MYD88 mutant lymphoplasmacytic lymphoma/Waldenström macroglobulinemia has distinct clinical and pathological features as compared to its mutation negative counterpart.

• 发表时间: Feb 2015
• 来源期刊: Leuk Lymphoma
• DOI: 10.3109/10428194.2014.924123
• PMID: 24828863
• 文章类型: Comparative Study
• 作者列表: Patkar N,Subramanian PG,Deshpande P,Ghodke K,Tembhare P,Mascarenhas R,Muranjan A,Chaudhary S,Bagal B,Gujral S,Sengar M,Menon H

关键词: ISSWM; India; Lymphoplasmacytic lymphoma; MYD88 mutation; Waldenström; macroglobulinemia

Mesh: Aged; Base Sequence; Biomarkers, Tumor / genetics; DNA Mutational Analysis; Disease Progression; Female; Humans; Male; Middle Aged; Molecular Sequence Data; Mutation, Missense; Myeloid Differentiation Factor 88 / genetics; Prognosis; Remission Induction; Retrospective Studies; Treatment Outcome; Waldenstrom Macroglobulinemia / drug therapy genetics pathology

Abstract

In a first series from India, we report 32 cases of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) over 7 years. Here, we analyzed 32 patients with LPL/WM for MYD88 L265P mutation and correlated mutation staus with hematological and biochemical parameters and also with the International Prognostic Scoring System (ISSWM) and treatment response. Twenty-seven out of 32 cases of LPL/WM (84.3%) harbored the MYD88 L265P mutation. MYD88 wild-type WM was associated with a lower number of tumor cells (p<0.01) and older age (p=0.02) and a lower ISSWM score at presentation (p=0.03) as compared to mutated LPL/WM. On evaluation of response (n=23), 44.4% of patients with MYD88 mutated LPL/WM had progressive disease, whereas no patient in the MYD88 unmutated group changed their baseline status. We confirm the high frequency of MYD88 mutations in LPL/WM. Although the number of MYD88 wild-type cases was limited, our data indicate that MYD88 may represent an adverse prognostic marker for LPL/WM.