Haematologica 2003 - Supplements

CLINICOPATHOLOGICAL DEFINITION OF
WALDENSTRÖM’S MACROGLOBULINEMIA:
CONSENSUS PANEL I RECOMMENDATIONS FROM
THE SECOND INTERNATIONAL WORKSHOP ON
WALDENSTRÖM’S MACROGLOBULINEMIA.
Roger G. Owen 1 , Steven P. Treon 2 , Ayad Al-Katib 3 , Rafael
Fonseca 4 , Philip R. Greipp 4 , Mary L McMaster 5 , Enrica
Morra 6 , Gerasimos A. Pangalis 7 , Jesus F. San Miguel 8 ,
Andrew R. Branagan 2 , Meletios A. Dimopoulos 7 .
Leeds General Infirmary, Leeds, UK 1 ; Dana-Farber Cancer
Institute and Harvard Medical School, Boston MA, USA 2 , Van
Elslander Cancer Center, Grosse Point Woods, MI, USA 3 ; Mayo
Clinic, Rochester, MN, USA 4 ; National Cancer Institute, Bethesda,
MD, USA 5 ; Niguarda Ca’Granda Hospital, Milan, ITALY 6 ; University
of Salamanca, Salamanca, SPAIN 8 , University of Athens, Athens,
Greece 7 .
WM is an uncommon lymphoproliferative disorder characterized
primarily by bone marrow infiltration and IgM monoclonal
gammopathy. It should be considered a distinct
clinicopathological entity rather than a clinical syndrome
secondary to IgM secretion. The underlying pathological
diagnosis in WM is lymphoplasmacytic lymphoma as defined by
the WHO and REAL classification criteria. The concentration of
monoclonal IgM can vary widely in WM and it is not possible to
define a concentration, which reliably distinguishes WM from
MGUS and other lymphoproliferative disorders. A diagnosis of
WM can therefore be made irrespective of IgM concentration if
there is evidence on a bone marrow trephine biopsy of bone
marrow infiltration by lymphoplasmacytic lymphoma with
predominantly an intertrabecular pattern and this is supported by
appropriate immunophenotypic studies. Simple criteria to
distinguish patients with symptomatic WM who require therapy
from those with asymptomatic WM and MGUS were also
proposed. Patients with clinical features attributable to IgM
monoclonal gammopathy but no overt evidence of lymphoma are
considered to constitute a distinct clinical group and the term
“IgM related disorders” is proposed.
PROGNOSTIC MARKERS AND CRITERIA TO INITIATE
THERAPY IN WALDENSTROM’S
MACROGLOBULINEMIA: CONSENSUS PANEL II
RECOMMENDATIONS FROM THE SECOND
INTERNATIONAL WORKSHOP ON WALDENSTROM’S
MACROGLOBULINEMIA.
Robert A. Kyle 1 , Steven P. Treon 2 , Raymond Alexanian 3,
Bart Barlogie 4 , Magnus Bjorkholm 5 , Madhav Dhodapkar 6, T.
Andrew Lister 7 , Giampaolo Merlini 8 , Pierre Morel 9 , Marvin
Stone 10 , Andrew R. Branagan 2 , Veronique Leblond 11
Mayo Clinic, Rochester, MN, USA 1 , Dana Farber Cancer Institute
and Harvard Medical School, Boston MA, USA 2 , The University of
Texas M.D. Anderson Cancer Center, Houston, TX, USA 3 ,
Myeloma Institute for Research and Therapy, Little Rock, AR,
USA 4 , Department of Medicine, Karolinska Hospital and Institutet,
Stockholm, SWEDEN 5 , Laboratory of Tumor Immunology and
Immunotherapy, The Rockefeller University, New York, NY 10021,
USA 6 , Department of Oncology, St Bartholomew's Hospital,
London, UK 7 , Scientific Biotechnology Research Laboratories,
University Hospital IRCCS Policlinico San Matteo, Pavia, ITALY 8 ,
Service d'Hematologie Clinique, Centre Hospitalier Schaffner,
Lens, FRANCE 9 , Baylor Charles A. Sammons Cancer Center,
Dallas, TX, USA 10 , and Departement d'Hematologie, Hopital Pitie-
Salpetriere, AP-HP, Paris, FRANCE 11 .
The panel recommended that initiation of therapy should not be
based on the IgM level per sé since this may not correlate with
the clinical manifestations of WM. The consensus panel agreed
that initiation of therapy was appropriate for patients with
constitutional symptoms such as recurrent fever, night sweats,
fatigue due to anemia, or weight loss. The presence of
progressive, symptomatic lymphadenopathy or splenomegaly
provide additional reasons to begin therapy. The presence of
anemia with a hemoglobin value of ≤ 10 g/dL or a platelet count