Disseminated Cryptococcal Disease in a Patient With Monoclonal Gammopathy of Undetermined Significance and Polycythemia Vera: A Case Report and Review of the Literature

Cryptococcosis is a life-threatening opportunistic infection caused by Cryptococcus gattii and Cryptococcus neoformans. It affects both immunocompetent and immunosuppressed hosts. Disseminated cryptococcal infection is rare in immunocompetent patients, but the cryptococcal disease's neurological sequelae may be more prominent in this group. We present a case of a 58-year-old male patient with medical comorbidities of monoclonal gammopathy of undetermined significance (MGUS) and polycythemia vera. The patient presented with gradual worsening of mental status over one week. He was found to have Cryptococcus neoformans meningoencephalitis and fungemia. The patient received two weeks of liposomal amphotericin B (LAmB) and flucytosine with excellent clinical response. He was discharged on high dose fluconazole, and he returned to the hospital in one week with new-onset hemiplegia and cryptococcomas on imaging. Prolonged intravenous (IV) treatment of six weeks duration resulted in significant clinical improvement and disease-free state at two years follow-up. This article aims to stress the importance of individualized prolonged IV treatment with liposomal amphotericin B and flucytosine despite good initial response in patients with polycythemia vera and MGUS. This is the first reported case of cryptococcal disease, to the best of our knowledge, in a patient with MGUS and the third case of cryptococcal infection in patients with polycythemia vera in a non-HIV non-transplant state. Prolonged individualized IV treatment should be considered in immunocompetent patients with the above conditions, as this condition, if not adequately treated and relapses, lead to high morbidity and mortality.