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Phase 4 Question Knowledge > Haematology > Flashcards

Haematology Flashcards

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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
B12 deficiency
- 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
26
B12 deficiency causes
- 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
27
B12 deficiency Px
- anaemia sx - glossitis - angular stomatitis - mild jaundice - neuro sx - peripheral neuropathy, subacute combined degeneration of spinal cord, neuropsych
28
B12 deficiency Ix
- 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
29
B12 deficiency Mx
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
30
Folate deficiency
- (Vit B9) - absorbed in jejunum, found in green veggies
31
Folate deficiency causes
- poor folate diet - poverty, alcoholics, elderly - malabsorption - Crohn's, Coeliac, alcoholism - pregnancy - anti-folate drugs - methotrexate, trimethoprim, AEDs
32
Folate deficiency Px
- anaemia sx - no neuropathy
33
Folate deficiency Ix
- FBC, blood film - macrocytic anaemia, hypersegmented polymorphs - increased LDH - low reticulocyte count
34
Folate deficiency Mx
- tx cause - folic acid
35
Haemolytic anaemia
- premature breakdown of RBCs - intra/extravascular
36
Haemolytic anaemia causes
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
37
Haemolytic anaemia causes by site
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
38
Haemolytic anaemia general Px
- anaemia sx - splenomegaly - jaundice
39
Haemolytic anaemia general Ix
- FBC, blood film - normocytic anaemia, schistocytes - Direct Coombs test (DAT) - positive in AIHA
40
Autoimmune haemolytic anaemia (AIHA)
- 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
41
Hereditary spherocytosis
- 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
42
Hereditary elliptocytosis
- as spherocytosis but RBCs ellipse-shaped - autosomal dominant - Px / Mx the same
43
G6PD deficiency
- 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
44
Alloimmune haemolytic anaemia
- 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
45
Paroxysmal nocturnal haemoglobinuria
- 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
46
Microangiopathic haemolytic anaemia (MAHA)
- 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
47
Prosthetic valve haemolysis
- turbulent flow around valve -> shearing of RBCs Mx - monitor - oral iron / folic acid - blood transfusions - revision surgery
48
Fanconi anaemia
- autosomal recessive cause of aplastic anaemia - bone marrow failure, increased AML risk, neuro sx, short stature, thumb / radius abnormalities, café au lait spots
49
Sickle cell anaemia (SCA)
- autosomal recessive - production of abnormal Hb leading to vaso-occlusive crises (disorder of quality)
50
SCA patho
- 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
51
SCA Px
- 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
52
SCA Ix
- 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)
53
SCA Mx
- avoid triggers, dehydration - pneumococcal vaccine (and others) - pen V abx prophylaxis - hydroxycarbamide - crizanlizumab - blood transfusions - bone marrow transplant
54
Sickle cell crisis
- 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
55
SCA Cx
- anaemia - infections - CKD - sickle cell crises - stroke - avascular necrosis - hypoxia -> fibrosis -> pulm HTN - gallstones - priapism - splenic infarction, hyposplenism etc
56
Thalassaemia
- autosomal recessive genetic defect in Hb protein chains -> underproduction of one globin chain - reduced production + premature destruction of RBCs - disorder of quantity
57
Thalassaemia patho
- HbA - 2 alpha, 2 beta chains - lack of production of a/b chain - reduced RBC production, fragile RBCs break down
58
Thalassaemia overall Px
- Microcytic anaemia - Fatigue - Pallor - Jaundice - Gallstones - Splenomegaly - Poor growth + development
59
Alpha thalassaemia
- 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
60
Beta thalassaemia
- 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
61
B-thalassaemia minor / trait
- asym, heterozygous carrier - mild/absent anaemia - monitor only
62
B-thalassaemia intermedia
- 2 defective genes - 2 abnormal / 1 abnormal + 1 deletion - moderate anaemia - splenomegaly - transfusions, iron chelation
63
B-thalassaemia major
- 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
64
Thalassaemia Ix
- 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
Thalassaemia Mx
- exercise good diet - folate supplements - regular blood transfusions - desferrioxamine / ascorbic acid - splenectomy - hormonal tx for endocrine cx - bone marrow transplant
66
Leukaemia
- cancer of immature blast blood cells / stem cells - precursors of RBCs, platelets, WBCs
67
Leukaemia patho
- 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
Leukaemia general Px
- Fatigue, fever - Pallor - anaemia - Petechiae / bruising - Abnormal bleeding - Lymphadenopathy - HSM - Failure to thrive
69
Leukaemia general Ix
- FBC <48hrs - Blood film - LDH raised - Bone marrow biopsy - from iliac crest - CT / PET - lymph node biopsy - genetic tests, immunophenotyping
70
Leukaemia general Mx
- chemo - targeted therapy - bone marrow transplant - surgery
71
Acute lymphoblastic leukaemia (ALL)
- malignant proliferation of lymphoblasts (B/T cell precursors) - 80% childhood leukaemias - Some Philadelphia chromosome involvement - radiation, Down's
72
ALL Px
- bone marrow failure - anaemia, infections, fever, bleeding, bruising - bone pain - HSM - lymphadenopathy - headache, CN palsies - fever - testicular swelling - unilateral
73
ALL Ix
- FBC - low Hb, platelets, WCC - Blood film - blast cells - Bone marrow aspirate - blast cells - CXR, CT, LP
74
ALL Mx
- blood / platelet transfusions - infection prophylaxis - chemo - marrow transplant
75
ALL poor prognostic factors
- Age <2yo, >10yo - WBC >20 at diagnosis - T/B cell surface markers - Non-Caucasian - Male
76
Acute myeloid leukaemia (AML)
- malignant proliferation of myeloblast cells (basophil, neutrophil, eosinophil precursor) - most common acute leukaemia in adults - chemo, radiation, Downs RFs
77
AML Px
- anaemia, infection, bleeding - bleeding gums, gum hypertrophy - bone pain - HSM - skin involvement - DIC
78
AML Ix
- FBC - increased WCC, anaemia, thrombocytopenia - Bone marrow biopsy - Auer rods, high proportion blast cells
79
AML Mx
- infection prophylaxis - blood / platelet transfusions - chemo - bone marrow transplant
80
Chronic myeloid leukaemia (CML)
- Malignant proliferation of myeloid cells (WBCs - neutrophils, basophils, eosinophils) - Adults 50-60yo - chronic, accelerated, blast phases - Philadelphia chromosome
81
CML Px
- Insidious onset - Fever, wt loss, fatigue - Gout (from purine breakdown) - Bleeding, bruising - Anaemia - HSM
82
CML Ix
- FBC - increased WCC, anaemia - Bone marrow biopsy - hypercellular - cytology - genetics
83
CML Mx
- chemo - imatinib - bone marrow transplant
84
Chronic lymphocytic leukaemia
- Malignant proliferation of mature B cells - accumulate + escape apoptosis - Elderly - associated with warm haemolytic anaemia - mutations, Downs, pneumonia may be trigger
85
Richter's transformation
- CLL may transform into rare type of aggressive non-Hodgkin lymphoma - suddenly unwell - lymphadenopathy, fever, wt loss, night sweats, nausea, abdo pain
86
CLL Px
- Often asym - Anaemia, infection - Wt loss, night sweats - Anorexia - HSM - Lymphadenopathy
87
CLL Ix
- FBC - anaemia, raised WCC, high lymphocytes, thrombocytopenia - Blood film - smudge / smear cells
88
CLL Mx
- Blood transfusion - chemo / radio - rituximab - ibrutinib - bone marrow transplant - 1/3 never progress, 1/3 progress slowly, 1/3 progress quickly
89
Tumour lysis syndrome
- release of chemicals from cells destroyed by chemo - high grade lymphomas / leukaemias
90
Tumour lysis syndrome patho
- High uric acid -> form crystals in kidneys -> AKI - High K -> cardiac arrhythmias - High phosph - Low Ca - from high phos - Cytokines -> systemic inflammation
91
Tumour lysis syndrome Px
- suspect in AKI with high phos, high uric acid
92
Tumour lysis syndrome Ix
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
Tumour lysis syndrome Mx
- good hydration / UO before chemo - IV fluids - allopurinol - rasburicase
94
Lymphoma
- cancer of lymphocytes in lymphatic system - proliferate in lymph nodes
95
Lymphoma Lugano classification
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
Ann Arbor Classification
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
Lymphadenopathy DDx
- infective - IM, HIV, eczema, rubella, toxoplasmosis, CMV, TB, roseola infantum - neoplastic - leukaemia, lymphoma - SLE, RA, graft vs host disease, sarcoidosis, drugs
98
Hodgkin's lymphoma
- specific type of lymphoma - 20-25yo, and 80yo
99
Hodgkin's patho
- nearby contiguous spread, rarely extranodal, Reed-Sternberg cells RFs - HIV, EBV, RA, sarcoidosis, FHx
100
Hodgkin's Px
- 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
Hodgkin's Ix
- FBC - raised WCC, anaemia - LDH - high - Lymph node biopsy - Reed-Sternberg cells (multinucleated) - CT / MRI / PET, CXR
102
Hodgkin's Mx
- Chemo - radiotherapy - stem cell transplant - surgical removal of lymph nodes
103
Non-Hodgkin's lymphoma
- all other types of lymphoma - 80% B cell, 20% T cell
104
Non-Hodgkin's patho
- 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
Non-Hodgkin's Px
- 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
Non-Hodgkin's Ix
- FBC - raised WCC, low Hb/platelets - raised LDH - lymph node biopsy - CT / MRI / PET
107
Non-Hodgkin's Mx
- chemo - pred - rituximab - radiotherapy - stem cell transplant
108
Myeloma
- 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
Myeloma patho
- 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
Myeloma Px
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
Myeloma Ix
- 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
Myeloma Dx criteria
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
Myeloma Mx
- chemo - radio - stem cell transplant - analgesia - bisphosphonates - surgery - IV Ig - transfusion
114
Myeloproliferative disorders
- 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
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Myeloproliferative disorders Px
- 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)
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Myeloproliferative disorders Ix
- genetic testing - bloods - low Hb, high WCC/platelets, high urate/LDH - blood film - teardrop shaped RBCs, blasts, anisocytosis - bone marrow biopsy
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Myeloproliferative disorders Mx
Primary myelofibrosis - supportive - chemo - ruxolitinib - allogenic stem cell transplant Essential thrombocytosis - aspirin - chemo - anagrelide
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Polycythaemia
- increase in RBC mass / Hb / packed cell volume
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Polycythaemia causes
Absolute - primary - polycythaemia vera (JAK2 mutation) - secondary - hypoxia, high EPO (RCC, HCC) Relative - apparent polycythaemia - obesity, HTN, alcohol, smoking - dehydration
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Polycythaemia Px
- asym - HTN - HSM - gout - VTE, arterial thrombosis - headaches - itching - after warmth - dizziness - tinnitus - visual disturbance - angina - red face
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Polycythaemia Ix
- FBC - high Hb, raised WCC/platelets - ferritin low in primary - genetic screen - bone marrow biopsy
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Polycythaemia Mx
- venesection - chemo - aspirin
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Thrombocytosis
- high platelet count Causes - reactive - stress, infection, surgery, IDA - malignancy - essential thrombocytosis - hyposplenism
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Myelodysplastic syndrome
- 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
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Thrombocytopenia
- low platelet count - normal 150-450 - from reduced production / increased destruction
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Thrombocytopenia causes
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
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Thrombocytopenia Px
mild - asym <50 - nosebleeds, bleeding gums, heavy periods, easy bruising, haematuria, PR bleed <10 - risk spont bleed - intracranial haemorrhage, GI bleed
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DDx abnormal bleeding
- Thrombocytopenia - Von Willebrand disease - Haemophilia - DIC (usually secondary to sepsis)
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Immune thrombocytopenic purpura (ITP)
- ABs created against platelets - T2 hypersensitivity - eg after infection, vaccination - more acute in children - adults - autoimmune disorders
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ITP Px
- bruising - petechial / purpuric rash - epistaxis, gingival bleeding
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ITP Ix
- FBC - low platelets - blood film - bone marrow biopsy - if atypical features
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ITP Mx
- 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
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Thrombotic thrombocytopenic purpura (TTP)
- 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
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TTP Px
- florid purpura (bright red) - fever, fatigue - haemolytic anaemia, may have AKI - bruising, bleeding - confusion, headache, cerebral dysfunction - from microemboli
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TTP Ix
- FBC - low platelets - U/E - LDH raised - blood film - schistocytes / fragmented RBCs - coag - normal
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TTP Mx
- plasma exchange - steroids - IV methylprednisolone - rituximab - splenectomy
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Heparin-induced thrombocytopenia (HIT)
- 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
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Von Willebrand Disease
- 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
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Haemophilia
- 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
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Disseminated intravascular coagulation (DIC)
- 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
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Thrombophilia
- 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
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Acute intermittent porphyria
- 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
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Cryoglobulinaemia
- 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
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Hereditary angioedema (HAE)
- 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
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Lead poisoning
- 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
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Methaemoglobinaemia
- 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
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Neutropenia
- 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
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Hyposplenism
Causes - Splenectomy - Sickle cell - Coeliacs, dermatitis herpetiformis - Graves - SLE - Amyloid Features - Howell-Jolly bodies - Siderocytes
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Splenomegaly
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
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Post-thrombotic syndrome
- 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
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Thymoma
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
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Waldenstrom's macroglobulinaemia
- 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
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Wiskott-Aldrich syndrome
- B/T cell dysfunction causing primary immunodeficiency - X-linked recessive, WASP gene mutation Px - recurrent infections, eg chest - eczema - thrombocytopenia - low IgM
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Graft-vs-host disease (GVHD)
- 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
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Billingham criteria for dx of GVHD
- Transplanted tissue contains immunologically functioning cells - Recipient + donor are immunologically different - Recipient immunocompromised
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Acute GVHD
- <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)
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Chronic GVHD
- >100d post-transplant - varied clinical picture - often lung + eye Px - skin, eye, GI, lung sx
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GVHD Ix
- organ dependent - LFTs, hepatitis screen, USS, abdo imaging - PFTs - biopsy
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GVHD Mx
- immunosuppression - IV steroids, biologics - supportive - prophylaxis - calcineurin inhibitors

Phase 4 Question Knowledge (36 decks)

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