Haematology Flashcards

1
Q

What is Hodgkin’s lymphoma?

A

Cancer of lymph nodes - typically B-cell origin with a bimodal age distribution , EBV association

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2
Q

Sx Hodgkin’s lymphoma

A
  • painless lymphadenopathy which can become painful after drinking alcohol, B-symptoms, Pel-Ebstein fever (cyclical)
  • Dx: Reed-Sternberg cells
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3
Q

Tx Hodgkin’s lymphoma

A

ABVD and surgery

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4
Q

what is NHL?

A
  • Cancer of lymph nodes - typically B-cell origin
  • Lots of sub-types - Burkitt’s, hairy cell leukaemia, mantle cell
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5
Q

Sx NHL

A

painless lymphadenopathy, B-sx

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6
Q

Tx NHL

A

RCHOP

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7
Q

Burkitt’s lymphoma

A
  • Translocation in c-myc gene
  • EBV association
  • Starry sky appearance on blood film
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8
Q

what is myeloma?

A

A malignancy of plasma cells, producing monoclonal antibodies IgG/IgA (Bence-Jones proteins, cause frothy urine)

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9
Q

sx myeloma

A
  • C = calcium increases
  • R = renal damage - Bence Jones proteins cause frothy urine
  • A = anaemia
  • B = bones (lytic lesions - raindrop skull)
  • B = bleeding from thrombocytopenia
  • I = infections
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10
Q

Ix myeloma

A

Raised ESR, calcium and plasma cell levels

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11
Q

tx myeloma

A

Chemotherapy, stem cell transplant and supportive

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12
Q

what is haemophilia?

A

Haemophilia is an x-linked recessive deficiency of either factor 8 or 9, leading to excessive bleeding

  • Haemophilia A: factor 8 deficiency
  • Haemophilia B: factor 9 deficiency
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13
Q

sx haemophilia

A
  • Hematomas, hemarthroses and melena
  • Long aPTT
  • Normal PTT
  • Normal bleeding time
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14
Q

mx haemophilia

A
  • Avoid NSAIDs and IM injections
  • Can give desmopressin in haemophilia A
  • Supplement deficient factor
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15
Q

what is vWF disease?

A

An autosomal dominant bleeding disorder caused by a deficiency of vWF and therefore factor 8

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16
Q

sx vWF disease

A
  • Commonly asymptomatic
  • Menorrhagia
  • Mucosal bleeding
  • Haematoma/haemarthroses are rare
  • Normal/high aPTT
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17
Q

tx vWF disease

A
  • Tranexamic acid for mild bleeding
  • Desmopressin
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18
Q

what is factor 5 Leiden?

A

Most common clotting disorder, autosomal dominant condition causing constant activation of factor 5 and therefore constant thrombin activation

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19
Q

sx factor 5 leiden

A

normally ASx but can form blood clots = VTE/PE

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20
Q

tx factor 5 leiden

A

anticoagulant with heparin

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21
Q

what is aplastic anaemia?

A

you stop producing RBCs at all - pancytopenia
can be caused by Fanconi anaemia, chloramphenicol, gold, hepatitis etc

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22
Q

sx aplastic anaemia

A

Normocytic anaemia with low reticulocytes
Can be associated with acute leukaemia

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23
Q

tx aplastic anaemia

A

bone marrow transplant, supportive tx

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24
Q

how does CKD cause anaemia?

A

Reduced EPO = less oxygenated Hb = anaemia

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25
Q

what is iron deficiency anaemia?

A

Decreased iron binding to protoporphyrin = decreased oxygenated Hb

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26
Q

causes iron deficiency anaemia

A
  • Menorrhagia
  • Gastrointestinal bleeding (colon cancer!)
  • Inadequate dietary intake
  • Poor intestinal absorption
  • Increased iron requirements - e.g. during growth spurts
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27
Q

sx iron deficiency anaemia

A

brittle nails, hair loss, PICA, fatigue, SOB

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28
Q

mx iron deficiency anaemia

A

treat underlying cause -> oral ferrous fumarate/sulfate for at least 3m

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29
Q

what does a high TIBC indicate?

A

iron deficiency anaemia

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30
Q

what is thalassaemia

A

Decreased production of a- or b- globin with varying levels of severity (can be silent, can cause hydrops fetalis)

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31
Q

sx thalassaemia

A
  • Microcytic anaemia with normal ferritin
  • High forehead with prominent cheeks and maxilla (see photo)
  • Anaemia sx
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32
Q

Mx thalassaemia

A

transfuse missing globulin

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33
Q

what is pernicious anaemia

A

Caused by vitamin B12 and folate deficiencies - normally caused by diet.

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34
Q

sx pernicious anaemia

A
  • Cognitive changes
  • Headache
  • Anorexia
  • Fatigue
  • Palpitations
  • Anti-IF ABs and neurological disturbance
  • Addison’s disease
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35
Q

mx pernicious anaemia

A

B12 and folate supplements - B12 always BEFORE folate

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36
Q

What is autoimmune haemolytic anaemia?

A

The body makes antibodies against its own RBCs, causing them to be broken down

37
Q

Which type of autoimmune haemolytic anaemia is more common?

A

Warm - haemolytic occurs >37c whereas it occurs <4c with cold

38
Q

Which type of autoimmune haemolytic anaemia involves IgM antibodies in the peripheries?

A

Cold - Warm is IgG in spleen

39
Q

Which type of autoimmune haemolytic anaemia is Coombs +ve

A

Both

40
Q

What does the Coombs test measure?

A

The Coombs test detects antibodies that are stuck to the surface of RBCs

41
Q

Which type of autoimmune haemolytic anaemia has a gradual onset?

A

Warm - cold is acute and has Raynaud’s feature

42
Q

Which type of autoimmune haemolytic anaemia can be treated with rituximab?

A

Cold - warm needs glucocorticoids and splenectomy

43
Q

which disorder has long aPTT?

A

haemophilia

44
Q

what is hereditary spherocytosis?

A

Autosomal dominant defect affecting people from Northern Europe causing RBCs to become spherical

45
Q

sx hereditary spherocytosis

A

Jaundice and gallstones, splenomegaly, aplastic crises precipitated by Parvo b19 virus

46
Q

Ix hereditary spherocytosis

A

spherocytes and reticulocytes on film, use EMA test if in doubt

47
Q

mx hereditary spherocytosis

A

generally supportive, can transfuse/splenectomy if needed

48
Q

what is sickle cell disease?

A

Autosomal recessive condition affecting people from the mediterranean/Afro-Caribbean, a defect in the haemoglobin B chain causes RBCs to become sickle shaped

49
Q

What does HbSA indicate?

A

a person with the trait

50
Q

What does HbSS indicate?

A

a person with sickle cell disease

51
Q

Ix sickle cell disease

A
  • Blood film - look for sickle cells
  • Newborn heel prick
52
Q

Symptoms - sickle cell trait

A

asymptomatic/visible haematuria

53
Q

symptoms sickle cell disease

A

Vaso-occulative crises: HbSS polymerises when deoxygenated
- Sequestration crises: sickling within spleen/lungs causes pools of blood and worsening anaemia
- Acute chest syndrome: vaso-occlusion within vasculature of lungs
- Aplastic crises: infection with parvovirus B19

54
Q

management sickle cell disease

A

penicillin prophylaxis until 5 y/o

55
Q

management sickle cell crisis

A

hydrate and analgesia

56
Q

how often should patients with sickle cell receive the pneumococcal vaccine?

A

every 5 years

57
Q

what is haemolytic uraemia syndrome?

A

children <5 y/o with diarrhoea are infected with the shiva toxin produced by e.coli
The toxin causes glomerular and vascular damage, which can cause haemolytic anaemia

58
Q

sx HUS

A

diarrhoea preceding diagnosis
thrombocytopenia + haemolysis + reduced eGFR
Anaemia
haematuria
proteinuria

59
Q

mx HUS

A

fluids and transfuse

60
Q

what is DIC?

A

a trigger (sepsis, malignancy, trauma, infection) causes activation of the coagulation cascade, leading to thrombi and platelet/coagulation factor consumption

61
Q

sx DIC

A

thrombi, organ failure, shock, long PTT, aPTT and increased D-dimer

62
Q

mx DIC

A

ESCALATE

63
Q

what is ITP?

A

immune thrombocytopenia - the body creates immunoglobulins against platelets so they are destroyed by the spleen

64
Q

sx ITP

A

purport and epistaxis

65
Q

mx ITP

A

prednisolone, IVIG, splenectomy

66
Q

what is TTP?

A

Increased vWF on endothelium → platelet adhesion → thrombi → schistocytes → haemolysis

Caused by pregnancy/meds/malignancy

67
Q

sx TTP

A
  • Fever
  • Neurological disturbance
  • Low platelets
  • Haemolysis
  • Impaired renal function
68
Q

mx TTP

A

fluids and supportive

69
Q

cause of CML

A

translocation (9:22) lead to BCR-ABL genes

70
Q

phases of CML

A

chronic - <5 years
Accelerated: blast cells become more numerous, symptoms occur
blast: fatal

71
Q

tx CML

A

imatinib - tyrosine kinase inhibitor

72
Q

Richter’s transformation

A

CLL into high grade lymphoma

73
Q

MALT lymphoma

A

affects stomach, caused by h.pylori

74
Q

what does a high LDH indicate?

A

high cell turnover - worse prognosis

75
Q

what is SVC obstruction?

A

oncological emergency caused by compression of SVC, associated with lung cancer and lymphoma

76
Q

sx superior vena cava obstruction

A

dyspnoea, flushed face, headache/visual disturbance, raised JVP and bulging veins

77
Q

mx for superior vena cava obstruction

A

dexamethasone for visual sx
endovascular stenting

78
Q

what is lymphoma?

A

malignant proliferation of lymphocytes

79
Q

painful lymphadenopathy with alcohol

A

Hodgkin’s lymphoma

80
Q

what is the Ann Arbor scoring system used for?

A

Hodgkin’s lymphoma

81
Q

Ann Arbor staging for lymphoma

A

1) one region of LN, either above or below diaphragm
2) more than one region of LN, one side of diaphragm
3) affected lymph nodes on both sides of diaphragm
4) spreads to organs

82
Q

what is tumour lysis syndrome?

A

oncological emergency caused by chemo
destruction of tumour cells leads to toxic intracellular material being released into blood

83
Q

sx tumor lysis syndrome

A

systemically unwell, anorexia, diarrhoea
arrhythmia
muscle cramps
AKI

84
Q

ix tumour lysis syndrome

A

Cairo-bishop score
electrolytes high apart from calcium

85
Q

cairo bishop score used for

A

tumour lysis syndrome

86
Q

mx tumour lysis syndrome

A

prophylactic allopurinol
treat hyperkalaemi

87
Q

what is myeloma

A

neoplasm of plasma cells - large quantities of a single antibody being produced

88
Q

stages of myeloma

A

MGUS - smouldering myeloma - myeloma