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Erdheim-Chester disease presenting with multisystem involvement: A case report.
Majeed Haq1, Syed Muhammad Rooh Ul Ain Naqi Bukhari2, Abdul Basit3
1Department of Medicine, Shaheed Suhrawardy Medical College and Hospital, Dhaka 1207, Bangladesh. b0807621@gmail.com.
Erdheim-Chester disease (ECD), a rare histiocytosis, was diagnosed in a Pakistani male via characteristic imaging, histology, and BRAF V600E mutation. Treatment with corticosteroids and interferon-alpha led to significant symptom improvement.
Area of Science:
- Histiocytosis
- Rare Diseases
- Oncology
Background:
- Erdheim-Chester disease (ECD) is an ultra-rare non-Langerhans cell histiocytosis.
- Characterized by clonal proliferation of lipid-laden histiocytes.
- Underreported in South Asia, with fewer than 1,000 global cases.
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