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MRCP 2 > Haematology > Flashcards

Haematology Flashcards

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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
heparin
binds to anti thrombin inhibits Xa and IIa
26
heparin contraindications
acute bacterial endocarditis peptic ulcer recent eye surgery thrombocytopenia
27
dabigatran reversal
idarucizumab
28
DOAC anti factor Xa reversal agent
andexanet
29
hereditary spherocytosis
negative direct antiglobulin > splenectomy for severe disease increased incidence of gallstones
30
hodgkins dx and tx
reed sternberg neutrophilia, eosinophilia, thrombocytosis normocytic anaemia raised ESR, LDH > doxorubicin, bleomycin, vincristine, dacarbazine ABVD > BEACOPP > RTx adjunct for stage I or II
31
Ann Arbor staging
I: one group of nodes II: two groups same side of diaphragm III: both sides diaphragm IV: extra nodal involvement incl bone marrow
32
primary immunodeficiency mechanisms
50% B cell defects 30% T cell 18% phagocytic 2% complement
33
patterns of immunodeficiency
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
effects of lead poisoning and treatment
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
methaemaglobinaemia causes
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
treating methaemoglobinaemia
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
MGUS diagnostic criteria
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
types of MGUS
IgG, IgA can progress to multiple myeloma IgM can progress to waldenstroms, CLL, NHL
39
multiple myeloma diagnostic criteria
clonal bone marrow plasma cells > 10% or plasmacytoma and end organ damage (hypercalcaemia or renal insufficiency or anaemia or bone lesions)
40
smouldering multiple myeloma diagnostic criteria
IgG or IgA > 30 or urinary monoclonal protein > 500 or clonal bone marrow plasma cells 10-60%
41
types of myeloma
IgG and IgA most common IgM and IgE rare
42
multiple myeloma pathophysiology
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
multiple myeloma presentation
CRAB hyperCalcaemia Renal impairment Anaemia Bone pain
44
staging multiple myeloma
beta2 microglobulin serum albumin
45
myelodysplastic syndrome
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
myeloproliferative disorders
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
myeloproliferative disorders presentation
PRV- hyperviscosity, thrombotic event, JAK2 ET- thrombosis, bleeding CML- raised WCC myelofibrosis often asymptomatic
48
treating myeloproliferative disorders
hydroxycarbamide, hydroxyurea antiplatelet, anticoagulant BCR-ABL tyrosine kinase inhibitor stem cell transplant
49
myeloproliferative disorders poor prognostic factors
age > 65y Hb < 100 WCC > 25 circulating blasts > 1% philadelphia negative can spontaneously transform to acute leukaemia
50
causes of neutropenia
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
NHL
arises from B cells or T cells of NK cells tx: CTx +/- RTx irregular pattern of spread extra nodal disease is common
52
NHL types
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
paroxysmal nocturnal haemaglobinuria
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
managing polycythaemia vera
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
infections associated with sickle cells
strep pneumoniae haemophilus meningococcus
56
thalassaemia diagnosis
microcytic anaemia mexican hat cells in alpha raised iron and ferritin
57
managing thalassaemia
tranfusions iron chelation hydroxyurea ascorbic acid for urine iron excretion splenectomy, bone marrow transplant
58
platelets and contact sports
avoid if < 50 000
59
warfarin induced skin necrosis
seen in protein c deficiency
60
tumour lysis syndrome
hyperuricaemia hyperkalaemia hyperphosphataemia > obstructive uropathy > hypocalcaemia common in high grade NHL
61
managing tumour lysis
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
vWD
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

MRCP 2 (12 decks)

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