Waldenström's macroglobulinemia natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

If left untreated, patients with asymptomatic Waldenström macroglobulinemia may progress to develop fatigue, weight loss, peripheral neuropathy and other symptoms of the disease. Common complications of Waldenström macroglobulinemia include hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, venous thromboembolism, primary amyloidosis, renal insufficiency, malabsorbtive diarrhea, and visual abnormalities. Prognosis varies depending on the various factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease.

Natural History, Complications, and Prognosis

Natural History

Complications

  • Common complications of Waldenström macroglobulinemia include:
    • Hyperviscosity syndrome[2]
    • Cold haemagglutinin disease[3]
    • Cryoglobulinemia[4]
    • Peripheral neuropathy[5]
    • Amyloidosis of the heart, kidney, liver, lungs, and joints[6]
    • Gastrointestinal malabsorption[7]
    • Renal insufficiency[8]
    • Fundoscopic abnormalities[2]

Prognosis

  • Prognosis is generally poor, and the median survival of patients with Waldenström macroglobulinemia varies from 5 to 11 years.[9]


Prognosis

The median survival of patients with waldenström macroglobulinemia varies from 5 to 11 years.[10]

Favorable prognostic factor:

  • Nodular type of bone marrow involvement

Adverse prognostic factors:

  • Age > 65 years
  • Hemoglobin < 11.5 g/dL
  • Platelet <1,000,000/microliter
  • Elevated beta-2-microglobulin level
  • Elevated paraprotein concentration > 7 g/dL

Risk stratification

Low-risk disease:[11]

  • 0-1 adverse prognostic factors excluding age
  • Five-year survival rate is 87%

Intermediate-risk disease:

  • 2 adverse prognostic factors or age
  • Five-year survival rate is 68%

High-risk disease:

  • > 2 adverse prognostic factors
  • Five-year survival rate is 36%

References

  1. Wang H, Chen Y, Li F, Delasalle K, Wang J, Alexanian R; et al. (2012). "Temporal and geographic variations of Waldenstrom macroglobulinemia incidence: a large population-based study". Cancer. 118 (15): 3793–800. doi:10.1002/cncr.26627. PMID 22139816.
  2. 2.0 2.1 García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A; et al. (2001). "Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases". Br J Haematol. 115 (3): 575–82. PMID 11736938.
  3. Owen RG, Pratt G, Auer RL, Flatley R, Kyriakou C, Lunn MP; et al. (2014). "Guidelines on the diagnosis and management of Waldenström macroglobulinaemia". Br J Haematol. 165 (3): 316–33. doi:10.1111/bjh.12760. PMID 24528152.
  4. Michael AB, Lawes M, Kamalarajan M, Huissoon A, Pratt G (2004). "Cryoglobulinaemia as an acute presentation of Waldenstrom's macroglobulinaemia". Br J Haematol. 124 (5): 565. PMID 14871241.
  5. Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T; et al. (2006). "Peripheral neuropathies in Waldenström's macroglobulinaemia". J Neurol Neurosurg Psychiatry. 77 (2): 224–8. doi:10.1136/jnnp.2005.071175. PMC 2077569. PMID 16421127.
  6. Zimmermann I, Gloor HJ, Rüttimann S (2001). "[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia]". Praxis (Bern 1994) (in German). 90 (47): 2050–5. PMID 11763619.
  7. Veloso FT, Fraga J, Saleiro JV (1988). "Macroglobulinemia and small intestinal disease. A case report with review of the literature". J Clin Gastroenterol. 10 (5): 546–50. PMID 3141496.
  8. Vos JM, Gustine J, Rennke HG, Hunter Z, Manning RJ, Dubeau TE; et al. (2016). "Renal disease related to Waldenström macroglobulinaemia: incidence, pathology and clinical outcomes". Br J Haematol. 175 (4): 623–630. doi:10.1111/bjh.14279. PMID 27468978.
  9. Morel, P.; Duhamel, A.; Gobbi, P.; Dimopoulos, M. A.; Dhodapkar, M. V.; McCoy, J.; Crowley, J.; Ocio, E. M.; Garcia-Sanz, R.; Treon, S. P.; Leblond, V.; Kyle, R. A.; Barlogie, B.; Merlini, G. (2009). "International prognostic scoring system for Waldenstrom macroglobulinemia". Blood. 113 (18): 4163–4170. doi:10.1182/blood-2008-08-174961. ISSN 0006-4971.
  10. Oza A, Rajkumar SV (2015). "Waldenstrom macroglobulinemia: prognosis and management". Blood Cancer J. 5: e296. doi:10.1038/bcj.2015.28. PMC 4382666. PMID 25815903.
  11. Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015

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