Ahad Azeem, MD
Creighton University Medical Center-Bergan Mercy
Omaha, NE
Disclosure information not submitted.
Nida Majeed, MBBS
Physician
Boys Town National Research Hospital
Boys Town, Nebraska, United States
Disclosure information not submitted.
Title: A Combination of Histoplasmosis and HLH can make your Transplanted Kidney Worthless.
Case Report Body:
Introduction: Recognizing hemophagocytic lymphohistiocytosis (HLH) is a challenge based on its atypical presentation. Its diagnosis is based on five of the following clinical criteria; fever, splenomegaly, cytopenia, elevated triglycerides ( >3 mmol/L), serum ferritin ( >500 ug/L), low fibrinogen levels, low activity of NK cells, and increased soluble CD25 concentration ( >2400 U/mL). Cases of HLH secondary to disseminated histoplasmosis are associated with high mortality.
Description: A 59-year-old male with a history of renal transplant 14 years ago was admitted to the hospital for fever, myalgia, and diarrhea of six weeks duration. On presentation, he was febrile up to 39.5 °C. Initial laboratory tests revealed serum creatinine of 4.25 mg/dL (normal range: 0.64 - 1.27) and dense granular casts in the urine. A computerized tomography scan of the chest and abdomen was unremarkable for any acute focus of infection. Based on his immunocompromised status and persistently elevated inflammatory markers, viral and fungal studies were checked which resulted in positive for urine histoplasma antigen of 7.8 ng/mL (reference range: < 0.3) and serum histoplasma antigen of 25 ng/mL (reference range: < 0.3). He continued to have worsening encephalopathy, diffuse diarrhea, renal failure requiring invasive mechanical ventilation, and continuous veno-venous hemodialysis. Treatment with amphotericin (3 mg/kg) was initiated for suspicion of disseminated histoplasmosis disease. Due to persistent pancytopenia, high interleukin-2 receptors, and elevated serum ferritin level of 8185 ng/mL (reference range: 23 – 340), dexamethasone (40 mg/day) was started, and bone marrow biopsy was performed which confirmed scattered foamy histocytes, hemophagocytic histiocytes along with budding yeast. Given overall clinical improvement and risks of nephrotoxicity with etoposide, it was not administered. Amphotericin was continued for two weeks until the therapeutic serum levels of itraconazole. He was discharged after 22 days of inpatient stay on scheduled hemodialysis three times/week.
Discussion: Treatment of acquired HLH depends on underlying etiology such as infection, malignancy, or autoimmune disease. Survival increases up to 54% at 6.2 years if prompt treatment is initiated. Early management can also limit the use of cytotoxic chemotherapy.