extravascular -> in the RES, causes splenomegaly

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25
Q

Describe hereditary spherocytosis

A

Congenital cause of haemolytic anaemia
AD
Defect on RBC membrane, causes the RBCs to be removed by the spleen leading to spherocytes remaining
S/Sx: gallstones, jaundice, pallor, splenomegaly
Bloods: Low Hb, high reticulocytes, high UCBR
Blood film: reticulocytes and spherocytes
Treatment: transfusions, folic acid supplementation, splenectomy, cholecystectomy

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26
Q

Describe warm and cold type autoimmune haemolytic anaemia and how they present/are investigated

A

Acquired types of haemolytic anaemia
Intravascular haemolysis
Warm -> IgG
Cold -> IgM
Can be idiopathic or caused by cold/infection/drugs
Antibody coats the RBC membrane and causes them to be destroyted
Produces spherocytes
Presents with: anaemia, high UCBR, reticulocytes, haemoglobinaemia/uria

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27
Q

Describe the treatment for warm and cold type autoimmune haemolytic anaemia

A

Warm:

  • steroids
  • transfusions
  • folic acid
  • splenectomy

Cold:

  • keep warm
  • Rituximab
  • Steroids
  • Splenectomy
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28
Q

Describe how the DAT (direct antiglobulin test)/direct Coombs test works

A

Detects antibody in vivo which is already coating RBC’s
Test by taking a sample of blood from the patient and adding anti-human immunoglobulin to the sample
If agglutination occurs this is a + test result

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29
Q

Describe how the indirect Coombs test works

A

Used pre-blood transfusion
Detects antibody in the donors serum which could attack the patients RBC’s
Test for by taking a sample of the recipients RBCs and adding some donors blood to it
A positive test result will cause agglutination

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30
Q

Describe isoimmune haemolytic anaemia

A

Antibodies are produced as a result of antigen from another person e.g. haemolytic disease of the newborn (e.g. mother Rh-, first baby Rh+, then the baby 1 sensitises the mother and means that baby 2 could be attacked during development)

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31
Q

Describe non-immune haemolytic anaemia

A

E.g. drug induced, which causes formation of RBC antibodies

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32
Q

Describe complications of splenectomy and what patients should be warned of

A

Complications:
- increased infections with encapsulated organisms (strep. pneumonia, haemophilus influenza, neisseria meningitidis)

Pts should be:
- on long term antibiotic prophylaxis (penicillin V), vaccinated (annual influenza and pneumococcal vaccines), and carry a splenectomy warning card

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33
Q

Define haemoglobinopathies

A

The term for a group of blood disorders affecting RBC’s

Mutations in the globin genes leads to production of structurally abnormal globin molecules

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34
Q

Describe thalassaemias

A

Underproduction of globin proteins due to gene mutations

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35
Q

What three types of haemoglobin are normally found in adults

A

HbA (2 x alpha and 2 x beta genes)
HbF (2 x alpha and 2 x gamma genes)
HbA2 (2 x alpha and 2 x delta genes)

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36
Q

What chromosomes are the alpha globin genes normally found on?

A

Chr 16

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37
Q

What chromosomes are the beta globin genes normally found on?

A

Chr 11

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38
Q

What are the three main categories of thalassaemias?

A

alpha gene thalassaeima
beta gene thalassaeima
sickle cell disease

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39
Q

Describe the types of alpha gene thalassaeima

A

1) loss of 1/2 genes = not associated with disease
2) loss of 3 alpha genes (HbH) = severe microcytic hypochromic anaemia and splenomegaly
3) loss of all alpha genes (hydrops fetalis) = not compatible with life, as these genes are essential to make fetal haemoglobin

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40
Q

Describe beta thalassaemia minor and major

A

Common in the meditarranean region

B thalassaemia major -> loss of both B genes, can make foetal Hb but not adult Hb, normal development until 6 months and then require blood transfusions

B thalassaemia minor -> loss of 1 B gene, not as serious as the major forms

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41
Q

Describe sickle cell anaemia

A

Amino acid substitution in the beta-globin gene
Valine -> glutamine
Results in abnormal haemoglobin production and HbS is made (2 x alpha and 2 x beta-sickle)
HbS is insoluble and forms crystals in the body, causing vaso-occlusion and RBC haemolysis

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42
Q

Define pancytopenia

A

Reduction in all major cell lines (RBC, WBC, plts)

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43
Q

Define agranulocytosis

A

Reduction in the production of granulocytes (neutrophils, basophils, eosinophils)

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44
Q

What are the causes of pancytopenia?

A

1) Reduced marrow production
- aplastic anaemia
- infiltration (cancer)
- megaloblastic anaemia
- myelofibrosis
- megaloblastic anaemia (lack of vit B12/folate)

2) Increased peripheral breakdown
- hypersplenism

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45
Q

What drug commonly causes agranulocytosis?

A

Carbimazole

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46
Q

Define multiple myeloma

A

Clonal proliferation of plasma cells in the bone marrow

Monoclonal proliferation of Ig (rather than the normal polyclonal)

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47
Q

What are the S/Sx of multiple myeloma?

A

Bone pain, pathological fractures
Hypercalcaemia (increased osteoclast activity)
Anaemia (low RBC)
Infections (neutropenia)
Fatigue
Renal impairment (deposition of immunological light chains in kidneys)

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48
Q

Describe the three key laboratory findings in myeloma

A

1) Paraprotein (monoclonal Ig in the blood, detected by electrophoresis)
2) Free light chains (Bence jones in the urine, or serum free kappa/lambda chains)
3) B2-microglobulin (this protein is raised in myeloma when the serum is measured, and it is a component of all MHC-I cells)

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49
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance (myeloma precursor)

50
Q

What investigations would you carry out for myeloma and what would they show?

A

Bloods:

  • FBC (normochromic normocytic anaemia)
  • U&E
  • Ca+
  • LFT
  • Bone profile
  • TFT
  • ESR/CRP

Film: Roleaux
Bone marrow
Serum/urine protein electrophoresis
Skeletal survey for bone involvement (whole body CT)

51
Q

What are the three criteria needed to diagnose myeloma?

A

1) Monoclonal plasma cells in the bone marrow >10%
2) Paraprotein in serum/urine
3) Evidence of organ damage (CRAB)
- > high calcium
- > renal insufficiency
- > anaemia
- > lytic bone lesions

52
Q

What are the complications of myeloma?

A 
Spinal cord compression
ESRF
Hypercalcaemia
Anaemia
Immunosuppression
53
Q

How is myeloma treated?

A

Supportive

  • analgesia
  • hydration
  • bisphosphonates
  • transfusions

Chemotherapy

  • thalidomide
  • cyclophosphamide
  • steroids
  • bortezomib (velcade)
  • bone marrow/stem cell transplant
54
Q

What part of the coagulation cascade does APTT measure?

A

Activated partial thromboplastin time

INTRINSIC

55
Q

What part of the coagulation cascade does PT measure?

A

Prothrombin time

EXTRINSIC

56
Q

What part of the coagulation cascade does TCT measure?

A

Thrombin clotting time

Measures the time take to convert fibrinogen -> fibrin (a measure of how much thrombin is available)

57
Q

What are the three classes of coagulation disorders and give an example of each:

A

1) vascular defects -> HSP, infection, steroids
2) platelet disorders -> ITP, TTP, DIC, HUS
3) coagulation cascade disorders -> VWD, haemophilia

58
Q

Describe haemophilia A

A

Factor 8 deficiency
High APTT
Treat by giving desmopressin (increases VIII) or recombinant factor 8

59
Q

Describe haemophilia B

A

Factor 9 deficiency
High APTT
Treat by giving recombinant factor 9

60
Q

Describe acquired haemophilia - type A

A

Life threatening where the body produces autoantibodies against factor 8
Presents as bruising, mucosal bleeding,
High APTT, high factor 8 autoantibodies, low factor 8 activity
Tx: steroids / IV-FEIBA

61
Q

Define immune thrombocytopenia (ITP) and its causes

A

Low platelet count <150, ooo
ITP, typically affects young women
Autoantibodies made against platelets causing them to be cleared faster than they are produced

62
Q

What are the S/Sx of ITP and how would you investigate it

A 
Easy bruising
Bleeding
Petechial rash
Blood in stool/urine
Epistaxis
Heavy periods

Bloods (low plts), blood films
?IDA (excessive bleeding)
Bone marrow (megakaryocytes)

63
Q

How is ITP treated?

A

Mild = no treatment
Steroids
Splenectomy
?Rituximab (attacks B cells which produce the autoantibodies)

64
Q

Describe DIC and its three key features

A

Disseminated intravascular coagulation
A consumptive coagulatopathy with widespread activation of the coagulation cascade
Features:
1) microthrombi/thrombus formation (causes organ failure and ischaemia)
2) microangiopathic haemolytic anaemia
3) bleeding (as clotting factors consumed)

65
Q

What causes DIC and how may it present?

A

Anything which causes clotting/inflammation in the body:
- sepsis, malignancy, pregnancy, trauma, toxins (snake bite), severe liver failure, pancreatitis, immunological (blood transfusion)

S/Sx: SOB, coma, bruising, bleeding, cyanosis, dusky feet, renal failure, oligouria, fever

66
Q

How is DIC investigated and what features may be seen on blood film?

A 
Clinical diagnosis + coag studies + blood film
High APTT/PT
Low fibrinogen
High D dimer
Low plt count
Film:
- spherocytes
- reticulocytes
- schistocytes
67
Q

How is DIC treated?

A

SUPPORTIVE

  • Platelet transfusions
  • FFP (contains coagulation factors)
  • Cryoprecipitate (contains fibrinogen)
68
Q

Describe VWD and the normal role of VWF

A

Von Willebrand disease = over 22 types
Normal role of VWD: brings platelets into contact with exposed endothelium, and makes platelets bind to each other, VWF bound to factor 8 in the circulation to stop it being destroyed

69
Q

What are the three most common types of VWD, how may it present and how is it investigated and treated?

A

1) low levels of VWF
2 abnormal VWF production
3) undetectable levels of VWF

Presents: bruising, epistaxis, heavy periods

Investigations: high APTT, low factor 8, low VWF, normal INR/plts

Treatment: avoid NSAIDs, desmopressin for minor bleeds (increases factor 8), replace VWF/F8 (especially before surgery)

70
Q

Describe TTP and its pathophysiology

A

Thrombotic thrombocytopenic purpura
Thrombosis with paradoxical thrombocytopenia
Extremely rare but F>M and ~40yr females affected

Antibodies made against ADAMST13 (the protein which normally fragments VWF in the circulation and prevents it circulation in large active polymers) therefore without working ADAMST13 the VWF is constantly active and binds coagulation factors and platelets causing inappropriate clot formation

Causes: SLE, cancer, pregnancy, HIV, drugs

71
Q

How does TTP present, how is it investigated and treated?

A

Presents: fever and confusion
Investigations: normal coag screen, raised/normal D dimer, blood film shows MAHA (microangiopathic haemolytic anaemia) -> schistocytes, spherocytes, reticulocytes
Treatment: plasma exchange, steroids, aspirin, vincristine

72
Q

What is HUS and describe its basic pathophysiology, presentation, investigations and treatment

A

Haemolytic uraemic syndrome
Caused by infection with a STEC organism (Shigella-Toxin producing E.Coli)
The STEC organism produces shigatoxins which bind to renal and GI endothelium causing cellular necrosis and apoptosis
Usually presents in children <5yrs with triad of:
- haemolytic anaemia
- thrombocytopenia
- renal failure
Presents: severe blood diarrhoea, low Hb, high WCC, low plts, high BR (haemolysis), abnormal U&E/eGFR
Treatment: dialysis, rehydration, replace electrolytes and maintain BP

73
Q

What is HIT and describe its basic pathophysiology, presentation, investigations and treatment

A

Heparin induced thrombocytopenia
A reaction which can occur to any heparin, but normally unfractionated heparin
A drop in platelets following heparin administration
Presents with low platelet count, new thrombosis/worsening of an existing thrombus
Tx: stop heparin, start alternative anticoagulant e.g. fondaparinux

74
Q

Define thrombophilia and its causes

A

An inherited/acquired coagulopathy which predisposes to THROMBUS FORMATION
Usually venous thrombi (DVT/PEs)
Causes: inherited or acquired
Inherited - APC resistant, prothrombin gene mutation, protein /S deficiency, antithrombin deficiency
Acquired - antiphospholipid syndrome, OCP/HTI, PRV

75
Q

Describe risk factors for arterial thrombosis

A

smoking
HTN
DM
hyperlipidaemia

76
Q

Describe risk factors for venous thrombosis

A 
Surgery
Trauma
Immobility
Age
Obesity
Varicose veins
77
Q

How is thrombophilia treated?

A

Acute anticoagulation in thrombosis

Consider lifelong anticoagulation

78
Q

Define MPN

A 
Myeloproliferative neoplasms
Bone marrow stem cell disorders resulting in clonal proliferation of a particular HSC 
4 types:
1) PRV
2) ET
3) Myelofibrosis
4) CML
79
Q

What category of patients may present with a raised EPO and a false PRV like picture?

A

Smokers (increased EPO production)

80
Q

Describe absolute vs relative PRV and its causes

A

Polycythaemia rubra vera
Relative: haematocrit is falsely high due to dehydration -> sepsis, burns, dehydration, enteropathy
Absolute: primary JAK2+ mutation (90%), secondary: smoking, high altitude, lung disease, HF, malignancy

81
Q

What are the S/Sx of PRV

A

Signs:
- HTN, splenomegaly, erythromelalgia, gouty tophi, DVTs
Symptoms: aquagenic pruritis, erythromelalgia, TIA/MI/stroke, dizzy, weight loss, GORD (high histamine and this increases stomach acid production!)

82
Q

How is PRV investigated and treated?

A 
Bloods: 
- high Hb, haematocrit, RBCs
- may also have high WCC/plts
- low EPO (- feedback)
Genetic testing: JAK2+
Treatment:
- aspirin and venesection, may need chemotherapy/pegylated interferon if severe
83
Q

What is ET and its causes?

A

Essential thrombocythaemia (high platelets due to megakaryocyte over-proliferation)
Causes can be essential or reactive
Essential: JAK2 mutation (50%), PRV, CML, myelofibrosis
Reactive: haemorrhage, malignancy, post-splenectomy, low haematinics (leads to megaloblastic anaemia and reduced RBC’s -> so an increase in the other cell line)

84
Q

What are the S/Sx of ET?

A 
THROMBOTIC RISK!
TIA/MI/stoke
Dizzy/headaches
DVT
HTN
Splenomegaly
Erythromelalgia
Easy bruising
85
Q

How is ET investigated and treated?

A 
Bloods: high plts, LDH, urea
Film: megakaryocytes (large with staghorn nuclei)
Genetic testing - JAK2 or CALR mutation
Treatment:
- aspirin
- hydroxycarbamide
- optimise CV risk factors
86
Q

Describe myelofibrosis and its causes

A
  • fibrosis of the bone marrow
  • associated with haematopoiesis in the liver and spleen
    PRIMARY - JAK2+ mutation
    SECONDARY - ET, PRV, myeloma, sarcoid, cancers
87
Q

What are the S/Sx of myelofibrosis

A 
lethargy
weight loss
night sweats
hepato/splenomegaly (extramedullary haematopoiesis)
anaemia
fever
bone pain
pancytopenia -> infections
bleeding
bruising
88
Q

What are the investigations and treatment for myelofibrosis?

A

Investigations:

  • JAK testing
  • blood film -> poikilocytes (tear drop shaped cells)
  • bone marrow -> hypercellular and fibrotic
  • bloods (high urea and LDH due to cell turnover)

Treatment:
- control symptoms: anaemia (transfusions) and splenomegaly (chemotherapy/splenectomy)

89
Q

Describe MDS and its features

A

Myelodysplastic syndromes
Type of bone marrow failure -> issues with haematopoietic stem cells leading to dysplastic changes in 1+ cell lines, results in CYTOPENIAS
Usually in elderly male smokers, but can be secondary to chemoradiotherapy
Fts: pancytopenia, bone marrow hypercellular and dysplastic, XS blast cells in bone marrow (but less than 20%)

90
Q

What are the differentials of MDS

A 
alcoholism
megaloblastic anaemia
drug side effects
HIV/viruses
copper deficiency (wilson's disease)
91
Q

How is MDS managed?

A

Low risk:
transfusions
thrombo/erythropoietin

High risk:
chemotherapy
SC/BM transplant

92
Q

Define lymphoma and its incidence peaks

A

Think solid lumps and bumps
Monoclonal proliferation of lymphoid stem cells creating malignant tumours
Divided into HL and NHL
2 peaks of incidence = 20s and 60s

93
Q

Name some risk factors for the development of lymphoma

A

EBV
H.pylori/coeliac (increased risk of MALT)
Older age
Immunosuppression
Autoimmune diseases (Sjrogren’s increases risk of salivary malignancies)

94
Q

Describe HL (Hodgin’s lymphoma) and how it normally presents

A 
Usually always nodal
40% EBV +ve
peripheral nodes affected, predictable course of spread
More commonly presents with B symptoms
Painless lymphadenopathy
Organomegaly
Anaemia, SOB, infections
REED-STERNBERG CELLS on blood film!
95
Q

Describe NHL (Hodgin’s lymphoma) and how it normally presents

A

50% nodal vs extranodal
deep LNs affected with unpredictable course
Divided into high and low grades
High grade examples - DLBCL, Burkitts (t8:14)
Low grade examples - follicular (t14:18), mantle cell (t11:14)

96
Q

What investigations should be carried out for lymphoma

A 
Bloods (low Hb and plts) -> WCC will be always high in NHL but vary in HL
LN biopsy
Bone marrow (aspirate and trephine)
97
Q

Describe the Ann Arbour staging for lymphoma

A

1) one LN group affected
2) 1+ LN groups/LN group + organ affected on same side of diaphragm
3) multiple LN groups/organs affected on different sides of the diaphragm
4) same as 3 + involvement of the liver

+ A/B depending on presence of B symptoms

98
Q

What is the classical chemotherapy regimen for lymphoma?

A 
R-CHOP
rituximab
cyclophosphamide
adriamycin
vincristine
prednisolone
99
Q

Compare autologous and allogenic stem cell transplants

A

Autologous - uses patients own cells

Allogenic - from a donor (usually a sibling)

100
Q

What is the commonest adult acute leukaemia?

A

AML

101
Q

Define AML

A

Malignancy of HSC resulting in accumulation of immature blast cells in the bone marrow +/- peripheral blood
A malignancy of myeloid derived cells and the median age is 70yrs
>20% blast cells in the bone marrow

102
Q

What is the most commonly mutated gene in AML?

A

FLT3

103
Q

Describe APML

A

Subtype of AML
t15:17 = PML (15)-RARa(17) gene fusion
On blood film you can see promyelocytes containing Auer rods
Causes procoagulopathy and DIC
Treat with ATRA (all-trans retinoic acid)

104
Q

Describe CML and it’s three phases

A

Proliferation of mature granulocytes and their precursors
Common 40-60yrs
Associated with the philadelphia chromosome (BCR-122, ABL-9)

1) Chronic = 3-5yrs, asymptomatic
2) Accelerated = 12-18 months, tired, weight loss and splenomegaly
3) Blast = 3-6 months, behaves like acute leukaemia

105
Q

What are the signs and symptoms of CML?

A

20-50% asymptomatic
Raised WCC and platelets
B symptoms
Splenomegaly

106
Q

What are the three treatment responses you aim for when treating CML?

A

1) Haematological response: aim to normalise blood counts
2) Cytogenetic response: no Ph+ chromosomes detectable
3) Molecular response: no BCR-ABL detectable

107
Q

What is the revolutionary treatment drug for CML?

A

TKI’s e.g. imatinib!

108
Q

What is the commonest childhood haematological cancer?

A

ALL

109
Q

Describe ALL

A

Rare in adults, common in children
Malignancy of B/T lymphocytes
t4:11

110
Q

How does ALL present?

A

Tired, pale, bruised limping child
Pancytopenia (anaemia, infection, bleeding)
Purpuric rash (due to low platelets)
Bone/joint pain
CNS features (headache and vomiting as the blast cells invade)

111
Q

What is the most common adult leukaemia?

A

CLL

112
Q

Describe CLL and its pathophysiology

A

Clonal expansion of lymphocytes (2M:1F)
Median age ~72yrs
B-lymphocytes build up in the bone marrow and blood

113
Q

How does CLL present and how is it investigated?

A 
High WCC
Anaemia
Painless lymphadenopathy
Infection
Splenomegaly

FBC -> high WCC, low RBC
Bone marrow -> increased B cells
Blood film -> smudge/smear cells (these are immature B cells which burst when the film is being smeared)
Blood -> may have a strawberry pink hue as it contains so many WBCs

114
Q

What staging system is used for CLL and describe it

A

Binet staging: predicts prognosis

  • A = <3 groups of enlarged LN’s + high WCC
  • B = >3 groups of enlarged LN’s + high WCC
  • C = enlarged LN’s/spleen + high WCC PLUS low RBC/plts
115
Q

List causes of microcytic anaemias

A

TAILS:

  • thalassaemia
  • Anaemia of chronic disease
  • IDA
  • Lead poisoning
  • Sideroblastic anaemia
116
Q

List causes of normocytic anaemia

A

HHAAA:

  • haemolytic anaemia
  • hypothyroidism
  • acute blood loss
  • anaplastic anaemia
  • anaemia of chronic disease
117
Q

List causes of macrocytic anaemia

A

Divided into megaloblastic or normoblastic

Megaloblastic = due to impaired DNA synthesis (prevents cell from dividing), Caused by B12/folate deficiencies!!!

Normoblastic causes:
Alcohol 
Reticulocytosis  
Hypothyroidism 
Liver disease 
Medications (azathioprine)
118
Q

What is the formula used to calculate TF saturation?

A

TF saturation = serum iron/TIBC

119
Q

List the 5 congenital types of haemolytic anaemia

A
  1. Hereditary spherocytosis
  2. Hereditary elliptocytosis
  3. Thalassaemia
  4. Sickle cell anaemia
  5. G6PD deficiency
120
Q

Describe G6PD deficiency

A

Defect in the RBC enzyme called G6PD
Common in africa/mediteranean
X-linked recessive (usually only affects males)

Causes crises triggered by fava beans, infection and medications 
Drugs which can trigger this: 
Quinines (anti-malarials) 
Ciprofloxacin 
Sulphasalizine
121
Q

List the 5 types of acquired haemolytic anaemias

A
  1. Autoimmune haemolytic anaemia -> tested with Coomb’s test!
  2. Alloimmune haemolytic anaemia (transfusion reactions and HDN (haemolytic disease of the newborn))
    (Either foreign RBC’s floating about in the pt (transfusions), OR foreign antibodies in the pt’s blood which attack their own RBC’s (HDN)
  3. Paroxysmal nocturnal haemoglobinuria
    (Rare! Genetic mutation in haematopoietic stem cells occurs at some point in the pts life. The RBC’s lose surface proteins which triggers the complement cascade and causes RBC destruction. Characteristically presents with red urine in the morning! Pts also pre-disposed to thrombosis!)
  4. Microangiopathic haemolytic anaemia (MAHA) - Small blood vessels have structural abnormalities (e.g. mesh) that causes haemolysis as RBC pass through
    Normally seen in HUS, DIC, TTP, SLE, or cancer
  5. Prosthetic valve related haemolysis
    - Caused by turbulence around heart valves and collision of RBC’s with the implanted valves
122
Q

List 4 differentials for abnormal bleeding (XS bleeding) in haematology

A
  1. ITP (lack of plts)
  2. DIC
  3. Haemophilias (lack of coag factors 8/9)
  4. VWD

Year 4 (28 decks)

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