Defining lymphoplasmacytic lymphoma: Does MYD88L265P define a pathologically distinct entity among patients with an IgM paraprotein and bone marrow-based low-grade b-cell lymphomas with plasmacytic differentiation?

Hong Fang, Prashant Kapoor, Wilson Gonsalves, Lori A. Frederick, David Viswanatha, Matthew T. Howard, Rong He, William G. Morice, Ellen McPhail, Patricia T Greipp, Stephen Maxted Ansell, Robert A. Kyle, Morie Gertz, Jonas Paludo, Jithma Abeykoon, Rebecca King

Research output: Contribution to journalArticle

Abstract

Objectives Lymphoplasmacytic lymphoma (LPL) remains a poorly defined entity, even with the discovery of MYD88 L265P mutations and association with Waldenström macroglobulinemia (WM). Among bone marrow (BM)-based, low-grade B-cell lymphoma with plasmacytic differentiation (LGBLPD) and immunoglobulin M (IgM) paraproteins, we sought to determine whether MYD88 L265P defines a distinct entity and can help refine diagnostic criteria for LPL. Methods BMs diagnosed with LGBLPD or LPL and serum IgM paraprotein were studied (2007-2013). Clinicopathologic features were reviewed and specimens were tested for MYD88 L265P. Results In total, 138 (87%) of 159 cases had MYD88 L265P, and 158 of 159 were clinically considered WM. MYD88 L265P cases had higher disease burden than MYD88 WT. Features associated with MYD88 L265P include increased mast cells and lymphocyte (not plasma cell)-predominant infiltrate. Hemosiderin, Dutcher bodies, and paratrabecular growth were not associated with MYD88 L265P. Conclusions Our data support a clinicopathologic approach to LPL diagnosis and recognition that it may manifest with varying morphologies, phenotypes, and molecular features.

Original languageEnglish (US)
Pages (from-to)168-176
Number of pages9
JournalAmerican Journal of Clinical Pathology
Volume150
Issue number2
DOIs
StatePublished - Jul 3 2018

Fingerprint

Paraproteins
Immunoglobulin M
Lymphoma
Bone Marrow
Waldenstrom Macroglobulinemia
B-Cell Lymphoma
Non-Hodgkin's Lymphoma
Hemosiderin
Plasma Cells
Mast Cells
Lymphocytes
Phenotype
Mutation
Growth
Serum

Keywords

  • Bone marrow
  • IgM paraprotein
  • Lymphoplasmacytic lymphoma
  • MYD88
  • Waldenström macroglobulinemia

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

@article{ab1010814a1b481ca5826f56af6a3b3d,
title = "Defining lymphoplasmacytic lymphoma: Does MYD88L265P define a pathologically distinct entity among patients with an IgM paraprotein and bone marrow-based low-grade b-cell lymphomas with plasmacytic differentiation?",
abstract = "Objectives Lymphoplasmacytic lymphoma (LPL) remains a poorly defined entity, even with the discovery of MYD88 L265P mutations and association with Waldenstr{\"o}m macroglobulinemia (WM). Among bone marrow (BM)-based, low-grade B-cell lymphoma with plasmacytic differentiation (LGBLPD) and immunoglobulin M (IgM) paraproteins, we sought to determine whether MYD88 L265P defines a distinct entity and can help refine diagnostic criteria for LPL. Methods BMs diagnosed with LGBLPD or LPL and serum IgM paraprotein were studied (2007-2013). Clinicopathologic features were reviewed and specimens were tested for MYD88 L265P. Results In total, 138 (87{\%}) of 159 cases had MYD88 L265P, and 158 of 159 were clinically considered WM. MYD88 L265P cases had higher disease burden than MYD88 WT. Features associated with MYD88 L265P include increased mast cells and lymphocyte (not plasma cell)-predominant infiltrate. Hemosiderin, Dutcher bodies, and paratrabecular growth were not associated with MYD88 L265P. Conclusions Our data support a clinicopathologic approach to LPL diagnosis and recognition that it may manifest with varying morphologies, phenotypes, and molecular features.",
keywords = "Bone marrow, IgM paraprotein, Lymphoplasmacytic lymphoma, MYD88, Waldenstr{\"o}m macroglobulinemia",
author = "Hong Fang and Prashant Kapoor and Wilson Gonsalves and Frederick, {Lori A.} and David Viswanatha and Howard, {Matthew T.} and Rong He and Morice, {William G.} and Ellen McPhail and Greipp, {Patricia T} and Ansell, {Stephen Maxted} and Kyle, {Robert A.} and Morie Gertz and Jonas Paludo and Jithma Abeykoon and Rebecca King",
year = "2018",
month = "7",
day = "3",
doi = "10.1093/ajcp/aqy041",
language = "English (US)",
volume = "150",
pages = "168--176",
journal = "American Journal of Clinical Pathology",
issn = "0002-9173",
publisher = "American Society of Clinical Pathologists",
number = "2",

}

TY - JOUR

T1 - Defining lymphoplasmacytic lymphoma

T2 - Does MYD88L265P define a pathologically distinct entity among patients with an IgM paraprotein and bone marrow-based low-grade b-cell lymphomas with plasmacytic differentiation?

AU - Fang, Hong

AU - Kapoor, Prashant

AU - Gonsalves, Wilson

AU - Frederick, Lori A.

AU - Viswanatha, David

AU - Howard, Matthew T.

AU - He, Rong

AU - Morice, William G.

AU - McPhail, Ellen

AU - Greipp, Patricia T

AU - Ansell, Stephen Maxted

AU - Kyle, Robert A.

AU - Gertz, Morie

AU - Paludo, Jonas

AU - Abeykoon, Jithma

AU - King, Rebecca

PY - 2018/7/3

Y1 - 2018/7/3

N2 - Objectives Lymphoplasmacytic lymphoma (LPL) remains a poorly defined entity, even with the discovery of MYD88 L265P mutations and association with Waldenström macroglobulinemia (WM). Among bone marrow (BM)-based, low-grade B-cell lymphoma with plasmacytic differentiation (LGBLPD) and immunoglobulin M (IgM) paraproteins, we sought to determine whether MYD88 L265P defines a distinct entity and can help refine diagnostic criteria for LPL. Methods BMs diagnosed with LGBLPD or LPL and serum IgM paraprotein were studied (2007-2013). Clinicopathologic features were reviewed and specimens were tested for MYD88 L265P. Results In total, 138 (87%) of 159 cases had MYD88 L265P, and 158 of 159 were clinically considered WM. MYD88 L265P cases had higher disease burden than MYD88 WT. Features associated with MYD88 L265P include increased mast cells and lymphocyte (not plasma cell)-predominant infiltrate. Hemosiderin, Dutcher bodies, and paratrabecular growth were not associated with MYD88 L265P. Conclusions Our data support a clinicopathologic approach to LPL diagnosis and recognition that it may manifest with varying morphologies, phenotypes, and molecular features.

AB - Objectives Lymphoplasmacytic lymphoma (LPL) remains a poorly defined entity, even with the discovery of MYD88 L265P mutations and association with Waldenström macroglobulinemia (WM). Among bone marrow (BM)-based, low-grade B-cell lymphoma with plasmacytic differentiation (LGBLPD) and immunoglobulin M (IgM) paraproteins, we sought to determine whether MYD88 L265P defines a distinct entity and can help refine diagnostic criteria for LPL. Methods BMs diagnosed with LGBLPD or LPL and serum IgM paraprotein were studied (2007-2013). Clinicopathologic features were reviewed and specimens were tested for MYD88 L265P. Results In total, 138 (87%) of 159 cases had MYD88 L265P, and 158 of 159 were clinically considered WM. MYD88 L265P cases had higher disease burden than MYD88 WT. Features associated with MYD88 L265P include increased mast cells and lymphocyte (not plasma cell)-predominant infiltrate. Hemosiderin, Dutcher bodies, and paratrabecular growth were not associated with MYD88 L265P. Conclusions Our data support a clinicopathologic approach to LPL diagnosis and recognition that it may manifest with varying morphologies, phenotypes, and molecular features.

KW - Bone marrow

KW - IgM paraprotein

KW - Lymphoplasmacytic lymphoma

KW - MYD88

KW - Waldenström macroglobulinemia

UR - http://www.scopus.com/inward/record.url?scp=85050734484&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85050734484&partnerID=8YFLogxK

U2 - 10.1093/ajcp/aqy041

DO - 10.1093/ajcp/aqy041

M3 - Article

C2 - 29868855

AN - SCOPUS:85050734484

VL - 150

SP - 168

EP - 176

JO - American Journal of Clinical Pathology

JF - American Journal of Clinical Pathology

SN - 0002-9173

IS - 2

ER -