Patient: Male, 55-year-old Final Diagnosis: CMV infection • Good syndrome • thymoma Symptoms: Recurrent infection preceded by thymoma Medication:— Clinical Procedure: — Specialty: Hematology • Immunology • Oncology • Ophthalmology • Surgery Objective: Rare disease Background: Good syndrome (thymoma with immunodeficiency) is a frequently missed and forgotten entity. It is a rare cause of combined B and T cell immunodeficiency in adults. To date, fewer than 200 patients with Good syndrome have been reported in the literature. Case Report: We report a case of type AB Masaoka-Koga stage I thymoma which predated the evidence of immune dysregulation by 5 years, manifesting as bilateral cytomegalovirus retinitis, multiple bouts of pneumonia, and bronchi-ectasis in a HIV-seronegative 55-year-old man. Intravitreal ganciclovir was administered in addition to intravenous systemic ganciclovir, which resulted in severe neutropenic sepsis. A thorough immunodeficiency workup confirmed the presence of hypogammaglobulinemia with complete absence of B cells and reduced CD4/CD8 ratio. The patient responded well to monthly intravenous immunoglobulin replacement therapy, with no further episodes of infection since then. The immunoglobulin level doubled after 1 year of treatment. However, as the patient refused further intravitreal and CMV-targeted treatment, his vision did not recover. Conclusions: Clinicians should be aware that thymoma can precede the onset of immunodeficiency. Clinical suspicion should be heightened in at-risk patients who present with multiple bouts of infection, particularly in thymoma cases with adult-onset immune dysfunction. It is of paramount importance to follow up those patients with annual clinical reviews and immunodeficiency screening.
Pulmonary nodular lymphoid hyperplasia (PNLH) is a rare, benign lymphoproliferative disorder often manifesting as a solitary pulmonary nodule (SPN). Once a malignant cause of SPN is ruled out, it presents a diagnostic dilemma to clinicians due to histopathological overlap with diseases like mucosa-associated lymphoid tissue (MALT) lymphoma and IgG4-related sclerosing disease. We report an adult patient who presented with incidental finding of a SPN, and was subsequently diagnosed with PNLH via a combined technique of radial-endobronchial ultrasound (R-EBUS), virtual bronchoscopic navigation (VBN), and cryobiopsy. We discuss the sonographic appearance and histopathological morphology of PNLH, and the merits of bronchoscopic techniques in averting surgical intervention of SPN. This is the first reported sonographic appearance of PNLH under radial-endobronchial ultrasound (R-EBUS).
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