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

Muhammad Khurram Guhjjar,Ajsza Matela,Maneesh Gaddam,Haider Ghazanfar,Shoaib Ashraf

doi:10.7759/cureus.12458

Abstract

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.

Highlights

Cryptococcal disease (CD) refers to an infection by the genus cryptococcus [1]
We report a case of Cryptococcus neoformans meningoencephalitis and fungemia in a patient with monoclonal gammopathy of undetermined significance (MGUS) and polycythemia vera on hydroxyurea
This article aims to highlight the rare cause of cryptococcal disease in immunocompetent patients and consideration of individualized prolonged intravenous (IV) treatment in patients with MGUS and polycythemia vera as there might be an association of nonmalignant myeloproliferative syndromes and cryptococcal infection

Summary

Introduction

Cryptococcal disease (CD) refers to an infection by the genus cryptococcus [1]. There are two common pathogenic species in humans, including Cryptococcus neoformans and Cryptococcus gatti [2]. The cryptococcal disease has been increasingly reported in HIV-negative patients and immunosuppression states such as solid organ transplantation, hematologic malignancy, glucocorticoid use for a prolonged time, and chronic liver disease, and sarcoidosis [4,5]. The patient was found to have positive serum cryptococcal antigen (titer 1:1024) He was later identified to have Cryptococcus neoformans on CSF final culture report. Repeat lumbar punctures (LP) was performed with an opening pressure of 19 cm H2O and CSF sample positive for cryptococcal antigen (titer 1: 1024). His left-sided weakness improved throughout treatment, with 3/5 in the left lower extremity and 4/5 in the left upper extremity He completed six weeks of inpatient IV L-AmpB plus flucytosine with remarkable improvement in his clinical status, and serum cryptococcal titer dropped to 1:16 and to 1:8. At two years of follow-up in the clinic, the patient has been maintained on oral fluconazole therapy, has shown no relapse, and has been actively living without any deficits

Discussion

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Findings

Antinori S