Summary
Definition
History and exam
Key diagnostic factors
- idade >70 anos
- sexo masculino
- ascendência branca
Other diagnostic factors
- história de gamopatia monoclonal de significado indeterminado (MGUS) monoclonal por IgM
- história familiar de doença linfoproliferativa de células B
- história familiar de MW (ou gamopatias monoclonais relacionadas)
- fadiga, fraqueza, dispneia
- anorexia
- infecções
- neuropatia periférica
- Sintomas B (perda de peso, febre, sudorese noturna)
- Síndrome de Raynaud
- esplenomegalia
- linfadenopatia
- hepatomegalia
- sangramento da pele e/ou mucosa (púrpura, epistaxe)
- sintomas oftalmológicos
- cefaleia
- tontura e/ou vertigem
- zumbido
- trombose
Risk factors
- Gamopatia de IgM monoclonal de significado indeterminado (MGUS)
- história familiar de doença linfoproliferativa de células B
- história familiar de MW
- vírus da hepatite C (HCV)
Diagnostic investigations
1st investigations to order
- Hemograma completo com diferencial
- teste hematínico (ferro, vitamina B12 e folato)
- esfregaço de sangue periférico
- ureia, creatinina, eletrólitos
- TFHs
- albumina sérica
- concentração sérica de lactato desidrogenase
- microglobulina beta-2 sérica
- ácido úrico sérico
- imunoglobulinas quantitativas séricas
- eletroforese de proteínas séricas com imunofixação
- avaliação da medula óssea
- teste de mutação genética
- TC de tórax, abdome e pelve
Investigations to consider
- Urina de 24 horas para proteína total e eletroforese de proteína urinária com imunofixação
- cadeias leves livres no soro
- viscosidade sérica
- crioaglutininas e crioglobulinas
- sorologia viral (hepatite B e C e HIV)
- anticorpos anti-glicoproteína associada à mielina (MAG)
- anticorpos anti-gangliosídeo M1 (anti-GM1)
- anticorpos IgM anti-sulfatídio
- estudos de condução nervosa/eletromiografia
- biópsia do coxim gorduroso com coloração com vermelho Congo
- fundoscopia
- tempo de protrombina (TP) e tempo de tromboplastina parcial ativada (TTPa)
- biópsia de linfonodos
- PET-CT com flúor-18 fluordesoxiglucose do tórax, abdome e pelve
Treatment algorithm
assintomático
sintomático com baixa carga tumoral
sintomático com alta carga tumoral
responsivos aos esquemas iniciais de quimioimunoterapia contendo rituximabe
recidiva ou doença refratária
Contributors
Authors
Guy Pratt, MD, FRCP, FRCPath
Honorary Consultant Haematologist
University Hospitals Birmingham NHS Foundation Trust
Professor of Haematology
Institute of Cancer and Genomic Sciences
College of Medical and Dental Sciences
University of Birmingham
Birmingham
UK
Disclosures
GP has received honoraria for serving on advisory boards for Janssen (J&J), BeOne Medicines (formerly known as BeiGene), and Astra-Zeneca. GP received funding from BeOne Medicines to attend an educational event. GP is an author of references cited within this topic.
Acknowledgements
Dr Guy Pratt wishes to gratefully acknowledge Dr Boris Kobrinsky and Dr Kenneth Hymes, the previous contributors to this topic.
Disclosures
BK and KH declare that they have no competing interests.
Peer reviewers
Shaji Kumar, MD
Consultant
Department of Hematology
Mayo Clinic
Rochester
MN
Disclosures
SK declares that he has no competing interests.
Madhav Dhodapkar, MD
Professor of Medicine
Chief, Section of Hematology
Department of Internal Medicine
Yale University School of Medicine
New Haven
CT
Disclosures
MD declares that he has no competing interests.
Xavier Leleu, MD, PhD
Instructor in Hematology
Department of Hematology
Hopital Huriez CHRU
Lille
France
Disclosures
XL has received lecture fees and research funding from Janssen-Cilag, Celgene, Chugai, Amgen, Novartis, Mundipharma, and Roche. XL is an author of a number of references cited in this topic.
Shayna Sarosiek, MD
Assistant Professor
Harvard Medical School
Boston
MA
Disclosures
SS has received research and consulting funding from BeiGene and ADC Therapeutics.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
Pratt G, El-Sharkawi D, Kothari J, et al. Diagnosis and management of Waldenström macroglobulinaemia - a British Society for Haematology guideline. Br J Haematol. 2022 Apr;197(2):171-87.Full text Abstract
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: Waldenström macroglobulinemia/lymphoplasmacytic lymphoma. [internet publication].Full text
Leblond V, Kastritis E, Advani R, et al. Treatment recommendations from the Eighth International Workshop on Waldenström's Macroglobulinemia. Blood. 2016 Sep 8;128(10):1321-8.Full text Abstract
Kastritis E, Leblond V, Dimopoulos MA, et al. Waldenström's macroglobulinaemia: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29 (suppl 4):iv41-50. Abstract
Kapoor P, Ansell SM, Fonseca R, et al. Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines 2016. JAMA Oncol. 2017 Sep 1;3(9):1257-65.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Mieloma múltiplo (MM)
- Linfomas de células B de baixo grau (por exemplo, linfoma folicular)
- Leucemia linfocítica crônica (LLC)
More DifferentialsGuidelines
- Clinical practice guidelines in oncology: hematopoietic cell transplantation (HCT)
- Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines
More Guidelines
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