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2017 ; 3
(9
): 1257-1265
Nephropedia Template TP
gab.com Text
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English Wikipedia
Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of
Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines 2016
#MMPMID28056114
Kapoor P
; Ansell SM
; Fonseca R
; Chanan-Khan A
; Kyle RA
; Kumar SK
; Mikhael JR
; Witzig TE
; Mauermann M
; Dispenzieri A
; Ailawadhi S
; Stewart AK
; Lacy MQ
; Thompson CA
; Buadi FK
; Dingli D
; Morice WG
; Go RS
; Jevremovic D
; Sher T
; King RL
; Braggio E
; Novak A
; Roy V
; Ketterling RP
; Greipp PT
; Grogan M
; Micallef IN
; Bergsagel PL
; Colgan JP
; Leung N
; Gonsalves WI
; Lin Y
; Inwards DJ
; Hayman SR
; Nowakowski GS
; Johnston PB
; Russell SJ
; Markovic SN
; Zeldenrust SR
; Hwa YL
; Lust JA
; Porrata LF
; Habermann TM
; Rajkumar SV
; Gertz MA
; Reeder CB
JAMA Oncol
2017[Sep]; 3
(9
): 1257-1265
PMID28056114
show ga
IMPORTANCE: Waldenström macroglobulinemia (WM), an IgM-associated
lymphoplasmacytic lymphoma, has witnessed several practice-altering advances in
recent years. With availability of a wider array of therapies, the management
strategies have become increasingly complex. Our multidisciplinary team appraised
studies published or presented up to December 2015 to provide consensus
recommendations for a risk-adapted approach to WM, using a grading system.
OBSERVATIONS: Waldenström macroglobulinemia remains a rare, incurable cancer,
with a heterogeneous disease course. The major classes of effective agents in WM
include monoclonal antibodies, alkylating agents, purine analogs, proteasome
inhibitors, immunomodulatory drugs, and mammalian target of rapamycin inhibitors.
However, the highest-quality evidence from rigorously conducted randomized
clinical trials remains scant. CONCLUSIONS AND RELEVANCE: Recognizing the paucity
of data, we advocate participation in clinical trials, if available, at every
stage of WM. Specific indications exist for initiation of therapy. Outside
clinical trials, based on the synthesis of available evidence, we recommend
bendamustine-rituximab as primary therapy for bulky disease, profound hematologic
compromise, or constitutional symptoms attributable to WM.
Dexamethasone-rituximab-cyclophosphamide is an alternative, particularly for
nonbulky WM. Routine rituximab maintenance should be avoided. Plasma exchange
should be promptly initiated before cytoreduction for hyperviscosity-related
symptoms. Stem cell harvest for future use may be considered in first remission
for patients 70 years or younger who are potential candidates for autologous stem
cell transplantation. At relapse, retreatment with the original therapy is
reasonable in patients with prior durable responses (time to next therapy ?3
years) and good tolerability to previous regimen. Ibrutinib is efficacious in
patients with relapsed or refractory disease harboring MYD88 L265P mutation. In
the absence of neuropathy, a bortezomib-rituximab-based option is reasonable for
relapsed or refractory disease. In select patients with chemosensitive disease,
autologous stem cell transplantation should be considered at first or second
relapse. Everolimus and purine analogs are suitable options for refractory or
multiply relapsed WM. Our recommendations are periodically updated as new,
clinically relevant information emerges.
|Adenine/analogs & derivatives
[MESH]
|Antineoplastic Combined Chemotherapy Protocols/*therapeutic use
[MESH]
free
free
free
