Iatrogenic Immunodeficiency-Associated Classical Hodgkin Lymphoma: A Clinicopathologic and Immunophenotypic Study of 9 Cases
Eric Y Loo, L Jeffrey Medeiros, Ken H Young, Pei Lin, Russell Brynes, Roberto Miranda. MD Anderson Cancer Center, Houston, TX; University of Southern California, Los Angeles, CA
Background: Iatrogenic immunodeficiency-associated lymphoproliferative disorders are rare and show a spectrum of morphologic and immunophenotypic features which can mimic those of typical Classical Hodgkin Lymphoma (CHL). We analyzed the salient features of iatrogenic immunodeficiency-associated CHL cases from a 10-year period at our institution.
Design: Records from the last 10 years were searched for cases of classical Hodgkin lymphoma in patients who received immunomodulatory therapy for disease unrelated to treatment of hematologic malignancy or immune suppression for transplant. The clinical, pathologic, and immunologic features were analyzed.
Results: 9 HIV negative patients were identified; 7 were females and 2 males with a median age of 49 years (range, 25 – 77 yrs). Underlying diseases included rheumatoid arthritis (n=5), systemic lupus erythematosus (SLE, n=1), SLE with concurrent dermatomyositis (n=1), dermatomyositis (n=1), and Crohn's disease (n=1). Patients had received methotrexate (MTX, n=6), azathioprine (n=2), and a TNF-α inhibitor (n=3, concurrent MTX in 2). Duration of therapy was documented in 7 cases and ranged from 3 to 20 years. Involved sites included lymph nodes (n=6), intestinal tract (n=2), and skin (n=1). CHL subtypes were nodular sclerosis (n=2) or not further classified (n=7). Seven cases had been staged, and were stage I in 2 cases, II in 1 case, III in 1 case, and IV in 3 cases. Four cases had the typical CHL immunophenotype (CD45-/CD30+/ CD15+/CD20-). Five cases differed by one marker; either CD20+ or CD 15-. EBV encoded RNA was positive in 6/8 cases and LMP-1 was positive in 2/3 cases, consistent with a type II latency infection. Follow-up was available in 4 cases with median period post-treatment being 4 years (range, 7 months – 6 years). All four received chemotherapy with cessation of immunomodulatory drugs, and 2 also received local radiation. Complete remission was achieved in 3/4 cases, all of whom received ABVD. The partial responder received an incomplete regimen of CVPP due to toxicity, but was alive and asymptomatic at 5 years. No patients died of disease.
Conclusions: Iatrogenic immunodeficiency-associated CHL is rare and usually has a nodal presentation. The disease may differ from usual CHL histologic appearances, often varies from the 'typical' immunophenotype, has frequent association with EBV, but appears to respond to chemotherapy and withdrawal of immunomodulatory drugs.
Monday, March 19, 2012 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 228, Monday Morning