Abstract
BackgroundCryptococcal meningoencephalitis is an opportunistic infection that predominantly affects immunocompromised patients. Hyper immunoglobulin M syndrome is a primary immunodeficiency syndrome that increases susceptibility to several opportunistic infections. Here, we report a case of cryptococcal meningoencephalitis in the context of hyper immunoglobulin M syndrome, a situation that has been reported very few times and whose management is not clearly defined. We describe our management of this case and the outcome of the patient to help in future similar situations.Case presentationThe patient is a 19-year-old Caucasian male student diagnosed with X-linked hyper immunoglobulin M syndrome and treated chronically with weekly intravenous immunoglobulin and daily sulfamethoxazole-trimethoprim. He was admitted to the infectious diseases ward because of headache, diplopia and a cerebral-spinal fluid analysis revealing cryptococcal meningoencephalitis. The patient was treated with liposomal amphotericin and flucytosine with a favorable outcome. Maintenance therapy with fluconazole has continued and will be sustained for 6 months following his upcoming bone marrow transplantation.ConclusionMonitoring for cryptococcal meningoencephalitis should be considered in patients with primary immunodeficiencies, as clinical manifestations may go unnoticed. In these patients, it is expected that chronic treatment with fluconazole will be the only treatment that will prevent reinfection or reactivation, and therefore should be kept at least until bone marrow transplant, the only curative treatment, is performed. It may, however, lead to intolerable side effects and hepatic toxicity.
Highlights
Cryptococcal meningoencephalitis is an opportunistic infection that predominantly affects immunocompromised patients
Monitoring for cryptococcal meningoencephalitis should be considered in patients with primary immunodeficiencies, as clinical manifestations may go unnoticed
It is expected that chronic treatment with fluconazole will be the only treatment that will prevent reinfection or reactivation, and should be kept at least until bone marrow transplant, the only curative treatment, is performed
Summary
X-linked HIGM syndrome is characterized by a high incidence of opportunistic infections with an unfavorable outcome, despite regular substitution therapy with IVIg. outcomes will likely be improved with additional therapies, like trimethoprim/sulfamethoxazole prophylaxis, and close follow-up. Outcomes will likely be improved with additional therapies, like trimethoprim/sulfamethoxazole prophylaxis, and close follow-up If these methods are successful, an older population of X-linked HIGM patients will develop in time, and new opportunistic infections that we are unaware of could appear. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests The authors declare that they have no competing interests. All authors read and approved the manuscript
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