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MRCP part 2 > Haematology > Flashcards

Haematology Flashcards

1
Q

Burkitt’s lymphoma pathology

A

c-myc gene
t8;14 translocation
Starry sky appearance: lymphocyte sheets, macrophages containing apoptotic tumour cells
EBV in African mandibular subtype
ileo-caecal / abdominal subtype is sporadic

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2
Q

Complication of Burkitt’s lymphoma treatment

A

Tumour lysis syndrome - give rasburicase

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3
Q

Chronic myeloid leukaemia

A

T9:22 - 9 ABL (oncogene - an aberrant tyrosine kinase) + 22 B cell receptor

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4
Q

Acute pro-myelocytic leukaemia

A

T15:17 - 15 Promyelocytic gene + 17 Retinoid acid receptor alpha (Fusion protein binds retinoid acid receptor and promotes transcription).
AUER RODS (also in AML)
Bilobed / multilobed granulocytes (immature)
Clinical features: splenomegaly, pancytopenia, can present as DIC
Mx: all-trans retinoic acid

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5
Q

Follicular Lymphoma

A

T14:18 - 14 Ig heavy constant region + 18 Bcl2 (anti-apoptotic gene)

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6
Q

Mantle Cell Lymphoma

A

T11:14 - 11 - Cyclin D (oncogene) + 14 Ig heavy constant region

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7
Q

Diabetes monitoring with abnormal haemoglobin

A

HbA1c does not work

Use total glycated haemoglobin or glycosylated fructosamine

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8
Q

Heinz bodies

A

Denatured haemoglobin inclusions in RBCs e.g. exposure to oxidative stress

G6PD

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9
Q

Acanthocytes

A

spiculated RBCs

hyposplenism, abetalipoproteinaemia

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10
Q

Basophilic stippling

A

lead poisoning, megaloblastic anaemia, thalassaemia, myelodysplasia

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11
Q

Burr cells

A

Irregularly shaped cells

Stomach cancer

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12
Q

Howell-Jolly bodies

A

nuclear remnant (purple spot) in RBC

hyposplenism: post-splenectomy, sickle cell, coeliac, UC/crohn’s
megaloblastic anaemia
hereditary spherocytosis

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13
Q

Leucoerythroblastic anaemia

A

marrow infiltration -> nucleated RBCs and immature WBCs in blood

Myelofibrosis, malignancy with marrow infiltration

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14
Q

Polychromasia

A

Reticulocytosis

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15
Q

Rouleaux foramation

A

chronic inflammation, parapropteinaemia, myeloma

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16
Q

schistocytes

A

Fragmentation haemolysis is occurring

  • DIC
  • HELLP syndrome
  • mechanical valve turbulence
  • TTP / HUS
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17
Q

Spherocytes

A

hereditary spherocytosis

Autoimmune haemolytic anaemia

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18
Q

Target cells

A

Increased cell membrane:cell volume size

  • IDA
  • thalassaemia
  • hyposplenism
  • liver disease
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19
Q

Hypersegmented neutrophil

A

megaloblastic anaemia - e.g. b12/folate defiency

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20
Q

Tear drop cells

A

extramedullary haemopoiesis e.g. myelodysplasia, myelofibrosis, thalassaemia

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21
Q

Poikilocytosis

A

Varied RBC shape

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22
Q

Anisocytosis

A

varied RBC size

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23
Q

Hyposplenism (and post splenectomy) blood film

A 
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
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24
Q

IDA blood fbc / film / haematinics

A

FBC

  • RDW increases early
  • microcytosis later (but more specific)

Blood film

  • target cells
  • ‘pencil’ poikilocytes

Haematinics

  • low ferritin
  • raised transferrin (compensatory)
  • reduced TIBC
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25
Q

Myelofibrosis

A

‘tear-drop’ poikilocytes
JAK2 mutation
Dry tap bone marrow

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26
Q

Indications for CLL treatment

A

> 6cm splenomegaly
10cm lymphadenopathy
B symptoms (weight loss, fever)
progressive lymphocytosis (>50% in 2 months, doubling time <6months)
progressive marrow failure (anaemia, thrombocytopenia)

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27
Q

CLL mx

A

FCR: fludarabine, cyclophosphamide, rituximab
- prophylactic co-trimoxazole (fludarabine)
Chlorambucil if not fit for FCR
Ibrutinib if fail to respond to the above

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28
Q

Unprovoked DVT ix

A

For over 40s…

1) CXR, bloods, urinalysis including PSA and dip
2) CT AP and mammogram
3) Consider antiphospholipid antibodies (do on younger patients)
4) hereditary thrombophilia screen only if 1st degree relative (or young?)

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29
Q

Beta thalassaemia major

A 
Severe microcytic anaemia
Target cells
Transfusion dependent
HbA2 > 5%
HbF up to 95%
No HbA
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30
Q

Beta thalassaemia intermedia

A

Mod microcytic anaemia
Not transfusion dependent
HbA2 >4%
HbF up to 50%

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31
Q

Beta thalassaemia minor

A

Mild microcytic anaemia
HbA2 >4%
HbF up to 5%

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32
Q

Alpha thalassaemia major

A 
Either hydrops faetalis in utero
OR
HbH disease
- HbH up to 30%
- HbA2 up to 4%
- moderate microcytic anaemia
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33
Q

Alpha thalassaemia minor

A 
Mild microcytic anaemia
Hb Barts (3 to 8%, only in the newborn period)
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34
Q

IgA deficiency features

A

Respiratory tract infections
Associated with atopy
Risk of severe transfusion reactions

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35
Q

Myelofibrosis mx

A

1) Allogenic stem cell transplant

2) Ruxolitinib, fedratinib, hydroxyurea

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36
Q

Cryoglobulinaemia features

A
  • Raynaud’s only seen in type I
  • cutaneous: vascular purpura, distal ulceration
  • arthralgia
  • renal involvement (diffuse glomerulonephritis)
  • peripheral neuropathy
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37
Q

Type 1 cryoglobulinaemia aetiology

A

Monoclonal IgM or IgG responsible

Causes: myeloma, waldenstrom macroglobulinaemia

Specific features: raynaud’s

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38
Q

Type 2 (mixed) cryoglobulinaemia aetiology

A
  • Mixed poly and monoclonal Ig
  • RF positive usually

Causes: hep C, HIV, rheumatoid arthritis, sjogren’s, lymhpoma

Mx: treat the cause, steroids / rituxumab
plasma exchange if hyperviscosity

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39
Q

Type 3 cryoglobulinaemia

A

Polyclonal Ig

Rheumatoid arthritis or Sjogren’s

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40
Q

Causes of paraprotein bands

A

MGUS

  • IgM / M protein < 3g/dL
  • < 10% lymphoplasmocytic / plasma cells on BM
  • abnormal kappa: lambda free light chain ratio
  • absence of end organ damage

Waldenstrom’s macroglobulinaemia

  • IgM gammopathy of any size
  • > 10% lymphoplasmocytic cells on BM
  • hyperviscosity, splenomegaly common (more than MM)

Multiple myeloma

  • more commonly IgG gammopathy
  • > 10% plasma cells on BM
  • evidence of end organ damage

Smouldering multiple myeloma

  • 10-60% plasma cells
  • absence of end organ damage
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41
Q

Acute (pro)myelocytic complications

A

(DIC), anaemia, thrombocytopenia, hyperviscosity

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42
Q

Hereditary angioedema - acute mx and prophylaxis

A

Acute: IV C1-inhibitory concentrate OR FFP
Prophylaxis: tranexamic acid, danazol

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43
Q

Mx polycythaemia rubra vera

A

Venesection aim hct < 0.45
Low dose aspirin
Hydroxyurea if >60 years old or hx of thrombosis

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44
Q

Neurovisceral only porphyrias

A

Acute intermittent porphyria

Aminolaevulinic acid dehydrogenase porphyria

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45
Q

Photosensitive only porphyrias

A

Porphyria cutanea tarda
Congenital erythropoietic porphyria
Erythropoietic protoporphyria

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46
Q

Mixed neurovisceral / photosensitive porphyria

A

Hereditary coproporphyria

Variegate porphyria

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47
Q

Stroke in vaso-occlusive sickle cell crisis

A

Exchange transfusion

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48
Q

Haemophilia A & B coagulation

A
  • normal PT / INR
  • raised APTT (decreases with mixing)
  • normal bleeding time
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49
Q

von Willebrand coagulation and ix

A
  • normal PT / INR
  • raised APTT
  • raised bleeding time
  • reduced vWF activity (ristocetin cofactor)
  • reduced factor VIII
50
Q

DIC

A
  • all raised with thrombocytopenia
  • raised d-dimer
  • reduced fibrinogen (but can be normal or raised in early disease)
51
Q

vitamin K deficiency

A
  • raised PT / INR
  • raised APTT
  • normal bleeding time
52
Q

Acute intermittent porphyria investigations

A

Raised urinary porphobilogen acutely
Raised serum porphobilogen, delta aminolaevulinic acid
porphobilogen deaminase defect in RBCs

53
Q

Paroxysmal nocturnal haemoglobinuria features

A
  • acquired mutation in haematopoietic stem cell lineage: de novo or aplastic anaemia / myelofibrosis / myelodysplasia
  • haemolytic anaemia
  • Thrombosis e.g. DVT, budd-chiari
  • haemoglobinuria
54
Q

Paroxysmal nocturnal haemoglobinuria ix

A

Positive Ham’s test
Flow cytometry CD59 and CD55
low haptoglobin, raised ldh

55
Q

Paroxysmal nocturnal haemoglobinuria mx

A

blood products
anticoagulation
eculizumab - give meningitis vaccines
stem cell transplant

56
Q

Indications for irradiated blood products include:

A

Immunocompromised marrow or organ transplant recipients
Patients with haematological disorders who will be undergoing allogeneic marrow transplantation imminently
Intrauterine transfusions
Patients with Hodgkin’s disease
Patients treated with purine analogue drugs (e.g. fludarabine)

57
Q

Glanzmann’s thrombasthenia

A

Autosomal recessive defect GIIb/IIIa -> thrombocytopenia and purpura -> excessive bleeding e.g. menorrhagia

58
Q

Poor prognostic features in multiple myelom

A

Raised B2 microglobulin

Low albumin

59
Q

AML blood film

A

Auer rods - needle shaped cytoplasmic inclusions (in myeloblasts)

60
Q

CLL prognostic factors

A

Poor

  • male sex
  • age > 70 years
  • lymphocyte count > 50
  • prolymphocytes comprising more than 10% of blood lymphocytes
  • lymphocyte doubling time < 12 months
  • raised LDH
  • CD38 expression positive
  • deletions short arm chromosome 17 (del 17p)

Good
- deletions long arm of chromosome 13 (del 13q)

61
Q

Exchange transfusion target in sickle cell

A

HbS < 30%

62
Q

CML management

A

1) imatinib
2) allogenic stem cell transplant - if young, chronic phase and not responding to imatinib OR blast phase
3) other agents: cytarabine, hydroxyurea

63
Q

Methaemoglobinaemia features

A
  • State of abnormally increased haem iron oxidation to Fe3+ impairing oxygen delivery to tissues
  • Usual range 1-3% HbMet
  • Respiratory and metabolic acidosis
  • Normal sats / PaO2 on ABG but reduced on sats probe
64
Q

Methaemoglobinaemia causes

A

Congenital

  • NADH methaemoglobin reductase deficiency
  • HbH or HbM haemoglobin chain variants

Acquired

  • poppers (alkyl nitrates)
  • dapsone
  • nitrates
  • sodium nitroprusside
  • primaquine
65
Q

Methaemoglobinaemia treatment

A

NADH methaemoglobin reductase deficiency
- ascorbic acid

Acquired causes

  • IV methylthioninium chloride / methylene blue
  • exchange transfusion
66
Q

Causes of splenomegaly

A

Massive splenomegaly

  • haem: CML, myelofibrosis
  • infection: visceral leishmaniasis, malaria
  • metabolic: Gaucher’s syndrome (AR lysosomal storage)

Other

  • haem: CLL, Hodgkin’s, haemolytic anaemia, thalassaemia, early sickle, polycythaemia rubra vera
  • infection: endocarditis, hepatitis, glandular fever
  • gastro: portal hypertension
  • rheum: Felty’s syndrome
67
Q

Paroxysmal cold haemoglobinuria pathogenesis

A
  • Type of autoimmune haemolytic anaemia

- antibodies bind to RBCs in the cold -> fix complement -> intravascular haemolysis -> spherocytes

68
Q

Paroxysmal cold haemoglobinuria causes

A

Acquired causes

  • Secondary or tertiary syphilis
  • Post-viral infection (eg, varicella, measles, mumps, EBV, CMV)
  • Post-bacterial infection (eg, mycoplasma, Klebsiella, Escherichia coli, Haemophilus influenzae)
  • Post-measles vaccine
  • Autoimmune disorders
  • Lymphoma or CLL
69
Q

Paroxysmal cold haemoglobinuria features

A

Haemoglobinuria after cold exposure, jaundice, anaemia, raynaud’s, urticaria

Ix: DAT / Coomb’s +ve, urinary haemosiderin

70
Q

TTP features

A
  • fever
  • fluctuating neurological signs
  • AKI
  • thrombocytopenia
  • microangiopathic haemolytic anaemia (schistocytes AND Coomb’s/DAT negative)
71
Q

TTP causes

A

Hereditary - AR deficiency ADAMTS13 gene

Acquired autoantibody vs ADAMTS13

  • pregnancy / post partum, COCP
  • drugs
  • SLE
  • infections inc. HIV, E Coli
72
Q

TTP management

A

1) plasma exchange for all

2) seroids +/- rituximab / caplacizumab (depending on severity e.g. neuro)

73
Q

CMV negative blood product indications

A
  • granulocyte transfusions
  • intrauterine transfusions
  • neonates up to 28 days adjusted
  • maternal transfusions during pregnancy
74
Q

Pain in lymph nodes when drinking alcohol

A

Hodgkin’s lymphoma

75
Q

Acute intermittent porphyria mx

A

Acute: glucose, hematin / heme arginate

Reduce frequency of attacks: prophylactic heme arginate, givosiran, liver transplant

76
Q

Causes of platelet transfusion failure to increment (<10)

A

splenomegaly
new HLA abs (repeated transfusions)
DIC
Sepsis

77
Q

HLH / MAS features

A

Fever
Splenomegaly
Mimics infection: encephalitis, hepatitis, PUO

Cytopenias
Deranged LFTs
Raised ferritin
Hypertryglyceridaemia
Haemophagocytosis on BM biopsy
78
Q

HLH / MAS treatment

A

Treat the cause

If critically unwell

  • etoposide & dexamethasone
  • intrathecal hydrocortisone & methotrexate if CNS features
79
Q

Acute promyelocytic leukaemia

  • emergency treatment
  • indications
A
  • all transretinoic acid (ATRA)

- blast crisis with severe anaemia, sepsis, DIC

80
Q

Hairy cell leukaemia features

A

Rare malignant proliferation of B cells
BRAF mutation

Males 4:1

  • pancytopenia
  • splenomegaly
  • skin vasculitis in 1/3 patients
  • ‘dry tap’ despite bone marrow hypercellularity
  • cells have ‘fried egg’ appearance
  • tartrate resistant acid phosphotase (TRAP) stain positive
81
Q

Hairy cell leukaemia management

A

1) chemotherapy: cladribine, pentostatin

2) immunotherapy: rituximab, interferon-alpha

82
Q

Germline TP53 mutation

A

Li Fraumeni syndrome

  • AD
  • sarcomas, sarcomas, breast carcinoma, glioblastoma, lymphoma and leukaemia.
83
Q

Hereditary spherocytosis mx

A

1) Folate replacement

2) Splenectomy if recurrent anaemia

84
Q

ITP pathogenesis

A

Acquired abs vs platelet antigens
Primary ITP - no cause / trigger found
Secondary ITP - infection / rheumatological conditions / alemtuzumab as trigger

85
Q

ITP features

A

Thrombocytopenia
- other cell lines completely normal
Bleeding
Petechiae (which coalesce into purpura)

86
Q

ITP management

A

Treat bleeding
Steroids if no bleeding
IVIG if bleeding or as second line treatment

87
Q

HUS vs TTP

A
  • less neuro signs
  • more significant renal failure
  • Shiga toxin as trigger - bloody diarrhoea
88
Q

Lead poisoning clinical features

A
  • fatigue
  • neuropsychiatric
  • peripheral neuropathy (motor predominant)
  • GI upset
  • blue lines on gums
89
Q

Lead poisoning ix

A
  • microcytic anaemia with basophilic stippling
  • raised urinary coproporphyrin
  • raised blood lead levels
90
Q

Lead poisoning mx

A

Chelating agents

  • dimercaptosuccinic acid (DMSA)
  • D-penicillamine
  • EDTA
  • dimercaprol
91
Q

Acute chest syndrome mx

A
  • IV fluids, oxygen, analgesia
  • IV antibiotics (if infective precipitant)
  • Transfuse to aim Hb >100 in mild cases
  • In more severe cases: exchange transfusion aim Hbs <30%
92
Q

Rivaroxaban drug withhold pre-op

A

1 day if normal egfr

93
Q

How long to stop apixaban pre-op

A

1-2 days if normal egfr

94
Q

How long to stop UFH pre-op

A

6 hours if IV

12 hours if SC

95
Q

LMWH

A

12 hours prophylactic

24 hours treatment dose

96
Q

Heparin induced thrombocytopenia

A

> 50% reduction in platelets (5-10 days onset)
HIT screen, serotonin release assay

Mx options

  • bivalirudin
  • lepirudin
  • danaparoid
  • fondaparinux
97
Q

Management of autoimmune haemolytic anaemia (e.g. secondary to CLL)

A

1) Steroids
2) Rituximab
3) Splenectomy

98
Q

Factor deficiencies leading to PT prolongation alone

A

VII

99
Q

Factor deficiencies leading to APTT prolongation alone

A

VIII, IX, XI, XII

100
Q

Factor deficiencies prolonging both PT ant APTT

A

II, V and X

101
Q

Role of 50:50 mixing studies

A

Simple factor deficiencies, coag corrects. If inhibitors are present e.g. lupus anticoagulant, these do not correct

102
Q

Acquired factor VIII deficiency

A 
- occurs in elderly
Causes
- psoriasis
- pemphigus
- cephalosporins, penicillins
103
Q

Myelodysplasia

A
  • occurs in elderly with anaemia / pancytopenia
  • no splenomegaly (vs CML)
  • precursor to AML (30% transform)
  • give chemo if elevated WCC
104
Q

Delayed transfusion reactions

A
  • pyrexia, jaundice, icteric, drop in hb
  • urine: urobilogen raised
  • blood film: spherocytosis
  • diagnosis: DAT / Coombs’
105
Q

INR target metal aortic valve

A

2-3

106
Q

INR target metal mitral valve

A

2.5-3.5

107
Q

Clotting in antiphospholipid

A

Raised APTT

Does not correct with mixing

108
Q

DIC acquired cause in elderly men

A

Prostate cancer invading marrow

109
Q

Dx primary polycythaemia

A

Maj

  • hb >185 OR >165 (women)
  • JAK2 mutation

Min

  • BM bx hypercelullarity
  • low serum EPO

Other
- raised LAP score

110
Q

Cause of TRALI

A

donor blood anti-granulocyte antibodies

111
Q

Non-hodgkin’s lymphoma treatment

A

R-CHOP

112
Q

C1 esterase deficiency

A

AD hereditary angioedema

  • mx: C1 inhibitory concentrate OR FFB
  • prophylaxis: tranexamic acid
113
Q

Anaphylaxis after RBC transfusion

A

IgA deficiency

114
Q

Antiphospholipid syndrome mx in pregnancy

A

Aspirin + LMWH

115
Q

Multiple myeloma mx

A

Good functional status?
- induction chemotherapy (proteasome inhibitor e.g. bortezomib + dexamethasone) -> autologous stem cell transplantation

Poor functional status?
- thalidomide + melphalan (alkylating agent) + dexamethasone

116
Q

How to reduce frequency of sickle cell crisis

A

Hydroxyurea (increases HbF production)

117
Q

von willebrand disease features and mx

A
  • inherited disease
  • M=F
  • bleeding gums, menorrhagia

Mx

1) tranexamic acid
2) desmopressin
3) factor VIII concentrate

118
Q

Homocystinuria features and mx

A
  • short-sightedness
  • marfanoid
  • ligamentous laxity
  • lens dislocation
  • thromboembolic events
  • neuropsych features
  • livedo reticularis
    Ix: urinary homocysteine
    Mx: pyridoxine / folic acid
119
Q

Causes of pure red cell aplasia

A
  • idiopathic / autoimmune
  • initial presentation of myelodysplastic syndrome
  • CLL
  • RA, SLE
  • EPO
  • phenytoin
  • co-trimoxazole
  • zidovudine
  • MMF
  • parvovirus B19
  • HIV
  • EBV
  • viral hepatitis
120
Q

Absolute and relative contraindications to thrombolysis

A

Absolute

  • previous ICH
  • intracranial neoplasm
  • seizure at stroke onset
  • stroke / TBI in last 3 months
  • LP last 7 days
  • GI bleed last 3 weeks
  • oesophageal varices
  • active bleeding
  • pregnancy
  • uncontrolled hypertension >200/120

Relative

  • INR >1.7
  • active diabetic haemorrhagic retinopathy
  • intracardiac thrombus
  • major surgery <2 weeks

MRCP part 2 (11 decks)

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