Waldenström’s  Macroglobulinemia:  Clinical Features, Complications, and  Management

Dimopoulos, Meletios A.; Panayiotidis, Panayiotis; Moulopoulos, Lia A.; Sfikakis, Petros P.; Dalakas, Marinos C.

doi:10.1200/jco.2000.18.1.214

Cited by 301 publications

(226 citation statements)

References 120 publications

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“…Waldenströ m Macroglobulinemia (WM) is a low grade lymphoma characterized by the presence of lymphoplasmacytic cells in the bone marrow and an increase in the IgM monoclonal protein in the serum [1][2][3][4]. Although indolent, it remains incurable with a median overall survival (OS) of 5 years, with a 36% 5-year survival in patients with high risk WM based on the ISS-WM scoring system [2,3,5,6].…”

Section: Introductionmentioning

confidence: 99%

Phase II trial of weekly bortezomib in combination with rituximab in untreated patients with Waldenström Macroglobulinemia

et al. 2010

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This study aimed to determine the activity and safety of weekly bortezomib and rituximab in patients with untreated Waldenströ m Macroglobulinemia (WM). Patients with no prior therapy and symptomatic disease were eligible. Patients received bortezomib IV weekly at 1.6 mg/m 2 on days 1, 8, 15, q 28 days 3 6 cycles, and rituximab 375 mg/m 2 weekly on cycles 1 and 4. Primary endpoint was the percent of patients with at least a minor response (MR). Twenty-six patients were treated. At least MR was observed in 23/26 patients (88%) (95% CI: 70-98%) with 1 complete response (4%), 1 near-complete response (4%), 15 partial remission (58%), and 6 MR (23%). Using IgM response evaluated by nephlometry, all 26 patients (100%) achieved at least MR or better. The median time to progression has not been reached, with an estimated 1-year event free rate of 79% (95% CI: 53, 91%). Common grade 3 and 4 therapy related adverse events included reversible neutropenia in 12%, anemia in 8%, and thrombocytopenia in 8%. No grade 3 or 4 neuropathy occurred. The combination of weekly bortezomib and rituximab exhibited significant activity and minimal neurological toxicity in patients with untreated WM. Am. J. Hematol. 85:670-674, 2010. V

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Section: Introductionmentioning

confidence: 99%

Phase II trial of weekly bortezomib in combination with rituximab in untreated patients with Waldenström Macroglobulinemia

et al. 2010

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“…11 The median age at diagnosis varies between 63 and 68 years, and slightly more common in males. 2 Our patient was 50 year old male, which is slightly younger than the cases described in other studies. The disease was originally described by Waldenstrom in 1944.…”

Section: Discussionmentioning

confidence: 54%

“…Patients may develop constitutional symptoms, pancytopenia, organomegaly, neuropathy, and symptoms associated with immunoglobulin deposition or hyperviscosity. 2,3) WM is incurable with current therapy, and half of the patients die of disease progression; median survival is approximately 5 years. (4) The incidence of development of second cancers was not significantly different between asymptomatic and symptomatic WM and between treated and untreated pati ents.…”

Section: Introductionmentioning

confidence: 99%

Waldenstrom’s Macroglobulinemia - a coincidental finding in a case of carcinoma of buccal mucosa

Salma¹,

Madhuri

Kumar

et al. 2014

Int Jour of Biomed Res

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“…20,28 The biological function of various tissues and/or organs may be altered by the formation and deposition of monoclonal IgM aggregates, however, the clinical manifestations related to their deposition are not frequent.…”

Section: Clinical Symptomsmentioning

confidence: 99%

“…It is also responsible for the development of distal symmetrical sensorimotor polyneuropathy or multiple mononeuropathy with axonal degeneration. 20,21 Monoclonal IgM can cause platelet dysfunction by binding to IIIa and Ib glycoproteins on the surface of platelets or due to nonspecific interactions with platelets. 9 It may also neutralize the activity of several coagulation factors (fibrinogen, prothrombin, factors V, VII, VIII, IX, X, and Von Willebrand factor), 9,22 triggering hemostatic disorders that are the source of hemorrhagic manifestations.…”

Section: Clinical Symptomsmentioning

confidence: 99%

Waldenström's macroglobulinemia - a review

Coimbra

Neves

Lima

et al. 2014

Rev. Assoc. Med. Bras.

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Waldenström's macroglobulinemia (WM) is a lymphoproliferative disease of B lymphocytes, characterized by a lymphoplasmocytic lymphoma in the bone marrow and by IgM monoclonal hypergammaglobulinemia. It was first described in 1944 by Jan Gösta Waldenström, reporting two patients with oronasal bleeding, lymphadenopathy, anemia, thrombocytopenia, high erythrocyte sedimentation rate and serum viscosity, normal radiography and bone marrow infiltrated by lymphoid cells. The WM is a rare disease with a typically indolent clinical course, affecting mainly individuals aged between 63 and 68 years. Most patients have clinical signs and symptoms related to hyperviscosity resulting from IgM monoclonal gammopathy, and/or cytopenias resulting from bone marrow infiltration by lymphoma. The differential diagnosis with other lymphomas is essential for the assessment of prognosis and therapeutic approach. Treatment of patients with asymptomatic WM does not improve the quality of life of patients, or increase their survival, being recommended, therefore, their follow-up. For the treatment of symptomatic patients, alkylating agents, purine analogs and anti-CD20 monoclonal antibodies are used. However, the disease is incurable and the response to therapy is not always favorable. Recent studies have shown promising results with bortezomib, an inhibitor of proteasomes, and some patients respond to thalidomide. In patients with relapse or refractory to therapy, autologous transplantation may be indicated. The aim of this paper is to describe in detail the current knowledge on the pathophysiology of WM, main clinical manifestations, diagnosis, prognosis and treatment.

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Waldenström’s Macroglobulinemia: Clinical Features, Complications, and Management

Cited by 301 publications

References 120 publications

Phase II trial of weekly bortezomib in combination with rituximab in untreated patients with Waldenström Macroglobulinemia

Phase II trial of weekly bortezomib in combination with rituximab in untreated patients with Waldenström Macroglobulinemia

Waldenstrom’s Macroglobulinemia - a coincidental finding in a case of carcinoma of buccal mucosa

Waldenström's macroglobulinemia - a review

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