Haematology Flashcards

1
Q

AIP precipitants

A

alcohol, starvation, infection
barbiturates
sulfonamides
carbamazepine, phenytoin
OCP
rifampicin

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2
Q

managing AIP

A

IV fluids
IV haemin for acute attack or dextrose
weekly haemin for recurrent non cyclic attacks
GnRH analogues for cyclic attacks in women

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3
Q

diagnosing ALL

A

> 20% lymphoblasts in bone marrow
peripheral blasts on blood film
t (9;22)/BCR-ABL1 philadelphia, associated with worse prognosis

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4
Q

AML associations/risk factors

A

Downs, Fanconi, neurofibromatosis
enzymatic polymorphisms
radiation, tobacco, benzene
previous CTx
myelodysplastic syndrome, aplastic anaemia

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5
Q

diagnosing AML

A

blood film- blasts and Auer rods
anaemia, macrocytosis, leukocytosis, neutropenia, thrombocytopenia
raised LDH
bone marrow- hypercellular, blast infiltration, > 20% blasts

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6
Q

APML

A

life threatening, requires urgent treatment

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7
Q

anisocytosis

A

variation in size of RBC

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8
Q

leukaemoid reaction

A

profound leucocytosis in severe illness and leukaemia

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9
Q

poikilocytosis

A

variation in shape of RBCs

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10
Q

acquired causes of aplastic anaemia

A

RTx, CTx
carbamazepine, phenytoin
sulfonamides, chlormaphenicol
indomethaxin
methimazole, propylthiouracil
gold, arsenic
benzene, glue
parvo, EBV, HIV
SLE
thymoma
PNH, MDS
pregnancy, anorexia

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11
Q

inherited causes of aplastic anaemia

A

fanconi:
auto rec or x linked
hearing loss, pigmentation, urogenital

dyskeratosis congenita:
x linked, TERC, TERT, TINF2, DKC1
nails, reticulated rashes, leukplakia

schwachman diamond:
auto rec, SBDS
exocrine pancreatic, skeletal

GATA2 def:
zinc finger dysfunction

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12
Q

aplastic anaemia mechanism

A

deficiency of CD34

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13
Q

autoimmune haemolytic anaemia

A

warm (commonest, extravasc)
cold (intravasc)
paroxysmal cold (only in < 5y)

assoc w/ SLE, lymphoma, CLL

warm > splenomegaly, thromboembolism
cold > livedo reticularis, ulceration, raynauds, haemaglobinuria

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14
Q

diagnosing AIHI

A

IgG positive at 4 degrees for paroxysmal cold
IgG positive at 37 degrees for warm
IgG negative for cold

C3 positive in all

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15
Q

managing AIHI

A

warm and cold paroxysmal:
glucocorticoids and/or rituximab
second line cyclophosphamide, MMF, aza or splenectomy

cold AIHI:
with Hb < 70 or asymptomatic requires RBC with plasmapheresis
rituximab or bortezomib

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16
Q

when to give anti D to pregnant people

A

if not already sensitised:
28 and 34 weeks
or large single dose at 28 weeks

also at 12 weeks if any PV bleeding

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17
Q

CLL features

A

smudge cells
raised WCC, lymphocytes
low Hb, platelets
CD5, CD19, CD20, CD23
poor prognosis associated w/: TP53, NOTCH1, SF3B1, ATM, BIRC3

> conservative tx if asymptomatic and early
CTx fludra + cyclo + ritux
stem cell transplant, esp if TP53 in remission (poor prognosis)

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18
Q

CLL poor prognostic factors

A

TP53, NOTCH1, SF3B1, ATM, BIRC3
beta2 microglobulin > 3.5
impaired renal function
male
elevated thymidine kinase
17p deletion

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19
Q

CML

A

BCR-ABL 9;22
chronic asymptomatic phase
> accelerated phase
> blast crisis

raised WCC, basophils, eosinophils, low Hb
mature myeloid cells
granulocytic hyperplasia, granulocyte left shift

tx w/ tyrosine kinase inhib imatinib, dasatinib, nilotinib

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20
Q

coag cascade and blood tests

A

PT: extrinsic system and final common pathway
INR is derived from PT

APTT: intrinsic system and final common pathway
if elevated, should assay factors VIII, IX, XI, XII

TT: final part of common pathway
prolonged by lack of fibrinogen

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21
Q

G6PD def

A

x linked recessive
triggers:
fluroquino, dapsone
nitrofurantoin
methylthionium
primaquine
rasburicase
sulfonamides

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22
Q

HUS

A

triad:
haemolytic anaemia
thrombocytopenia
AKI

treat with heparin, urokinase, FFP, shiga binding, steroids

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23
Q

atypical HUS

A

causes:
tacrolimus, ciclosporin
complement mutations
SLE, pregnancy

should test for complement mutations and ADAMTS13
treat with plasmapheresis

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24
Q

haemophilia

A

A: VIII def
B: IX def
x recessive
prolonged APTT, normal PT and vWF

tx:
VII/IX concentrate
severe bleeding in acquired VIII def requires PTC or recombinant VIIa
vasopressin (not effective for haemophiliaB)
TXA

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25
Q

heparin

A

binds to anti thrombin
inhibits Xa and IIa

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26
Q

heparin contraindications

A

acute bacterial endocarditis
peptic ulcer
recent eye surgery
thrombocytopenia

27
Q

dabigatran reversal

A

idarucizumab

28
Q

DOAC anti factor Xa reversal agent

A

andexanet

29
Q

hereditary spherocytosis

A

negative direct antiglobulin
> splenectomy for severe disease
increased incidence of gallstones

30
Q

hodgkins dx and tx

A

reed sternberg
neutrophilia, eosinophilia, thrombocytosis
normocytic anaemia
raised ESR, LDH
> doxorubicin, bleomycin, vincristine, dacarbazine ABVD
> BEACOPP
> RTx adjunct for stage I or II

31
Q

Ann Arbor staging

A

I: one group of nodes
II: two groups same side of diaphragm
III: both sides diaphragm
IV: extra nodal involvement incl bone marrow

32
Q

primary immunodeficiency mechanisms

A

50% B cell defects
30% T cell
18% phagocytic
2% complement

33
Q

patterns of immunodeficiency

A

B cell:
sinoplumonary infections
e.g. common variable, selevtive IgA, CLL

T cell:
opportunistic infections, invasive viral infections, intracellular bacterial (salmonella, mycobacterium)
e.g. digeorge, SCID, ataxia telangiectasia

neutrophil:
bacterial/fungal skin infections
e.g. chronic granulomatous disease, DM

complement:
recurrent/invasive neisseria infections
assoc w/ SLE, hereditary angioedema

34
Q

effects of lead poisoning and treatment

A

renal: PCT toxicity > Fanconi syndrome
CVS: HTN, CAD, PAD
neurodevelopmental esp in children, cerebellar signs
blue gum line
tx: dimercaprol, succimer or sodium calcium edetate chelation
long term follow up due to storage in bones

35
Q

methaemaglobinaemia causes

A

congenital:
cytochrome B5 reductase def
pyruvate kinase def
haemaglobin M disease

acquired
local anaesthetics
aniline dyes, benzenes
chloroquine, nitrites, metoclopramide
dapsone, sulfonamide
smoke

Fe2+ oxidised to Fe3+, unable to bind oxygen
> dissociation curve left shift (increased oxygen affinity)

36
Q

treating methaemoglobinaemia

A

paO2 might be normal but saO2 reduced

treat if > 30% or symptomatic
IV dextrose
oxygen
methylene blue to reduce Fe3+
ascorbic acid if MB contraindicated (e.g. G6PD def)

37
Q

MGUS diagnostic criteria

A

monoclonal M protein in serum or urine
< 10% plasma cells in bone marrow
absence of lytic lesions/anaemia/hypercalcaemia/renal insufficiency/amyloidosis

osteoporosis, risk of fracture
more common in older, male, black people

38
Q

types of MGUS

A

IgG, IgA can progress to multiple myeloma
IgM can progress to waldenstroms, CLL, NHL

39
Q

multiple myeloma diagnostic criteria

A

clonal bone marrow plasma cells > 10% or plasmacytoma and end organ damage (hypercalcaemia or renal insufficiency or anaemia or bone lesions)

40
Q

smouldering multiple myeloma diagnostic criteria

A

IgG or IgA > 30
or urinary monoclonal protein > 500
or clonal bone marrow plasma cells 10-60%

41
Q

types of myeloma

A

IgG and IgA most common
IgM and IgE rare

42
Q

multiple myeloma pathophysiology

A

clonal proliferation and accumulation of plasma cells in bone marrow
secrete Ig or Ig light chains
cytokines activate osteoclasts and suppress osteoblasts > bone lesions
bence jones proteins deposited in DCT > kidney disease and hypercalcaemia

43
Q

multiple myeloma presentation

A

CRAB
hyperCalcaemia
Renal impairment
Anaemia
Bone pain

44
Q

staging multiple myeloma

A

beta2 microglobulin
serum albumin

45
Q

myelodysplastic syndrome

A

predisposition for AML
cytopenia is most common presentation
hypercellular marrow
diagnosis of exclusion
Pappenheimer bodies, basophilic stippling

> stem cell transplant only cure
CTx FLAG
azacitidine if not suitable for SCT

46
Q

myeloproliferative disorders

A

abnormal proliferation of RBC or WBC or platelet progenitors in bone marrow
increased fibrosis
> extramedullary haematopoiesis

  • PRV (RBC)
  • CML (WBC)
  • essential thrombocythaemia (platelets)
  • myelofibrosis (fibroblasts)
47
Q

myeloproliferative disorders presentation

A

PRV- hyperviscosity, thrombotic event, JAK2
ET- thrombosis, bleeding
CML- raised WCC
myelofibrosis often asymptomatic

48
Q

treating myeloproliferative disorders

A

hydroxycarbamide, hydroxyurea
antiplatelet, anticoagulant
BCR-ABL tyrosine kinase inhibitor
stem cell transplant

49
Q

myeloproliferative disorders poor prognostic factors

A

age > 65y
Hb < 100
WCC > 25
circulating blasts > 1%
philadelphia negative can spontaneously transform to acute leukaemia

50
Q

causes of neutropenia

A

infection most common
drugs
primary autoimmune (most common cause in children)

congenital:
kostmann, x linked agammaglobulinaemia, schwachman diamond
ethnic variation (african)
cyclical in children rarely

acquired:
malignant marrow infiltration
aplastic anaemia
B12, folate deficiency
CTx, RTx
phenytoin, alcohol, chloramphenicol
EBV, hep B, hep C, HIV, CMV, typhoid
hypersplenism, malaria
NSAIDS, abx, anticonvulsants, diuretics, diabetic meds, antidepressants

51
Q

NHL

A

arises from B cells or T cells of NK cells
tx: CTx +/- RTx
irregular pattern of spread
extra nodal disease is common

52
Q

NHL types

A

B cell high grade:
diffuse large
mediastinal large
primary CNS
burkitts
mantle

B cell low grade:
follicular
MALT
waldenstrom

T high grade:
enteropathy type
peripheral
subcut paniculitis
systemic anaplastic
angio immunoblastic

T low grade:
mycosis fungoides
cutaneous

53
Q

paroxysmal nocturnal haemaglobinuria

A

acquired haemolysis
lack of GPI CD55 and CD59
PIGA gene on x chromosome

triad:
haemolytic anaemia
pancytopenia
large vessel thromboses

direct coombs neg
tx: eculizumab, SCT

54
Q

managing polycythaemia vera

A

low dose aspirin (avoid if vW syndrome)
venesection
maintain hct < 45%
do not supplement iron
high risk: hydroxyurea, INF, bisulfan
INF if young or pregnant

55
Q

infections associated with sickle cells

A

strep pneumoniae
haemophilus
meningococcus

56
Q

thalassaemia diagnosis

A

microcytic anaemia
mexican hat cells in alpha
raised iron and ferritin

57
Q

managing thalassaemia

A

tranfusions
iron chelation
hydroxyurea
ascorbic acid for urine iron excretion
splenectomy, bone marrow transplant

58
Q

platelets and contact sports

A

avoid if < 50 000

59
Q

warfarin induced skin necrosis

A

seen in protein c deficiency

60
Q

tumour lysis syndrome

A

hyperuricaemia
hyperkalaemia
hyperphosphataemia
> obstructive uropathy
> hypocalcaemia

common in high grade NHL

61
Q

managing tumour lysis

A

rapid expansion of volume with crystalloids
allopurinol
recombinant urate oxidase
sodium bicarb to alkalinise urine
calcium
dialysis
febuxostat

avoid gent, NSAIDs, iodine contrast
prophylactic xanthine oxidase inhib

62
Q

vWD

A

auto dom
presents with bruising or mucocutaneous bleeding
more severe bleeding in type III (factor VIII reduction)

ix: normal FBC, aPTT, low vWF
tx: desmopressin for type I, II. concentrates for type III

63
Q
A