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Nina Lu,1 Chin Loon Neoh,2 Zhengying Ruan,3 Lei Zhao,4 Limei Ying,1 Xiaochang Zhang,1 Sai Chen,1 Linglong Xu1 1Department of Hematology, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, Zhejiang 318000, People’s Republic of China; 2Department of Hematology, Royal Marsden Hospital, London SW3 6JJ, UK; 3Department of Pathology, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, Zhejiang 318000, People’s Republic of China; 4Department of Laboratory Medicine, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, Zhejiang 318000, People’s Republic of ChinaCorrespondence: Linglong Xu Email daniongniong@163.comAbstract: Essential thrombocythaemia (ET) and Waldenström macroglobulinaemia (WM) are two distinct disorders. Studies have reported several cases of myeloproliferative neoplasms (MPNs) with concomitant plasma cell dyscrasia. However, there were no reported cases of ET with concomitant WM to date. Here, we present a 55-year-old Chinese man with thrombocytosis and raised immunoglobulin level. Further investigations led to a diagnosis of ET and coexistent WM. Next-generation sequencing (NGS) of his bone marrow identified 3 mutated genes: JAK2 V617F, MYD88 L265P, and ATM F1036L. After being treated with pegylated interferon and low-dose aspirin, his platelet count normalized and immunoglobulinM (IgM) level reduced. To the best of our knowledge, this is the first reported case of dual pathology ET with WM.Keywords: myeloproliferative neoplasm, Waldenström macroglobulinaemia, Janus kinase 2, myeloid differentiation factor 88

Linglong Xu, Xiaoxiao Ding, Limei Ying, Xiaochang Zhang, Nina Lu Department of Hematology, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, Zhejiang, People’s Republic of ChinaCorrespondence: Linglong XuDepartment of Hematology, Taizhou Central Hospital (Taizhou University Hospital), 999 Donghai Road, Taizhou, Zhejiang 318000, People’s Republic of ChinaTel +86 576 8852 6258Email daniongniong@163.comAbstract: MYD88 mutation has been reported in various lymphomas, specifically in lymphoplasmacytic lymphoma. Yet, the mutation has not been reported in primary follicular lymphoma. Here, we present a 62-year-old male with follicular lymphoma who had an MYD88 L265P somatic mutation and monoclonal IgM gammopathy. He received four cycles of R-CHOP immunochemotherapy. Interim PET/CT evaluation indicated a state of stable disease (SD). Neither did serum IgM remarkably drop. He was then given a bortezomib-contained regimen which significantly reduced the level of serum IgM. To the best of our knowledge, this is the first report of follicular lymphoma with monoclonal IgM and MYD88 L265P mutation. The present case indicated bortezomib may benefit these patients.Keywords: follicular lymphoma, monoclonal gammopathy, immunochemotherapy, MYD88 mutation