Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare and often deadly syndrome characterized by severe inflammation and cytokine dysregulation. The disease is defined by the HLH-2004 criteria, requiring five of eight findings, and is further differentiated into either primary or secondary causes. Primary HLH tends to be of genetic etiology, while secondary HLH results from other insults such as infection. Secondary HLH is most commonly associated with viral infections in immunocompromised patients. Acute cytomegalovirus (CMV) associated HLH in the immunocompetent host is exceedingly rare and only documented in four case reports to date. We describe the fifth documented case of CMV-associated HLH in an immunocompetent patient, and furthermore, we demonstrate that this patient is the first published case of its type to satisfy all eight of HLH-2004 criteria.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a devastating and rare disorder with an incidence of only 1.2 cases per million patients per year, with a startlingly high mortality rate of 47% [1]. This disease entity is characterized by defective natural killer cell cytotoxicity, which results in an inappropriately robust activation of macrophages and leads to engulfment of other blood cells
Et al found that 60% of 96 patients who met the 2004 Hemophagocytic Lymphohistiocytosis (HLH) diagnostic criteria had leukopenia, 20% had hepatosplenomegaly, and 20% developed jaundice [2]
HLH is frequently mistaken for a septic syndrome, leading to profound delays in time to diagnosis of this rare condition
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a devastating and rare disorder with an incidence of only 1.2 cases per million patients per year, with a startlingly high mortality rate of 47% [1]. On the fifth day of admission, the patient suffered acute hypoxemic respiratory failure believed to be secondary to pneumonia and developing acute respiratory distress syndrome She was transferred to the medical intensive care unit for more aggressive care and diuresis. The patient met all eight HLH diagnostic criteria She had demonstrated acute CMV infection via robust viremia and positive CMV PCR on BAL. The patient eventually recovered after suffering several complications during therapy, including an episode of Proteus mirabilis septicemia secondary to central venous catheter line infection, multiple intubations, and acute renal failure secondary to acute tubular necrosis, which required renal replacement therapy She was discharged on hospital day 58. Hepatitis B virus - HBV, Anti-neutrophil cytoplasmic antibodies - ANCA, Complete blood count - CBC
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