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Multicentric Castleman Disease Case Studies

Episode 3: Dr. Fajgenbaum’s Commentary

Published Online:Apr 06, 2016
Dr. David Fajgenbaum, Perelman School of Medicine, University of Pennsylvania, says that this is a very complicated case, with a patient who is very, very ill. Within just a few weeks, this patient went from being a healthy young adult to being hospitalized in the intensive care unit.

Differential Diagnosis

  • Patient treated with rituximab
  • Discharged from hospital
  • Received weekly rituximab for 3 weeks
  • After 3-week period, patient began to have recurrence of symptoms
    • Elevated CRP, low albumin, and low platelets
  • Patient hospitalized and given very high doses of methylprednisolone, expected to help improve symptoms, but with no response
  • Patient treated with double-dose rituximab; still no response
  • CRP climbed over 300 and patient had no response to steroids or rituximab
  • Then treated with very intense combination of cytotoxic chemotherapy:
    • Bortezomib, dexamethasone, thalidomide, adriamycin, Cytoxan, etoposide, rituximab, siltuximab.
  • Combination resulted in immediate improvement in symptoms
  • Patient was on dialysis 3 times/week and was able to go from dialysis frequently, then no longer needed dialysis
  • Albumin began to improve
  • Previous pancytopenia began to improve following chemotherapy
  • CRP began to drop very quickly
  • Patient demonstrated positive response to cytotoxic chemotherapy
  • Based on now-approved drug siltuximab, anti-interleukin-6 therapy, patient was put on maintenance anti-IL-6 therapy following intense hospitalization and discharge
  • Patient was on maintenance anti-interleukin-6 therapy for full year

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

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