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doi: 10.1097/01.HS9.0000547927.01953.62
Advanced Stages

László Pinczés, Zsófia Miltényi, Ádám Jóna, Árpád Illés

Division of Hematology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen

Introduction: Autoimmune cytopenias (AICP), namely autoimmune hemolytic anaemia (AIHA) and autoimmune thrombocytopenia (AITP) often complicate the course of malignant lymphomas. However, the incidence and clinical significance of AICPs associated with Hodgkin lymphoma (HL) have not been defined thoroughly.

Patients and methods: Seven hundred and fifty-six HL patients were diagnosed and treated at the University of Debrecen between 1980 and 2017. We retrospectively assessed the incidence and clinical features of HL-associated autoimmune phenomena, also compared the distribution of clinical, laboratory and pathologic characteristics between HL patients presenting with AICP and those who had no clinical evidence of autoimmune events. Statistical analysis was performed using Fisher's exact test.

Results: We identified 8 cases of AIHA and 4 cases of AITP among 11 patients altogether. The incidence of AICPs in HL patients was 1.59%. Three cases presented at initial diagnosis, 8 cases during follow-up after first line therapy. Ninety-two percent of the AICPs required therapy, 72% of these cases responded well to intravenous steroid. In steroid-refractory AICPs azathioprine, rituximab and romiplostim therapy were effective. No splenectomy was needed. Third of the AICPs was disease-defining event: 2 cases led to the diagnosis of HL, 1 indicated relapse and 1 event revealed a secondary hematological malignancy. AIHAs and AICPs altogether were more frequently observed in patients presenting with advanced stage disease at initial diagnosis (p < 0.012 and p < 0.028 respectively).

Conclusion: The occurrence of AICPs in patients with HL can imply clinical significance. Patients diagnosed in advanced stage are at increased risk of developing AICPs. Our data also emphasise the importance of taking the possibility of underlying hematological malignancy into consideration in newly diagnosed AIHA/AITP cases.

Copyright © 2018 The Authors. Published by Wolters Kluwer Health Inc., on behalf of the European Hematology Association.