Hodgkin's lymphoma with concurrent systemic amyloidosis, presenting as acute renal failure, following lymphomatoid papulosis.

BACKGROUND: Hodgkin's lymphoma (HL) has been reported to occur infrequently in association with lymphomatoid papulosis (LP). On the other hand, amyloidosis may also occur as a complication of lymphomas including HL but has never been reported in association with LP. We herein present an unusual case of a patient with LP who 3 years later developed concomitant HL, as well as kidney and brain amyloidosis, leading to nephrotic syndrome, acute renal failure, coma and death within 3 weeks. In our patient the simultaneous development of amyloidosis and HL suggests that amyloidosis is secondary to LP, an association never reported before.
METHODS: A 74-year-old man with a 3-year history of LP presented with oliguria. He was found to have acute renal failure and 37 g of proteinuria. The patient had had a right nephrectomy performed 27 years before because of a small nonfunctioning kidney. He was found to have paraaortic and mesenteric lymphadenopathy that were biopsied percutaneously. Despite supportive dialysis the patient continued to do poorly, went into coma and died.
RESULTS: Biopsy of his single left kidney showed enlarged glomeruli completely replaced by amyloidosis confirmed by Congo red stain and electron microscopy. His paraaortic lymph nodes exhibited classic HL with CD30- and CD15-positive Reed-Sternberg cells in a background of mixed inflammatory cells including prominent eosinophils. A CT scan of the brain was negative; however, the MRI showed multiple periventricular parenchymatous lesions and changes of cerebral amyloid angiopathy.
CONCLUSION: In this case, the concomitant presentation of systemic amyloidosis and HL post-LP suggests that amyloidosis is a late sequela of LP, an association that has never been reported before.