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.