4. THE FACTORY – BONE
MARROW
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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
5. 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.
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6. 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
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7. 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.
8. •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.
9. 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
10. • 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
11. 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.
15. 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
16. 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.
17. PATIENT HISTORY
• Acute or chronic
• Medication/Drug precipitants
G6PD
AIHA
• Family history
• Concomitant medical illnesses
• Clinical presentation
27. 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))