Hemolyticanemia afnan

HEMOLYTIC ANEMIA
Afnan Shamraiz

OBJECTIVES
• Lab indication of hemolysis
• Intravascular v/s extravascular hemolysis
• D/D of hemolytic anemia
• Diagnose hemo.anemia with peripheral smear &
ancillary lab tests

NORMAL RED CELLS
drpankajyadav05@gmail.com
No nucleus
Biconcave discs
Center 1/3 pallor
Pink cytoplasm (Hb filled)
Cell size 7- 8 µ - capill.
Negative charge
100-120 days life span

THE FACTORY – BONE
MARROW
Sternum, pelvis, vertebrae, long
bones, skull bones, Tibia (paed)
From stem cells (pleuripotent)
75% of marrow for WBC
25% of BM for Red cells
Erythrod / Granulocyte Ratio 1:3
Large white areas are marrow
fat

ERYTHROPOIESIS
15-20µm- basophilic cytoplasm,
nucleus with nucleoli.
14-17µm-mitosis, basophilic
cytoplasm, nucleoli disappears.
10-15µm-’POLYCHROMASIA’
Hb appears, nucleus condenses.
7-10µm- PYKNOTIC Nucleus.
Extrusion, Hb is maximum.
7.3µm- Reticulum of basophilic
material in the cytoplasm.
7.2µm- Mature red cell with Hb.

RETICULOCYTE
• Reticular nuclear
fragments
• Nucleus extruded
• Slightly larger than
RBCs
• Fully mature with in 2
days as their contents
are degraded by
intracellular enzymes.
• Count = 1-2% of red
cells
• Provide an index of
rate of RBC formation

HAEMOLYTIC ANAEMIAS
•The normal red cell life is 110-120(half life 55-
60 days) days after which the senile cells are
removed by bone marrow and splenic
macrophages.
•Reduced red cell survival leads to increased
red cell production due to erythropoietin
drive that can compensate for the reduced
red cell life and maintain a normal Hb level.
•The mean red cell life is affected by
molecular changes in either the red cell
membrane or haemoglobin.

•A haemolytic state exists when the in vivo survival
of the RBC is shortened.
•Anaemia occurs if the onset of haemolysis is
sudden with no time for marrow compensation or in
severe chronic haemolysis when the mean red cell
life is very short.
•The usual marrow response in acute hemolytic
anemia is reflected by a reticulocyte index of 2–3,
whereas in long-standing chronic hemolysis, the
increase in erythropoiesis is approximately 6-fold.

HAEMOLYTIC ANAEMIA
Intravascular
• Intravascular
• red cells lyse in the
circulation and release
their products into the
plasma fraction.
• Anemia
• Decreased Haptoglobin
• Hemoglobinemia
• Hemoglobinuria
• Urine hemosiderin
• Increased LDH
Extravascular
• Increased LDH

• Extravascular
• ingestion of red cells by
macrophages in the liver,
spleen and bone marrow
• Little or no hemoglobin
escapes into the
circulation
• Anemia
• Decreased Haptoglobin
• Normal plasma
hemoglobin

CLINICAL FEATURES
Jaundice: generally mild and often
not noticed by the patient.
Anaemia: recent onset = acquired
long-standing = possibly congenital.
Haemoglobinuria: intravascular haemolysis.
Urobilinogenuria: increased Hb catabolism.
Splenic pain: spenomegaly or splenic
infarction.
Leg ulcers: intrinsic red cell disorders, e.g. sickle cell disease.
Dactylitis; in sickle cell ds
Skeletal hypertrophy: severe congenital haemolytic anaemias
and thalassaemias.

HEMOLYTIC FACIES- CHIPMUNK
FACIES

EVIDENCE OF
ERYTHROPOIESIS
• Polychromasia
• Increased reticulocyte
• “Shift” macrocytosis
• Hypercelluar BM

Nelson texrbook of paediatrics 19the.
Hemolytic
anemia
CELLULAR DEFECTS
Membrane defects
Enzyme defects
Hemoglobin abnormalities
EXTRACELLULAR DEFECTS
Autoimmune H.A
Fragmentation hemolysis
Hypersplenism
Pllasma Factors

CELLULAR DEFECTS
Membrane defects
 Hereditary
spherocytosis
 Hereditary
elliptocytosis
 Hereditary
pyropoikilocytosis
 Hereditary
stomatocytosis
(possibly Rh null)
 PNH (sensitivity to
complement lysis --
sugar water test,
Ham’s test)
 Acanthocytosis
Enzyme defects
 G-6-P-D defficiency
 Pyruvate kinase
deficiency
 Other glycolytic enzyme
deficiencies
 Phosphofructokinase d.
 Triose phosphate
isomerase(TPI) d.
 Phosphoglycerate
kinase (PKG) d.
Hemoglobin
abnormalities.
 Unstable hemoglobin
disease
 Sickle cell anemia
 Other homozygous
hemoglobinopathies
(CC, DD, EE;
 Thalassemia major
 Hemoglobin H disease
 Doubly heterozygous
disorders (such as
hemoglobin SC disease
and sickle thalassemia)
 Hereditary
methemoglobinemia

EXTRACELLULAR DEFECTS
Immune H.A
Alloimmune
Hemolytic Ds of NB
 ABO I.C
 Rh. I.C
 Other B.group I.C
 Hemolytic Transfusion Reaction
Autoimmune
 Idiopathic(primary)
 Cold antibody
 Warm antibody
 Secondary or
symptomatic (in case of
lymphoma, chronic
lymphocytic leukemia,
Other malignant disease,
Immune-deficiency states,
Systemic lupus
erythematosus and other
autoimmune disorders,
Virus and mycoplasma
infections)
 Paroxysmal Cold
hemoglobinuria
Fragmentation
hemolysis
 DIC, TTP. HUS
 Extracarporeal
membrane oxigenation
 Prosthetic heart valves
 Burns-thermal injury
 Venom - Snake, Spider,
Bee
 Hypersplenism
Plasma factors
 Liver disesaes
 Abetalipoproteinemia
 Infections(Malaria Babesia
Clostridium Gram negative
endotoxin)
 Wilsons disesae
 Venom - Snake, Spider,
Bee
Nelson text book of paediatrics 19th e.

PATIENT HISTORY
• Acute or chronic
• Medication/Drug precipitants
G6PD
AIHA
• Family history
• Concomitant medical illnesses
• Clinical presentation

FINDINGS IN HEMOLYTIC
ANEMIA
Reticulocyte count Increased
Serum Unconjugated Bilirubin Increased
Serum LDH Increased
Serum Haptoglobin Decreased
Urine Hemoglobin Present
Urine Hemosiderin Present
Urine Urobilinogen Increased
Cr 51 labeled RBC life span Decreased

FEATURES OF HEMOLYSIS
Bilirubin
LDH
Reticulocytes, n-RBC
Haptoglobulins
+ve Urinary hemosiderin, Urobilinogen
Blood Film
Spherocytes No spherocytes Fragmentation
DCT +ve DCT –ve
AI Hemolysis H. Sherocytosis Malaria,
Clostidium
Hereditery enzymopathies Microangiopathic,
Traumatic

TESTS
• CBC with retics……Hb, reticulocytosis…hemolysis.TLC for
infection
• Thrombocytopenia and neutopenia….hypersplenism
• LDH….increased
• Haptoglobins….decreased
• Urinary and fecal urobilinogen….increasesd
• hemoglobinuria
• Serum Blbn levels….hyperbilirubinemia(indirect)
• Bioten & Na2 Cr4…. Labelling…reduced RBC survival
• METHALBUMEN….INCREASED(oxidised heme binds to albumin)
• Pink colour plasma…free Hb in plasma

TESTS….
• IDA…. Secondary to chronichemolysis
• End tidal CO…hemolysis
• Bone marrow….hyperplastic(erythroid hyperplasia)
hypoplastic, aplastic in parvo B19 infection
• Coombs test(direct and indirect)…imunune H.A
• Warm n cold antibody
• G6PD assay….G6PD deficiency
• PK assay…..Pk deficiency
• Osmotic fragility test…H.S
• Abnormal cytoskeltal proteins analysis…H.elliptiocytosis
• Thermal sensitivity….fragmentation at 45 c for15 min…pyropiokilicytosis
• Hb electropherisis…..hemoglobinopathies

TESTS…
• Decreased WBC CD55 and Cd 59, decreased RBC CD59 by
flow cytometry,…..PNH
• Ham test, Sucrose lysis test….PNH
• PT,ApTT, d-dimers,FDPs….DIC
• Altered plasma cholesterol and phospholipids…liver Ds
• Absence of apolipoprotein β….abetalipoproteinmia
• Blood cultures and serlogy…for infectiuos etiology
• S.copper, seruloplasmin, penicillamin challenge test, urinary
cupper……wilsons ds
• ANA…..SLE

PERIPHERAL SMEAR

SPHEROCYTES
- hereditary
spherocytosis
- acquired hemolytic
anemia (e.g. AIHA)
- physical or chemical
injury
- heat

ELLIPTOCYTES
- heredirary elliptocytosis
- iron def. anemia
- myelofibrosis with
myeloid
metaplasia
- - normal (<10% of cells)

STOMATOCYTES
SLIT LIKE CENTRAL PALLOR IN RBC
1. Liver Disease
2. Acute Alcoholism
3. H Stomatocyosis
4. Malignancies
(normal red cell, a stomatocyte has a
centrally located linear slit or stoma (fish
mouth) and the MCV is usually increased.
On scanning EM, a stomatocyte looks like a
ball with a single concavity (cup-shaped))

ACANTHOCYTES
(IRREGULAR SURFACE SPICULES)
Irregularly spiculated
cells with
bulbous/rounded
ends of spicules
- abetalipoproteinemia
- liver disease

ECHINOCYTES
(CRENATED CELLS, BURR CELLS,PRICKLE
CELLS)
Regularly contracted
cells with smooth
surface undulation
- PK-D
- uremia

BITE CELLS
Removal (“bites”) of
membrane by
splenic
macrophages
- G6PD deficiency

HEINZ BODIES
denatured
hemoglobin
- G6PD deficiency

DREPANOCYTES
(SICKLE CELLS)
- sickle cell anemia

DACROCYTES
(TEARDROP CELLS)
- thalassemia
- myelofibrosis

LEPTOCYTES
(TARGET CELLS)
- liver disease
(obstructive jaundice)
- post splenectomy
- hemoglobinopathies
(hypochromic anemias)
thalassemia
Hgb C disease
Hgb H disease
relative increase of cell
membrane --> “target” formation

ROULEAUX
lined up RBCs in a
row
- multiple myeloma
Red cells stuck together by abnormal
protein (rouleaux)

HOWELL-JOLLY BODY
remnant of nuclear
chromatin
single:
•megaloblastic anemia
•hemolytic anemia
•post splenectomy
multiple:
•megaloblastic anemia
•other abnormal
erythropoiesis

ACANTHOCYTES
5-8 SPIKES OF VARYING LENGTH, IRREGULAR
INTERVALS
Called Spur Cells

SCHISTOCYTES
(CELL FRAGMENTS)
indication of hemolysis
- megaloblastic
anemia
- severe burns
- traumatic hemolysis
- microangiopathic
hemolytic anemia
(helmet cells, triangular
cells)
“helmet cell”

RADIOLOGY
• air on end appearnce
• Thalassemia
• Sickle cell

TEATMENT
• Blood transfusion
• Steriods
• Imunosupression
• Exchange transfusion
• Imunosupression
• IVIG
• Spleenectomy
• BMT

Hemolyticanemia afnan

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