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rbc 3 hemolytic anemia (membrane (Paroxysmal Nocturnal Hemoglobinuria…
rbc 3 hemolytic anemia
intrinsic: hereditary
membrane
Paroxysmal Nocturnal Hemoglobinuria
treatment
complement
eculizumab
thrombosis
warfarin
marrow
HSCT hematopoetic stem cell transplantation
diagnosis
flow cytometry
no GPI achor
aetiology
X chromosome: somatic mutation
2 venous thrombosis, 3 bone marrow failure
1 intravascular hemolysis
complement
no CD55, CD59
no GPI anchor
south east asian ovalocytosis
ovalocytes, stomatocytes
hereditary elliptocytosis
elliptocytes
hereditary spherocytosis
treatment
(overwhelming post-splenectomy infection OPSI) splenectomy
diagnosis
(-ve Coombs)
flow cytometry
spherocytes
blood film
spherocytes
CBC
high MCHC
aetiology
autosomal dominant
vertical interactions
enzyme
G6PD
aetiology
X-linked recessive
intravascular hemolysis
oxidized Hb
oxidative stress
diagnosis
blood film
Heinz body, bite/blister cell
biochemistry
normal G6PD (reticulocyte high G6PD)
treatment
x oxidant stress
maintain urine output (Hb: nephrotoxic)
unstable Hb: malaria
thalamssemia
variant Hb
extracorpuscular: acquired
x immune
red cell fragmentation syndromes
schistocytes
microangiopathic hemolytic anemia
TTP/HUS
diagnosis
micrangiopathic hemolytic anemia
thrombocytopenia
renal impairent
fever
fluctuating neurlogical signs
aetiology
HUS
thrombosis
O157 O104/Shigella: Shiga(-like) toxins
TTP
thrombosis
ultra-large vWF
DIC
aetiology
consumption: bleeding
microthrombi: infarct
diagnosis
increase clotting time: APTT, PT
thrombocytopenia
decrease fibrinogen
increase fibrin degradation products e.g. D-dimer
pre-eclampsia/HELLP
AVM
heart
infection
chemical
v immune
autoimmune hemolytic anemia
cold AIHA
IgM autoantibody on RBC
intravas hemolysis
diagnosis
cold environment: red cell agglutination
treatment
v monoclonal antibody: CD20: rituximab
x splenctomy (post-splenectomy sepsis)
v warm
warm AIHA
IgG autoantibody on RBC
extravas hemolysis
treatment
x cold
v splenctomy (overwhelming post-splenectomy infection OPSI)
v monoclonal antibody: CD20: rituximab
diagnosis
(+ve Coombs)
flow cytometry
spherocytes
blood film
spherocytes
CBC
spherocytes
warm AIHA + ITP
Evans syndrome
alloimmune hemolytic anemia