Hemophagocytic Lymphohistiocytosis in a Lung Transplant Recipient

• DOI: 10.1016/j.healun.2021.01.2060
• Publication Date: 2021-03-20T23:10:03Z
• Authors: D. Rudym; M. Lesko; S. Chang; Z. Kon; K. Sureau; V. LaMaina; T. Lewis; L. Angel
• Keywords: Hemophagocytic Lymphohistiocytosis

Introduction Hemophagocytic Lymphohistiocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome. Few case reports exist describing this entity in lung transplant recipients. Case Report A 56-year-old woman with scleroderma and pulmonary hypertension presented two months after double lung transplantation with acute onset abdominal pain, nausea, and vomiting. Her physical examination was notable for mild tenderness to palpation over right upper quadrant. Admission laboratory values revealed aspartate transaminase of 337 U/L, alanine aminotransferase of 430 U/L, alkaline phosphatase of 1083 U/L, total bilirubin of 3.9 mg/dL, and direct bilirubin of 2.9 mg/dL. Abdominal computed tomography (CT) was unremarkable aside from prior cholecystectomy. Subsequent liver biopsy was consistent with drug-induced liver injury, which led to discontinuation of possible culprits. There was no evidence of a hemophagocytic process. As her transaminases recovered to near-normal, she abruptly became leukopenic to 0.1 × 103μ/L and thrombocytopenic to 17 × 109μ/L. She developed new parotid lymphadenopathy and was febrile. Further work-up showed hyperferritinemia of 21899 ng/mL, hypertriglyceridemia of 294 mg/dL, and elevated soluble CD25 of 17780 IU/mL. Repeat abdominal CT showed splenic enhancement. A bone marrow biopsy revealed numerous histiocytes with hemophagocytosis (see image). She was treated with high dose steroids, cyclophosphamide, and a dose of rituximab for Epstein-Barr virus PCR of 390 copies/mL. As all her markers worsened, she began therapy with anakinra. Concurrently, she developed polymicrobial bacteremia, fungemia, and renal failure. Despite aggressive efforts, she deteriorated and passed away. Summary HLH carries a high mortality rate and is commonly underrecognized due to the non-specific nature of its clinical and laboratory findings. Particularly in transplant recipients, where differentials are broader at baseline, early consideration, rapid diagnosis, and prompt treatment are critical.