This document discusses different hemoglobinopathies including HbCC, HbEE, and their combinations. HbCC is caused by a mutation in the beta chains and results in insoluble hemoglobin and hemolytic anemia of varying severity due to crystal formation inside or outside red blood cells. HbEE also results from a beta chain mutation and causes unstable hemoglobin and microcytic anemia similar to beta-thalassemia. Electrophoresis can be used to diagnose HbCC and HbEE homozygous and heterozygous states. Common sickle cell syndromes include sickle cell disease (HbSS), sickle cell trait (HbAS), sickle cell hemoglobin C disease (HbSC), and sick

1. HEMOGLOBIN HbCC-HbEE
Developed by-Dr.Abdulrazzaq Othman Alagbare
M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
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2. HEMOGLOBIN HbCC
Definition and
causes
HbC  caused by a mutation in β chains (β6 Glu→ Lys) which
is associated Insoluble hemoglobin like HbSS.
Hemolytic anemia varies from moderate to severe, similar With
crystals forming in or out of RBC and folded cells
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3. HEMOGLOBIN HbCC
Diagnostic test Electrophoresis: HbCC
HbA Nil
HbA2: Normal
HbF: 7%
HbC: >90 %
Hemoglobin trait HbAC
HbA: 55-60 %
HbA2: Normal
HbF: Normal
HbC: 35-45 %
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4. HEMOGLOBIN E–β-THALASSEMIA
Definition
and causes
HbE  caused by a mutation in β chains (β26 Glu→ Lys)
which is associated with the phenotype of β thalassaemia.
Hemolytic anemia varies from moderate to severe, similar to β-
thalassemias  Very small RBC with MCV lower than 60 fl
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5. HEMOGLOBIN E–β-THALASSEMIA
Diagnostic
test
Electrophoresis: HbEE (Homo.)
HbA Nil
HbA2: Normal
HbF: 7%
HbE: >90 %
Hemoglobin trait (Hetro.) HbEA
HbA: 55-60 %
HbA2: Normal
HbF: Normal
HbE: 35-45 %
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6. β- chain - Hemoglopinopathies
Hb Defect Hb type RBC changes
HbSS Valine Replace Glu at
6th position of β- chain
Produce insoluble Hb Sickling shape
HbCC Lysin Replace Glu at 6th
position of β- chain
Produce insoluble Hb Crystals inside the RBC
And folded cells
HbEE Lysin Replace Glu at
26th position of β-
chain
Produce unstable Hb Microcyte with MCV 55
fl
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7. 7
β/βcβ/βs
β/β0
βs/βc
Sickle-cell / HbC disease
β/βs
β0/βs
 Sickle-cell / β-thalassaemia
Hemoglopinopathies combinations
Hemoglopinopathies combinations OCCUR among the heterozygous individuals

8. Hemoglopinopathies combinations
SC disease is a variant of homozygous sickle
cell anemia (SS disease) which is of almost
equal severity and has a similar clinical
presentation
Clinical phenotype of
HbE/β thalassaemia resembles
either thalassaemia major
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9. SUMMARY ……Sickle-cell syndromes
Mostly the following clinical syndrome types
are observed
Type
Sickle-cell Disease – HbSS Homozygous βs/βs
Sickle-cell trait - HbAS Heterozygous β/βs
Sickle-cell haemoglobin / C disease βs/βc
 Sickle-cell / β-thalassaemia βs/β0 9

10. ‫رب‬ ‫هلل‬ ‫الحمد‬
‫العالمين‬
‫وجه‬ ‫ابتغاء‬ ‫الجهد‬ ‫هذا‬
‫الكريم‬ ‫هللا‬