(472) Hemophagocytic Lymphohistiocytosis: Insidious Fever and Shock in the Critical Care Setting

Introduction: We present a patient with Cutaneous T- Cell Lymphoma (CTCL) and Disseminated Candida (DC) presenting with circulatory shock unresponsive to anti fungal treatment eventually diagnosed with Hemophagocytic Lymphohistiocytosis (HLH).

Description: 69 years old male with history of CTCL, disseminated Mycobacterium Avium Complex (MAC) infection, erythroderma mycosis fungoides (on Bexarotene) and monthly extracorporeal photopheresis, recently discharged after being treated for septic shock, presented with fever and altered mental status for 1 day. Broad spectrum antibiotics were initiated. Treatment for disseminated MAC was withheld due to worsening renal function. Vasopressor requirements escalated due to worsening hemodynamic instability. CT abdomen showed hepatosplenomegaly (HSM) with multiple lesions in the liver and spleen, biopsy of which was deferred due to circulatory shock. During day 2 of ICU stay, the patient developed progressive hypoxia and respiratory distress, eventually requiring intubation. X ray showed worsening opacity in the right lung. Bronchoscopy showed White yellow, irregular lesions at the secondary carina into the Left Upper/Left Lower lobe with some vesicular appearing lesions. Herpes Simplex Virus-1 PCR was positive. Acyclovir was initiated. Micafungin was later added due to positive serum Candida antibodies. Bacterial cultures continued to show no growth. Due to fever, HSM, history of malignancy and unresponsiveness to antibiotics and antifungals, testing for HLH was initiated. Triglycerides, Fibrinogen and Ferritin were 247 mg/dl (increased to 491 mg/dl), 98 mg/dl and 58488 ng/dl, respectively. Soluble CD25 level was 81400 pg/ml (resulted posthumously). Patient and family eventually opted for comfort measures, and the patient eventually passed away from worsening hemodynamic instability.

Discussion: HLH can rarely complicate rare, cytotoxic types of CTCL. Frequent coexistence of pathologies with similar presentations makes HLH notoriously deceptive. DC poorly responsive to anti fungals was a clue towards an alternate pathology in our case. Incorporation of Ferritin, Soluble CD25 and NK-cell activity in the revised 2004 Histiocytic Society Criteria for HLH does improve diagnostic accuracy, although the diagnostic process needs further refinement.