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Received Sep 29, 2017; Revised Dec 14, 2017; Accepted Jan 1, 2018
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1. Introduction
Castleman’s disease is a lymphoproliferative disorder associated in some cases with the human immunodeficiency virus (HIV) and human herpes virus 8 (HHV8). It can be unicentric or multicentric. HHV8 related clinical manifestations range from Kaposi Sarcoma (KS), intracavity primary effusion lymphoma (PEL) and systemic multicentric Castleman disease (MCD). HHV8 is more prevalent in sub-Saharan African, Latin American, Caribbean, Mediterranean, and Middle Eastern countries. This is commonly a donor derived disorder and may in fact involve the transplanted organ [1].
2. Case
A 59-year-old Caucasian male with a history of hepatitis C cirrhosis and history of hepatitis B exposure presented 8 months after orthotopic liver transplant (LT) with fever, fatigue, myalgia, night sweats, nonproductive cough, and shortness of breath. He received tacrolimus (TCR) 3 mg twice daily and prednisone 7.5 mg daily for immunosuppression. He was afebrile at the time of admission and vitals were stable. His physical exam was normal. White blood cell count was 2.9, hemoglobin 7.1, hematocrit 23, platelets 94,000, AST 44, ALT 64, total bilirubin 1.5, albumin 2.7, protein 6.4, and INR 1.5. The basic metabolic panel was normal. Infectious workup including blood culture, urine culture, CMV, HIV, hepatitis A/B/C serologies, and urine histoplasma antigen was negative. CT scan of the chest showed borderline enlarged axillary lymph nodes. CT scan of the abdomen/pelvis showed moderate retroperitoneal adenopathy and ill-defined soft tissue density within the region of the porta hepatis. Bone marrow biopsy was performed for pancytopenia and showed hypercellular bone marrow (80%) with erythroid hyperplasia and dyserythropoiesis. Flow...