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Hemoglobin E disorders in the north east India
15 Air Force Hospital.
The Journal of the Association of Physicians of India
|April 20, 2001
Summary
This study on Hemoglobin E (HbE) variant disease found that while most patients experienced symptoms like jaundice and fatigue, splenomegaly and microcytosis were consistently present. Clinical features were compared to existing data, noting increased splenomegaly frequency.
Area of Science:
- Hematology
- Genetics
- Clinical Medicine
Background:
- Hemoglobin E (HbE) variant is a common inherited blood disorder.
- HbE can manifest as homozygous HbE disease or in combination with beta-thalassemia.
- Understanding the clinical and hematological spectrum is crucial for patient management.
Purpose of the Study:
- To prospectively investigate the clinical and hematological features of patients with Hemoglobin E variant.
- To compare observed features with previously reported series.
Main Methods:
- Prospective study over three years.
- Inclusion of nine patients with Hemoglobin E variant (3 HbE-beta thalassemia, 6 homozygous HbE).
- Clinical assessment and hematological parameter evaluation.
Main Results:
- Splenomegaly and microcytosis were present in all nine patients.
- Mild anemia (mean hemoglobin 11.3 gm/dl) was observed in the majority.
- Jaundice was the most frequent symptom; other symptoms included fatigue and abdominal pain.
Conclusions:
- Splenomegaly and microcytosis are consistent findings in HbE variant disease.
- Clinical presentation can range from asymptomatic to symptomatic with jaundice and anemia.
- Observed splenomegaly frequency was higher than in some reported series.