ABSTRACT
We report an association between histologically confirmed progressive multifocal leukoencephalopathy (PML) and an extremely rare humoral immunodeficiency disease, Franklin disease. In our patient, clinical presentation has been typical and prompted us, together with radiological findings, to perform a brain biopsy to confirm the diagnosis even if there was no evidence of any other risk factor except hypogammaglobulinemia. We suggest that PML should be suspected in patients in whom immunosuppression is not obvious (i.e. not only in the setting of HIV infection or disseminated end-stage lymphomas) and involves defects in humoral immunity.
Subject(s)
Agammaglobulinemia/complications , Heavy Chain Disease/complications , JC Virus/isolation & purification , Leukoencephalopathy, Progressive Multifocal/etiology , Adenocarcinoma/complications , Agammaglobulinemia/immunology , Aged , Antiviral Agents/therapeutic use , Brain/pathology , Brain/virology , Brain Neoplasms/diagnosis , Brain Neoplasms/secondary , Cidofovir , Cytosine/analogs & derivatives , Cytosine/therapeutic use , Diagnosis, Differential , Fatal Outcome , Heavy Chain Disease/immunology , Humans , Immunocompromised Host , Immunoglobulins, Intravenous/therapeutic use , JC Virus/growth & development , Leukoencephalopathy, Progressive Multifocal/diagnosis , Leukoencephalopathy, Progressive Multifocal/drug therapy , Leukoencephalopathy, Progressive Multifocal/immunology , Leukoencephalopathy, Progressive Multifocal/pathology , Male , Organophosphonates/therapeutic use , Paraneoplastic Syndromes/diagnosis , Rectal Neoplasms/complications , Risk Factors , Virus ActivationABSTRACT
We herein present a case of primary malignant lymphoma of the saphenous vein. A 72-year-old man suffered from tumor and pain of the anteromedial aspect of the left upper thigh. Local thigh ultrasound scanning and computed tomography revealed a mass within the superior third of the internal saphenous vein. The patient underwent surgical exploration and removal of the saphenous vein between the groin and the upper third of the leg. The resected vessel was surrounded and infiltrated by a whitish, rubbery tissue all along its course. The histologic findings were consistent with high-grade, diffuse, large-cell lymphoma of peripheral B lymphocyte origin, primarily arising in the saphenous vein. Antiactin monoclonal antibodies depicted the venous vascular wall infiltrated by tumor cells, confirming the lymphomatous localization within the saphenous vein. The patient is now alive and free of tumor 10 months after the operation.
