Haem Flashcards
Acute manifestations of sickle cell
- sickle cell crisis
- AVN + ischaemia due to vasoocclusive crisis
- Acute chest syndrome
- Spleen sequestration = severe anaemia + hypovolaemic shock + hyposplenism
- Aplastic crisis
Features of thalaessemia
microcytic anaemia
anaemia symptoms
splenomegaly
jaundice
Hereditary thrombotic disorders
- factor lv leiden (activated protein c resistance) - thrombophilia
- prothrombin gene mutation
- antithrombin 3 def
- protein s def
- protein c def
Causes of cold hemolytic anaemia vs warm
cold: lymphoma, mycoplasma, ebv
warm: cll, sle, lymphoma, idiopathic - extravascular
What to see on blood film for sickle cell
sickle celled rbcs
Howell jolly bodies
nucleated rbcs
Screening for hemolysis
LDH - released by cells
Blood film - shistocytes
Haptoglobin - is low as binds free hb
Bilirubin
Reticulocytes - high if bm is functioning
Coombs test
(MCV = normal or high)
Features paroxysmal nocturnal hemoglobinuria
piga gene
hams test +
anaemia
hemolglobinuria in morning
thrombosis
needs anticoagulant
Features of G6PD deficiency
reduced NADPH so inc oxidative stress to rbcs
mostly males as x linked
Neonatal jaundice
Splenomegaly
Heinz bodies, blister cells
Inc risk when primaquine, ciprofloxacin, sulfate drugs, fava beans, gliclazide
G6PD enzyme assay 3 months after episode
Features wvf disease
most common bleeding disorder, needed for platelet aggregation in damaged vessels
auto dom
mucosal bleeding
menorrhagia
Is a carrier for factor 8 in intrinsic pathway
Inc APTT, inc bleeding time
Factor 8 reduced
desmopressin, tranexamic acid, vwf infusion + factor 8
Causes normocytic anaemia
Anaemia chronic disease
Acute blood loss
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
Features haemophilias
Mainly males as x linked recessive
haemoarthrosis
mucosal bleeding
affects intrinsic pathway hence
inc aptt
normal bleeding time, thrombin and PT
Liver disease clotting results
dec 7-11, fibrinogen
- inc inr, inc aptt, fibrinogen dec, platelets dec
Causes of normoblastic macrocytic anaemia
Alcohol
Reticulocytosis
hypothyroidism
liver disease
Features of hereditary spherocytosis
auto dom
fragile sphere shaped rbcs
anaemia
jaundice
splenomegaly
gallstones
aplastic crisis if parvovirus
Inc MCHC, reticulocytes
Spherocytes
Needs folate, transfusions, splenectomy
Causes of microangiopathic hemolytic anaemia
destruction rbcs as they travel through circulation via mini thrombi causing obstruction
Usually due to : DIC, HUS, TTP, SLE, cancer
Schistocytes
b thalaessemia features
trait: mild microcytic anaemia
intermedia: anaemia, occasional blood transfusions + desferrioxamine as iron overload likely
major: severe, frontal bossing, enlarged maxilla, protruding upper teeth, may also need splenectomy
Heinz bodies!
Clotting cascade pathways
Intrinsic activated when damaged surface: factor 12 -> factor 8
Extrinsic releases tissue factors: factor 7
Combined: factor 10 which can then turn prothrombin to thrombin which turns fibrinogen to fibrin making a clot
Clotting screen
PT (extrinsic pathway): therefore affected by liver, DIC, vit k def, warfarin levels
APTT (intrinsic pathway): so haemophilias, vwf
Bleeding time: overall platelet function
Thrombin time: tests how fast fibrinogen turns into fibrin
Causes of low platelets
b12/f def
malignancy
liver failure
ITP
HIT
HUS
TTP
Features of ITP
ab against platelets
purpura non blanching rash on legs
bruising/bleeding
Needs pred, ivig if active bleeding, rituximab
Features of thrombotic thrombocytopenic purpura
tiny thrombi using up platelets
purpura
tissue ischaemia
Plasma exchange, steroids, rituximab (can’t treat with a platelet transfusion !!)
Features of DIC
Widespread clotting and resultant bleeding
Inc APTT
Inc PT
Dec platelets
Dec fibrinogen
Inc fibrinogen degradation products
Inc bleeding time
Causes DIC
sepsis
trauma
malignancy
obstetrics: haemorrhage, HELLP
What deficiency increases the risk of anaphylactic blood transfusion
IgA deficiency
Reversal of dabigitran
Idarucizumab
Lymph node biopsy for HL
reed Sternberg cells
Type cell in CLL
B cell
Binets staging for CLL
A - <2 lymphoid sites
B - >3 lymphoid sites
C - anaemia and/or thrombocytopenia
CLL blood film
smear/smudge cells
Prognostic indicators for myeloma
serum b2 microglobulin
albumin
Prognostic factors for ALL
Age 1-10 yrs better
WCC>50 at presentation poor
Female better prognosis
CNS involvement poor
Hyperdiploid blast cells is good
Prognostic factors for AML
age >60, poor performance status, comorbs, previous dysplasia, previous chemo/radio exposure
Spreads to CNS, skin, gums
What’s in cryoprecipitate
Factor 8, fibrinogen, von Willebrand factor, and Factor XIII
Blood film for primary myeloproliferative disorder
poikilocytes
Features of amyloidosis
kidneys: nephrotic syndrome
gi: hepatosplenomeg
neurological
vasculature: periorbital purpura
joints: large joint inflamm
heart: cardiomyopathy
apple green birefringence when stained with Congo red
Mx amyloidosis
dexamethasone
bortezomib
assess response via free light chain assay
Warfarin clotting results
2,7,9,11
Intrinsic and extrinsic
PT inc, APTT
Indications: mech HV, antiphospholipid
Interacts with certain meds, come to a&e if any bleeding, no contact sports
Test to diagnose hereditary spherocytosis
auto dom, high MCHC
EMA test
Splenectomy indications
hereditory spherocytosis
SCD
trauma
ITP
Complications of hemolytic anaemia
gallstones - pigmented as inc bilirubin
HF due to high output
Kernicterus in child: bilirubin crosses BBB causing weak cry, floppy and then when gets bad then hypertonia + high pitched cry + irritable - needs plasma exchange
Complication of polycythaemia vera
Myelofibrosis
AML
budd chairi
Leukaemia complications
AVN
TLS
Growth stunt
Secondary cancers
Infertility
Myeloma diagnosis
> 30g/l paraproten on serum (if < then MGUS)
10% plasma cells on bm biopsy
CRAB - ca inc, renal impair, anaemia, bone pain due to lesions
Diagnosis of leukaemia
leukaemia- >20% blast cells in bm for diagnosis
How come if mum is different blood group to baby no hemolytic anaemia but there is if different rhesus status
bc igM doesnt cross placenta
but igG does
Symptoms of ABO mismatch
fever
abdo pain
urticaria
hypotension
AKI/hemoglobinuria
Irradiated blood
Hodgkins
fludarabine
hsct
T cell immunodeficiency
CMV- blood
intrauterine transfusion
elective pregnancy transfusions
Threshold for rbc transfusion
<70 normal
<80 cvs disease
<90 critical source of bleeding
Differentials for DVT in pregnant lady
cellulitis
venous insufficiency
pre-eclampsia (oedema)
How to decrease periop bleeding
What causes renal impairment in myeloma
Hypercalcaemia
Light chain deposition
Hyperviscosity
Renal stones
