Edited :

Dr.Dairion Gatot Sp.PD-KHOM

Hematology & Medical Onkology Division

Internal Departement of Medical Faculty
Universitas Sumatera Utara/H Adam Malik General Hospital
MEDAN-2018
 BIO-DATA
Nama : Dr. Dairion Gatot M.Ked, SpPD-KHOM
Perkerjaan : Dosen Fakultas Kedokteran USU Medan
Alamat : Taman Binjai Indah No. F-4 Jl. T Amir Hamzah
Kelurahan Jati karya Kota Binjai- 20746 Sumatera Utara.
Kantor : Divisi Hematologi & Onkologi Medik Dep Ilmu Penyakit Dalam
FK- USU/RSUP H Adam Malik, Jl Bunga Lau No 17 Medan 20136
HP/Email : 082167626666/ drdairion@yahoo.com

Pendidikan/ 1. Fakultas Kedokteran USU Medan Tahun 1988

Pelatihan : 2. Akupunkur Jakarta-RSCM Tahun 1995
3. Spesialis Penyakit Dalam FK-USU Medan Tahun 2003
4. Turbo-Hipnotis Tahun 2004
5. Hipnotis & Hipnoterapi (IHA/IBH) Tahun 2006/2007
6. Konsultan Hematologi & Onkologi Tahun 2009
7. Certifiated Instruktur Hipnoterapi Tahun 2010
7. Profesional Hipnoterapi Tahun 2013
8. NLP Practitioner Tahun 2016
9. International Body Language Tahun 2016
10. Magister Kedokteran Tahun 2016
 WHEN BY THEN

Hb 9 g% transfusion Hb 4 g%

No sign of bleeding

HEMOLYTIC ?
Definition
• Any situation in which there is a
reduction in RBC life-span due to
increase RBC destruction. Failure of
compensatory marrow response
results in anemia. Predominant site
of RBC destruction is red pulp of the
spleen.
Used Drug or
traditional
Hb
medicine
decreased

no sign of bleeding

Hemolytic ?
 Infection

Anemia +
Hemolytic ?
icterus (mild)
 INTERPRETATION
LABORATORY TEST
NORMOCYTIC
PERIPHERAL SMEAR ANAEMIA
RETICULOCYTE COUNT INCREASED REDUCED

BLOOD IN STOOL OR
OTHER SOURCE OF POSITIVE NEGATIVE
BLEEDING IDENTIFIED

ANTIHUMAN GLOBULIN POSITIVE NEGATIVE NEGATIVE

(COOMBS’TEST)

BONE MARROW AND BONE HYPERCELLULAR HYPERCELLULAR DECREASED REPLACEMENT OF

MARROW BIOPSY ERYTHROID ERYTHROID CELLULARITY NORMAL MARROW
HYPERPLASIA HYPERPLASIA ELEMENTS

DIAGNOSIS BLOOD AUTO IMMUNO OTHER HAEMOLYTIC OTHER MYELOPHTHISIC

LOSS HAEMOLYTIC ANAEMIAS Renal disease Tumor
ANAEMIA ANAEMIA
Parasites Infection Myelofibrosis
Erythroblastosis
Hypersplenism Malnutrition Infection
foetalis
Microangiopathic Aplastic Leukaemia
Transfusion
haemolysis anaemia
reaction
Hereditary Radiation
Collagen
spherocytosis
vascular
Paroxysmal noctural
disease
haemoglobinuria
Enzyme deficiencies
Drug or toxin
Haemoglobinopathies
normositic normochromic anemia
• Diagnostic:
– MCV 80-100 fL and
– MCH > 27 pg or MCHC ³ 30 g/dL
• Cause :
– Distribution failure .
– Lack of production,
– Rate of RBC destruction
 reduced red-cell life span
Haemolytic anaemia
• Rate of RBC destruction

RBC production

Reticulocyte counts
Classification of Hemolytic anemias

I. Red cell abnormality (Intracorpuscular

factors)
A. Hereditary
1. Membrane defect (spherocytosis, elliptocytosis)
2. Metabolic defect (Glucoze-6-Phosphate-Dehydrogenaze
(G6PD) deficiency, Pyruvate kinase (PK) 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
Mechanisms of hemolysis:

- Intravascular
- Extravascular
Inravascular hemolysis (1):

- 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)
Inravascular hemolysis (2):

- laboratory signs of intravascular hemolysis:

indirect hyperbilirubinemia
erythroid hyperplasia
hemoglobinemia
methemoalbuminemia
hemoglobinuria
absence or reduced of free serum haptoglobin
hemosiderinuria
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
 Extravascular hemolysis (2):

Laboratory signs of extravascular hemolysis:

indirect hyperbilirubinemia
increased excretion of bilirubin by bile
erythroid hyperplasia
hemosiderosis
Hemolytic anemia - clinical features:

- pallor
- jaundice
- splenomegaly
Laboratory features:
1. Laboratory features
- normocytic/macrocytic, hyperchromic anemia
- reticulocytosis
- increased serum iron
- antiglobulin Coombs’ test is positive

2. Blood smear
- anisopoikilocytosis, spherocytes
- erythroblasts
- schistocytes

3. Bone marrow smear

- erythroid hyperplasia
DIAGNOSIS OF HEMOLYTIC ANAEMIA

Basic steps are :

1. Evaluation of clinical information from a review of the
history & physical examination
2. Evaluation of the basic blood examination &
specialized laboratory examination
3. Bone marrow examination
Diagnosis of hemolytic syndrome:

1. Anemia
2. Reticulocytosis
3. Indirect hyperbilirubinemia
Autoimmune hemolytic anemia -
diagnosis

- positive Coombs’ test

Treatment:
- steroids
- splenectomy
- immunosupressive agents
- transfusion
G-6PD deficiency
G6-PD activity
G-6PD deficiency
• X-linked disorders, heterozygote females only
rarely have significant haemolysis
• Haemolysis cause by infection, acidosis, drugs & toxins
• Red blood cells membrane oxidation
• Precipitation of haemoglobin Heinz bodies
• PB smear bite cells
• In acute haemolytic episode G-6PD activity
maybe normal
Diagnosis :
Low level G-6PD concentration
Treatment :

1. Avoid of drugs exposure.

2. Transfusion .
3. No spesific treatment.
4. Anti oksidan, ???.
 Drug exposure of hemolytic.

• Asetanilid primakuin
• Furazolidon sulfasetamid
• Biru metilen sulfametoksazol
• Asam nalidixat sulfanilamid
• Naftalen sulfapiridin
• Nitrofurantoin biru toluidin
• Fenazopirid trinitrotoluen
• Fenilhidrazin jamu
4. Treatment :

- washed RBC transfusion

- iron therapy
- allogenic bone marrow transplantation
Haemolytic crisis
 Increased rate of haemolysis
 Reticulocytosis
 Painful crisis stasis blood vessel
auto
splenectomia
priapism

retinopathy
necrosis
papilla renalis