Acquired hemolytic anemia

Dairion Gatot, Savita Handayani, Heny, Andri M

Divisi Hematology -Onkology Medic

Internal Departement of Medical Faculty of North Sumatera University /
Haji Adam Malik General Hospital,
Medan 2015
 Definition
• Anemia due to shortened survival of red cells in the
circulation coupled with increased erythropoiesis not
due to genetic or congential disorder.
• Anemia is the result of premature destruction
of red cells exceeding the erythropoietic
capacity of the bone marrow.
 Classification of Hemolytic anemias
I. Red cell abnormality (Intracorpuscular factors)
A. Hereditary
1. Membrane defect (spherocytosis, elliptocytosis)
2. Metabolic defect (Glucoze-6-Phosphate Dehydrogenaze
deficiency, Pyruvate kinase deficiency)
3. Hemoglobinopathies (unstable hemoglobins,
thalassemias, sickle cell anemia )

B. Acquired
1. Membrane abnormality : paroxysmal nocturnal
hemoglobinuria (PNH)
II. Extracorpuscular factors
A. Immune hemolytic anemias
1. Autoimmune hemolytic anemia
- caused by warm-reactive antibodies
- caused by cold-reactive antibodies
2. Transfusion of incompatible blood

B. Nonimmune hemolytic anemias

1. Chemicals
2. Bacterial infections, parasitic infections (malaria),
venons
3. Hemolysis due to physical trauma
- Hemolytic Uremic Syndrome (HUS)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Prosthetic heart valves
4. Hypersplenism
 Pathophysiology

Hemolysis may occur in two compartments

I. Intravascular
II. Extravascular
Intravascular hemolysis :

- red cells destruction occurs in vascular space

- clinical states associated with Intravascular hemolysis:
acute hemolytic transfusion reactions
severe and extensive burns
paroxysmal nocturnal hemoglobinuria
severe microangiopathic hemolysis
physical trauma
bacterial infections and parasitic infections (sepsis)
 INTRAVASCULAR HAEMOGLOBIN DEGRADATION

Free Hb in blood
Haptoglobin (102 mg/dL)

Hb-haptoglobin liver (catabolism same as extravascular)

Hb
in excess of haptoglobin
ab dimers
Methaemoglobin
kidney
globin amino acid pool
tubular
reabsorption
Heme
(Fe+++)
Urine Urine
haemoglobin haemosiderin haemopexin
haemopexin-heme

albumin
methemalbumin

albumin
heme

RE cells in liver
Extravascular hemolysis :
- red cells destruction occurs in reticuloendothelial
system
- clinical states associated with extravascular hemolysis
:
autoimmune hemolysis
delayed hemolytic transfusion reactions
hemoglobinopathies
hereditary spherocytosis
hypersplenism
hemolysis with liver disease
 Clinical Manifestations in Summary
• Symptoms and signs of anemia
• Onset may be acute or insidious
• Jaundice
– Acholuric
– Without pruritus
• Splenomegaly
• Changes in urine color
• Symptoms and signs spesific to the type of hemolytic anemia
• Symptoms related to the underlying disease
Laboratory features:
1. Increased RBC destruction
- reduces RBC life span
- increased indirect bilirubin
- increased serum LDH
- decreased or absent haptoglobin

2. Increased bone marrowactivity and RBC production

a. Blood smear
- reticulositosis
- anisopoikilocytosis, spherocytes
- erythroblastosis
- schistocytes

b. Bone marrow smear

- erythroid hyperplasia
• Signs of intravascular hemolysis
– Hemoglobinemia*
– Hemoglobinuria*
– Hemosiderinuria*
– Methemalbuminemia

 Sign of extravascular hemolysis :

- hemosiderosis
DIAGNOSIS OF HEMOLYTIC ANAEMIA
1. Confirm hemolysis is present
2. Determine the etiology
Basic steps are :
3. Evaluation of clinical information from a review of the
history & physical examination
4. Evaluation of the basic blood examination &
specialized laboratory examination
5. Bone marrow examination
 Hemolytic Anemia (CRC>2% + no blood loss)

Sphero- Sickled Bite Target Schisto- parasite Acantho-

cytes cells cells cells cytes inclusions cytes

DAT DAT Hgb G6PD PT/PTT

(+) (-) e-phoresis level Crea
platelets

Thalas- TTP-HUS
G6PD Malaria
Auto- Heredi- semias DIC
Deficient Babe-
Immune tary Hemo- Prosthe- Liver
Sickle Vs siosis
Hemo- Sphero- globino- Tic Valve Ds
Cell Unstable Barto-
lytic cytosis pathy Malignant
Ds Hgbs nella
Anemia Liver Ds HTN
 Treatment :

• Treatment of underlying disorder

• Removal of offending drug
• Steroids, splenectomy, IV gamma globulin
• Washed RBC transfusion