Survivorship and Patients Perspective
Ayse Salihoglu1, Tugrul Elverdi1, Dilek Keskin1, Ahmet Emre Eskazan1, Muhlis Cem Ar1, Seniz Ongoren1, Nukhet Tuzuner2, Teoman Soysal1, Zafer Baslar1
1 Department of Hematology, Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey, 2 Department of Pathology, Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey
Background: The incidence of nephrotic syndrome in Hodgkin lymphoma (HL) is less than 1%. The principal glomerular pathology is minimal change and systemic AA amyloidosis associated with HL is exceptionally rare. Here we describe two patients with HL and AA amyloidosis. Case -1. A 24-year-old male with an unremarkable past medical history was admitted because of fever, edema, weight loss and fatigue. Nephrotic range proteinuria was detected (9.7 g/day). Conglomerates of infradiaphragmatic lymph nodes were seen on imaging. Renal biopsy showed glomerular AA amyloid deposition and core-needle biopsy was consistent with HL. He was considered to have stage IIB disease and ABVD was started. After the first cycle a critical condition with generalized gross edema developed and was treated with diuretics and albumin infusions. After 6 cycles of ABVD he achieved CR. Colchicine and an angiotensin converting enzyme (ACE) inhibitor were initiated after chemotherapy. Nine years and 3 months off therapy he is well and no recurrence of proteinuria or HL has been noted. Case-2. A 31-year-old male was diagnosed with stage IIIB HL of the mixed cellularity type. CR was obtained following 8 ABVD cycles. After 6 years he experienced HL relapse considered as stage IIIB again with concomitant nephrotic range proteinuria (9.3 g/day). Bone marrow biopsy revealed AA amyloid deposits in blood vessel walls. He received 2 cycles of platinum based salvage therapy followed by Dexa-BEAM for stem cell mobilization during which he developed septic shock and admitted to an intensive care unit. Colchicine and an ACE inhibitor were initiated after chemotherapy. He was considered too fragile for transplantation and was followed without therapy. Currently 10 years since off therapy he remained in CR although proteinuria continues during follow-up evaluations. Conclusions. The timing of presentation of AA amyloidosis in respect of HL varies in literature. AA amyloidosis was detected during the first presentation in one patient and during HL relapse in the other. Selective serious albuminuria with normal renal function was the typical manifestation in both patients. Risk of serious complications during therapy (septic shock and development of anasarca in our patients) might be increased for HL patients with AA amyloidosis. Both patients achieved long-lasting CRs and reversal of proteinuria following HL remission occurred in one of the patients.