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 He was diagnosed with acute EBV mononucleosis early in admission, but the diagnosis was rescinded when he was found to be EBV IgG+.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 &ldquo;cytological atypia of the megakaryocyte lineage (FVIII+, CD61+)&hellip;&rdquo;
• &ldquo;increased CD68+ macrophages (10%)&hellip;&rdquo;
• &ldquo;myeloid: erythroid ratio of 6:1&rdquo;
• &ldquo;Large CD34- cKit+ blasts.&rdquo;
• &ldquo;Rare macrophages and megakaryocytes that contain red and white blood cells.&rdquo;

A lymph node biopsy was scheduled to be performed.