Thoracic lymphadenopathy due to vascular transformation of lymph node sinuses associated with upper limb edema in a chronic hemodialysis patient with congestive heart failure

BACKGROUND: Vascular transformation of lymph node sinuses (VTLS) is a rare disorder characterized by transformation of lymph node sinuses into endothelium-lined capillary-like channels. This phenomenon was originally discovered by accident whilst examining regional lymph nodes draining cancer. However, it has been found in association with other conditions associated with lympho venous congestion and distension, such as congestive heart failure (CHF) or even lymphoadenopathy alone. CASE REPORT: We describe the clinical case of a male dialysis patient with CHF (secondary to ischemic-hypertensive cardiac failure) who developed gross edema of the upper left limb on the arteriovenous fistula (AVF) side. Edema appeared within a month after carotid endoarteriectomy following approximately twenty years of chronic hemodialysis. Doppler ultrasound with other investigations showed that subclavian and upper cava veins were patent, but revealed many enlarged lymph nodes in the upper left thorax and in the left axilla. Suspicion of lymphoproliferative disease or metastatic involvement was raised and a lymph node biopsy was performed, revealing VTLS. Bone marrow biopsy and abdominal tomographies showed no mass or a proliferative disorder. Based on a hypothesis of an association between upper limb edema and ipsilateral AVF, the AVF was tied. The upper limb edema decreased dramatically within weeks, whilst RRT was continued by means of a central venous catheter. However, a few months later the patient's condition worsened; he developed relapsing pleural effusions and eventually died. Post-mortem examination revealed severe ischemic-calcific cardiopathy and showed that major thoracic and brachial vessels were patent whilst most thoracic and hilar lymph nodes showed VTLS and fibrosis. CONCLUSIONS: We believe that in our patient CHF was the primary cause of thoracic adenomegaly and that CHF, together with venous hypertension at the left fistula's arm, caused ipsilateral limb edema. Thus, adenomegaly due to VTLS could represent an accompanying feature even in upper limb edema in chronic hemodialysis patients. To our knowledge, this is the first report of such an association. In our patient months were "lost" because we thought that limb edema was secondary to the adenomegaly. It is important that clinicians working in dialysis units are aware that when upper limb edema is present, adenomegaly might just be an accompanying symptom, especially in case of concomitant diagnosis of CHF.