Hemoglobin Lepore-beta-thalassemia…

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Hemoglobin Lepore-beta-thalassemia syndrome

ORPHA:330032Disease

Also called HbLepore-beta-thalassemia syndrome · Lepore-beta-thalassemia syndrome

What it is

A rare beta-thalassemia associated with another hemoglobin anomaly characterized by the presence of the hemoglobin Lepore variant in association with beta-thalassemia. Clinical presentation is highly variable, depending on the type of beta-thalassemia, and ranges from severe hypochromic microcytic anemia and complete transfusion dependency to moderate, compensated anemia without a need for regular blood transfusions.

Key facts

Age of onset
No data available
Inheritance
Autosomal recessive
Classified as
Disease

Genes reported in subtypes

HBBHBD

Orphanet records these genes on 2 more specific entries under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

ICD-10 codes

D56.8filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0018022UMLS C5679855

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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