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Finals PACES > Haematology > Flashcards

Haematology Flashcards

1
Q

Plummer-Vinson syndrome

A

Post-cricoid web that is associated with iron deficiency anaemia

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2
Q

Iron deficiency anaemia haematinics

A

↓ferritin, ↑TIBC, ↓ transferrin saturation

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3
Q

Sideroblastic anaemia haematinics

A

↑Ferritin, ↑ se Fe, ↔TIBC

Rx: Can give pyroxidine

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4
Q

B Thalassaemia trait

A

↓ MCV

↑ HbA2 (α2δ 2) and ↑HbF (α2γ2)

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5
Q

B Thalassaemia Major

A

↓Hb, ↓MCV, ↑↑HbF, ↑HbA2 variable

Film: Target cells and nucleated RBCs

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6
Q

Folate deficiency

A

Typically decreased intake or high demand (pregnancy or medication e.g. methotrexate/phenytoin)
Rx = give B12 first unless this is normal as can worsen SCDC; give folate PO 5mg/d

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7
Q

Subacute Combined Degeneration of the Cord

A

Combined symmetrical dorsal column loss and corticospinal tract loss with a mixed UMN and LMN picture.
Pain and temperature intact
Usually only caused by pernicious anaemia

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8
Q

G6PD deficiency triggers

A

 Broad (Fava) beans
 Mothballs (naphthalene)
 Infection
 Drugs: antimalarials, henna, dapsone, sulphonamides

Can see Heinz bodies

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9
Q

Warm AIHA

A

IgG mediated with extravascular haemolysis and spherocytes and is DAT +ve
Linked to SLE, RA and Evan’s
Rx = immunosuppression +/- splenectomy

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10
Q

Cold AIHA

A

IgM mediated with intravascular haemolysis and is DAT +ve
Linked to mycoplasma
Rx = avoid cold and rituximab

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11
Q

Paroxysmal cold haemoglobinuria

A

IgG “Donath-Landsteiner” Abs bind RBCs in the cold → complement-mediated lysis on rewarming
Associated with Measles, mumps, chickenpox

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12
Q

Paroxysmal nocturnal haemoglobinuria

A

Features
 Visceral venous thrombosis (hepatic, mesenteric, CNS)
 IV haemolysis and haemoglobinuria

Ix
 Anaemia ± thrombocytopenia ± neutropenia
 FACS: ↓CD55 and ↓CD59

Rx
 Chronic disorder therefore long-term anticoagulation
 Eculizumab (prevents complement MAC formation)

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13
Q

HUS

A

E. coli O157:H7 from undercooked meat

Bloody diarrhoea and abdominal pain precedes:
 MAHA (schistocytes)
 Thrombocytopenia
 Renal failure

Spontaneously resolves

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14
Q

TTP

A

Pentad = fever, CNS, MAHA, thrombocytopenia, renal failure

Rx = Plasmapheresis, immunosuppression, splenectomy

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15
Q

Hereditary spherocytosis Ix + Rx

A

 ↑ osmotic fragility
 Spherocytes
 DAT-ve

Rx = folate + splenectomy (after childhood)

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16
Q

Pyruvate Kinase Deficiency

A

Features: splenomegaly, anaemia +/- jaundice

Rx = often not required

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17
Q

SCD presentation

A 
SICKLED
Splenomegaly
Infarction - stroke, AVN, spleen (hyposplenism)
Crises - pulmonary, mesenteric, sequestration crises (splenic pooling --> shock and severe anaemia)
Kidney disease
Liver disease
Erection
Dactylitis
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18
Q

Increased PT

A

 Warfarin / Vit K deficiency
 Hepatic failure
 DIC

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19
Q

Increased APTT

A 
 Lupus anti-coagulant
 Haemophilia A or B
 vWD (carries factor 8)
 Unfractionated heparin
 DIC
 Hepatic failure
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20
Q

Increased bleeding time

A

 ↓ plats number or function
 vWD
 Aspirin
 DIC (increased thrombin time as well)

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21
Q

Haemophilia A Ix and Mx

A

Ix
↑APTT, normal PT, ↓F8 assay

Mx
 Avoid NSAIDs and IM injections
 Minor bleeds: desmopressin + tranexamic acid
 Major bleeds: rhF8

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22
Q

vWD Ix and Mx

A

Ix
↑ APTT, ↑ bleeding time, normal plat, ↓ vWF AG

Rx
Desmopressin + transexamic acid

23
Q

Thrombophilia conditions

A 
Factor V Leiden
Prothrombin gene mutation (elevated prothrombin levels)
Protein C and S deficiency
Antithrombin III deficiency
Antiphospholipid syndrome
OCP (progesterone)

Make sure to anticoagulate these patients

24
Q

Massive transfusion reaction

A

Whole blood volume in 24 hrs (10 units)

Features:
↑K
↓ Ca (citrate chelation) ↓ F5 and F8
↓ plats
Hypothermia

Rx:
Massive Transfusion Protocol
1:1:1 ratio of PRBC:FFP:PLT + Warm the blood

25
Q

Delayed transfusion reactions

A

Delayed haemolytic - jaundice, anaemia

Fe overload - typically from chronic transfusion in SCA or Thal Major

Post-transfustion purpura

GvHD - diarrhoea, skin rash, raised LFTs, pancytopenia

26
Q

Aplastic anaemia causes

A

Inherited
 Fanconi’s anaemia: Ashkenazi, short, pigmented
 Dyskeratosis congenita: premature ageing
 Swachman-Diamond syn.: pancreatic exocrine
dysfunction

Acquired
 Drugs
 Viruses: parvovirus, hepatitis
 Autoimmune: SLE

Rx = transfusions, ATG for immunosuppression
Allogenic BMT is curative

27
Q

Myelodysplastic syndrome

A

 Cytopenias with <20% blasts
 Hypercellular BM
 Defective cells: e.g. ringed sideroblasts or Pelger-Huet anamoly
 30% → AML

Rx = transfusions, EPO, G-CSF
Allogenic BMT is curative

28
Q

CML disorders

A

 RBC → Polycythaemia Vera (secondary is physiological, or pseudo if reduced plasma volume)
Rx = aspirin 75mg OD, venesection if young / hydroxycarbamide if older

 WBC → CML

 Platelets → Essential thrombocythaemia
Rx = aspirin if 400-1000; >1000=hydroxycarbamide. Can also use anagralide

 Megakaryocytes → Myelofibrosis. 5 year median survival; supportive with blood products and can perform splenectomy. BMT can be curative in younger pts

29
Q

ALL

A

All children get ALL
Mainly B lineage

RF: radiation during pregnancy and Down’s

Mx:
Supportive - blood products, allopurinol, portacath, Rx for infxns
Chemotherapy - remission induction –> consolidation and CNS Rx –> maintenance for 2-3 years
BMT best in younger pts

30
Q

AML FAB classification:

A

 M2: granulocyte maturation
 M3: acute promyelocytic leukaemia – t(15;17). Rx = ATRA
 M4: acute myelomonocytic leukaemia
 M7: megakaryoblastic leukaemia – trisomy 21

31
Q

AML

A 
RF:
 Chromosomal abnormalities
 Radiation
 Down’s
 Chemotherapy: e.g. for lymphoma
 Myelodysplastic and myeloproliferative syndromes

Mx:
Supportive: as for ALL
Chemotherapy
 V. intensive → long periods of neutropenia and ↓ plats
 ATRA for APML
BMT allogenic if poor prognosis and autogenic if intermediate

32
Q

CLL

A

↑ WCC = lymphocytosis; Smear cells; +ve DAT; immunophenotyping to distinguish from NHL

Can have Richter Transformation: CLL → large B cell lymphoma

Rx:
Indications
 Symptomatic
 Ig genes un-mutated (bad prognostic indicator)
 17p deletions (bad prognostic indicator)
Supportive care
Chemotherapy
 Cylophosphamide
 Fludarabine
 Rituximab
Radiotherapy
 Relieve LN or splenomegaly

Prognosis:
 1/3 never progress
 1/3 progress with time
 1/3 are actively progressing

33
Q

Evan’s syndrome

A

CLL, AIHA, ITP

34
Q

CML

A

t(9,22) = Philadelphia chr

Natural Hx
 Chronic phase: <5% blasts in blood or DM
 Accelerated phase: 10-19% blasts
 Blast crisis: usually AML, ≥20% blasts

Rx:
Imatinib: tyrosine kinase inhibitor
 → >90% haematological response
 80% 5ys
Allogeneic SCT
 Indicated if blast crisis or TK-refractory
35
Q

Non-hodgkin lymphoma classification

A

B Cell (commonest)
 Low Grade: usually indolent but often incurable
 Follicular
 Small cell lymphocytic (=CLL)
 Marginal Zone (inc. MALTomas)
 Lymphoplamsacytoid (e.g. Waldenstrom’s)

 High Grade: aggressive but may be curable
 Diffuse large B cell (commonest NHL)
 Burkitt’s

T Cell
 Adult T cell lymphoma: Caribs and Japs – HTLV-1
 Enteropathy-assoc. T cell lymphoma: chronic coeliac
 Cutaneous T cell lymphoma: e.g. Sezary syn.
 Anaplastic large cell

Mx:
RCHOP for high grade
BMT if relapse

36
Q

Hodgkin’s lymphoma

A

Pel Ebstein fever - cyclical fever
Use Ann Arbor system (NHL as well) for staging
1. Single LN region
2. ≥2 nodal area on same side of diaphragm
3. Nodes on both sides of diaphragm
4. Spread byond nodes: e.g. liver, BM
 + B if constitutional symptoms

Mx:
 Chemo, radio or both
 ABVD regimen
 BMT for relapse

37
Q

MM

A 
CRAB
 Ca ↑ (>2.6mM)
 Renal insufficiency
 Anaemia (<10g/dL)
 Bone lesions
X-ray: Skeletal Survey
 Punched-out lytic lesions
 Pepper-pot skull
 Vertebral collapse
 Fractures

Mx:
Supportive with bisphosphonates and analgesia
Fit pts = Induction chemo: lenalidomide + low-dose dex then allogenic BMT
 Unfit pts = Chemo only: melphalan + pred + lenalidomide. Bortezomib (also used for mantle cell lymphoma) for relapse

Poor prognostic indicators
 ↑ β2-microglobulin
 ↓ albumin

38
Q

Causes of massive spleen (>20cm)

A 
 CML
 Myelofibrosis
 Malaria
 LeishManiasis
 Gaucher’s (AR, glucocerebrosidase deficiency)
39
Q

Splenectomy signs

A

Film
 Howell-Jolly bodies
 Pappenheimer bodies
 Target cells

40
Q

Smoldering myeloma

A

 Se monoclonal protein and/or BM plasma cells ≥10%

 No CRAB

41
Q

MGUS

A

 Se monoclonal protein <30g/L
 Clonal BM plasma cells <10%
 No CRAB

42
Q

Waldenstrom’s Macroglobulinaemia

A

 Lymphoplasmacytoid lymphoma→ monoclonal IgM band

Features:
 Hyperviscosity: CNS and ocular symptoms
 Lymphadenopathy + splenomegaly
 Ix: ↑ESR, IgM paraprotein

43
Q

AL Amyloidosis

A

Features
 Renal: proteinuria and nephrotic syndrome
 Heart: restrictive cardiomyopathy, arrhythmias, echo - “Sparkling” appearance on echo
 Nerves: peripheral and autonomic neuropathy, carpal tunnel.
 GIT: macroglossia, malabsorption, perforation,
haemorrhage, hepatomegaly, obstruction.
 Vascular: periorbital purpura (characteristic)

AL amyloid may respond to therapy for myeloma

44
Q

AA Amyloidosis

A

Amyloid derived from serum amyloid A, an acute phase protein therefore chronic inflammation causes e.g.
 RA
 IBD
 Chronic infection: TB, bronchiectasis

Features
 Renal: proteinuria and nephrotic syndrome
 Hepatosplenomegaly

AA amyloid may improve with underlying condition

45
Q

Familial Amyloidosis

A

 Group of AD disorders caused by mutations in
transthyretin (produced by liver)
 Features: sensory or autonomic neuropathy
 Liver Tx may be curative for familial amyloidosis

46
Q

non-Systemic Amyloidosis

A

 β-amyloid: Alzheimer’s
 β2 microglobulin: chronic dialysis
 Amylin: T2DM

47
Q

Hyperviscosity Syndrome

A

Causes
 ↑↑RBC / Hct >0.5: e.g. PV
 ↑↑WCC > 100: e.g. leukaemia
 ↑↑ plasma proteins: Myeloma, Waldenstrom’s

Features
 CNS: headache, confusion, seizures, faints
 Visual: retinopathy → visual disturbance
 Bleeding: mucus membranes, GI, GU
 Thrombosis

Ix
 ↑ plasma viscosity (PV)
 FBC, film, clotting
 Se + urinary protein electrophoresis

Rx
 Polycythaemia: venesection
 Leukopheresis: leukaemia
 Avoid transfusing before lowering WCC
 Plasmapheresis: myeloma and Waldenstrom’s
48
Q

Tumour Lysis Syndrome

A

Massive cell destruction
 High count leukaemia or bulky lymphoma

↑K, ↑urate → renal failure

Prevention: ↑ fluid intake + allopurinol

49
Q

Basophilia causes

A

 Parasitic infection
 IgE-mediated hypersensitivity: urticarial, asthma
 CML

50
Q

Eosinophilia causes

A

 Parasitic infection
 Drug reactions: e.g. c¯ EM
 Allergies: asthma, atopy, Churg-Strauss
 Skin disease: eczema, psoriasis, pemphigus

51
Q

ΔΔ of ESR >100

A 
 Myeloma
 SLE
 GCA
 AAA
 Ca prostate
52
Q

Myeloblasts cytochemistry

A

 Sudan black B
 MPO
So used for CML

53
Q

Hairy cell leukaemia cytochemistry

A

tartrate resistant acid phosphatase

54
Q

Leukocyte alkaline phosphatase

A

 ↑: PV, ET, MF

 ↓: CML, PNH

Finals PACES (12 decks)

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