Episode 2: Dr. Fajgenbaum’s Commentary

• After episode 1, patient was discharged and out of hospital for almost 4 weeks
  • Diuresed 42 pounds of fluid and began to feel well again
• Toward end of 4-weeks, patient started to experience same symptoms he had before first hospitalization:
  • Enlargement of lymph nodes, fatigue and night sweats, and abdominal pain
• Lab tests performed, as well as CT scan, which found extensive lymphadenopathy throughout the body
• Lymph node biopsy performed
  • 4 important findings:
    • Regressed germinal centers
    • Plasmacytosis
    • Hyperplastic follicles
    • Extreme vascularization throughout lymph node
• Multicentric Castleman Disease suspected
• Additional testing warranted to rule out other disorders
• No signs of monoclonality within lymph node; other disorders already ruled out
• Determined to be Multicentric Castleman Disease
• Tests conducted to determine whether HHV-8—negative or HHV-8–positive Multicentric Castleman Disease
  • Based on negative LANA-1 staining and negative PCR for HHV-8, HHV-8 negative Multicentric Castleman Disease was determined
• Patient sent back to hospital and started on rituximab
• Patient did not respond right away
• Within 48 hours, improvements in laboratory values observed
  • BUN, which peaked over 200, began to improve
  • Creatinine, which was over 2, began to improve
  • CRP, which was over 300 at its peak, started to improve within couple days of starting rituximab
• Patient achieves partial remission on rituximab and is healthy enough to leave hospital
• Not yet in complete remission

Read through the episodes of one patient’s journey toward an accurate diagnosis and listen to an expert’s analysis on each episode.