TY - JOUR
T1 - Waldenström macroglobulinemia
T2 - What a hematologist needs to know
AU - Kapoor, Prashant
AU - Paludo, Jonas
AU - Vallumsetla, Nishanth
AU - Greipp, Philip R.
N1 - Publisher Copyright:
© 2015 Elsevier Ltd.
PY - 2015/9/1
Y1 - 2015/9/1
N2 - Waldenström macroglobulinemia (WM) is a distinct hematologic malignancy characterized by a lymphoplasmacytic bone marrow infiltration and the presence of immunoglobulin (Ig)M monoclonal protein. Patients typically present at an advanced age, and a substantial proportion are asymptomatic at diagnosis. A unifying diagnosis of WM may be missed by an unsuspecting hematologist, as symptomatic patients present with a multitude of non-specific manifestations. Although constitutional and neuropathy-related symptoms predominate, concomitant IgM-induced hyperviscosity-associated features can provide useful diagnostic clues. There are specific indications for initiation of therapy. This review focuses on the most up-to-date management strategies of WM, in addition to highlighting the recent discoveries of MYD88 and CXCR4 mutations that have shed unprecedented light on the complex signaling pathways, and opened avenues for novel therapeutic targeting. Although WM remains incurable, with the rapid emergence and integration of effective novel therapies, its clinical course appears poised to improve in the foreseeable future.
AB - Waldenström macroglobulinemia (WM) is a distinct hematologic malignancy characterized by a lymphoplasmacytic bone marrow infiltration and the presence of immunoglobulin (Ig)M monoclonal protein. Patients typically present at an advanced age, and a substantial proportion are asymptomatic at diagnosis. A unifying diagnosis of WM may be missed by an unsuspecting hematologist, as symptomatic patients present with a multitude of non-specific manifestations. Although constitutional and neuropathy-related symptoms predominate, concomitant IgM-induced hyperviscosity-associated features can provide useful diagnostic clues. There are specific indications for initiation of therapy. This review focuses on the most up-to-date management strategies of WM, in addition to highlighting the recent discoveries of MYD88 and CXCR4 mutations that have shed unprecedented light on the complex signaling pathways, and opened avenues for novel therapeutic targeting. Although WM remains incurable, with the rapid emergence and integration of effective novel therapies, its clinical course appears poised to improve in the foreseeable future.
KW - IgM monoclonal gammopathy
KW - Indolent lymphoma
KW - Lymphoplasmacytic lymphoma
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U2 - 10.1016/j.blre.2015.03.001
DO - 10.1016/j.blre.2015.03.001
M3 - Article
C2 - 25882617
AN - SCOPUS:84941261534
SN - 0268-960X
VL - 29
SP - 301
EP - 319
JO - Blood Reviews
JF - Blood Reviews
IS - 5
ER -