Cytomegalovirus disease in patients with impaired cell mediated immunity deemed at risk

Cytomegalovirus (CMV) disease in patients with impaired cell-mediated immunity (Orphanet code 137698) refers to symptomatic CMV infection occurring in individuals whose T-cell-mediated immune responses are compromised. This includes patients who have undergone solid organ or hematopoietic stem cell transplantation, individuals receiving immunosuppressive therapies, and those with advanced HIV/AIDS or primary immunodeficiency disorders affecting cellular immunity. Unlike CMV infection in immunocompetent individuals, which is typically asymptomatic or causes mild mononucleosis-like illness, CMV disease in immunocompromised hosts can be severe and life-threatening. The disease can affect multiple organ systems. Common manifestations include CMV pneumonitis (affecting the lungs with cough, dyspnea, and hypoxia), CMV retinitis (causing visual impairment and potentially blindness), CMV colitis and esophagitis (causing gastrointestinal ulceration, diarrhea, abdominal pain, and bleeding), CMV hepatitis, and CMV encephalitis. Disseminated disease involving multiple organs simultaneously can also occur. CMV viremia with fever, malaise, and cytopenias (particularly leukopenia and thrombocytopenia) is frequently observed. The virus can also cause graft rejection or graft-versus-host disease exacerbation in transplant recipients. Treatment relies primarily on antiviral therapy with ganciclovir (intravenous) or its oral prodrug valganciclovir as first-line agents. Foscarnet and cidofovir serve as second-line alternatives, particularly in cases of ganciclovir-resistant CMV. Letermovir is approved for CMV prophylaxis in hematopoietic stem cell transplant recipients. Maribavir has been approved for treatment of refractory or resistant CMV infection in transplant patients. Reduction of immunosuppression, when feasible, is an important adjunctive strategy. CMV-specific immunoglobulin may be used as adjunctive therapy in certain settings. Monitoring of CMV viral load by quantitative PCR is essential for guiding preemptive therapy and assessing treatment response.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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