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Approach to Hemolysis

Kuang Pu
Division of Hematology, West China Hospital
May 15, 2015

Pathogenesis in Hemolysis
Unconjugated
Bilirubin

Lactose
Dehydrogenase
( )

Reticulocyte

Lifespan of RBC
Hemopoiesis

Hemoglobin/HCT
/

Haptoglobin
( )

Clinical manifestation of Hemolysis

Anemia,

Icterus,
Discloration of Urine,

Extravascular
Hereditary
Membrane Defects
Spherocytosis

Elliptocytosis

Intravascular
Hereditary
Enzyme Deficiency
G6PD Deficiency
Pyruvate Kinase Deficiency

Acquired

Paroxysmal Nocturnal
Hemolysis
Hemoglobinopathies
Hemoglobinuria

Sickle Cells Anemia


Unstable Hemoglobins

Acquired
Autoimmune Hemolytic
Anemias( )
Associated with warm
antibodies
Associated with cold
antibodies

Hypersplenism( )

Drug induced Hemolytic


Anemias
Infections

PlasmodiamU( ),
Clostridium ( )

Isoimmune Hemolytic
Anemias
ABO mismatched
transfusion

Microangiopathic Hemolytic
Anemias
DIC
HUS-TTP

Unconjugated
bilirubin

Macrophage

Liver
Globin

Amino
Acids

Iron

Heme

Conjugated
bilirubin

Binds to
transferrin

Reabsorbed
Urobillinogen

(urine)

Spleen

Stercobillinogen

(feces)

Extra vascular hemolysis


Destruction of RBC occurs extra-vascularly.
Most commonly seen.
Macrophages in the reticuloendothelial

system, particularly the spleen are


responsible for clearance of RBC fragments

Renal tubule
(proximal)

Kidney

Hemoglobulin

Haptoglobin

Liver

Hemoglobinuria

Hemosideruria

Intra vascular Hemolysis


Destruction of RBC occurs intravascularly
Not commonly seen, acute onset
Hemoglobin released into the blood
Metabolize through
Haptoglobin( ) Liver
Proximal tubule of kidney
Hemosiderin Prussian Blue( )
or Hemoglobin in urine

Extra vascular Intra vascular


History
Onset
Splenomegaly

Chronic->acute
Yes

acute
no

yes

yes

Absent
N
)variable)

Absent

(variable)

- ve

+ ve

Serum
Anemia
Bilirubin
(unconjugated)
Haptoglobin
Plasma Hb
Lactate
Dehydrogenase
Urine
Bilirubin
Hemosiderin/Rous

Any Questions

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