ONCAlert | 2017 San Antonio Breast Cancer Symposium
Multicentric Castleman Disease Case Studies

Case Studies: MCD with David Fajgenbaum, MD, MBA, Msc

Garrett is a 47-year old male carpenter who was formerly a Marine, with a 4-week history of fatigue, night sweats, and weight loss. He reported difficulty breathing for 2 weeks, and 1 week of fevers. He also reported that he’s noted fluid in his legs. He presented to the emergency department for a work-up. His past medical history was only notable for Raynaud’s phenomenon. His family history included a mother that died from a myocardial infarction at 71 and a father who died from lung cancer at 61.

MCD with David Fajgenbaum, MD, MBA, Msc: Case 2

Multicentric Castleman Disease: Case 2

Garrett is a 47-year old male carpenter who was formerly a Marine, with a 4-week history of fatigue, night sweats, and weight loss. He reported difficulty breathing for 2 weeks, and 1 week of fevers. He also reported that he’s noted fluid in his legs. He presented to the emergency department for a work-up. His past medical history was only notable for Raynaud’s phenomenon. His family history included a mother that died from a myocardial infarction at 71 and a father who died from lung cancer at 61. His physical exam was notable for bilateral cervical and inguinal lymphadenopathy (1-2 cm), moderate edema (10 lb weight gain in past 7 days), and pleural effusions.

Laboratory findings showed anemia (Hgb: 11 gm/dl), low platelets (109k), and elevated alkaline phosphatase levels. The patient was admitted with a presumed viral illness and then moved to MICU when the patient began experiencing severe difficulty breathing, transaminitis, and increased fluid gain (30 lbs). Further testing showed his CRP >300 mg/L, albumin 1.2 g/dL, renal dysfunction, and Hgb trending downward (now 9 gm/dl), and PLTs trending downward (now 35k). His infectious workup was pan-negative, except for possible EBV infection (8/29/10: EBV PCR positive). He was diagnosed with acute EBV mononucleosis early in admission, but the diagnosis was rescinded when he was found to be EBV IgG+. A rheumatology workup was negative except for a positive ANA (1:120).

The patient was started on 125mg BID of solumedrol without improvement. His hematology/oncology workup was notable for: .

  • Elevated B-2-microglobulin
  • CT scan: diffuse LAD, splenomegaly
  • PET: patchy FDG uptake in SI only (while on high dose steroids)
  • Normal Igs (IgG: 930, range: 650-2000; IgM 63, range: 40-270; IgA: 202, range: 50-500)
  • Normal/moderately elevated IL-6 (6, nml <5)
  • No light chain restriction
  • Negative SPEP and UPEP

A bone marrow biopsy reported: 

  • Hypercellular marrow (90%) with myeloid and megakaryocytic hyperplasia, and emperipoesis.
  • Small perivascular lymphohistiocytic aggregate
  • Reticulin fibrosis was also noted with “cytological atypia of the megakaryocyte lineage (FVIII+, CD61+)…”
  • “increased CD68+ macrophages (10%)…”
  • “myeloid: erythroid ratio of 6:1”
  • “Large CD34- cKit+ blasts.”
  • “Rare macrophages and megakaryocytes that contain red and white blood cells.”

A lymph node biopsy was scheduled to be performed.

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