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Phase 4 Question Knowledge > Haematology > Flashcards

Haematology Flashcards

1
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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2
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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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

Infective

A
  • transmission of infection, eg vCJD

Px

  • Fever
  • rigors
  • hypotension
  • tachycardia
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9
Q

Delayed haemolytic transfusion reaction

A
  • fever, failure to respond to transfusion, unexplained bilirubin rise
  • Dx on lab tests
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10
Q

Post-transfusion purpura

A
  • immune mediated - 5-12d after transfusion
  • platelet drop
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11
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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12
Q

Anticoagulants reversal

A

See notes for pharm

dabigatran - idarucizumab

heparin - protamine sulfate

apixaban/rivaroxaban - andexanet alfa

edoxaban - ?andexanet alfa

warfarin - vit K, FFP, PCC

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13
Q

Anaemia

A
  • low Hb conc
  • Men Hb 130-180, women Hb 120-165
  • MCV 80-100
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14
Q

Anaemia causes

A

MCV<80 - microcytic

  • iron deficiency
  • anaemia of chronic disease
  • thalassaemia
  • congenital sideroblastic anaemia
  • lead poisoning

MCV80-100 - normocytic

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

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

Haemolytic anaemia

  • abnormal breakdown of RBCs
  • see reticulocytosis, increased bilirubin, LDH, mildly jaundiced…
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15
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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16
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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17
Q

Iron deficiency anaemia (IDA)

A
  • low Hb due to low iron
  • iron absorbed in duodenum/jejunum - needed for haem formation
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18
Q

IDA causes

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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19
Q

IDA Px

A
  • anaemia - fatigue, SOB, pallor, dizzy/faint, palpitations, headaches
  • brittle hair, nails
  • atrophic glossitis
  • koilonychia
  • angular stomatitis / cheilitis
  • pica
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20
Q

IDA 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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21
Q

IDA Mx

A
  • oral iron - ferrous sulfate / fumarate
  • iron-rich diet
  • iron infusions - IV CosmoFer
  • blood transfusion
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22
Q

Sideroblastic anaemia

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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23
Q

Anaemia of chronic disease

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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24
Q

Aplastic anaemia

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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25
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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26
Q

B12 deficiency causes

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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27
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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28
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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29
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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30
Q

Folate deficiency

A
  • (Vit B9) - absorbed in jejunum, found in green veggies
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31
Q

Folate deficiency causes

A
  • poor folate diet - poverty, alcoholics, elderly
  • malabsorption - Crohn’s, Coeliac, alcoholism
  • pregnancy
  • anti-folate drugs - methotrexate, trimethoprim, AEDs
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32
Q

Folate deficiency Px

A
  • anaemia sx
  • no neuropathy
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33
Q

Folate deficiency Ix

A
  • FBC, blood film - macrocytic anaemia, hypersegmented polymorphs
  • increased LDH
  • low reticulocyte count
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34
Q

Folate deficiency Mx

A
  • tx cause
  • folic acid
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35
Q

Haemolytic anaemia

A
  • premature breakdown of RBCs - intra/extravascular
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36
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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37
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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38
Q

Haemolytic anaemia general Px

A
  • anaemia sx
  • splenomegaly
  • jaundice
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39
Q

Haemolytic anaemia general Ix

A
  • FBC, blood film - normocytic anaemia, schistocytes
  • Direct Coombs test (DAT) - positive in AIHA
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40
Q

Autoimmune haemolytic anaemia (AIHA)

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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41
Q

Hereditary spherocytosis

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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42
Q

Hereditary elliptocytosis

A
  • as spherocytosis but RBCs ellipse-shaped
  • autosomal dominant
  • Px / Mx the same
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43
Q

G6PD deficiency

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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44
Q

Alloimmune haemolytic anaemia

A
  • in response to foreign ABs / RBCs

Haemolytic transfusion reactions

  • ABs produced against foreign RBCs in transfusion - destroys these RBCs

Haemolytic disease of newborn

  • 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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45
Q

Paroxysmal nocturnal haemoglobinuria

A
  • acquired genetic mutation - loss of proteins on RBC that inhibit complement - complement destroys RBCs

Px

  • red urine in morning
  • haemolytic anaemia
  • thrombosis - DVT, PE, hepatic vein thrombosis
  • smooth muscle dystonia

Ix

  • flow cytometry of blood
  • Ham’s test

Mx

  • eculizumab
  • blood transfusion
  • bone marrow transplantation
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46
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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47
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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48
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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49
Q

Sickle cell anaemia (SCA)

A
  • autosomal recessive - production of abnormal Hb leading to vaso-occlusive crises (disorder of quality)
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50
Q

SCA patho

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

Sickle cell crises

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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51
Q

SCA 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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52
Q

SCA 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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53
Q

SCA Mx

A
  • avoid triggers, dehydration
  • pneumococcal vaccine (and others)
  • pen V abx prophylaxis
  • hydroxycarbamide
  • crizanlizumab
  • blood transfusions
  • bone marrow transplant
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54
Q

Sickle cell crisis

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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55
Q

SCA Cx

A
  • anaemia
  • infections
  • CKD
  • sickle cell crises
  • stroke
  • avascular necrosis
  • hypoxia -> fibrosis -> pulm HTN
  • gallstones
  • priapism
  • splenic infarction, hyposplenism etc
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56
Q

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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57
Q

Thalassaemia patho

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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58
Q

Thalassaemia overall Px

A
  • Microcytic anaemia
  • Fatigue
  • Pallor
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Poor growth + development
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59
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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60
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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61
Q

B-thalassaemia minor / trait

A
  • asym, heterozygous carrier
  • mild/absent anaemia
  • monitor only
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62
Q

B-thalassaemia intermedia

A
  • 2 defective genes - 2 abnormal / 1 abnormal + 1 deletion
  • moderate anaemia
  • splenomegaly
  • transfusions, iron chelation
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63
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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64
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
65
Q

Thalassaemia Mx

A
  • exercise good diet
  • folate supplements
  • regular blood transfusions
  • desferrioxamine / ascorbic acid
  • splenectomy
  • hormonal tx for endocrine cx
  • bone marrow transplant
66
Q

Leukaemia

A
  • cancer of immature blast blood cells / stem cells - precursors of RBCs, platelets, WBCs
67
Q

Leukaemia patho

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
68
Q

Leukaemia general Px

A
  • Fatigue, fever
  • Pallor - anaemia
  • Petechiae / bruising
  • Abnormal bleeding
  • Lymphadenopathy
  • HSM
  • Failure to thrive
69
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
70
Q

Leukaemia general Mx

A
  • chemo
  • targeted therapy
  • bone marrow transplant
  • surgery
71
Q

Acute lymphoblastic leukaemia (ALL)

A
  • malignant proliferation of lymphoblasts (B/T cell precursors)
  • 80% childhood leukaemias
  • Some Philadelphia chromosome involvement
  • radiation, Down’s
72
Q

ALL Px

A
  • bone marrow failure - anaemia, infections, fever, bleeding, bruising
  • bone pain
  • HSM
  • lymphadenopathy
  • headache, CN palsies
  • fever
  • testicular swelling - unilateral
73
Q

ALL Ix

A
  • FBC - low Hb, platelets, WCC
  • Blood film - blast cells
  • Bone marrow aspirate - blast cells
  • CXR, CT, LP
74
Q

ALL Mx

A
  • blood / platelet transfusions
  • infection prophylaxis
  • chemo
  • marrow transplant
75
Q

ALL poor prognostic factors

A
  • Age <2yo, >10yo
  • WBC >20 at diagnosis
  • T/B cell surface markers
  • Non-Caucasian
  • Male
76
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
77
Q

AML Px

A
  • anaemia, infection, bleeding
  • bleeding gums, gum hypertrophy
  • bone pain
  • HSM
  • skin involvement
  • DIC
78
Q

AML Ix

A
  • FBC - increased WCC, anaemia, thrombocytopenia
  • Bone marrow biopsy - Auer rods, high proportion blast cells
79
Q

AML Mx

A
  • infection prophylaxis
  • blood / platelet transfusions
  • chemo
  • bone marrow transplant
80
Q

Chronic myeloid leukaemia (CML)

A
  • Malignant proliferation of myeloid cells (WBCs - neutrophils, basophils, eosinophils)
  • Adults 50-60yo
  • chronic, accelerated, blast phases
  • Philadelphia chromosome
81
Q

CML Px

A
  • Insidious onset
  • Fever, wt loss, fatigue
  • Gout (from purine breakdown)
  • Bleeding, bruising
  • Anaemia
  • HSM
82
Q

CML Ix

A
  • FBC - increased WCC, anaemia
  • Bone marrow biopsy - hypercellular
  • cytology
  • genetics
83
Q

CML Mx

A
  • chemo
  • imatinib
  • bone marrow transplant
84
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
85
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
86
Q

CLL Px

A
  • Often asym
  • Anaemia, infection
  • Wt loss, night sweats
  • Anorexia
  • HSM
  • Lymphadenopathy
87
Q

CLL Ix

A
  • FBC - anaemia, raised WCC, high lymphocytes, thrombocytopenia
  • Blood film - smudge / smear cells
88
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
89
Q

Tumour lysis syndrome

A
  • release of chemicals from cells destroyed by chemo
  • high grade lymphomas / leukaemias
90
Q

Tumour lysis syndrome patho

A
  • High uric acid -> form crystals in kidneys -> AKI
  • High K -> cardiac arrhythmias
  • High phosph
  • Low Ca - from high phos
  • Cytokines -> systemic inflammation
91
Q

Tumour lysis syndrome Px

A
  • suspect in AKI with high phos, high uric acid
92
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

93
Q

Tumour lysis syndrome Mx

A
  • good hydration / UO before chemo - IV fluids
  • allopurinol
  • rasburicase
94
Q

Lymphoma

A
  • cancer of lymphocytes in lymphatic system
  • proliferate in lymph nodes
95
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

96
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)

97
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
98
Q

Hodgkin’s lymphoma

A
  • specific type of lymphoma
  • 20-25yo, and 80yo
99
Q

Hodgkin’s patho

A
  • nearby contiguous spread, rarely extranodal, Reed-Sternberg cells

RFs

  • HIV, EBV, RA, sarcoidosis, FHx
100
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
101
Q

Hodgkin’s Ix

A
  • FBC - raised WCC, anaemia
  • LDH - high
  • Lymph node biopsy - Reed-Sternberg cells (multinucleated)
  • CT / MRI / PET, CXR
102
Q

Hodgkin’s Mx

A
  • Chemo
  • radiotherapy
  • stem cell transplant
  • surgical removal of lymph nodes
103
Q

Non-Hodgkin’s lymphoma

A
  • all other types of lymphoma
  • 80% B cell, 20% T cell
104
Q

Non-Hodgkin’s patho

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
105
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

106
Q

Non-Hodgkin’s Ix

A
  • FBC - raised WCC, low Hb/platelets
  • raised LDH
  • lymph node biopsy
  • CT / MRI / PET
107
Q

Non-Hodgkin’s Mx

A
  • chemo
  • pred
  • rituximab
  • radiotherapy
  • stem cell transplant
108
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
109
Q

Myeloma patho

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
110
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

111
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
112
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 levels
113
Q

Myeloma Mx

A
  • chemo
  • radio
  • stem cell transplant
  • analgesia
  • bisphosphonates
  • surgery
  • IV Ig
  • transfusion
114
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
115
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)
116
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
117
Q

Myeloproliferative disorders Mx

A

Primary myelofibrosis

  • supportive
  • chemo
  • ruxolitinib
  • allogenic stem cell transplant

Essential thrombocytosis

  • aspirin
  • chemo
  • anagrelide
118
Q

Polycythaemia

A
  • increase in RBC mass / Hb / packed cell volume
119
Q

Polycythaemia causes

A

Absolute

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

Relative

  • apparent polycythaemia - obesity, HTN, alcohol, smoking
  • dehydration
120
Q

Polycythaemia Px

A
  • asym
  • HTN
  • HSM
  • gout
  • VTE, arterial thrombosis
  • headaches
  • itching - after warmth
  • dizziness
  • tinnitus
  • visual disturbance
  • angina
  • red face
121
Q

Polycythaemia Ix

A
  • FBC - high Hb, raised WCC/platelets
  • ferritin low in primary
  • genetic screen
  • bone marrow biopsy
122
Q

Polycythaemia Mx

A
  • venesection
  • chemo
  • aspirin
123
Q

Thrombocytosis

A
  • high platelet count

Causes

  • reactive - stress, infection, surgery, IDA
  • malignancy
  • essential thrombocytosis
  • hyposplenism
124
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
125
Q

Thrombocytopenia

A
  • low platelet count - normal 150-450
  • from reduced production / increased destruction
126
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
127
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

128
Q

DDx abnormal bleeding

A
  • Thrombocytopenia
  • Von Willebrand disease
  • Haemophilia
  • DIC (usually secondary to sepsis)
129
Q

Immune thrombocytopenic purpura (ITP)

A
  • ABs created against platelets
  • T2 hypersensitivity
  • eg after infection, vaccination - more acute in children
  • adults - autoimmune disorders
130
Q

ITP Px

A
  • bruising
  • petechial / purpuric rash
  • epistaxis, gingival bleeding
131
Q

ITP Ix

A
  • FBC - low platelets
  • blood film
  • bone marrow biopsy - if atypical features
132
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
133
Q

Thrombotic thrombocytopenic purpura (TTP)

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
134
Q

TTP Px

A
  • florid purpura (bright red)
  • fever, fatigue
  • haemolytic anaemia, may have AKI
  • bruising, bleeding
  • confusion, headache, cerebral dysfunction - from microemboli
135
Q

TTP Ix

A
  • FBC - low platelets
  • U/E
  • LDH raised
  • blood film - schistocytes / fragmented RBCs
  • coag - normal
136
Q

TTP Mx

A
  • plasma exchange
  • steroids - IV methylprednisolone
  • rituximab
  • splenectomy
137
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
138
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

Mx

  • Mx if bleeding / operations
  • desmopressin
  • TXA
  • VWF infusion
  • Factor VIII
139
Q

Haemophilia

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

Px

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

Ix

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

Mx

A

  • desmopressin
  • recombinant factor 8 infusion
  • FFP - acute bleed
  • emicizumab

B

  • recombinant factor 9

Acquired

  • steroids
140
Q

Disseminated intravascular coagulation (DIC)

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

Causes

sepsis, trauma, advanced cancer, obstetric cx

Px

  • ill pt, shocked
  • bleeding, bruising
  • confusion
  • purpura, petechiae, infarctions

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
141
Q

Thrombophilia

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

142
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
143
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
144
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
145
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
146
Q

Methaemoglobinaemia

A
  • Hb where Fe2+ oxidised to Fe3+
  • normally regulated by NADH methaemoglobin reductase
  • Fe3+ cannot bind to O2 -> O2 dissociation curve moves left, tissue hypoxia

Causes

  • congenital
  • acquired - drugs, aniline dyes

Px

  • chocolate cyanosis
  • SOB, anxiety, headache
  • severe - acidosis, arrhythmias, seizures, coma
  • normal pO2, decreased O2 sats

Mx

  • enzyme deficiency - ascorbic acid
  • methylene blue if acquired
147
Q

Neutropenia

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
148
Q

Hyposplenism

A

Causes

  • Splenectomy
  • Sickle cell
  • Coeliacs, dermatitis herpetiformis
  • Graves
  • SLE
  • Amyloid

Features

  • Howell-Jolly bodies
  • Siderocytes
149
Q

Splenomegaly

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
150
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
151
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

152
Q

Waldenstrom’s macroglobulinaemia

A
  • Lymphoplasmacytoid malignancy – secretion of a monoclonal IgM paraprotein

Px

  • wt loss, lethargy
  • hyperviscosity syndrome - visual disturbance
  • HSM
  • lymphadenopathy
  • cryoglobulinaemia

Ix

  • Monoclonal IgM paraproteinaemia
  • Bone marrow biopsy - malignant cell infiltration

Mx

  • rituximab combo chemo
153
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
154
Q

Graft-vs-host disease (GVHD)

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
155
Q

Billingham criteria for dx of GVHD

A
  • Transplanted tissue contains immunologically functioning cells
  • Recipient + donor are immunologically different
  • Recipient immunocompromised
156
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)
157
Q

Chronic GVHD

A
  • > 100d post-transplant
  • varied clinical picture - often lung + eye

Px

  • skin, eye, GI, lung sx
158
Q

GVHD Ix

A
  • organ dependent
  • LFTs, hepatitis screen, USS, abdo imaging
  • PFTs
  • biopsy
159
Q

GVHD Mx

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

Phase 4 Question Knowledge (30 decks)

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