A 58-year-old Caucasian male with a medical history of hypertension, remote history of coccidioidomycosis, and tobacco use presented with acute-onset altered mental status and 70-pound unintentional weight loss over the previous 6 months. The patient was found to be in status epilepticus refractory to anti-epileptic drugs secondary to traumatic brain injury (TBI) from multiple ground-level falls, and magnetic resonance imaging (MRI) of the brain demonstrated bifrontal hemorrhagic contusions (larger on the left), scattered subarachnoid hemorrhages without demonstrable mass effect or midline shift, and few scattered tiny hypointense areas on gradient echo (Figure 1). Subsequent magnetic resonance angiography (MRA) showed no demonstrable intracranial aneurysm or occlusive vascular disease in the anterior or posterior circulations.
The patient went into septic shock, presumably due to aspiration pneumonia and urinary tract infection, requiring vasopressors and intravenous antibiotics. He was intubated and transferred to the intensive care unit for acute respiratory failure, severe anion gap metabolic acidosis with pH 6.9, HCO3 8 mmol/L, and a corrected anion gap of 22 mmol/L, acute renal failure with a creatinine of 14.8 mg/dL, and hyperkalemia of 6.6 mmol/L (Table 1), and started on hemodialysis. Initial chest x-ray revealed left basilar atelectasis, left hemidiaphragm elevation, and a right upper lobe irregular calcification (Figure 2).
The patient was also found to have pancytopenia and required multiple packed red blood cell and platelet transfusions. Given this along with recent weight loss, the team performed cancer workup that included a computed tomography (CT) without contrast of the abdomen and pelvis, which revealed hepatomegaly with a craniocaudal length of 21 cm, splenomegaly with a length of 18.2 cm, bilateral adrenal masses measuring 4 cm each, and enlarged pericaval lymph nodes with a length of 2.3 cm x 1.7 cm x 4 cm (Figures 3 and 4). Adrenal insufficiency was ruled out with morning cortisol of 23.1 microg/dL. A transthoracic echogram revealed no evidence of heart or valvular disease.
Despite empiric antibiotics throughout the hospital course, the patient continued to maintain low-grade fevers. Initial laboratory values noted to have an elevated ferritin 1186 microg/L, and the patient remained transfusion dependent due to pancytopenias with hemoglobin 7.9 g/dL and platelets 66 x 109/L. Splenomegaly was seen on CT scan, raising the specter of possible hemophagocytic lymphohistiocytosis (HLH) in the setting of potential malignancy or possible infection.
All cultures, including fungal cultures, from cerebrospinal fluid (CSF), blood, and urine had no growth. Laboratory results were negative for HIV, hepatitis B and C, syphilis, and QuantiFERON-TB Gold. Given the patient's history of coccidioidomycosis infection living in an endemic area, serology was ordered and showed immunodiffusion IgM weakly reactive and immunodiffusion IgG non-reactive, with complement fixation <1:2. A lumbar puncture was performed, which ruled out meningitis with an opening pressure of 19 cm H2O, white blood cell (WBC) count of 4 cells/microL, red blood cell (RBC) count of 55 cells/microL, glucose level of 55 mg/dL, and protein level of 66 mg/dL.