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Clinical > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the pathology behind transfusion-associated graft vs host disease? Who is at an increased risk?

A

T-cells in blood products attack the recipient’s tissues.
Increased risk pts include immunosuppressed or previous Hodgkin’s lymphoma.

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2
Q

What is Transfusion associated circulatory overload (TACO)?

A

Pulmonary oedema due to circulatory excess, occurs in those who cannot cope with the excess fluid after blood transfusion (elderly, young, heart failure).

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3
Q

How can you mitigate the risk of transfusion associated circulatory overload?

A

Prophylactic furosemide to those at higher risk (elderly, children, heart failure). Also ensure to only give them 1 unit at a time.

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4
Q

What is transfusion associated lung injury (TRALI)?

A

Non-cardiogenic pulmonary oedema and ARDS as a result in activation of immune cells in the lungs.

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5
Q

Which factors are assessed by the APTTR and the INR?

A

APTTR: 8, 9, 11,12
INR: 10, 9, 7, 2

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6
Q

What is the ISTH BAT score?

A

ISTH ‘bleeding assessment score’ for assessing the severity of potential bleeding disorders.
>3 men and >5 women is abnormal.

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7
Q

What is a mixing study?

A

Mixing pt plasma with normal plasma (50:50) to see if a problem is corrected.
If it is just a deficiency it will completely correct the problem.
If it is autoimmune due to autoantibody destruction then it won’t correct as the antibodies would destroy it in normal blood too.

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8
Q

What is DIC? What are the lab findings?

A

Loads of small clots throughout the body meaning clotting factors are used up and the pt is at a significantly increased risk of bleeding.
Secondary to infection, injury, and malignancy.

Labs: elevated D-dimer, increased APTT and PT, low platelets and fibrinogen.

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9
Q

What is HUS? What is it most commonly caused by?

A

Haemolytic uremic syndrome: haemolytic anaemia (schistocytes), thrombocytopenia (bleeding/bruising), AKI.
Typically caused by diarrhoeal/foodborne illness (E.coli 0157) when they break down and release toxins.

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10
Q

What is Atypical HUS?

A

Caused by complement deficiencies rather than infection.

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11
Q

What causes intravascular haemolysis?

A

Something within the vessels causing RBCs to become shredded/fragmented (schistocytes). E.g., mechanical heart valve and the fibrin clots in DIC.

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12
Q

What blood results might indicate an intravascular haemolysis?

A

Schistocytes and reticulocytes on blood film.
Raised bilirubin and LDH, haemoglobinuria (due to free Hb in urine), decreased haptoglobin.

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13
Q

How do you manage a febrile non-haemolytic transfusion reaction?

A

Stop transfusion, give paracetamol (anti-pyretic), and recommence at a slower rate once stable.

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14
Q

What are the signs of essential thrombocytosis? What is it caused by?

A

Dizziness, bleeding (including melena), thrombocytosis, raised platelets, headaches, splenomegaly, erythromelalgia (burning, red, hot skin), livedo reticularis (nettle reddish blue pattern on skin).
Caused by JAK2 V617F mutation.

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15
Q

What is the inheritance pattern of sickle cell anaemia? How can it be diagnosed?

A
  • Autosomal recessive
  • A-haemoglobin electrophoresis shows sickling of cells and genetic testing will identify allele
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16
Q

What are the 4 main Crises that someone with sickle cell may present with?

A
  • Aplastic crisis: profound anaemia, likely shows pancytopenia, caused by infections. present with tachycardia and tachypnoea in absence of splenomegaly.
  • Acute sequestration crisis: excessive blood trapped in spleen causes reduction in circulating volume.
  • Vaso-occlusive crisis: microvascular obstruction due to sickling, inflammation, and vasoconstriction. Cold, dehydrated, and after exercise trigger this. Very painful.
  • Chest crisis: presents with tachypnoea, wheeze, and cough, with hypoxia and pulmonary infiltrates on chest X-ray. Unknown cause.
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17
Q

Why do sickle cell pts experience hyposplenism? What is the consequence of this?

A

Repeated splenic infarctions which can cause reduced splenic function.
Increased susceptibility to encapsulated organisms ie, pseudomonas aeruginosa, haemophilus influenza, strep pneumoniae, etc.

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18
Q

A sickle cell pt with >3 painful crisis in 12 months may indicate the need for what medication? How does it work?

A

Hydroxyurea - increases foetal Hb (has no beta subunit so not susceptible to sickling).

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19
Q

What is the inheritance pattern for Hereditary Haemorrhagic Telangiectasia (Osler-Weber-Rendu syndrome)? What are the complications?

A

Autosomal dominant ENG and ACVRL1 genes.
Recurrent epistaxis (malformations in nasal mucosa), widespread telangiectasia, AVM malformations.

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20
Q

What is the most common hereditary thrombophilia? What is its inheritance pattern?

A

Factor V Leiden mutation.
Autosomal dominant.

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21
Q

Why does a Factor V Leiden mutation increase the risk of thromboembolic disease?

A

Factor V is resistant to degradation by Protein C, increasing thrombin formation and creating a prothrombotic state.

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22
Q

What is the inheritance pattern for G6PD deficiency? What is it?

A

X-linked.
G6PD (glucose-6 phosphate dehydrogenase) deficiency is an enzyme deficiency increasing susceptibility to the development of haemolytic anaemia.

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23
Q

What are the common triggers of haemolysis in G6PD deficiency pts?

A

Medications- sulphonylureas, ciprofloxacin, nitrofurantoin.

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24
Q

How might someone in a haemolytic crisis with a background of G6PD deficiency present? What might you see on blood film?

A

SOB, exhaustion, recent hx of starting a new medication, dark urine, scleral ictus,
Blood smears showing RBC fragments (schistocytes), bite cells (misshaped RBCs) and heinz bodies (denatured Hb).

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25
Q

What is the inheritance pattern for congenital sideroblastic anaemia? What is it?

A

X-lined recessive (predominantly affecting males)
Defect in haem synthesis .

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26
Q

What type of anaemia is seen in congenital sideroblastic anaemia?

A

Microcytic anaemia but normal iron levels.
Inefficient erythropoiesis means the iron isn’t used and becomes deposited in mitochondria and various organs.

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27
Q

What is seen on bone marrow aspiration for congenital sideroblastic anaemia? What do you see on blood film?

A

BM - Ring sideroblasts (abnormal RBCs).
BF - pappenheimer bodies (basophilic granules of iron).

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28
Q

How may congenital sideroblastic anaemia present?

A

Fatigue, dyspnoea, palpitations, microcytic anaemia, hepatosplenomegaly.

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29
Q

What causes paroxysmal nocturnal haemoglobinuria (PNH)?

A

Mutation in the PIGA gene in haematopoietic stem cells causing a haemolytic anaemia.

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30
Q

How do we investigate for paroxysmal nocturnal haemoglobinuria (PNH)?

A

Flow cytometry for CD55 and CD59.

31
Q

How does paroxysmal nocturnal haemoglobinuria (PNH) present? What are the complications?

A

Sx of anaemia, dark urine (@night or in morning), abdominal pain, easy bruising/bleeding.

Cx include dysphagia, erectile dysfunction, thrombosis (hepatic, cerebral, abdominal), pulmonary HeTN.

32
Q

What is the inheritance pattern for Thalassaemia? How might it present?

A

Autosomal recessive.
Jaundice, sx of anaemia, facial bone deformities (extreme cases).

33
Q

What causes Beta-thalassaemia? What are the subtypes? Where is it most common?

A
  • Defect in the beta globin gene of Hb,
  • Major: both subunits affected, Minor/trait: 1 affected
  • Mediterranean, Middle East, Asia, parts of Africa
34
Q

What might you see on blood film for someone with beta-thalassaemia?

A

Hypochromic, microcytic target cells and nucleated RBCs.

35
Q

How might you detect Beta-Thalassaemia Minor/Trait?

A

Found incidentally in someone with microcytic anaemia where MCV in much lower than you would expect of Hb level. Can also get a high HbA2.

36
Q

Alpha thalassaemia is caused by what? What is the consequence?

A

Defect in the alpha globin gene in Hb,
Can result in death in utero.

37
Q

What is the mainstay treatment for thalassaemia? What is a complication of this?

A

Blood transfusions or stem cell transplants.
Blood transfusions can result in iron overload (joint sx especially in metacarpophalangeal joints, hyperpigmentation)

38
Q

What is the pathophysiology of hereditary hemochromatosis? How does this show on biochemistry?

A

Autosomal recessive mutation in the HFE gene (most commonly C282Y then H63D is next most common)
This causes a dysfunction in iron excretion.
Biochem: increased iron, ferritin and transferrin saturation, with a low total iron binding capacity.

39
Q

How may someone present with hereditary hemochromatosis? What are the complications?

A
  • Cirrhosis (Jaundice), diabetes, Arthritis (joint pain), fatigue, erectile dysfunction, etc
  • Complications (damaged tissues due to increased iron deposition):
    Diabetes
    Cirrhosis
    heart failure (dilated cardiomyopathy)
40
Q

How is hereditary haemochromatosis managed?

A

Venesection to trigger erythropoiesis to remove iron from sites around the body.

41
Q

What is the pathophysiology behind Aacute intermittent porphyria? How might it present?

A

Autosomal dominant disease causing deficiency of the enzyme hydroxymethylbilane.
Generalsied sx of abdo pain, N+V, weakness, neurological sx, convulsions, hyponatraemia.

42
Q

What can trigger acute intermittent porphyria?

A

Usually starting new drugs e.g., COCP, antidepressants.

43
Q

How do we investigate for and manage acute intermittent porphyria?

A

Inx- urine sample (stored in brown bag to protect from changing colour in light) test creatinine, PBG (porphobilinogen), ALA (aminolevulinic acid) , and total porphyrins.

Mx - acute attacks are treated with IV heme arginate

44
Q

What are the clotting screen findings for Von-Willebrand disease? How is it managed?

A

Normal platelet, thrombin time and prothrombin time.
Prolonged bleeding time, prolonged APTT.
Mx - desmopressin.

45
Q

How is APTT corrected in someone with Von-Willebrand Disease?

A

50:50 mixing with normal plasma

46
Q

Which autoantibodies are +ve in anti-phospholipid syndrome?

A
  • Anti-beta-2-glycoprotein
  • Anti-cardiolipin
  • Lupus anticoagulant
47
Q

How do you manage antiphospholipid syndrome?

A
  • If pregnant: LMWH and low dose aspirin
  • Not pregnant: warfarin
48
Q

What are the components of TTP (thrombotic thrombocytopenic purpura)?

A
  • Purpuric (red/purple spots that do not blanch) rash
  • thrombocytopenia
  • anaemia
  • unconjugated hyperbilirubinemia
  • can get a raised LDH.
49
Q

What causes TTP? What is seen on blood film?

A

Formation of antibodies against ADAMTS13 protein.
BF - schistocytes (fragmented RBCs).

50
Q

What is autoimmune haemolytic anaemia? What do you see on blood film?

A

Antibodies develop against RBCs cause destruction and deformities.
Spherocytes: damage to RBC membrane causes change of shape.

51
Q

Which antibody develops in cold autoimmune haemolytic anaemia? How do we test for this?

A

IgM- but this becomes unbound by the time sample reaches lab so you test for C3d.

52
Q

Which antibody develops in warm autoimmune haemolytic anaemia?

A

IgG.

53
Q

ITP (immune thrombocytopenic purpura) is characterised why reduction in circulating what?

A

Platelets.

54
Q

What are components of ITP?

A

Usually post viral infection in children but chronic and relapsing in adults…
- easy or excessive bruising (purpura)
- superficial bleeding into the skin appearing as a rash of pin-point sized reddish purple spots (petechiae) usually on shins
- prolonged bleeding from cuts
- spontaneous mucocutaneous bleeding (nose and gums)
- haematuria and blood in stools
- unusually heavy menstrual flow

55
Q

How is ITP managed?

A

Corticosteroids to decrease platelet destruction.

56
Q

What is Pernicious anaemia? What are the symptoms? What do you seen on blood film?

A

Vitamin B12 deficiency causing macrocytic anaemia.
Causes fatigues, pallor, SOB, dizziness, memory loss, confusion, jaundice, atrophy of lingual papillae.
You get hypersegmented neutrophils and macrocytic RBCs on blood film.

57
Q

What type of jaundice do you get in haemolytic anaemia?

A

Unconjugated hyperbilirubinemia.
Increased breakdown of Hb into globin + haem, haem is broken down into iron and unconjugated bilirubin, but liver cannot compensate for increase in breakdown = raised unconjugated bilirubin = jaundice.

58
Q

What is seen on bone marrow analysis of Aplastic anaemia?

A

Hypocellular marrow due to pancytopenia.

59
Q

What happens when you aspirate bone marrow from someone with myelofibrosis? What will blood film show?

A

You get a dry tap due to fibrosis of bone marrow.
Dacrocytes (tear drop poikilocytes which are abnormally shaped RBCs) on BF.

60
Q

How does myelofibrosis present?

A

Constitutional sx (fever, weight loss, night sweats),
Sx of anaemia (fatigue, pallor), leukopenia (recurrent infection),
Sx of thrombocytopenia (bleeding/bruising),
Hepatosplenomegaly.

61
Q

What is the pathophysiology behind amyloidosis? What are the most common affects?

A

Deposition of insoluble fibrins in organs and blood vessels.
- Kidneys (commonly nephrotic syndrome)
- GIT (malabsorption, chronic diarrhoea)
- neuro (peripheral neuropathy, carpel tunnel)
- joints (rheumatoid arthritis)

62
Q

What are the types of amyloidosis?

A
  • AA: amyloid A build up (chronic inflammatory disorders)
  • AL: light chain deposition (leukaemia)
  • Beta 2: beta 2 microglobulin (dialysis)
63
Q

How do you diagnose amyloidosis?

A

Tissue biopsy of affected organs - apple green birefringence when stained with congo red.

64
Q

Smoking can cause a persistent what?
a) neutropenia b) neutrophilia c) thrombophilia

A

b) neutrophilia

65
Q

How do you manage idiopathic autoimmune haemolytic anaemia?

A

Corticosteroids

66
Q

What are the manifestations of Hereditary spherocytosis? Mx?

A

Hyperchromic, normocytic, haemolytic anaemia,
Jaundice,
Gallstones,
Splenomegaly.
Mx - folate suplements and for moderate-severe disease splenectomy.

67
Q

What is Waldenstroms Macroglobulinaemia?

A

Low grade lymphoma with monoclonal plasmacytoid lymphocytes.
Clinical features include IgM deposition (hyperviscosity, polyneuropathy), organ infiltration (organomegaly), and bone marrow infiltration (pancytopenia).

68
Q

What is methemoglobinemia? What are the complications?

A

Accumulation of methaemoglobin (contains Fe3+) instead of normal haemoglobin (contains Fe2+) meaning less effective oxygen binding. You get a low saturation but high PaO2 when you supplement oxygen.
Cx: seizures, coma, cardiorespiratory collapse.

69
Q

Management of methemoglobinemia?

A

Oxygen therapy, methylene blue administration, ascorbic acid supplementation.

70
Q

How do you measure Iron when investigating for IDA?

A

Ferritin <30 = IDA.

71
Q

What is Transferrin? At what level is it considered raised? What is this suggestive of?

A

Iron + Protein
Transferrin saturation >45% is raised and suggestive of hemochromatosis.

72
Q

What is the most common cause of inherited aplastic anaemia?
What type of inherotance?

A

Fanconi anaemia.
Autosomal recessive.

73
Q

What antibodies are associated with pernicious anaemia? What % of pernicious anaemias are they present in?

A
  • Anti-parietal cell (high sensitivity, low specificity), present in 70%
  • Anti-intrinsic factor (high specificity, low sensitivity), present in 40%
74
Q
A

Clinical (27 decks)

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