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: Sara Mohsin, M.D.[2]Roukoz A. Karam, M.D.[3]
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, 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
- The symptoms of Waldenström macroglobulinemia usually develop in the seventh and eighth decade of life, and it will typically start with symptoms such as fatigue, unexplained weight loss, peripheral neuropathy, shortness of breath, purpura, raynaud's phenomenon, and vision problems.[1]
Complications
- Common complications of Waldenström macroglobulinemia include:
- Hyperviscosity syndrome[2]
- Cryoglobulinemia[3]
- Peripheral neuropathy(15%).[4]
- Amyloidosis of the heart, kidney, liver, lungs, and joints[5]
- Cold haemagglutinin disease/Autoimmune hemolytic anemia(<10%).[6]
- Gastrointestinal malabsorption[7]
- Renal insufficiency[8]
- Fundoscopic abnormalities[2]
- Congestive heart failure.
- Schnitzler syndrome which is an autoimmune complication associated with elevated IgM levels, that leads to fever, itchy skin lesions, and joint aches.
Late and rare complications
- Large cell transformation (Richter syndrome).
- Dr. Richter of the University of Minnesota first recognized the blockage in maturity of the lymphoma cells at a point when they can't mature beyond the large cell stage thus, leading to large cell transformation.
- Central Nervous system Lymphoma (Bing-Neel syndrome).
- The development of Waldenström macroglobulinemia cells in the central nervous system was first described by Drs. Bing and Neel and carries their names as the Bing-Neel syndrome. WM involves CNS in following two forms:
- Actual tumor developing in the brain substance causing seizures and paralysis.
- Tumor cells invading meninges and cranial nerves without causing the actual tumors and with or without CSF cryoglobulinemia,[9] leading to following symptoms:[10]
- Headache.
- Confusion.
- Neck stiffness.
- Sporadic loss of motor function.
- Facial paralysis.
- Drooping eyelid.
- Double vision.
- Difficult swallowing.
- Visual loss.
- Hearing loss.
- The development of Waldenström macroglobulinemia cells in the central nervous system was first described by Drs. Bing and Neel and carries their names as the Bing-Neel syndrome. WM involves CNS in following two forms:
Prognosis
- Prognosis is generally poor.
- The median survival from the time of diagnosis is 6.4 years.[11]
- The median disease-specific survival is 11.2 years.[11]
- Approximately 10% patients still live at 15 years.[12][13]
- 5-year survival rate is 78%.
- After 2000, a 2-fold increased mortality is reported in patients diagnosed with WM when compared with expected population mortality.[14]
- The presence of symptoms is associated with a particularly poor prognosis among patients with the disease.
- Prognosis of asymptomatic patients is similar to that of the general population.[15]
Adverse prognostic factors
- Some of the pretreatment factors associated with shorter survival in WM patients are:[16][11]
- Age >/=65 years.
- Organomegaly (Hepatosplenomegaly).
- B-symptoms (weight loss, fever or night sweats).
- Anaemia (Hb < 10.0 g/dl).
- Platelets <100 x 10(6)/dl.
- Albumin <3.5 g/dl.
- Bone marrow lymphoplasmacytic infiltrate >/=50%.
- Elevated beta2-microglobulin ( associated with 3-fold increase in death).
- Leucopenia (<4.0 x 10(9)/l).
- Thrombocytopenia (<150 x 10(9)/l).
- Quantitative IgM < 0.4 g/l.
- Hyperviscosity.
- Other prognostic factors recently studied are:
Risk Stratification Criteria
Several systems have been proposed to risk stratify patients with Waldenström macroglobulinemia; however, recently, a standardized scoring system has been developed: the International prognostic scoring system for Waldenström macroglobulinemia (IPSSWM):[20]
| Risk factors | Score |
|---|---|
| Age > 65 | 1 |
| Hemoglobin ≤ 11.5g/dl | 1 |
| Platelet ≤ 100,000μl | 1 |
| β-microglobulin > 3mg/l | 1 |
| IgM > 70g/l | 1 |
| Risk group | Score | 5-year survival |
|---|---|---|
| Low | 0-1 (except age) | 87% |
| Intermediate | 2 or age>65 | 68% |
| High | ≥3 | 36% |
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH (2009). "Bing-Neel Syndrome revisited". Clin Lymphoma Myeloma. 9 (1): 104–6. doi:10.3816/CLM.2009.n.028. PMID 19362988.
- ↑ Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG (2009). "Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options". Clin Lymphoma Myeloma. 9 (6): 462–6. doi:10.3816/CLM.2009.n.091. PMID 19951888.
- ↑ 11.0 11.1 11.2 Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM; et al. (2006). "Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia". Br J Haematol. 133 (2): 158–64. doi:10.1111/j.1365-2141.2006.06003.x. PMID 16611306.
- ↑ 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.
- ↑ Kyle RA, Greipp PR, Gertz MA, Witzig TE, Lust JA, Lacy MQ; et al. (2000). "Waldenström's macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil". Br J Haematol. 108 (4): 737–42. PMID 10792277.
- ↑ Kyle, Robert A.; Larson, Dirk R.; McPhail, Ellen D.; Therneau, Terry M.; Dispenzieri, Angela; Kumar, Shaji; Kapoor, Prashant; Cerhan, James R.; Rajkumar, S. Vincent (2018). "Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review". Mayo Clinic Proceedings. 93 (6): 739–746. doi:10.1016/j.mayocp.2018.02.011. ISSN 0025-6196.
- ↑ Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P; et al. (2005). "Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance?". Clin Cancer Res. 11 (5): 1786–90. doi:10.1158/1078-0432.CCR-04-1899. PMID 15756000.
- ↑ Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P; et al. (2003). "Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia". Ann Oncol. 14 (8): 1299–305. PMID 12881396.
- ↑ Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R; et al. (2008). "Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia". Leuk Lymphoma. 49 (6): 1104–7. doi:10.1080/10428190802074619. PMID 18452095.
- ↑ Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A; et al. (2009). "Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase". Clin Lymphoma Myeloma. 9 (1): 50–2. doi:10.3816/CLM.2009.n.012. PMID 19362972.
- ↑ Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X; et al. (2008). "CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia". Blood. 112 (12): 4683–9. doi:10.1182/blood-2007-04-084525. PMC 2597134. PMID 18216294.
- ↑ Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J; et al. (2009). "International prognostic scoring system for Waldenstrom macroglobulinemia". Blood. 113 (18): 4163–70. doi:10.1182/blood-2008-08-174961. PMID 19196866.