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Haematology Flashcards

1
Q

Non-haemolytic febrile reaction

A
  • temp increase by 1-2, or >38
  • ABs reacting to WBC fragments/cytokines in blood product

Px
- fever, chills

Mx
- slow / stop transfusion
- paracetamol
- monitor

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2
Q

Blood product transfusion reactions

A

Acute reactions
- Immunological – acute haemolytic, non-haemolytic febrile, allergic / anaphylaxis
- Infective
- Transfusion-related acute lung injury (TRALI)
- Transfusion-associated circulatory overload (TACO)
- Hyperkalaemia, iron overload, clotting
- hypotension in isolation
- transfusion associated dyspnoea

Delayed reactions
- Delayed haemolytic transfusion reaction
- Post transfusion purpura
- Transfusion associated graft vs host disease

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3
Q

Minor allergic reaction

A
  • ?from foreign plasma proteins, hypersensitivity reaction
    Px
  • pruritis
  • urticaria
    Mx
  • stop transfusion
  • antihistamine
  • monitor
  • continue once sx resolve
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4
Q

Anaphylaxis

A

Px
- hypotension, SOB, wheeze, angioedema

Mx
- stop the transfusion
- IM adrenaline
- A-E, O2, fluids, antihistamine, corticosteroids, bronchodilators, ITU review

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5
Q

Acute haemolytic reaction

A
  • ABO-incompatible blood -> massive intravascular haemolysis

Px
- begin in minutes
- fever
- abdo/chest pain
- hypotension
- UO falls

Mx
- stop transfusion
- check pt identity, name on blood product
- Send bloods - DAT, G/S, crossmatch
- fluid resus
- discuss with haem / ITU - maybe steroids +/- IV Ig

Cx
- DIC
- renal failure

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6
Q

Transfusion-associated circulatory overload (TACO)

A
  • excessive rate of transfusion, eg pre-existing HF
  • immediately, can be <24hrs
    Px
  • pulm oedema
  • ?hypertension
    Mx
  • Slow / stop transfusion
  • Consider furosemide, O2
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7
Q

Transfusion-related acute lung injury (TRALI)

A
  • non-cardiogenic pulmonary oedema - increased vascular permeability from host neutrophils
  • hypoxia / ARDS <6hrs post-transfusion
    Px
  • hypoxia
  • fever
  • hypotension
    Ix
  • CXR - pulm infiltrates
    Mx
  • stop transfusion
  • O2, supportive
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8
Q

Delayed haemolytic transfusion reaction

A
  • fever, failure to respond to transfusion, unexplained bilirubin rise
  • Dx on lab tests
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9
Q

Post-transfusion purpura

A
  • immune mediated - 5-12d after transfusion
  • platelet drop
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10
Q

Indications for blood transfusion

A
  • acute bleeding, haemodynamically unstable
  • Hb <70, stable
  • Hb <80, ACS
  • chronic transfusion-dependent anaemia
  • radiotherapy (aim Hb>100)
  • exchange transfusion
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11
Q

Anticoagulants reversal

A

dabigatran - idarucizumab
heparin - protamine sulfate
apixaban/rivaroxaban - andexanet alfa
edoxaban - ?andexanet alfa
warfarin - vit K, FFP, PCC

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12
Q

anaemia - definition

A
  • low Hb conc
  • Men Hb 130-180, women Hb 120-165
  • MCV 80-100
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13
Q

causes of microcytic anaemia?

A

MCV<80 - microcytic
- iron deficiency
- anaemia of chronic disease
- thalassaemia
- congenital sideroblastic anaemia
- lead poisoning

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14
Q

causes of normocytic anaemia?

A

MCV80-100 - normocytic
- acute blood loss
- anaemia of chronic disease
- combined haematinic deficiency - iron + B12
- aplastic anaemia
- pregnancy
- haemolytic anaemia
- hypothyroidism

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15
Q

causes of macrocytic anaemia?

A

MCV>100 - macrocytic
- B12/folate deficiency - megaloblastic - pernicious anaemia
- alcohol excess / liver disease
- hypothyroid
- bone marrow failure / infiltration
- drugs - azathioprine, methotrexae
- reticulocytosis - rapid turnover, eg haemolytic anaemia
- myelodysplasia

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16
Q

what is haemolytic anaemia?

A
  • abnormal breakdown of RBCs
  • see reticulocytosis, increased bilirubin, LDH
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17
Q

Anaemia general Px

A
  • fatigue, SOB, faint, dizzy
  • palpitations, headaches, tinnitus, anorexia
  • angina, HF
  • conjunctival pallor, pale skin
  • hyperdynamic circulation - tachycardia, flow murmurs, cardiac enlargement
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18
Q

Anaemia general Ix

A
  • FBC - Hb, MCV
  • reticulocyte count
  • blood film
  • renal profile
  • LFTs
  • ferritin, B12, folate
  • TFTs
  • IF ABs
  • Coeliac ABs
  • HB electrophoresis
  • DAT
  • colonoscopy + OGD
  • bone marrow biopsy
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19
Q

iron deficiency anaemia

A
  • low Hb due to low iron
  • iron absorbed in duodenum/jejunum - needed for haem formation
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20
Q

causes of iron deficiency anaemia?

A
  • low iron diet - vegans, lack of green leafy veg
  • blood loss - menorrhagia, GI bleed, hookworm
  • malabsorption - coeliac, Crohn’s
  • higher iron demand - pregnancy, growing children
  • PPIs
  • paeds - excessive cow’s milk intake, CMPA
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21
Q

iron deficiency anaemia px

A
  • anaemia - fatigue, SOB, pallor, dizzy/faint, palpitations, headaches
  • brittle hair, nails
  • atrophic glossitis
  • koilonychia
  • angular stomatitis / cheilitis
  • pica
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22
Q

iron deficiency anaemia Ix

A
  • FBC, blood film - hypochromic, microcytic anaemia, anisocytosis, poikilocytosis
  • iron studies - low serum ferritin, high TIBC / transferrin
  • reduced reticulocyte count
  • colonoscopy + OGD
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23
Q

iron deficiency anaemia Mx

A
  • oral iron - ferrous sulfate / fumarate
  • iron-rich diet
  • iron infusions - IV CosmoFer
  • blood transfusion
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24
Q

Sideroblastic anaemia - Px, Ix and Mx

A
  • RBCs fail to completely form haem
  • congenital / acquired

Px
- anaemia sx

Ix
- FBC - hypochromic, microcytic anaemia
- blood film - basophilic stippling of RBCs
- iron studies - high ferritin/iron/transferrin saturation
- bone marrow - Prussian blue staining – ringed sideroblasts

Mx
- tx cause, supportive
- pyridoxine (vit B6)
- transfusions

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25
Q

anaemia of chronic disease - Px, Ix and Mx

A
  • anaemia secondary to chronic disease
  • poor use of iron / decreased RBC survival / decreased EPO
    Causes
  • Crohn’s, RA, TB, malignancy, CKD, SLE

Px
- anaemia sx

Ix
- FBC, blood film - microcytic/normocytic, hypochromic
- iron studies - low serum iron/TIBC/transferrin sat, increased/normal serum ferritin

Mx
- tx cause
- recombinant EPO
- ?IV iron

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26
Q

aplastic anaemia - causes, Px, Ix and Mx

A
  • bone marrow failure - pancytopenia

Causes
- Fanconi, acquired, chemo, infections (EBV, HIV, TB, hepatitis), pregnancy, radiation

Px
- anaemia sx
- infections, bruising, bleeding

Ix
- FBC - normochromic, normocytic anaemia, low WCC, low platelets
- low reticulocyte count
- bone marrow biopsy - hypocellular marrow, increased fat spaces

Mx
- remove cause
- blood / platelet transfusion
- bone marrow transplant
- immunosuppression

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27
Q

B12 deficiency

A
  • needed for RBC production / DNA synthesis
  • megaloblastic / macrocytic anaemia
  • absorbed in terminal ileum bound to IF (produced by parietal cells in stomach)
  • also important for myelination of nerves
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28
Q

causes of B12 deficiency

A
  • pernicious anaemia - associated with thyroid, T1DM, Addison’s, RA, vitiligo
  • low dietary intake - vegans
  • alcoholism, malnutrition
  • atrophic gastritis
  • gastrectomy
  • Coeliac’s, Crohns
  • drugs - colchicine, AEDs, PPIs, metformin
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29
Q

B12 deficiency Px

A
  • anaemia sx
  • glossitis
  • angular stomatitis
  • mild jaundice
  • neuro sx - peripheral neuropathy, subacute combined degeneration of spinal cord, neuropsych
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30
Q

B12 deficiency Ix

A
  • FBC, blood film - macrocytic anaemia, hypersegmented polymorphs, low WCC/platelets
  • Auto-ABs - IF ABs, parietal cell ABs
  • serum B12 low
  • Schilling test not done
  • LDH - increased
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31
Q

B12 deficiency Mx

A

Initial
- IM hydroxocobalamin - 3x weekly for 2wks

Maintenance
- pernicious - 2-3monthly injections
- diet - oral cyanocobalamin / 2x yearly injections
- do not give folic acid at same time - wait for B12 to resolve

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32
Q

folate deficiency

A
  • (Vit B9) - absorbed in jejunum, found in green veggies
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33
Q

causes of folate deficiency

A
  • poor folate diet - poverty, alcoholics, elderly
  • malabsorption - Crohn’s, Coeliac, alcoholism
  • pregnancy
  • anti-folate drugs - methotrexate, trimethoprim, AEDs
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34
Q

folate deficiency Px

A
  • anaemia sx
  • no neuropathy
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35
Q

folate deficiency Ix

A
  • FBC, blood film - macrocytic anaemia, hypersegmented polymorphs
  • increased LDH
  • low reticulocyte count
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36
Q

folate deficiency Mx

A
  • tx cause
  • folic acid
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37
Q

Haemolytic anaemia

A
  • premature breakdown of RBCs - intra/extravascular
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38
Q

Haemolytic anaemia causes

A

Hereditary
- membrane - hereditary spherocytosis / elliptocytosis
- enzymes - G6PD deficiency, pyruvate kinase deficiency
- haemoglobinopathies - sickle cell, thalassaemia

Acquired - immune (Coombs+)
- autoimmune - warm/cold
- alloimmune - transfusion, haemolytic disease of newborn
- Drug - methyldopa, penicillin

Acquired - non-immune (Coombs-)
- microangiopathic haemolytic anaemia - TTP, HUS, DIC, malignancy, pre-eclampsia
- prosthetic heart valves
- paroxysmal nocturnal haemoglobinuria
- infections - malaria

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39
Q

Haemolytic anaemia causes by site

A

Intravascular
- Mismatched blood transfusion
- G6PD deficiency (bit of both)
- RBC fragmentation – heart valves, TTP, DIC, HUS
- Paroxysmal nocturnal haemoglobinuria
- Cold autoimmune haemolytic anaemia

Extravascular
- SCA, thalassaemia
- Hereditary spherocytosis
- Haemolytic disease of newborn
- Warm autoimmune haemolytic anaemia

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40
Q

Haemolytic anaemia general Px

A
  • anaemia sx
  • splenomegaly
  • jaundice
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41
Q

Haemolytic anaemia general Ix

A
  • FBC, blood film - normocytic anaemia, schistocytes
  • Direct Coombs test (DAT) - positive in AIHA
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42
Q

Autoimmune haemolytic anaemia (AIHA) - Ix and Mx

A
  • body creates ABs against RBCs -> haemolysis
  • warm / cold types

Ix
- FBC - anaemia
- increased reticulocytes
- low haptoglobin
- raised LDH + indirect bilirubin
- blood film - spherocytes, reticulocytes
- positive DAT (Coombs)

Mx
- blood transfusions
- prednisolone
- rituximab
- splenectomy

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43
Q

Hereditary spherocytosis - Px, Ix and Mx

A
  • autosomal dominant - defect in RBC cytoskeleton -> fragile RBCs break down in spleen

Px
- failure to thrive
- anaemia
- jaundice
- gallstones
- splenomegaly
- aplastic crisis (parvovirus)

Ix
- FBC - raised MCHC, raised reticulocytes
- Blood film - spherocytes
- EMA binding test

Mx
- acute - blood transfusions, supportive
- long-term - folate, splenectomy, cholecystectomy

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44
Q

Hereditary elliptocytosis

A
  • as spherocytosis but RBCs ellipse-shaped
  • autosomal dominant
  • Px / Mx the same
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45
Q

G6PD deficiency - Px, Ix and mx

A
  • x-linked recessive - defect in gene for G6PD -> RBCs susceptible to oxidative stress
  • triggers - infections, fava beans, meds

Px
- jaundice
- gallstones
- anaemia
- splenomegaly

Ix
- FBC
- blood film - Heinz bodies, bite+blister cells
- G6PD enzyme assay - dx

Mx
- remove trigger
- supportive
- transfusions

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46
Q

what is haemolytic disease of the newborn?

A
  • If fetus is RhD+ (has RhD antigens on RBCs), and mother is RhD-, then during sensitisation event, mother can be exposed to fetal RhD, produce anti-D ABs against RhD
  • In future, these ABs can cross placenta, cause haemolysis, destroying fetal RBCs
  • To prevent sensitisation – give anti-D – Ig against RhD antigens that may have come from fetal blood into maternal bloodstream
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47
Q

Microangiopathic haemolytic anaemia (MAHA)

A
  • destruction of RBCs as they travel through circulation
  • abnormal activation of clotting cascade - thrombi obstruct vessels, churn RBCs -> haemolysis
  • HUS, DIC, TTP, SLE, cancer
  • schistocytes on blood film
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48
Q

Prosthetic valve haemolysis

A
  • turbulent flow around valve -> shearing of RBCs
    Mx
  • monitor
  • oral iron / folic acid
  • blood transfusions
  • revision surgery
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49
Q

fanconi anaemia

A
  • autosomal recessive cause of aplastic anaemia
  • bone marrow failure, increased AML risk, neuro sx, short stature, thumb / radius abnormalities, café au lait spots
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50
Q

Sickle cell anaemia (SCA)

A
  • autosomal recessive - production of abnormal Hb leading to vaso-occlusive crises (disorder of quality)
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51
Q

sickle cell pathophysiology

A
  • HbS produced rather than HbA - which polymerises when deoxygenated - RBCs deform, produce fragile sickle cells -> intravascular haemolysis, obstruction, infarction
  • HbSS/HbAS - anaemia / trait

Triggers
- spontaneous
- dehydration, infection, stress, cold weather, high altitude

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52
Q

sickle cell crises

A

Vaso-occlusive crisis - RBCs clog capillaries -> distal ischaemia
Acute chest syndrome - lung vessels blocked
Splenic sequestration crisis - RBCs block flow through spleen
Aplastic crisis - cessation of RBC formation - parvovirus B19 trigger - anaemia

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53
Q

sickle cell Px

A
  • anaemia
  • jaundice
  • infection, fever etc
  • vaso-occlusive crisis - pain/swelling in hands, feet (also chest, back, elsewhere)
  • acute chest syndrome - fever, SOB, chest pain, cough, hypoxia
  • aplastic crisis - anaemia
  • splenic sequestration crisis - painful splenomegaly, blood pooling -> anaemia, shock
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54
Q

sickle cell Ix

A
  • newborn blood spot screening
  • test pregnant women at risk of being carrier
  • FBC - low Hb, increased reticulocytes, increased bilirubin
  • Blood film - sickle cells
  • Hb electrophoresis - definitive
  • CXR - pulm infiltrates (acute chest syndrome)
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55
Q

sickle cell Mx

A
  • avoid triggers, dehydration
  • pneumococcal vaccine (and others)
  • pen V abx prophylaxis
  • hydroxycarbamide
  • crizanlizumab
  • blood transfusions
  • bone marrow transplant
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56
Q

sickle cell crises management

A
  • low threshold to admit
  • abx for infections
  • keep warm, hydrate, IV fluids, analgesia, O2
  • blood transfusion
  • exchange transfusion
  • splenectomy
  • acute chest syndrome - incentive spirometry, resp support
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57
Q

sicke cell complications

A
  • anaemia
  • infections
  • CKD
  • sickle cell crises
  • stroke
  • avascular necrosis
  • hypoxia -> fibrosis -> pulm HTN
  • gallstones
  • priapism
  • splenic infarction, hyposplenism etc
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58
Q

what is thalassaemia

A
  • autosomal recessive genetic defect in Hb protein chains -> underproduction of one globin chain
  • reduced production + premature destruction of RBCs - disorder of quantity
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59
Q

thalassaemia pathophysiology

A
  • HbA - 2 alpha, 2 beta chains
  • lack of production of a/b chain
  • reduced RBC production, fragile RBCs break down
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60
Q

thalassaemia Px

A
  • Microcytic anaemia
  • Fatigue
  • Pallor
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Poor growth + development
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61
Q

alpha thalassaemia

A
  • Defect on gene for alpha-globin, 2 separate genes on each c16 (4 total)

1 deletion
- blood picture normal
2 deletions
- asym, mild microcytic anaemia
3 deletions
- Hb H disease
- severe haemolytic anaemia, splenomegaly
4 deletions
- alpha thalassaemia major - no a-chain, stillborn infant

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62
Q

beta thalassaemia

A
  • defect in b-globin gene c11
  • Homozygous B-thalassaemia -> little/no B chain production -> excess A-chain production -> A-chains combining with whatever B, delta, gamma chains available -> increased production of HbA2 and HbF -> ineffective erythropoiesis and haemolysis
  • either abnormal gene / deletion of gene
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63
Q

B-thalassaemia minor / trait

A
  • asym, heterozygous carrier
  • mild/absent anaemia
  • monitor only
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64
Q

B-thalassaemia intermedia

A
  • 2 defective genes - 2 abnormal / 1 abnormal + 1 deletion
  • moderate anaemia
  • splenomegaly
  • transfusions, iron chelation
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65
Q

B-thalassaemia major

A
  • homozygous - 2 deletion genes
  • severe anaemia, failure to thrive
  • bony abnormalities - frontal bossing, enlarged maxilla, depressed nasal bridge, protruding upper teeth, prominent frontal/parietal bones
  • HSM
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66
Q

thalassaemia ix

A
  • screen in pregnancy at booking
  • FBC - hypochromic, microcytic anaemia, raised HbA2 (HbF also in major)
  • Blood film - RBCs pale, irregular
  • increased reticulocytes
  • ferritin raised in iron overload
  • Hb electrophoresis - dx
  • DNA testing
  • Skull XR
67
Q

thalassaemia Mx

A
  • exercise good diet
  • folate supplements
  • regular blood transfusions
  • desferrioxamine / ascorbic acid
  • splenectomy
  • hormonal tx for endocrine cx
  • bone marrow transplant
68
Q

leukaemia

A
  • cancer of immature blast blood cells / stem cells - precursors of RBCs, platelets, WBCs
69
Q

leukaemia pathophysiology

A
  • energy wasted making useless cells
  • cells take up space from nutrients / other cells
  • fewer functioning blood cells
  • pancytopenia
  • acute leukaemia - cells don’t differentiate (partially differentiate in chronic)
  • Lymphoid - adaptive immune system, myeloid - innate immune system
70
Q

leukaemia - general Px

A
  • Fatigue, fever
  • Pallor - anaemia
  • Petechiae / bruising
  • Abnormal bleeding
  • Lymphadenopathy
  • HSM
  • Failure to thrive
71
Q

leukaemia - general Ix

A
  • FBC <48hrs
  • Blood film
  • LDH raised
  • Bone marrow biopsy - from iliac crest
  • CT / PET
  • lymph node biopsy
  • genetic tests, immunophenotyping
72
Q

leukaemia - general Mx

A
  • chemo
  • targeted therapy
  • bone marrow transplant
  • surgery
73
Q

Acute lymphoblastic leukaemia (ALL)

A
  • malignant proliferation of lymphoblasts (B/T cell precursors)
  • 80% childhood leukaemias
  • Some Philadelphia chromosome involvement
  • radiation, Down’s
74
Q

ALL Px

A
  • bone marrow failure - anaemia, infections, fever, bleeding, bruising
  • bone pain
  • HSM
  • lymphadenopathy
  • headache, CN palsies
  • fever
  • testicular swelling - unilateral
75
Q

ALL Ix

A
  • FBC - low Hb, platelets, WCC
  • Blood film - blast cells
  • Bone marrow aspirate - blast cells
  • CXR, CT, LP
76
Q

ALL Mx

A
  • blood / platelet transfusions
  • infection prophylaxis
  • chemo
  • marrow transplant
77
Q

ALL poor prognostic factors

A
  • Age <2yo, >10yo
  • WBC >20 at diagnosis
  • T/B cell surface markers
  • Non-Caucasian
  • Male
78
Q

Acute myeloid leukaemia (AML)

A
  • malignant proliferation of myeloblast cells (basophil, neutrophil, eosinophil precursor)
  • most common acute leukaemia in adults
  • chemo, radiation, Downs RFs
79
Q

AML Px

A
  • anaemia, infection, bleeding
  • bleeding gums, gum hypertrophy
  • bone pain
  • HSM
  • skin involvement
  • DIC
80
Q

AML Ix

A
  • FBC - increased WCC, anaemia, thrombocytopenia
  • Bone marrow biopsy - Auer rods, high proportion blast cells
81
Q

AML Mx

A
  • infection prophylaxis
  • blood / platelet transfusions
  • chemo
  • bone marrow transplant
82
Q

Chronic myeloid leukaemia (CML)

A
  • Malignant proliferation of myeloid cells (WBCs - neutrophils, basophils, eosinophils)
  • Adults 50-60yo
  • chronic, accelerated, blast phases
  • Philadelphia chromosome
83
Q

CML Px

A
  • Insidious onset
  • Fever, wt loss, fatigue
  • Gout (from purine breakdown)
  • Bleeding, bruising
  • Anaemia
  • HSM
84
Q

CML Ix

A
  • FBC - increased WCC, anaemia
  • Bone marrow biopsy - hypercellular
  • cytology
  • genetics
85
Q

CML Mx

A
  • chemo
  • imatinib
  • bone marrow transplant
86
Q

Chronic lymphocytic leukaemia

A
  • Malignant proliferation of mature B cells - accumulate + escape apoptosis
  • Elderly
  • associated with warm haemolytic anaemia
  • mutations, Downs, pneumonia may be trigger
87
Q

Richter’s transformation

A
  • CLL may transform into rare type of aggressive non-Hodgkin lymphoma
  • suddenly unwell - lymphadenopathy, fever, wt loss, night sweats, nausea, abdo pain
88
Q

CLL Px

A
  • Often asym
  • Anaemia, infection
  • Wt loss, night sweats
  • Anorexia
  • HSM
  • Lymphadenopathy
89
Q

CLL Ix

A
  • FBC - anaemia, raised WCC, high lymphocytes, thrombocytopenia
  • Blood film - smudge / smear cells
90
Q

CLL Mx

A
  • Blood transfusion
  • chemo / radio
  • rituximab
  • ibrutinib
  • bone marrow transplant
  • 1/3 never progress, 1/3 progress slowly, 1/3 progress quickly
91
Q

Tumour lysis syndrome

A
  • release of chemicals from cells destroyed by chemo
  • high grade lymphomas / leukaemias
92
Q

tumour lysis syndrome pathophysiology

A
  • High uric acid -> form crystals in kidneys -> AKI
  • High K -> cardiac arrhythmias
  • High phosph
  • Low Ca - from high phos
  • Cytokines -> systemic inflammation
93
Q

Tumour lysis syndrome Px

A
  • suspect in AKI with high phos, high uric acid
94
Q

Tumour lysis syndrome Ix

A

Clinical tumour lysis syndrome is - lab tumour lysis syndrome plus 1+ of:
- Increased serum creatinine >1.5x upper limit normal
- Cardiac arrhythmia / sudden death
- Seizure

95
Q

Tumour lysis syndrome Mx

A
  • good hydration / UO before chemo - IV fluids
  • allopurinol
  • rasburicase
96
Q

lymphoma

A
  • cancer of lymphocytes in lymphatic system
  • proliferate in lymph nodes
97
Q

Lymphoma Lugano classification

A

Stage 1 – one node / group of nodes
Stage 2 – 1+ group of nodes, same side of diaphragm
Stage 3 – lymph nodes above + below diaphragm
Stage 4 – widespread, inc organs eg lungs, liver

98
Q

Ann Arbor Classification

A

I – confined to single lymph node region
II – involvement of 2+ nodal areas on same side of diaphragm
III – involvement of nodes on both sides of diaphragm
IV – spread beyond lymph nodes, eg liver, bone marrow

Each stage is A/B
A – no systemic symptoms other than pruritis (severe itching of skin)
B – presence of B symptoms (fever, wt loss, night sweats)

99
Q

Lymphadenopathy DDx

A
  • infective - IM, HIV, eczema, rubella, toxoplasmosis, CMV, TB, roseola infantum
  • neoplastic - leukaemia, lymphoma
  • SLE, RA, graft vs host disease, sarcoidosis, drugs
100
Q

Hodgkin’s lymphoma

A
  • specific type of lymphoma
  • 20-25yo, and 80yo
101
Q

Hodgkin’s pathophysiology + RFx

A
  • nearby contiguous spread, rarely extranodal, Reed-Sternberg cells
    RFs
  • HIV, EBV, RA, sarcoidosis, FHx
102
Q

Hodgkin’s Px

A
  • lymphadenopathy - worse after alcohol
  • B sx - fever, wt loss, night sweats, anorexia
  • fatigue, itching, cough, SOB, abdo pain, recurrent infections
  • compression - MSCC, SVC, DVT
103
Q

Hodgkin’s Ix

A
  • FBC - raised WCC, anaemia
  • LDH - high
  • Lymph node biopsy - Reed-Sternberg cells (multinucleated)
  • CT / MRI / PET, CXR
104
Q

Hodgkin’s Mx

A
  • Chemo
  • radiotherapy
  • stem cell transplant
  • surgical removal of lymph nodes
105
Q

Non-Hodgkin’s lymphoma

A
  • all other types of lymphoma
  • 80% B cell, 20% T cell
106
Q

Non-Hodgkin’s patho + RFx

A
  • non-contiguous spread
  • extranodal - GI tract, skin, brain
  • low/high grade

Diffuse large B cell lymphoma
- rapidly growing, painless mass, older pts

Burkitt’s lymphoma
- HIV, EBV, malaria association
- starry sky appearance on microscopy

MALT lymphoma
- affects mucosa-associated lymphoid tissue around stomach
- H pylori association

RFs
- HIV, EBV, H pylori, hep B/C, pesticides, industrial chemicals, FHx

107
Q

Non-Hodgkin’s Px

A
  • lymphadenopathy

Extranodal disease
- Gastric - dyspepsia, dysphagia, wt loss, abdo pain
- Bone marrow - pain, bruising, infections - pancytopenia, anaemia
- Lungs
- Skin
- CNS - nerve palsies, spinal cord

B sx
- fever, wt loss, night sweats, anorexia

108
Q

Non-Hodgkin’s Ix

A
  • FBC - raised WCC, low Hb/platelets
  • raised LDH
  • lymph node biopsy
  • CT / MRI / PET
109
Q

Non-Hodgkin’s Mx

A
  • chemo
  • pred
  • rituximab
  • radiotherapy
  • stem cell transplant
110
Q

Myeloma

A
  • cancer of plasma cells in bone marrow
  • multiple myeloma - myeloma in multiple sites of bone marrow
  • MGUS - production of paraprotein without myeloma/cancer
  • smouldering myeloma - abnormal plasma cells/paraproteins, no sx
111
Q

myeloma pathophysiology

A
  • cancer of plasma cell - large production of one antibody (IgG most commonly)
  • other Ig levels low - immunoparesis, infections
  • bone marrow failure - anaemia, low WCC, low platelets
  • increased bone turnover
  • renal impairment
  • hyperviscosity syndrome
  • relapsing/remitting disease
112
Q

Myeloma Px

A

OLD - old age
C - Ca high
R - renal failure - thirst
A - anaemia, neutropenia, thrombocytopenia - infection, bleeding, fatigue, pallor
B - bone lytic lesions - bone/back pain, fractures

113
Q

myeloma Ix

A
  • FBC - anaemia, low WCC
  • Blood film - Rouleaux formation
  • raised ESR, ALP, Ca
  • Urea + creatinine raised
  • serum electrophoresis
  • serum-free light-chain assay
  • urine protein electrophoresis - Bence-Jones protein
  • XR - lytic, punched out lesions, fractures
  • CT / MRI / skeletal survey
  • bone marrow biopsy
114
Q

Myeloma Dx criteria

A

1 major + 1 minor, or 3 minor + sx

Major
- Plasmacytoma – tumour of plasma cells on biopsy
- 30% plasma cells in bone marrow sample
- Elevated M protein levels in blood / urine
Minor
- 10-30% plasma cells in bone marrow sample
- Minor elevation of M protein in blood / urine
- Osteolytic lesions – on imaging
- Low AB level

115
Q

myeloma Mx

A
  • chemo
  • radio
  • stem cell transplant
  • analgesia
  • bisphosphonates
  • surgery
  • IV Ig
  • transfusion
116
Q

Myeloproliferative disorders

A
  • uncontrolled proliferation of one stem cell - form of cancer
  • scarring of bone marrow -> fibrosis -> bone marrow failure

Primary myelofibrosis
- haematopoietic stem cells
- low Hb, high/low WCC, high/low platelets

Polycythaemia vera
- RBCs
- high Hb

Essential thrombocytosis
- megakaryocyte
- high platelets

117
Q

Myeloproliferative disorders Px

A
  • asym
  • fatigue, wt loss, night sweats, fever
  • sx of specific disorder
  • anaemia, HSM, portal HTN, bleeding, petechiae
  • infections, gout, thrombosis, raised Hb (itchy, headaches, red face)
118
Q

Myeloproliferative disorders Ix

A
  • genetic testing
  • bloods - low Hb, high WCC/platelets, high urate/LDH
  • blood film - teardrop shaped RBCs, blasts, anisocytosis
  • bone marrow biopsy
119
Q

Myeloproliferative disorders Mx

A

Primary myelofibrosis
- supportive
- chemo
- ruxolitinib
- allogenic stem cell transplant

Essential thrombocytosis
- aspirin
- chemo
- anagrelide

120
Q

Polycythaemia

A
  • increase in RBC mass / Hb / packed cell volume
121
Q

causes of polycythaemia

A

Absolute
- primary - polycythaemia vera (JAK2 mutation)
- secondary - hypoxia, high EPO (RCC, HCC)

Relative
- apparent polycythaemia - obesity, HTN, alcohol, smoking
- dehydration

122
Q

polycythaemia Px

A
  • asym
  • HTN
  • HSM
  • gout
  • VTE, arterial thrombosis
  • headaches
  • itching - after warmth
  • dizziness
  • tinnitus
  • visual disturbance
  • angina
  • red face
123
Q

polycythaemia Ix

A
  • FBC - high Hb, raised WCC/platelets
  • ferritin low in primary
  • genetic screen
  • bone marrow biopsy
124
Q

polycythaemia Mx

A
  • venesection
  • chemo
  • aspirin
125
Q

thrombocytosis

A
  • high platelet count

Causes
- reactive - stress, infection, surgery, IDA
- malignancy
- essential thrombocytosis
- hyposplenism

126
Q

myelodysplastic syndrome

A
  • Cancer caused by mutation in myeloid cells (haematopoietic stem cells) in bone marrow -> inadequate production of blood cells
  • potential to transform into AML

Px
- pancytopenia - anaemia, infections, bleeding etc
- may be asym

Ix
- FBC - pancytopenia
- blood film - blast cells
- bone marrow biopsy

Mx
- W+W
- blood / platelet transfusions
- EPO
- granulocyte colony-stimulating factor
- chemo
- targeted therapies
- allogenic stem cell transplant

127
Q

Thrombocytopenia

A
  • low platelet count - normal 150-450
  • from reduced production / increased destruction
128
Q

Thrombocytopenia causes

A

Reduced production
- EBV, CMV, HIV
- B12, folate deficiency
- Liver failure – reduced thrombopoietin production
- Leukaemia
- Myelodysplastic syndrome
- Chemotherapy

Increased destruction
- meds - valproate, methotrexate
- alcohol
- ITP
- TTP
- HIT
- HUS
- hypersplenism - portal HTN, splenomegaly

129
Q

Thrombocytopenia Px

A

mild - asym

<50 - nosebleeds, bleeding gums, heavy periods, easy bruising, haematuria, PR bleed

<10 - risk spont bleed - intracranial haemorrhage, GI bleed

130
Q

DDx abnormal bleeding

A
  • Thrombocytopenia
  • Von Willebrand disease
  • Haemophilia
  • DIC (usually secondary to sepsis)
131
Q

Immune thrombocytopenic purpura (ITP)

A
  • ABs created against platelets
  • T2 hypersensitivity
  • eg after infection, vaccination - more acute in children
  • adults - autoimmune disorders
132
Q

ITP Px

A
  • bruising
  • petechial / purpuric rash
  • epistaxis, gingival bleeding
133
Q

ITP Ix

A
  • FBC - low platelets
  • blood film
  • bone marrow biopsy - if atypical features
134
Q

ITP Mx

A
  • children - resolves alone
  • avoid contact sports / trauma

If v low platelets / bleeding
- oral / IV corticosteroid
- IV Ig
- platelet transfusions

Adults
- oral prednisolone
- IV Ig
- avatrombopag - thrombopoietin receptor agonist
- rituximab
- splenectomy

135
Q

Thrombotic thrombocytopenic purpura (TTP) - definition and causes

A
  • widespread aggregation of platelets
  • deficiency in ADAMTS13 protein - usually inactivates vW - lack of it increases clot formation
  • thrombi -> purpura, tissue ischaemia

Causes
- post-infection
- pregnancy
- drugs
- tumours
- SLE, HIV

136
Q

TTP Px

A
  • florid purpura (bright red)
  • fever, fatigue
  • haemolytic anaemia, may have AKI
  • bruising, bleeding
  • confusion, headache, cerebral dysfunction - from microemboli
137
Q

TTP Ix

A
  • FBC - low platelets
  • U/E
  • LDH raised
  • blood film - schistocytes / fragmented RBCs
  • coag - normal
138
Q

TTP Mx

A
  • plasma exchange
  • steroids - IV methylprednisolone
  • rituximab
  • splenectomy
139
Q

Heparin-induced thrombocytopenia (HIT)

A
  • ABs against platelets produced in response to heparin (also LMWHs)
  • Px is 5-10d after heparin tx
  • ABs bind to platelets, activate clotting cascade, cause thromboses, break down platelets
  • pt on heparin + low platelets + abnormal clots
    Ix
  • for HIT ABs
    Mx
  • stop heparin
  • use alt anticoagulant
140
Q

Von Willebrand Disease

A
  • malfunctioning of vWF - important for platelet adhesion/aggregation

Px
- prolonged / heavy bleeding
- bleeding gums, epistaxis
- easy bruising
- menorrhagia
Ix
- Clotting - prolonged bleeding time, APTT prolonged
- Factor VIII assay - reduced
- Von Willebrand screen - factor 8, VWF:Ag, VWF activity

141
Q

haemophilia defintion

A
  • inherited bleeding disorder from deficiency of clotting factors
  • Haemophilia A - factor 8
  • Haemophilia B - factor 9
  • x-linked recessive
142
Q

haemophilia px

A
  • easy bruising, haematomas, prolonged bleeding, haematuria, epistaxis, haemarthrosis, GI bleeds, brain bleeds
143
Q

haemophilia ix

A

Ix
- PTT, vWF normal (extrinsic)
- APTT prolonged (intrinsic)
- factor auto-AB
- factor 8/9 assay

144
Q

haemophilia Mx

A

Mx
A
- desmopressin
- recombinant factor 8 infusion
- FFP - acute bleed
- emicizumab
B
- recombinant factor 9

Acquired
- steroids

145
Q

Disseminated intravascular coagulation (DIC) - what is it and what causes it ?

A
  • massive activation of clotting cascade - widespread clotting + bleeding
  • extensive damage to vascular endothelium

Causes
sepsis, trauma, advanced cancer, obstetric cx

146
Q

DIC Px

A
  • ill pt, shocked
  • bleeding, bruising
  • confusion
  • purpura, petechiae, infarctions
147
Q

DIC Ix and Mx

A

Ix
- FBC - low platelets
- blood film - schistocytes
- coag - prolonged PTT, APTT, thrombin time (TT)
- decreased fibrinogen, elevated FDPs

Mx
- tx cause
- transfuse - platelets, RBCs, FFP, cryo

148
Q

thrombophilia - definition and causes

A
  • overtendency for blood to clot

Causes
Inherited
- factor V Lieden
- Prothrombin gene mutation
- Antithrombin III deficiency
- Protein C deficiency
- Protein S deficiency
Acquired
APL syndrome
cOCP

149
Q

Acute intermittent porphyria

A
  • autosomal dominant - defect in enzyme for biosynthesis of haem
  • results in toxic accumulation of delta aminolaevulinic acid and porphobilinogen

Px
- Abdo pain, vomiting
- Motor neuropathy
- Depression
- HTN, tachycardia
- Urine turns deep red on standing
- Will have attacks of sx
Ix
- raised urinary porphobilinogen
- assay of RBCs for porphobilinogen deaminase
- raised serum delta aminolaevulinic acid, porphobilinogen
Mx
- avoid triggers
- acute attacks - IV haematin / haem arginate / IV glucose

150
Q

Cryoglobulinaemia

A
  • Igs which undergo reversible precipitation at 4 deg, dissolve when warmed to 37
  • 3 types - eg IgG/M, mixed, polyclonal….
    Px
  • Raynaud’s in type 1
  • Vascular purpura
  • Distal ulceration
  • Arthralgia
  • Renal involvement – glomerulonephritis
    Ix
  • low complement, esp C4
  • high ESR
    Mx
  • Tx underlying condition
  • immunosuppression
  • plasmapheresis
151
Q

Hereditary angioedema (HAE)

A
  • autosomal dominant - low levels of C1 inhibitor
  • bradykinin -> oedema
    Px
  • painful macular rash
  • painless, non-pruritic swelling
  • upper airway, skin, abdo organs
  • no urticaria
    Ix
    -C1-INH level low during attack
  • Low C2 and C4
    Mx
  • Acute - IV C1-inhibitor conc, FFP
  • prophylaxis - anabolic steroid danazol
152
Q

lead poisoning

A
  • Results in defective ferrochelatase and ALA dehydratase function
    Px
  • Abdo pain
  • Peripheral neuropathy – motor mainly
  • Neuropsych sx
  • Fatigue
  • Constipation
  • Blue lines on gum margin (20% adults)
    Ix
  • high serum lead
  • FBC - microcytic anaemia
  • Blood film - basophilic stippling, clover-lead morphology
  • raised serum + urine delta aminolaevulinic acid
  • increased urine coproporphyrin
    Mx
  • Chelating agents - DMSA, D-penicillamine, EDTA, dimercaprol
153
Q

neutropenia - definiton and causes

A
  • low neutrophil count <1.5

Causes
- viral
- drugs
- Benign ethnic neutropenia – common in black ethnicity, no tx
- Haem malignancies – myelodysplastic, aplastic
- Rheum
- SLE
- RA, eg hypersplenism in Felty’s syndrome
- Severe sepsis
- Haemodialysis

154
Q

hyposplenism - causes

A

Causes
- Splenectomy
- Sickle cell
- Coeliacs, dermatitis herpetiformis
- Graves
- SLE
- Amyloid

Features
- Howell-Jolly bodies
- Siderocytes

155
Q

splenomegaly causes

A

Massive splenomegaly
- Myelofibrosis
- CML
- Visceral leishmaniasis (kala-azar)
- Malaria
- Gaucher’s syndrome

Others
- Portal HTN
- CLL, Hodgkins
- Haemolytic anaemia
- Hepatitis, glandular fever
- IE
- Sickle cell (majority have atrophied spleen from repeated infarction)
- Thalassaemia
- RA – Felty syndrome

156
Q

post-thrombotic syndrome

A
  • cx after DVT
  • venous outflow obstruction + venous insufficiency -> chronic venous HTN
    Px
  • painful, heavy calves
  • pruritis
  • swelling
  • varicose veins
  • venous ulceration
    Mx
  • compression stockings
  • elevate leg
157
Q

thymoma

A

Most common tumour of anterior mediastinum
Usually 60-70yo

Associated with:
- Myasthenia gravis - 30-40% of pts with thymoma
- Red cell aplasia
- Dermatomyositis
- SLE, SIADH

Death from
- Airway compression
- Cardiac tamponade

158
Q

Wiskott-Aldrich syndrome

A
  • B/T cell dysfunction causing primary immunodeficiency
  • X-linked recessive, WASP gene mutation
    Px
  • recurrent infections, eg chest
  • eczema
  • thrombocytopenia
  • low IgM
159
Q

Graft-vs-host disease (GVHD) - definition and RF

A
  • Multi-system cx of allogeneic bone marrow transplantation (from host)
  • autologous - from own pt
  • also solid organ transplant / transfusion
  • T cells in donor tissue mount immune response to host cells

RFs
- poorly matched donor/recipient (HLA)
- types of conditioning prior to transplant
- gender disparity graft/host
- graft source - eg bone marrow / peripheral blood source

160
Q

Billingham criteria for dx of GVHD

A
  • Transplanted tissue contains immunologically functioning cells
  • Recipient + donor are immunologically different
  • Recipient immunocompromised
161
Q

acute GVHD

A
  • <100d post-transplant
  • skin, liver, GI tract
    Px
  • Painful maculopapular rash – may progress to erythroderma or TEN-like syndrome
  • Jaundice
  • Watery / bloody diarrhoea
  • Persistent N+V
  • Fever (culture negative)
162
Q

chronic GVHD

A
  • > 100d post-transplant
  • varied clinical picture - often lung + eye
    Px
  • skin, eye, GI, lung sx
163
Q

GVHD Ix

A
  • organ dependent
  • LFTs, hepatitis screen, USS, abdo imaging
  • PFTs
  • biopsy
164
Q

GVHD Mx

A
  • immunosuppression - IV steroids, biologics
  • supportive
  • prophylaxis - calcineurin inhibitors

finals (21 decks)

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