CYTOMEGALOVIRUS (CMV) INFECTION CAUSING HEMOPHAGOCYTIC LEUKOHISTIOCYTOSIS (HLH) IN A PATIENT WITH MYASTHENIA GRAVIS

Abstract

TYPE: Abstract Publication TOPIC: Critical Care PURPOSE: We describe a rare case of CMV-induced HLH in an immunocompromised adult with myasthenia gravis being managed with azathioprine, illustrating the challenge of making the diagnosis of HLH and initiating early treatment. METHODS: A 63-year-old female with medical history significant for myasthenia gravis managed with azathioprine (AZA) presented with fevers, shortness of breath, malaise and night sweats for one week. She was tachycardic, and initial labs were significant for pancytopenia. CT pulmonary embolism and CT abdomen/pelvis studies showed splenomegaly. Further labs suggested CMV infection; she also had hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia, suggestive of HLH, prompting bone marrow biopsy, which was consistent with CMV with early HLH; NK cell activity was undetectable, with high sCD25. She was started on ganciclovir and dexamethasone; once her liver failure and CMV viremia improved, she received etoposide. Fibrinogen, ferritin levels and CMV titers improved but her cytopenias worsened - further etoposide was held. Unfortunately, the patient’s clinical status deteriorated and she passed. RESULTS: HLH is a rare, underdiagnosed disease characterized by hyperinflammation and cytokine dysregulation, leading to severe infections and death. Five of eight HLH-2004 diagnostic criteria are required for acquired HLH, including fever; splenomegaly; cytopenias; hyperferritinemia; hypertriglyceridemia, hypofibrinogenemia; histological evidence of hemophagocytosis; low/absent NK-cell activity, and high levels of sCD25. Treatment involves eight weeks of corticosteroids and etoposide. CONCLUSIONS: CMV-induced HLH is a rare disease and few cases have been reported. Clinicians should consider HLH as a differential diagnosis as early treatment may reduce mortality. CLINICAL IMPLICATIONS: HLH is an underdiagnosed disease associated with high mortality, making a timely diagnosis imperative. DISCLOSURE: No significant relationships. KEYWORDS: hemophagocytic leukohistiocytosis, cytomegalovirus