Haematology Flashcards
AIP precipitants
alcohol, starvation, infection
barbiturates
sulfonamides
carbamazepine, phenytoin
OCP
rifampicin
managing AIP
IV fluids
IV haemin for acute attack or dextrose
weekly haemin for recurrent non cyclic attacks
GnRH analogues for cyclic attacks in women
diagnosing ALL
> 20% lymphoblasts in bone marrow
peripheral blasts on blood film
t (9;22)/BCR-ABL1 philadelphia, associated with worse prognosis
AML associations/risk factors
Downs, Fanconi, neurofibromatosis
enzymatic polymorphisms
radiation, tobacco, benzene
previous CTx
myelodysplastic syndrome, aplastic anaemia
diagnosing AML
blood film- blasts and Auer rods
anaemia, macrocytosis, leukocytosis, neutropenia, thrombocytopenia
raised LDH
bone marrow- hypercellular, blast infiltration, > 20% blasts
APML
life threatening, requires urgent treatment
anisocytosis
variation in size of RBC
leukaemoid reaction
profound leucocytosis in severe illness and leukaemia
poikilocytosis
variation in shape of RBCs
acquired causes of aplastic anaemia
RTx, CTx
carbamazepine, phenytoin
sulfonamides, chlormaphenicol
indomethaxin
methimazole, propylthiouracil
gold, arsenic
benzene, glue
parvo, EBV, HIV
SLE
thymoma
PNH, MDS
pregnancy, anorexia
inherited causes of aplastic anaemia
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
aplastic anaemia mechanism
deficiency of CD34
autoimmune haemolytic anaemia
warm (commonest, extravasc)
cold (intravasc)
paroxysmal cold (only in < 5y)
assoc w/ SLE, lymphoma, CLL
warm > splenomegaly, thromboembolism
cold > livedo reticularis, ulceration, raynauds, haemaglobinuria
diagnosing AIHI
IgG positive at 4 degrees for paroxysmal cold
IgG positive at 37 degrees for warm
IgG negative for cold
C3 positive in all
managing AIHI
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
when to give anti D to pregnant people
if not already sensitised:
28 and 34 weeks
or large single dose at 28 weeks
also at 12 weeks if any PV bleeding
CLL features
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)
CLL poor prognostic factors
TP53, NOTCH1, SF3B1, ATM, BIRC3
beta2 microglobulin > 3.5
impaired renal function
male
elevated thymidine kinase
17p deletion
CML
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
coag cascade and blood tests
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
G6PD def
x linked recessive
triggers:
fluroquino, dapsone
nitrofurantoin
methylthionium
primaquine
rasburicase
sulfonamides
HUS
triad:
haemolytic anaemia
thrombocytopenia
AKI
treat with heparin, urokinase, FFP, shiga binding, steroids
atypical HUS
causes:
tacrolimus, ciclosporin
complement mutations
SLE, pregnancy
should test for complement mutations and ADAMTS13
treat with plasmapheresis
haemophilia
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
heparin
binds to anti thrombin
inhibits Xa and IIa