Case Presentation:
A 40 year old woman with a recent allergic reaction from olive oil was seen and treated in the dermatology clinic, presented with a sudden onset of right‐sided chest pain, shortness of breath, and a recurrent fever of more than 101.3 degrees Fahrenheit for one day. She received broad spectrum intravenous antibiotics initially in the hospital. Since the symptoms were not improving, she was evaluated by multiple subspecialty physicians including infectious disease, hematology, rheumatology, pulmonary disease, dermatology, nephrology, and gastroenterology. She underwent an extensive work up including various imaging studies, multiple blood tests, bone marrow biopsy, thoracentesis, bronchoscopy, special tests for fungal, tuberculosis and bacterial infections. All results were negative. However, she did have a recurrent fever >101.3 degrees Fahrenheit (38.5 degrees Celsius), hepatomegaly, elevated ferritin levels of 13125 ng/mL, elevated lactate dehydrogenase (LDH) levels of 843 IU/L, and elevated triglyceride (TG) levels of 303 mg/dL. Therefore, hemophagocytic lymphohistiocytosis (HLH) was suspected. CMV and Epstein‐ Barr virus (EBV) titers were ordered which revealed positive for CMV. She was transferred to the intensive care unit where she received intravenous steroids for 6 days and tapered to oral tablets plus ganciclovir. She responded to the treatment with a marked decreased in ferritin level from 35673 to 1181 ng/mL, LDH from 843 to 386 IU/L, and TG from 303 to 162 mg/dL in 2‐3 weeks.
Discussion:
HLH is a severe inflammatory disorder characterized by the activation and proliferation of lymphocytes and histiocytes with cytokine release and uncontrolled hemophagocytosis, leading to severe sepsis and multi‐organ failure. Considering the fatal course of the disease, even though only 4 criteria were met in this patient, CD4 lymphocytopenia with CMV viremia and HLH was suspected. She received treatment with steroids plus antiviral therapy and recovered well. She was followed up in the clinic and referred to a specialized center for further work up and management.
Conclusions:
HLH should be considered when a patient has recurrent fever, high ferritin levels, high TG levels, and hepatospleenomegaly. Results from the HLH‐94 study indicated that in the proper clinical settings, ferritin levels >500 ng/mL was only specific for the diagnosis of HLH. A bone marrow biopsy can be negative in 20% of cases. Internists, hospitalists, medical residents, and subspecialists need to understand the pathophysiology of this disease to facilitate early recognition and treatment.