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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
what is iron deficiency anaemia?
Decreased iron binding to protoporphyrin = decreased oxygenated Hb
26
causes iron deficiency anaemia
- Menorrhagia - Gastrointestinal bleeding (colon cancer!) - Inadequate dietary intake - Poor intestinal absorption - Increased iron requirements - e.g. during growth spurts
27
sx iron deficiency anaemia
brittle nails, hair loss, PICA, fatigue, SOB
28
mx iron deficiency anaemia
treat underlying cause -> oral ferrous fumarate/sulfate for at least 3m
29
what does a high TIBC indicate?
iron deficiency anaemia
30
what is thalassaemia
Decreased production of a- or b- globin with varying levels of severity (can be silent, can cause hydrops fetalis)
31
sx thalassaemia
- Microcytic anaemia with normal ferritin - High forehead with prominent cheeks and maxilla (see photo) - Anaemia sx
32
Mx thalassaemia
transfuse missing globulin
33
what is pernicious anaemia
Caused by vitamin B12 and folate deficiencies - normally caused by diet.
34
sx pernicious anaemia
- Cognitive changes - Headache - Anorexia - Fatigue - Palpitations - Anti-IF ABs and neurological disturbance - Addison’s disease
35
mx pernicious anaemia
B12 and folate supplements - B12 always BEFORE folate
36
What is autoimmune haemolytic anaemia?
The body makes antibodies against its own RBCs, causing them to be broken down
37
Which type of autoimmune haemolytic anaemia is more common?
Warm - haemolytic occurs >37c whereas it occurs <4c with cold
38
Which type of autoimmune haemolytic anaemia involves IgM antibodies in the peripheries?
Cold - Warm is IgG in spleen
39
Which type of autoimmune haemolytic anaemia is Coombs +ve
Both
40
What does the Coombs test measure?
The Coombs test detects antibodies that are stuck to the surface of RBCs
41
Which type of autoimmune haemolytic anaemia has a gradual onset?
Warm - cold is acute and has Raynaud's feature
42
Which type of autoimmune haemolytic anaemia can be treated with rituximab?
Cold - warm needs glucocorticoids and splenectomy
43
which disorder has long aPTT?
haemophilia
44
what is hereditary spherocytosis?
Autosomal dominant defect affecting people from Northern Europe causing RBCs to become spherical
45
sx hereditary spherocytosis
Jaundice and gallstones, splenomegaly, aplastic crises precipitated by Parvo b19 virus
46
Ix hereditary spherocytosis
spherocytes and reticulocytes on film, use EMA test if in doubt
47
mx hereditary spherocytosis
generally supportive, can transfuse/splenectomy if needed
48
what is sickle cell disease?
Autosomal recessive condition affecting people from the mediterranean/Afro-Caribbean, a defect in the haemoglobin B chain causes RBCs to become sickle shaped
49
What does HbSA indicate?
a person with the trait
50
What does HbSS indicate?
a person with sickle cell disease
51
Ix sickle cell disease
- Blood film - look for sickle cells - Newborn heel prick
52
Symptoms - sickle cell trait
asymptomatic/visible haematuria
53
symptoms sickle cell disease
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
management sickle cell disease
penicillin prophylaxis until 5 y/o
55
management sickle cell crisis
hydrate and analgesia
56
how often should patients with sickle cell receive the pneumococcal vaccine?
every 5 years
57
what is haemolytic uraemia syndrome?
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
sx HUS
diarrhoea preceding diagnosis thrombocytopenia + haemolysis + reduced eGFR Anaemia haematuria proteinuria
59
mx HUS
fluids and transfuse
60
what is DIC?
a trigger (sepsis, malignancy, trauma, infection) causes activation of the coagulation cascade, leading to thrombi and platelet/coagulation factor consumption
61
sx DIC
thrombi, organ failure, shock, long PTT, aPTT and increased D-dimer
62
mx DIC
ESCALATE
63
what is ITP?
immune thrombocytopenia - the body creates immunoglobulins against platelets so they are destroyed by the spleen
64
sx ITP
purport and epistaxis
65
mx ITP
prednisolone, IVIG, splenectomy
66
what is TTP?
Increased vWF on endothelium → platelet adhesion → thrombi → schistocytes → haemolysis Caused by pregnancy/meds/malignancy
67
sx TTP
- Fever - Neurological disturbance - Low platelets - Haemolysis - Impaired renal function
68
mx TTP
fluids and supportive
69
cause of CML
translocation (9:22) lead to BCR-ABL genes
70
phases of CML
chronic - <5 years Accelerated: blast cells become more numerous, symptoms occur blast: fatal
71
tx CML
imatinib - tyrosine kinase inhibitor
72
Richter's transformation
CLL into high grade lymphoma
73
MALT lymphoma
affects stomach, caused by h.pylori
74
what does a high LDH indicate?
high cell turnover - worse prognosis
75
what is SVC obstruction?
oncological emergency caused by compression of SVC, associated with lung cancer and lymphoma
76
sx superior vena cava obstruction
dyspnoea, flushed face, headache/visual disturbance, raised JVP and bulging veins
77
mx for superior vena cava obstruction
dexamethasone for visual sx endovascular stenting
78
what is lymphoma?
malignant proliferation of lymphocytes
79
painful lymphadenopathy with alcohol
Hodgkin's lymphoma
80
what is the Ann Arbor scoring system used for?
Hodgkin's lymphoma
81
Ann Arbor staging for lymphoma
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
what is tumour lysis syndrome?
oncological emergency caused by chemo destruction of tumour cells leads to toxic intracellular material being released into blood
83
sx tumor lysis syndrome
systemically unwell, anorexia, diarrhoea arrhythmia muscle cramps AKI
84
ix tumour lysis syndrome
Cairo-bishop score electrolytes high apart from calcium
85
cairo bishop score used for
tumour lysis syndrome
86
mx tumour lysis syndrome
prophylactic allopurinol treat hyperkalaemi
87
what is myeloma
neoplasm of plasma cells - large quantities of a single antibody being produced
88
stages of myeloma
MGUS - smouldering myeloma - myeloma

AMK (33 decks)

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