Blood Podcast Review Series on Rare Systemic Hematologic Disorders
Apr 16, 2020
Julian Haroche, an internist at Hôpital Pitié-Salpêtrière in Paris, dives deep into the realm of rare systemic hematologic disorders that perplex clinicians. He shares insights on Erdheim-Chester disease, detailing its challenging diagnosis and the significance of BRAF mutations in treatment breakthroughs. The discussion reveals the hurdles faced in accessing novel therapies across Europe, emphasizing innovative approaches and the importance of collaboration in improving patient outcomes. Tune in for an enlightening exploration of these complex conditions!
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Challenges in Rare Hematologic Disorders
- Rare systemic hematologic disorders are often anxiety-provoking for clinicians unfamiliar with them. - Knowing how to recognize and treat these diseases is complex even for experienced professionals.
Unique Features of Lymphomatoid Granulomatosis
- Lymphomatoid granulomatosis (LYG) is an EBV-driven disorder primarily affecting extranodal sites like lungs and CNS. - It features minority EBV-infected B cells with abundant angioinvasive T cells causing tissue damage.
LYG and Immune Deficiencies
- Over half of LYG cases occur without any known immunodeficiency. - Defective immune surveillance of EBV-infected B cells, especially CD8+ T cells, underpins LYG pathobiology.