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Pathology > Haematology > Flashcards

Haematology Flashcards

1
Q

peripheral blood film: acanthocytes (spur/spike cells)
description, underlying condition

A

RBCs show many spicules

liver disease, hyposplenism, abetalipoproteinaemia (rare)

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2
Q

peripheral blood film: basophilic RBC stippling
description, underlying condition

A

accelerated erythropoiesis or defective Hb synthesis, small dots at the periphery are seen (rRNA)

lead poisoning, megaloblastic anaemia, myelodysplasia, liver disease, haemoglobinopathies e.g., thalassaemia

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3
Q

peripheral blood film: burr cells (echinocyte)
description, underlying condition

A

like a sea urchin with regular spicules

often an artefact if blood has sat in EDTA prior to film being made
uraemia, renal failure, GI bleeding, stomach carcinoma

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4
Q

peripheral blood film: heinz bodies
description, underlying condition

A

inclusions on very edge of RBCs due to denaturated Hb

glucose-6-phosphate dehydrogenase deficiency, chronic liver disease

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5
Q

peripheral blood film: Howell-Jolly bodies
description, underlying condition

A

basophilic (purple spot) nuclear remnants in RBCs
NB: much bigger purple spots in nucleated RBCs

post-splenectomy/hyposplenism (e.g., sickle cell disease, coeliac disease, congenital/UC/crohn’s, myeloproliferative disease, amyloid)
megaloblastic anaemia, hereditary spherocytosis

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6
Q

peripheral blood film: leucoerythroblastic
description, underlying condition

A

a phrase to denote the presence of nucleated red blood cells and myeloid precursors in peripheral blood

bone marrow infiltration i.e., myelofibrosis, malignancy

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7
Q

peripheral blood film: Pelger Huet cells
description, underlying condition

A

hyposegmented neutrophil with 2 lobes like a dumbbell
pseudo-pelger huet cells are also hypogranular

congenital (lamin B receptor mutation)
acquired (myelogenous leukaemia and myelodysplastic syndromes)

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8
Q

peripheral blood film: polychromasia
description, underlying condition

A

bluish red blood cells due to the presence of DNA. polychromatic cells are usually reticulocytes which are immature RBCs

usually increased naturally in response to shortened RBC life
increased in haemolytic anaemias
reduced in aplastic anaemias, chemo

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9
Q

peripheral blood film: right shift
description, underlying condition

A

hypermature white cells - hypersegmented polymorphs (>5 lobes to nucleus)

megaloblastic anaemia, uraemia, liver disease

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10
Q

peripheral blood film: rouleaux formation
description, underlying condition

A

red cells stacked on each other

chronic inflammation, paraproteinaemia, myeloma

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11
Q

peripheral blood film: schistocytes
description, underlying condition

A

fragmented parts of RBCs - typically irregularly shaped with sharp edges and no central pallor

microangiopathic anaemia e.g., DIC, haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura, pre-eclampsia

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12
Q

peripheral blood film: spherocytes
description, underlying condition

A

sphere shaped RBCs
often a little smaller

hereditary spherocytosis, autoimmune haemolytic anaemia

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13
Q

peripheral blood film: stomatocytes
description, underlying condition

A

central pallor is straight, curved or rod-like shape. RBCs appear as ‘smiling faces’ or ‘fish mouth’

can be an artefact during slide preparation.
if not: hereditary stomatocytosis, high alcohol intake, liver disease

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14
Q

peripheral blood film: target cells (codocytes)
description, underlying condition

A

bull’s-eye appearance in central pallor

liver disease, hyposplenism, thalassaemia, IDA

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15
Q

anaemia - causes, symptoms, signs

A

causes:
- reduced production of RBCs
- increased loss of RBCs (haemolytic anaemias)
- increased plasma volume (pregnancy)

symptoms:
- fatigue
- dyspnoea
- faintness
- palpitations
- headache
- tinnitus
- anorexia

signs:
- pallor
- in severe anaemia (Hb<80g/L): hyperdynaemic circulation e.g., tachycardia, flow murmurs (ejection-systolic loudest over apex) - heart failure

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16
Q

causes of microcytic anaemia

A
  • iron-deficiency anaemia
  • anaemia of chronic disease
  • sideroblastic anaemia
  • thalassaemia
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17
Q

causes of normocytic anaemia

A
  • acute blood loss
  • anaemia of chronic disease
  • BM failure
  • renal failure
  • hypothyroidism
  • haemolysis
  • pregnancy
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18
Q

causes of macrocytic anaemia

A

FATRBC
- fetus (pregnancy)
- antifolates (e.g., phenytoin)
- thyroid (hypothyroidism)
- reticulocytosis (release of larger immature cells e.g., within haemolysis)
- B12/folate deficiency
- cirrhosis (alcohol excess/liver disease)
- myelodysplastic syndromes

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19
Q

iron-deficiency anaemia - signs, blood film, causes

A

signs:
- koilonychia
- atrophic glossitis
- angular cheilitis
- post-cricoid webs
- brittle hair and nails

blood film:
- microcytic, hypochromic, anisocytosis (varying size), poikilocytosis (shape) pencil cells

causes:
- bleeding until proven otherwise
menorrhagia in young women

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20
Q

anaemia of chronic disease

A

cytokine driven inhibition of red cell production
- inflammatory markers like IFNs, TNF and IL1 reduce EPO receptor production (and thus EPO synthesis) by kidneys
- iron metabolism is dysregulated, IL6 and LPS stimulate the liver to make hepcidin, which decreases iron absorption from gut (by inhibiting transferrin) and also cause iron accumulation in macrophages

causes:
- chronic infection (e.g., TB, osteomyelitis)
- vasculitis
- rheumatoid arthritis
- malignancy

ferritin is high

NB: in renal failure; not cytokine driven but do to EPO deficiency

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21
Q

sideroblastic anaemia

A

ineffective erythropoiesis - iron loading (bone marrow) causing haemosiderosis (endocrine, liver and cardiac damage due to iron deposition)

diagnosis: ring sideroblasts seen in the bone marrow (erythroid precursors with iron deposited in mitochondria in a ring around the nucleus)

causes: myelodysplastic disorders, following chemotherapy, irradiation, alcohol excess, lead excess, anti-TB drugs or myeloproliferative disease

treatment: remove the cause and consider Pyridoxine (vit B6 promotes RBC production). consider giving EPO

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22
Q

is ferritin always reliable?

A

no, because it is an acute phase protein and increases with inflammation
check CRP with every ferritin you send
if there is an inflammatory state, transferrin saturations are more useful

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23
Q

pancytopenia investigation

A

examination for splenomegaly - associated w/ myelofibrosis and lymphoproliferative disorders

investigations:
- B12/folate/iron
- abdominal examination (splenomegaly)
- reticulocyte count (if low, think of aplastic anaemia, Fanconi anaemia, dyskeratosis congenita)
- blood film (look for abnormal cells e.g., blasts, haematological malignancies, dysplasia)
- myeloma screen
- parvovirus PCR
- ask for medications

unless there is a clear cause on the above tests, patients are likely to require a bone marrow biopsy to help diagnose

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24
Q

causes of macrocytosis

A

megaloblastic:
- B12/folate deficiency
- cytotoxic drugs

non-megaloblastic
- alcohol (most common cause of macrocytosis without anaemia)
- reticulocytosis (e.g., in haemolysis)
- liver disease
- hypothyroidism
- pregnancy

other haematological disease:
- myelodysplasia, myeloma, myeloproliferative disorders, aplastic anaemia

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25
Q

describe a megaloblastic blood film

A

hypersegmented polymorphs, leukopaenia, macrocytosis, anaemia, thrombocytopenia with megaloblasts.
megaloblasts are red cell precursors with an immature nucleus and mature cytoplasm.
B12 and folate are required for nucleus maturation.

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26
Q

sources of B12

A

meat and dairy
(we have large body stores)

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27
Q

causes of B12 deficiency

A

dietary (e.g., vegans)

malabsorption:
- stomach (lack of IF which is produced by gastric parietal cells; pernicious anaemia, post gastrectomy, achlorhydria-induced malabsorption)
- terminal ileum (absorption) due to ileal resection, Crohn’s, coeliac, bacterial overgrowth, tropical sprue and tapeworms
- nitrous oxide abuse

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28
Q

clinical features of B12 deficiency

A

mouth: glossitis, angular cheilitis
neuropsychiatric: irritability, depression, psychosis, dementia
neurological: paraesthesia, peripheral neuropathy

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29
Q

pernicious anaemia

A
  • autoimmune atrophic gastritis; achlorhydria and lack of intrinsic factor
  • most common cause of macrocytic anaemia in Western countries
  • specific tests: parietal cell antibodies (90%), intrinsic factor antibodies (50%), schilling test (outdated)

treatment:
- IM hydroxocobalamin (B12) in 6 injections over 2 weeks

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30
Q

folate sources

A

green vegetables, nuts, yeast & liver, synthesised by gut bacteria

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31
Q

causes of folate deficiency

A
  • poor diet
  • increased demand: pregnancy, increased cell turnover (haemolysis, malignancy, inflammatory disease, renal dialysis)
  • malabsorption (coeliac, tropical sprue)
  • drugs: alcohol, anti-epileptics (phenytoin), methotrexate, trimethoprim
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32
Q

treatment of folate deficiency

A

oral folic acid
ensure B12 is replaced prior to folic acid, otherwise folic acid may exacerbate the neuropathy of B12 deficiency and lead to subacute combined degeneration of the spinal cord

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33
Q

causes of haemolytic anaemia

A

Inherited
membrane defect:
- hereditary spherocytosis
- hereditary elliptocytosis
enzyme defect:
- G6PD deficiency
- pyruvate kinase deficiency
haemoglobinopathies:
- sickle cell disease
- thalassaemia

Acquired
immune:
- autoimmune - warm or cold
- alloimmune - haemolytic transfusion reactions
non-immune:
- mechanical e.g., metal vakves, trauma
- PNH, MAHA
- infections (i.e., Malaria), drugs

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34
Q

hereditary spherocytosis

A
  • autosomal dominant
  • spectrin or ankyrin deficiency (membrane proteins)
  • susceptible to effect of parvovirus B19 and often develop gallstones
  • extravascular haemolysis; splenomegaly
  • diagnosis: spherocytes, increased osmotic fragility (lysis in hypotonic solutions), [-ve DAT (Coombs) - not autoimmune Ab mediated], flow cytometry eosin-5’-maleimide (EMA) binding test
  • treatment: folic acid, some require splenectomy
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35
Q

hereditary elliptocytosis

A

almost all forms are autosomal dominant - spectrin mutations
- mostly asymptomatic but some forms can be more severe
- erythrocytes are elliptical in shape on blood film

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36
Q

glucose-6-phosphate dehydrogenase (G6PD) deficiency

A
  • most common RBC enzyme defect; X linked
  • prevalent in areas of malarial ethnicity (African/Mediterranean/Middle Eastern)
  • Attacks - rapid anaemia and jaundice, with bite cells and Heinz bodies (blue deposits, oxidised Hb)
  • precipitated by oxidants as G6PD helps RBCs make glutathione which protects them from oxidant damage
  • precipitants include: drugs, broad beans, acute stressors, moth balls, acute infection
  • intravascular haemolysis: dark urine

diagnosis:
- enzyme assay 2-3 months after a crisis: young RBCs may have sufficient enzyme so results may appear normal

treatment:
- avoid precipitants
- transfuse if severe, genetic screening (rare subtypes give chronic haemolysis for which splenectomy can be needed)

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37
Q

pyruvate kinase deficiency

A
  • autosomal recessive (but autosomal dominant has been observed with the disorder)
  • clinical features: can be severe neonatal jaundice, splenomegaly, haemolytic anaemia
  • treatment: most do not require treatment (can inc blood transfusion/splenectomy)
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38
Q

structure of haemoglobin molecule

A
  • 4 globins and heme
  • the 4 globins arrange around the heme molecule in 2 pairs
  • normally we have 4 alpha globin genes (2 from each parent)
  • normally we have 2 beta globin genes (1 from each parent)
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39
Q

which globin gene is affected in sickle cell disease and beta thalassaemia

A

beta globin genes

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40
Q
A
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41
Q

sickle cell disease diagnosis

A

sickle cells and target cells on blood film, sickle solubility test, Hb electrophoresis, Guthrie test (birth) to aid prompt pneumococcal prophylaxis (+FHx)

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42
Q

chronic treatment of sickle cell disease

A

all should be on:
- penicillin V
- pneumovax
- HIB vaccine
- folic acid

some benefit from:
- hydroxycarbamide (increases HbF%); reduces crises and prevent organ damage e.g., kidney, heart
- regular exchange blood transfusions
- carotid Doppler monitoring in early childhood with prophylactic exchange transfusion if turbulent carotid flow
- Crizanlizumab - recently approved by NICE, reduces painful crises
- voxelotor: increases haemoglobin, not frequently used
- allogeneic stem cell transplant (not funded in the UK in adults but done in other countries or considered in children with good sibling donors)

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43
Q

beta thalassaemia

A

point mutations, reduced beta chain synthesis (spectrum of disease), excess alpha chains
increased HbA2 and HbF

skull bossing
maxillary hypertrophy
hair-on-end skull X-ray
hepatosplenomegaly

varying severity
- B0 - no expression of gene
- B+ - some expression of gene
- B - normal gene

beta thalassaemia minor - asymptomatic carrier, mild hypochromic microcytic anaemia

beta-thalassaemia intermedia - moderate anaemia, splenomegaly, bone deformity, gallstones

beta-thalassaemia major - 3-6mnths severe microcytic anaemia, absent HbA, failure to thrive, hepatosplenomegaly, bone deformity, severe anaemia + heart failure

diagnosis: Hb electrophoresis

treatment:
- minor and some intermedia forms may not need regular treatment
- blood transfusions with iron chelation (desferrioxamine) to stop iron overload, plus folic acid
- regular screening for iron overload in heart and liver

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44
Q

alpha thalassaemia

A

deletions - reduced alpha-chain synthesis, excess beta-chains

4 alpha genes, severity depends on number deleted

  • alpha-thalassaemia trait (1/2 deleted); asymptomatic, mild anaemia
  • HbH disease (3 deleted); moderate anaemia, splenomegaly
  • haemoglobin Bart’s hydrops foetalis (4 deleted); incompatible with life and death in utero
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45
Q

warm autoimmune haemolytic anaemia

A

features:
- 37 degrees Celcius
- IgG
- positive Coombs test
- blood film-spherocytes

causes:
- mainly primary idiopathic
- lymphoma, CLL, SLE, methyldopa

management:
- steroids
- splenectomy
- immunosuppression (Rituximab)

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46
Q

autoimmune haemolytic anaemia - cold agglutinin disease

A

features:
- <37 degrees celcius
- IgM
- positive coombs test
- often with Raynaud’s and acrocyanosis

causes:
- primary idiopathic
- lymphoma, infections: EBV, mycoplasma

management:
- treat underlying condition
- avoid the cold
- chemotherapy if lymphoma

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47
Q

paroxysmal cold haemoglobinuria (PCH)

A
  • haemoglobin in the urine usually caused by a viral infection e.g., measles, syphilis, VZV
  • Donath-Landsteiner antibodies - stick to RBCs in cold - complement-mediated haemolysis on rewarming (self-limiting as IgG so dissociate at higher temp than IgM)
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48
Q

paroxysmal nocturnal haemoglobinuria

A

non-immune (Coombs -ve)
very rare

  • acquired loss of protective surface GPI markers on RBCs (platelets + neutrophils); complement-mediated lysis; chronic intravascular haemolysis, especially at night
  • morning haemoglobinuria, thrombosis (+Budd-Chiary syndrome - hepatic vein thrombosis)
  • diagnosis: immunophenotype shows altered GPI or Ham’s test (in vitro acid-induced lysis)
  • treatment: iron/folate supplements, prophylactic vaccines/antibiotics. expensive monoclonal antibodies (eculizumab) that prevents complement from binding RBCs
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49
Q

microangiopathic haemolytic anaemia (MAHA)

A

mechanical RBC destruction (forced through fibrin/plt mesh in damaged vessels); schistocytes

causes:
- HUS, TTP, DIC, pre-eclampsia, eclampsia

management: usually plasma exchange

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50
Q

thrombotic thrombocytopenic purpura (TTP) symptoms

A
  • anaemia [microangiopathic haemolytic anaemia (MAHA)]
  • fever
  • renal impairment (less pronounced than HUS)
  • neuro abnormalities
  • thrombocytopenia

haematological emergency! urgent plasma exchange

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51
Q

haemolytic uraemic syndrome

A
  • typically caused by Shiga toxin - producing E coli 0157:H7
  • toxin damages endothelial cells, forms fibrin mesh and damages RBCs + impaired renal function + microangiopathic haemolytic anaemia + thrombocytopenia
  • diarrhoea, renal failure, no neuro problems, children and elderly
  • treatment: supportive (fluids, blood transfusion, and dialysis if required), plasma exchange, eculizumab (a C5 inhibitor monoclonal antibody)
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52
Q

intrinsic pathway of coagulation cascade

A
  • activated partial thromboplastin time (APTT): monitor heparin therapy
  • starts with factor TWELVE
  • remember the next factor starts with the last letter of the previous factor

TWELVE, ELEVEN, NINE, EIGHT, TEN

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53
Q

extrinsic pathway of coagulation

A
  • prothrombin time (PT) - monitor warfarin therapy (INR)
  • starts with factor SEVEN
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54
Q

common pathway of coagulation

A
  • thrombin time (TT)
  • starts with activated factor FIVE
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55
Q

differences in clinical presentation of vascular/platelet defects and coagulation disorders

A

platelet/vascular:
- superficial bleeding into skin, mucosal membranes
- bleeding immediate after injury

coagulation:
- bleeding into deep tissues, muscles, joints
- delayed, but severe bleeding after injury
- bleeding often prolonged

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56
Q

platelet disorders

A

reduced platelet function
acquired:
- aspirin, cardiopulmonary bypass
- uraemia
congenital:
- storage pool disease
- thrombasthenia (glycoprotein deficiency)

decreased production
- BM failure

increased destruction
- auto-immune thrombocytopenic purpura (AITP) - formally idiopathic (ITP)
- drugs (e.g., hepatin-induced thrombocytopenia), DIC, HUS, TTP

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57
Q

haemophilia A & haemophilia B

A

Haemophilia A
- factor VIII deficiency
- X-linked recessive affecting 1/10,000 males
- presentation: often early in life or prolonged bleeding after surgery/trauma

diagnosis:
- increased APTT
- normal PT
- reduced factor VIII assay

management: avoid NSAIDs and IM injections, prophylaxis with factor VIII in more severe cases or treatment only for bleeds in milder cases

Haemophilia B
- factor IX deficiency
- X-linked recessive affecting 1/50,000 males
- clinically like haemophilia A

management: factor IX concentrates either as prophylaxis or just for bleeds

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58
Q

types of Von Willebrand’s disease + presentation

A

variable phenotype from complete deficiency to asymptomatic mild deficiency
- type 1: low levels of VWF
- type 2: deficiency in function of VWF compared to level (mutations causing poor function)
- type 3: absent of VWF - can present like haemophilia

presentation: often bleeding indicative of platelet disorders (i.e., mucocutaneous bleeding) but can also include bleeding indicative of coagulation disorders

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59
Q

diagnosis of Von Willebrand’s Disease

A
  • can be difficult to diagnose particularly type 2 - would need haematology input!
  • increased APTT, normal PT/INR (but both APTT + INR may be completely normal)
  • reduced factor VIII
  • reduced vWF Ag (or normal antigen level with reduced function in type 2)
  • normal platelet count
  • ristocetin co-factor
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60
Q

management of Von Willebrand’s disease

A
  • prophylaxis is indicated in some patients
  • treatment of bleeds: tranexamic acid, desmopressin (some patients respond - raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells), combined VWF and factor VIII concentrates
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61
Q

disseminated intravascular coagulation (DIC)

A
  • widespread activation of coagulation
  • clotting factors and platelets are consumed; increased risk of bleeding
  • causes: malignancy, sepsis, trauma, obstetric complications, toxins
  • low plts, low fibrinogen, high FDP/D-dimer, prolonged PT/INR
  • treat the cause and give transfusions, FFP, platelets, cryo, etc
62
Q

coagulation disorders due to liver disease

A
  • reduced synthesis of II, V, VII, IX, X, XI and fibrinogen
  • high levels of VIII/VWF
  • reduced absorption of vitamin K
  • abnormalities of platelet function
  • note that chronic liver disease is often a prothrombotic state despite having prolonged INR/APTT
63
Q

coagulation disorder - vitamin K deficiency

A
  • vit K needed for synthesis of factors II, VII, IX and X
  • and protein C/S (this is why warfarin may be pro-coagulant initially)
  • causes: warfarin, vitamin K malabsorption/malnutrition, Abx therapy, biliary obstruction
  • treatment: IV vitamin K or FFP for acute haemorrhage
64
Q

venous thrombosis - Virchow’s triad

A
  1. endothelial injury
  2. stasis
  3. hypercoagulability
65
Q

wells DVT calculator, wells PE calc

A

High Wells score (>4 points):
- ultrasound affected limb for DVT/CTPA for PE

Intermediate Wells score (4 points or less):
- D-Dimer: if high, ultrasound/CTPA. if low, rule out

Low Wells score:
- consider other diagnosis

66
Q

risk factors for venous thrombosis

A

inherited:
- antithrombin deficiency
- protein C deficiency
- protein S deficiency
- Factor V Leiden
- prothrombin G20210A
- lupus anticoagulant
- coag excess - VIII (10%), II (2%), fibrinogen

acquired:
- age, obesity
- previous DVT/PE
- immobilisation
- major surgery - esp. ortho, >30 mins, plaster cast immobilisation
- long distance travel
- malignancy - esp. pancreas
- pregnancy, COCP, HRT
- antiphospholipid syndrome
- polycythaemia
- thrombocythaemia

67
Q

prevention and treatment of VTE

A

DVT prophylaxis:
- daily subcutaneous LMWH (prophylactic dose), TED stockings
NB: some DOACs are now licensed for DVT prophylaxis e.g., in post-op ortho patients

treatment of DVT/PE:
- LMWH (treatment dose) followed by warfarin or apixaban/rivaroxaban/edoxaban (DOACs)
- LMWH stopped once INR in therapeutic range (2-3) (with some DOACs LMWH can be stopped immediately)
- reason for continuing LMWH while warfarin started: warfarin also affects protein C/S and often leads to procoagulant state in the first few days before anticoagulant effect

68
Q

duration of treatment for DVT/PE

A
  • 3 months minimum
  • for clearly provoked VTE consider stop at this point
  • unprovoked VTE needs anticoagulation for 6 months
  • otherwise, needs a clinical decision to be made: there are risk stratification tools used for this. young men and patients with high baseline D-dimer are at greater risk
  • recurrent VTE usually needs lifelong treatment
69
Q

heparin

A
  • potentiates antithrombin III which inactivates thrombin and factors 9, 10, 11
  • LMWH: give SC once daily, does not require monitoring (except late pregnancy and renal failure when anti-Xa levels can be monitored)
  • unfractionated heparin (used if renal impairment): given IV, loading dose then infusion, monitor APTT
  • antidote: profamine sulphate
  • side effects: bleeding and heparin induced thrombocytopenia (HIT), osteoporosis with long-term use (HIT and osteoporosis more common with UFH)
70
Q

warfarin

A
  • inhibits the vitamin K epoxide reductase enzyme responsible for regenerating the active form of vitamin K and therefore inhibits the synthesis of factors 2, 7, 9, 10 and protein C, S and Z
  • risk of teratogenicity, skin necrosis and purple toes

reversal:
- IV vitamin K (takes 6 hours)
- prothrombin complex concentrate (Octaplex/Beriplex; takes 30 mins)

  • dose adjusted to maintain INR in therapeutic range
  • factors that may potentiate warfarin: liver disease, P450 enzyme inhibitors (amiodarone, ciprofloxacin), cranbery juice, NSAIDs
71
Q

target INR post-DVT/PE

A

2.5
- 1st episode DVT/PE, atrial fibrillation (2-3), cardiomyopathy, symptomatic inherited thrombophilia, mural thrombus, cardioversion

3.5
- recurrent DVT or PE, mechanical prosthetic valve (2.5-3.5), coronary artery graft thrombosis, antiphospholipid syndrome

72
Q

anticoagulation post-DVT/PE: what to do in cases of raised INR?

A

5-8, no bleeding:
- withold few doses, reduce maintenance
- restart when INR < 5

5-8, minor bleeding:
- stop warfarin
- vit K slow IV
- restart when INR < 5

> 8, no bleed/minor bleed:
- stop warfarin
- vit K (oral/IV)
- check INR daily

major bleeding (inc. intracranial haemorrhage):
- stop warfarin
- give prothrombin complex concentrate
- if unavailable, give FFP
- also give vit K IV

73
Q

bleeding and DOACs

A

life/organ threatening bleeds:
- a normal APTT/PT does not exclude presence of an anticoagulant effect
- depends on half life of particular agent as to whether effect likely present
- dabigatran - idracizumab can be used to reverse depending on local availability
- rivaroxaban and apixaban - andexanet alfa can be used but most trusts do not have this due to high cost. prothrombin complex concentrate is often used instead

non-life threatening bleeds, pre-op:
- half-lives are approximately 12 hours so withholding doses may be enough

74
Q

haematological changes in pregnancy

A

increase:
- plasma volume
- red cell mass
- MCV
- WCC
- factors VII, VIII, IX, X, XII

reduce:
- haemoglobin
- haematocrit
- platelets
- factor XI
- protein S

75
Q

HELLP syndrome

A
  • haemolysis, elevated liver enzymes, low plateelts
  • life-threatening complication associated with pregnancy, specifically pre-eclampsia
  • key features - MAHA, increased AST, increased ALT, low platelets, normal APTT, PT
  • differentials include DIC (increased APTT, increased PT, low fibrinogen), AFLP (marked transaminitis)
  • management: supportive, delivery of foetus
76
Q

haemolytic disease of the newborn (HDN)

A
  • usually only a problem in 2nd pregnancy
  • in an RhD-ve mother’s first pregnancy, she may be exposed to foetal RhD Ag (RhD +ve)
  • as a result, the mother becomes sensitised and forms RhD antibodies
  • during a subsequent pregnancy, if the foetus is RhD +ve, maternal antibodies can destroy foetal red cells -> foetal anaemia + jaundice
  • only IgG can cross placenta
  • Ab most often responsible is anti-D, therefore always transfuse RhD negative blood to RhD negative women of childbearing age
  • other Abs: anti-c, anti-K, IgG ABO

preventing anti-D formation:
in women who are RhD negative
give intra-muscular anti-D Ig to:
- women at high risk of feto-maternal haemorrhage
- routine antenatal prophylaxis at 28 and 34 weeks
- during pregnancy if sensitising event occurs (abortion, miscarriage, abdo trauma, ECV, amniocentesis)
- at delivery if baby is RhD positive

77
Q

clinical features of acute leukaemia (ALL, AML)

A

bone marrow function failure (common to many haematological processes)
1. anaemia
2. thrombocytopaenia (bleeding)
3. neutropenia (infection)

organ infiltration
1. hepatosplenomegaly
2. lymphadenopathy
3. others: bone pain, CNS, skin, gum hypetrophy

78
Q

aetiology of acute leukaemia (ALL, AML)

A
  • unknown, most of the time no clear triggers
  • ionising radiation; radiotherapy
  • cytotoxic drugs; chemotherapy
  • pre-leukaemic disorders e.g., myelodysplastic syndromes (MDS)/myeloproliferative disorders (MPD)
  • Down’s: significantly increased risk of AML/ALL (AML>ALL)
  • neonates: often (30%) develop transient abnormal myelopoiesis (TAM); resembles AML but resolves spontaneously and completely after few weeks
79
Q

diagnosis of haematological malignancy

A
  • morphology +/- cytochemistry (stains)
  • immunophenotyping using flow cytometry (lineage, differenatiation)
  • cytogenetics (chromosomal translocations)
  • molecular genetics (PCR, point mutations etc)
80
Q

differences in clinical features between ALL and AML

A

ALL:
- lymphadenopathy +++
- CNS involvement +++
- testicular enlargement
- thymic enlargement (mediastinum)

“extra-medullary” disease more common i.e., solid leukaemia deposits outside in bone marrow

AML:
- lymphadenopathy is less common

81
Q

differences in investigation findings between ALL and AML

A

ALL:
- high WCC (blasts)
- blasts often have tails/blebs of cytoplasm
flow cytometry:
- CD34 = precursor/stem cells
- CD3, 4, 8 = T lymphocytes

AML:
- high WCC (blasts)
- Auer rods and granules
(Auer rods are not always present)
Auer rods are cytoplasmic inclusions containing enzymes such as MPO
flow cytometry:
CD34 = precursor/stem cells, CD33, CD13, CD117, MPO= myeloid cells

82
Q

differences in treatment between ALL and AML

A

ALL:
chemotherapy
- remission induction: chemo agents often given with steroids
- consolidation: high dose multi drug chemotherapy. CNS treatment (intrathecal chemo)
- maintenance: 2 years in girls and adults, 3 years in boys
consider allo-stem cell transplant if high risk of relapse

supportive: blood products, ABx, allopurinol, fluid, electrolytes - to prevent tumour lysis syndrome

AML:
chemotherapy
- remission induction: daunorubicin, cytarabine
- consolidation: cytarabine
- no CNS prophylaxis/maintenance therapy needed usually
- consider allo-SCT if high risk of relapse

supportive: similar principles to ALL
prognosis worse with age

83
Q

summary of chronic myeloid leukaemia (CML)

A
  • a myeloproliferative disease
  • middle-aged typically (40 to 60)
  • often diagnosed on routine bloods (large number of differentiated neutrophils)
  • or present with generally feeling unwell/weight loss/infection/bruising
  • 95% remission rate with imatinib
  • O/E: splenomegaly - (“massive splenomegaly”)
84
Q

investigations for chronic myeloid leukaemia (CML)

A
  • Ph+ve (philadelphia chromosome) in 80% = chromosomal translocation (9;22)
  • PCR for BCR-ABL (philadelphia Chr) fusion gene; monitor disease and therapeutic response by monitoring BCR-ABL levels
  • high white blood cell count with high neutrophils and high basophils
  • hypercellular BM with spectrum of immature (e.g., myelocytes) and mature granulocytic cells in the blood
85
Q

stages of CML

A

stage 1 - chronic phase
<5% blasts
Tx = imatinib (BCR-ABL tyrosine kinase inhibitor)

stage 2 - accelerated phase
10-20% blasts
less responsive to therapy

stage 3 - blast phase
>20% blast (resembles AML)
Tx = similar to AML
young patients can be possibly tx with allogenic SCT

86
Q

chronic lymphocytic leukaemia - background

A
  • a “lymphoproliferative disease” like lymphoma
  • CLL and small lymphcytic lymphoma (SLL) are essentially the same disease process with slightly different presentations - CLL is primarily seen in the BM, SLL in the LNs
  • M>F, elderly (median 65-70)
87
Q

clinical features of CLL

A
  • may be asymptomatic, often diagnosed on routine blood tests `(80%)
  • symmetrical painless lymphadenopathy
  • BM failure - anaemia & thrombocytopenia symptoms, recurrent infections (50% deaths)
  • hepatosplenomegaly
  • FLAWS

associated with autoimmunity (Evan’s syndrome) - AIHA, ITP
Richter’s transformation - whereby CLL transforms to more aggressive large cell lymphoma

88
Q

chronic lymphocytic leukaemia (CLL) investigations

A

high WBC with lymphocytosis >5 (high % of WBC composed of lymphocytes, small mature)
low serum immunoglobulin

flow cytometry to confirm a monoclonal population
- usually CD5+ CD23+

blood film
- smear cells - seen on blood film Ix

mutation status:
- TP53 = worse
- IGHV rearrangement = better

89
Q

chronic lymphocytic leukaemia (CLL) staging

A

Binet staging A, B & C (Rai staging I-IV could also be used)

Stage A
- high WBC
- <3 groups of enlarged lymph nodes
- usually no treatment required

Stage B
- >3 groups of enlarged lymph nodes

Stage C
- anaemia or thrombocytopenia

90
Q

CLL treatment

A
  • many patients benefit from watchful waiting if they are asymptomatic with slowly progressive disease
  • 1/3 patients never need any treatment
  • supportive treatment with transfusions, infection prophylaxis
  • options are: anti-CD20 (rituximab or Obinutuzumab) with chemotherapy; oral BTK inhibitors (ibrutinib); BCL2 inhibitor (venetoclax)
91
Q

clinical features of Hodgkin’s lymphoma

A
  • asymmetrical painless lymphadenopathy +/- obstructive/mass effect symptoms
  • B symptoms:
    fever
    drenching sweats at night
    weight loss >10% in 6 months
  • pain in affected nodes after alcohol
  • nodes tend to be mediastinal/cervical but not always
92
Q

hallmark cell for Hodgkin’s

A

Reed-Sternberg cell
(bi-nucleate/multinucleate ‘owel eyed’ cell on a background of lymphocytes & reactive cells)

93
Q

subtypes of Hodgkin’s lymphoma

A

classical 95% (in order of prevalence):
1. nodular sclerosing (NS)
2. mixed cellularity (MC)
3. lymphocyte rich (LR)
4. lymphocyte depleted (LD)

non-classical 5%
1. nodular lymphocyte predominant

94
Q

Ann-Arbor staging for Hodgkin’s lymphoma

A

stage 1 - one LN region (LN region can include spleen)

stage 2 - 2 or more LN regions on the same side of the diaphragm

stage 3 - 2 or more LN regions on opposite sides of the diaphragm

stage 4 - extra nodal sites (liver, BM)

A: no constitutional symptoms
B: constitutional symptoms

95
Q

treatment for Hodgkin’s lymphoma

A

prognosis excellent, esp in the young but intensive treatment

  1. combination chemotherapy
  2. radiotherapy (often used alongside chemo in bulky areas or limited disease - very high risk of breast cancer in women)
  3. relapsed patients
    - 2nd line chemotherapy agents
    - may need autologous or allogeneic stem cell transplant
96
Q

where are stem cells harvested for for stem cell/BM transplant?

A
  • peripheral blood (following stimulation by G-CSF)
  • BM
  • umbilical cord blood
97
Q

autologous SCT

A
  • patients own SCs are harvested and frozen
  • enables high dose chemo +/- radiotherapy to eradicate malignant cells at the cost of partial or even complete bone marrow ablation
  • frozen SCs then reintroduced into patient
  • used more in multiple myeloma and lymphoma, particularly with relapse, not used in leukaemia so much
  • no “graft vs leukaemia” effect
  • no graft versus host disease (GVHD) risk and lower risk of infection
98
Q

allogeneic SCT

A
  • HLA-matched donor SCs are harvested
  • patients own BM completely eradicated by high-dose chemo +/- radiotherapy
  • donor SCs are introduced and colonise “empty” BM
  • used more in leukaemia due to “graft vs leukaemia” effect (graft manipulation allowing doner immune cells to eliminate host leukaemic cells)
  • GVHD risk, risk of opportunistic infections, infertility and secondary malignancies
99
Q

high and low grade lymphomas

A

high grade:
- very aggressive - Burkitt’s
- aggressive - diffuse large B cell, mantle cell

low grade:
- indolent - follicular, marginal zone, small lymphocytic

NB: higher grade lymphomas are easier to treat!

100
Q

Burkitt’s lymphoma

A

3 types:
- all very aggressive, fast growing
- t(8;14) translocation
- c-myc oncogene expression
- rapidly responsive to Rx

endemic:
- most common malignancy in equatorial Africa
- EBV-associated
- characteristic jaw involvement and abdominal jasses

sporadic:
- found outside of Africa
- EBV-associated
- jaw less commonly involved

immunodeficiency:
- non-EBV-associated
- HIV/post-transplant patients

histology:
- “starry sky” appearance
(macrophages filled with cellular debris)

treatment:
- chemotherapy (rituximab) & secondary CNS prophylaxis

101
Q

diffuse large B-cell lymphoma

A
  • middle aged/elderly
  • aggressive
  • can be transformed from low grade lymphoma

histology:
- sheets of large lymphoid cells

treatment:
- rituximab-CHOP
- auto-SCT or CAR-T for relapse

NB: R-CHOP= rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone

102
Q

Mantle cell lymphoma

A
  • middle aged, M>F
  • aggressive
  • disseminated at presentation
  • median survival 3-5 yrs
  • t(11;14) translocation
  • cyclin D1 deregulation

histopathology:
- “angular/clefted nuclei”

treatment:
- rituximab-CHOP and high dose cytarabine
- auto-SCT for relapse
- oral options for less fit

NB: R-CHOP= rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone

103
Q

follicular lymphoma

A
  • indolent
  • mostly incurable
  • median survival 12-15 urs
  • t(14;18) translocation

histology:
- “follicular pattern”
- “nodular appearance”

treatment:
- watch and wait
- rituximab or obinutuzumab + chemo

104
Q

mucosal associated lymphoid tissue (MALT) lymphoma

A
  • marginal zone NHL
  • middle-aged
  • chronic antigen stimulation:
    H pylori - gastric MALT lymphoma
    Sjogren’s syndrome - parotid lymphoma

treatment:
- remove antigenic stimulus e.g., H pylori triple therapy
- chemotherapy

105
Q

T cell lymphomas

A
  • anaplastic large cell lymphoma
  • peripheral T-cell lymphoma
  • adult T cell leukaemia/lymphoma
  • enteropathy-associated T cell lymphoma (EATL)
  • cutaneous T cell lymphoma
106
Q

anaplastic large cell lymphoma

A

T cell lymphoma
- children and young adults
- aggressive
- large “epithelioid” lymphocytes
- t(2;5)
- Alk-1 protein expression

107
Q

peripheral T cell lymphoma

A
  • middle-aged and elderly
  • aggressive
  • large T cells
108
Q

adult T cell leukaemia/lymphoma

A
  • caribbean and japanese
  • HTLV-1 infection, aggressive
109
Q

enteropathy-associated T cell lymphoma (EATL)

A

associated with longstanding coeliac disease

110
Q

cutaneous T cell lymphoma

A

associated with mycosis fungoides

111
Q

burkitt’s lymphoma translocation

A

t(8;14)

112
Q

mantle cell lymphoma translocation

A

t(11;14)

113
Q

follicular cell lymphoma translocation

A

t(14;18)

114
Q

anaplastic large cell lymphoma translocation

A

t(2;5)

115
Q

multiple myeloma clinical features

A

CRAB:
- calcium high
thirst, moans, groans, stones, bones
myeloma cells release osteoclastic bone resorption - promoting cytokines such as IL-1

  • renal failure (plus amyloidosis and nephrotic syndrome)
    deposition of light chains within renal tubules
  • anaemia (+pancytopaenia)
  • bones
    pain, osteoporosis, osteolytic lesions, fractures e.g., wedge compression, pepper pot skull
    + hyperviscosity syndrome
116
Q

investigations for multiple myeloma

A

dense narrow band on serum electrophoresis (compared with broad band in polyclonal)
- in “gamma region”
- will then be identified as IgG/IgM/IgA/IgD/IgE
- will be identified as either kappa or lambda light chain

blood film: Rouleaux (RBC stacking)

look for CRAB symptoms:
- bone profile to check calcium
- U&Es to assess renal function
- FBC for anaemia
- low dose CT body/MRI whole body to look for bony lesions
- X-rays: ‘rain-drop skull’

Bence-Jones protein in urine

ESR very high

> 10% plasma cells in BM

staging: Durie-Salmon staging system/ISS

117
Q

treatment for multiple myeloma

A
  • supportive for CRAB symptoms inc. bisphosphonates e.g., alendronate
  • aim of treatment: induce remission for consideration of autologous stem cell transplant which will prolong remission
  • not curable
  • average survival 5-7 yrs but improving with new treatments

options:
- 1st line - Bortezomib +/- dexamethasone, cyclophosphamide, lenalidomide
- when in remission; auto-SCT - best for younger patients as prolongs remission
- if not suitable for SCT - multiple other new agents e.g., daratumumab (anti-CD38) carfilzomb/ixazomib (protease inhibitors) panobiostat

118
Q

Waldenstrom’s macroglobinaemia (lymphoblasmacytoid lymphoma - LPL)

A
  • elderly men typically
  • low-grade NHL; lymphoplasmacytoid cells producing monoclonal serum IgM infiltrate the LNs/BM
  • weight loss, fatigue, hyperviscosity syndrome (visual problems, confusion, CCF, muscle weakness)
  • associated with Raynaud’s

treatment: plasmapheresis for hyperviscosity; rituximab/bedamustine or ibrutinib for active disease

119
Q

stain (and result) to confirm amyloidosis on biopsy

A

congo-red stain –> apple green birefringence

120
Q

systemic amyloidosis

A
  • different types of amyloidosis
  • AL amyloidosis is due to build of mis-folded light chains
  • this can be in the presence or absence of myeloma
  • misfolded light chains deposit in the tissues & cause problems
  • other types of amyloidosis involve different types of misfolded proteins AL amyloidosis will result in an abnormal kappa:lamba light chain ratio
  • definitely diagnosed via biopsy of affected organ using congo-red stain –> apple green birefringence
  • new diagnostic test is the serum amyloid precursor (SAP) scan at the national amyloidosis centre at the Royal Free
  • presents with macroglossia, carpal tunnel syndrome, peripheral neuropathy, HF, RF
121
Q

what are myelodysplastic syndromes?

A

heterogeneous group of progressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells
- characterised by: peripheral cytopenia; qualitative abnormalities of cell maturation; risk of AML transformation
- typically seen in the elderly; symptoms usually develop over weeks/months (incidental)
- by definition all patients have <20% blasts (>20% blasts = acute leukaemia)

122
Q

clinical features of myelodysplastic syndromes

A
  • BM failure and cytopenias: infection, bleeding, fatigue
  • hypercellular BM
  • defective cells:
    a. RBCs e.g., ring sideroblasts (nucleated blast surrounded by iron granule ring)
    b. WBCs; hypogranulation. pseudo-pelger-huet anomaly (hyposegmented neutro)
    c. platelets; micromegakaryocytes, hypolobated nuclei
123
Q

treatment of myelodysplastic syndromes

A
  • supportive - tranfusions, EPO, C-CSF, ABx
  • biological modifiers - immunosuppressive drugs, lenalidomide, azacytidine
  • chemotherapy - similar to AML
  • allogeneic SCT
124
Q

what conditions is aplastic anaemia closely linked to?

A

leukaemia
paroxysmal nocturnal haemoglobinuria (PNH)

125
Q

classification of aplastic anaemia

A

primary:
- idiopathic (70%) - vast majority unexplained pathology but increasingly finding mutations with NGS
- inherited (10%)

secondary (10-15%)
- due to malignant infiltration, radiation, drugs inc. chemo, viruses, AI e.g., SLE

126
Q

management of aplastic anaemia

A
  • supportive - transfusions, ABx, iron chelation
  • drugs - to promote marrow recovery - growth factors and oxymethalone (androgen)
  • immunosuppressants - idiopathic AA
  • stem cell transplant
127
Q

4 types of inherited aplastic anaemia/BM failure syndrome

A
  • Fanconi anaemia
  • Dyskeratosis congenita
  • Schwachman-Diamond syndrome
  • Diamond-Blackfan syndrome
128
Q

fanconi anaemia

A
  • autosomal recessive
  • pancytopenia
  • presents at 5-10 yrs
  • skeletal abnormalities (radii, thumbs), renal malformations, microophthalmia, short stature, skin pigmentation
  • MDS (30%), AML risk (10% progress)
129
Q

dyskeratosis congenita

A
  • X-linked. chromosome instability (telomere shortening)
  • skin pigmentation, nail dystrophy, oral leukoplakia (triad) + BM failure
130
Q

Schwachman-Diamond syndrome

A
  • autosomal recessive. primarily neutropenia +/- others
  • skeletal abnormalities, endocrine and pancreatic dysfunction, hepatic impairment, short stature
  • AML risk
131
Q

Diamond-Blackfan syndrome

A
  • pure red-cell aplasia; normal WCC and platelets
  • presents at 1yr/neonatal
  • dysmorphology
132
Q

myeloproliferative disorders

A

a group of conditions characterised by clonal proliferation of one or more haemopoietic component i.e., increased production of mature cells

philadelphia chromosome positive:
- chronic myeloid leukaemia (CML)

philadelphia chromosome negative:
- polycythaemia vera (PV)
- myelofibrosis (MF)
- essential thrombocytosis (ET)

Ph-ve associated with JAK2 mutations, particularly PV (>95%)

associated with variable increases in reactive polyclonal BM fibrosis and terminal acute leukaemia transformation

all are at risk of transformation to myelofibrosis and acute leukaemias.
for many patients this risk is very low

133
Q

polycythaemia (+ causes)

A

raised red cell mass, Hb, red cell count and packed cell volume

primary causes:
- polycythaemia vera
- familial polycythaemia

secondary causes (raised EPO):
- disease states (renal Ca), high altitude, chronic hypoxia e.g., COPD

134
Q

relative (pseudo) polycythaemia

A

red cell mass normal but plasma volume is reduced
- dehydration, burns, vomiting, diarrhoea, cigarette smoking

135
Q

polycythaemia rubra vera (PRV) [+ mutations, clinical features, investigations, treatment]

A

an MPD where erythroid precursors dominate the BM. incidence rises with age

point mutations: JAK2 (V617F). independent of normal mechanisms of regulation.

clinical features:
- hyperviscosity/hypervolaemia/hypermetabolism
- blurred vision, headache
- plethoric (“red nose”), gout, thrombosis and stroke, retinal vein engorgement, erythromelagia
- splenomegaly
- histamine release –> aquagenic pruritis (contact with water) and peptic ulcers

investigations:
- raised Hb, HCT; also possibly platelets, WCC (neutrophils & basophils)
- low serum EPO

treatment:
- venesection
- hydroxycarbamide (maintenance), aspirin

136
Q

myelofibrosis [clinical features, investigations, treatment]

A

a MPD where myeloproliferation –> fibrosis of BM or replacement with collagenous tissue

primary (idiopathic) vs secondary following PRV, ET, leukaemia etc

clinical features:
- usually elderly
- pancytopenia - related symptoms
- extramedullary haematopoiesis - hepatomegaly, massive splenomegaly, WL, fever
- can present with Budd-Chiary syndrome

investigations:
- blood film: tear-drop poikilocytes (dacrocyte), leukoerythroblasts (primitive cells)
- BM: fibrosis, “dry tap”
- molecular tests: JAK2 mutation (60%), MPL mutation
- high urate and LDH (increased cell turnover)

treatment:
- support with blood products, in some cases splenectomy
- stem cell transplant is the only curative option
- ruloxitinib, hydroxycarbamide, thalidomide, steroids

137
Q

essential thrombocythaemia (or thrombocytosis)

A

an MPD where megakaryocytes dominate the BM
50% associated with JAK2
also associated with MPL mutation and CALR

clinical features:
- incidental finding in 50%
- venous and arterial thrombosis (stroke & MI), gangrene and haemorrhage
- erythromelalgia
- splenomegaly, dizziness, headaches, visual disturbances

investigations:
- platelet count >600 x 10[9]
- blood film: large platelets and megakaryocyte fragments
- increased BM megakaryocytes (not reactive)

treatment:
- aspirin
- anagrelide - reduce formation of platelets from megakaryocytes
- hydroxycarbamide

138
Q

what is the red cell threshold for transfusion?

A

70g/l if asymptomatic
80g/l if symptomatic
higher threshold of up to 90-100g/l for patients with coronary heart disease

139
Q

transfusion: shelf-life for red cells + storage temperature

A

35 days
4 degrees Celsius

140
Q

threshold for platelet transfusion

A

<10bn/litre
higher threshold of 20 in sepsis

141
Q

transfusion: shelf-life for platelets and storage temperature

A

7 days
22 degrees Celsius

142
Q

blood transfusion complications

A

Immediate (within 24 hrs)
immune:
- wrong blood: ABO
- febrile non-haemolytic
- allergic/anaphylaxis
- transfusion related acute lung injury (TRALI)
non-immune:
- bacterial infection
- transfusion associated cardiac overload (TACO)

Delayed (>24 hrs)
immune:
- delayed haemolytic transfusion reaction (DHTR)
- post-transfusion purpura
- transplant-associated GVHD
non-immune:
- viral infections
- iron overload

143
Q

transfusion: anaphylaxis

A
  • symptoms occur within minutes
  • IgE-mediated
  • risk increases in patients with IgA deficiency
144
Q

transfusion: ABO incompatibility

A
  • symptoms occur within minutes to hours
  • intravascular haemolysis - IgM mediated
145
Q

transfusion: bacterial contamination

A
  • symptoms occur within minutes to hours
  • more commonly occurs with platelet transfusion
146
Q

transfusion: febrile non-haemolytic transfusion reaction

A
  • rise in temperature of less than/equal to 1 degree Celsius without circulatory collapse
  • caused by release of cytokines by leukocytes and prevented by leukodepletion
  • Rx = slow transfusion and/or paracetamol
147
Q

transfusion: transfusion-related circulatory overload

A
  • caused by lack of attention to fluid balance
  • symptoms of pulmonary oedema/fluid overload occur within hours
  • look for signs of heart failure: raised JVP, raised PCWP (pulmonary capillary wedge pressure; used to assess LV filling, represents LA pressure)
148
Q

transfusion: transfusion-related acute lung injury

A
  • fever
  • caused by interaction with anti-WBC antibodies in donor blood and recipient WBCs
  • this causes immune complex deposition in pulmonary capillaries
  • absence of heart failure
149
Q

transfusion: delayed-haemolytic reaction

A
  • occurs within 1 week
  • extravascular haemolysis - IgG mediated
150
Q

transfusion: graft vs host disease

A
  • symptoms include diarrhoea, liver failure, skin desquamation and bone marrow failure
  • donor lymphocytes recognise recipient’s HLA as foreign and attack gut, liver, skin and bone marrow
  • prevent by irradiating blood components for immunosuppressed recipients
151
Q

transfusion: CMV infection

A
  • very immunosuppressed patients
  • leukodepletion removes CMV in WBCs
  • give CMV negative products to pregnant women
152
Q

transfusion: iron overload

A
  • chronic transfusion recipients, iron will accumulate in their body
  • this can damage their liver, heart and endocrine organs

Pathology (5 decks)

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