Lymphoplasmacytic lymphoma: Difference between revisions
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==Risk Factors== | ==Risk Factors== | ||
Common risk factors in the development of Waldenström macroglobulinemia include:<ref name="RF">Waldenström's macroglobulinemia. American Cancer Society (2015)http://www.cancer.org/cancer/waldenstrommacroglobulinemia/detailedguide/waldenstrom-macroglobulinemia-risk-factors Accessed on November 6, 2015</ref> | |||
* | * [[Monoclonal gammopathy of undetermined significance]] (MGUS): | ||
** Pre-existing [[monoclonal gammopathy of undetermined significance]] is the most common [[Risk factors|risk factor]], associated with 40 times more likelihood of developing Waldenström's macroglobulinemia. | |||
* [[Heredity]]: | |||
** Patients with Waldenström's macroglobulinemia usually have a close/first-degree relative with the disease or with a related [[B-cell leukemia|B-cell disease]], such as [[MGUS]] or certain types of [[lymphoma]] or [[leukemia]].<ref name="pmid16357024">{{cite journal| author=Treon SP, Hunter ZR, Aggarwal A, Ewen EP, Masota S, Lee C et al.| title=Characterization of familial Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2006 | volume= 17 | issue= 3 | pages= 488-94 | pmid=16357024 | doi=10.1093/annonc/mdj111 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16357024 }} </ref><ref name="pmid17785558">{{cite journal| author=McMaster ML, Csako G, Giambarresi TR, Vasquez L, Berg M, Saddlemire S et al.| title=Long-term evaluation of three multiple-case Waldenstrom macroglobulinemia families. | journal=Clin Cancer Res | year= 2007 | volume= 13 | issue= 17 | pages= 5063-9 | pmid=17785558 | doi=10.1158/1078-0432.CCR-07-0299 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17785558 }} </ref><ref name="pmid18703425">{{cite journal| author=Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O| title=Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. | journal=Blood | year= 2008 | volume= 112 | issue= 8 | pages= 3052-6 | pmid=18703425 | doi=10.1182/blood-2008-06-162768 | pmc=2569164 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18703425 }} </ref> | |||
* [[Hepatitis C]]: | |||
** Patients with chronic [[hepatitis C]] infection have an overall 20-30% increased risk for developing non-Hodgkin lymohoma and 3-fold increased risk for WM.<ref name="pmid18703425">{{cite journal| author=Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O| title=Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. | journal=Blood | year= 2008 | volume= 112 | issue= 8 | pages= 3052-6 | pmid=18703425 | doi=10.1182/blood-2008-06-162768 | pmc=2569164 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18703425 }} </ref><ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref><ref name="pmid18387498">{{cite journal| author=de Sanjose S, Benavente Y, Vajdic CM, Engels EA, Morton LM, Bracci PM et al.| title=Hepatitis C and non-Hodgkin lymphoma among 4784 cases and 6269 controls from the International Lymphoma Epidemiology Consortium. | journal=Clin Gastroenterol Hepatol | year= 2008 | volume= 6 | issue= 4 | pages= 451-8 | pmid=18387498 | doi=10.1016/j.cgh.2008.02.011 | pmc=3962672 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18387498 }} </ref> | |||
* [[Autoimmune Disease|Autoimmune Diseases]]: | |||
**Personal and family history of autoimmune diseases with autoantibodies leads to 2-3 fold higher risk of developing WM, especially elevated risks are associated with following: | |||
***HIV.<ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref> | |||
***Rickettsiosis.<ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref> | |||
***[[Sjögren syndrome]].<ref name="pmid20181958">{{cite journal| author=Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I et al.| title=Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. | journal=J Natl Cancer Inst | year= 2010 | volume= 102 | issue= 8 | pages= 557-67 | pmid=20181958 | doi=10.1093/jnci/djq043 | pmc=2857799 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20181958 }} </ref><ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref> | |||
***Autoimmune hemolytic anemia.<ref name="pmid20181958">{{cite journal| author=Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I et al.| title=Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. | journal=J Natl Cancer Inst | year= 2010 | volume= 102 | issue= 8 | pages= 557-67 | pmid=20181958 | doi=10.1093/jnci/djq043 | pmc=2857799 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20181958 }} </ref><ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref> | |||
***Sarcoidosis.<ref name="pmid16985251">{{cite journal| author=Landgren O, Engels EA, Pfeiffer RM, Gridley G, Mellemkjaer L, Olsen JH et al.| title=Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia. | journal=J Natl Cancer Inst | year= 2006 | volume= 98 | issue= 18 | pages= 1321-30 | pmid=16985251 | doi=10.1093/jnci/djj361 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16985251 }} </ref> | |||
***SLE.<ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref> | |||
* [[Allergic]] conditions: | |||
**[[Hay fever]] is also known to be associated with increased risk of WM. | |||
==Screening== | ==Screening== |
Revision as of 17:44, 11 February 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Synonyms and keywords: Waldenstrom's macroglobulinemia; Waldenstrom's disease; Primary macroglobulinemia; Hyperviscosity syndrome
Overview
Historical Perspective
- In 1936, Jens Bing and Axel Valdemar Neel, discovered a late and rare complication of WM known as Bing-Neel syndrome (BNS), who observed a case of 2 women, 56 and 39 years old, presenting with rapid neurodegeneration in the setting of hyperglobulinemia.
- In 1944, Jan G. Waldenstrom, a Swedish doctor of internal medicine, first discovered Waldenstrom macroglobulinemia(WM). He reported an unusal presentation of fatigue, lymphadenopathy, bleeding from nose and mouth, worsening anemia, elevated sedimentation rate, low serum fibrinogen levels (hypofibrinogenemia), hyperviscosity, and hypergammaglobulinemia in two patients due to increased levels of a class of an abnormal high molecular weight serum protein called macroglobulins.[1][2]
- In 1962, the first report on familiality in WM was published, and since then many cohort studies as well as small case-control studies have been published showing familial aggregation of WM.[3][4][5][6][7][8][9][10][11] [12][13][14][15]
- In 1994, a Revised European-American classification of lymphoid neaoplasms (REAL) was published by International Lymphoma Study Group which placed WM in the category of lymphoplasmacytic lymphoma (an indolent subtype of non-hodgkins lymphoma). The REAL classification is based on the morphology, immunophenotype, genetic features, and clinical features.[16][17]
- In 2001, WHO also classified the pathology of WM as lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia based on REAL classification.[2]
- In September 26-30, 2002, a consensus group at the Second International Workshop on WM in Athens, Greece, defined WM as a distinct clinicopathologic entity with characteristics of bone marrow infiltration associated with IgM monoclonal gammopathy by WM and proposed a diagnostic criteria forn WM.[2][18]
- In 2009, in Arkansas, a patient of Bing-Neel syndrome discontinued the treatment for BNS which included, "intrathecal chemotherapy with several cycles of systemic chemotherapy followed by autologous stem cell-supported High-dose chemotherapy and bone marrow transplant|high-dose therapy transplant", and in 2013, was still asymptomatic when a follow-up report was published.[19]
Classification
There is no established system for the classification of Waldenström's macroglobulinemia. However, according to a devised criteria based upon patient's symptoms, it can be classified into:[18]
- Symptomatic Waldenstrom macroglobulinemia.
- Asymptomatic/Smoldering Waldenstrom macroglobulinemia (SWM).[20]
Criteria | Symptomatic WM | Asymptomatic WM | IgM-Related Disorders | MGUS |
---|---|---|---|---|
IgM monoclonal protein | + | + | + | + |
Bone marrow infiltration | + | + | - | - |
Symptoms attributable to IgM | + | - | + | - |
Symptoms attributable to tumor infiltration | + | - | - | - |
Pathophysiology
- Waldenström macroglobulinemia arises from terminally differentiated B lymphocytes, which are normally involved in humoral immunity.
- It is understood that Waldenström macroglobulinemia is mediated by 2 major factors:
- The secretion of IgM paraprotein
- Causes symptoms of hyperviscosity syndrome
- The infiltration of tissues with neoplastic lymphoplasmacytic cells
- Mainly the bone marrow, spleen,and lymph nodes
- Sometimes the liver, gastrointestinal tract, lungs, kidneys, skin, eyes, and central nervous system
- The secretion of IgM paraprotein
Genetics
- The exact pathogenesis of Waldenström macroglobulinemia is not completely understood; however, its familial pattern of involvement supports the role played by genetic factors in the pathogenesis of this disease.[21][13]
- Development of Waldenström macroglobulinemia is the result of multiple genetic mutations.[22]
- Somatic mutations as well as chromosomal abnormalities play a part in the pathogenesis of this disease:
- A mutation of the MYD88 gene (L265P) has been found in more than 90% of patients with Waldenström macroglobulinemia, while it has rarely presented in patients with other types of mature B-cell tumors.[23][24][25][26][27][28][29][30]
- MYD88: The activating point mutation of MYD88 augments growth and survival of both normal and neoplastic B cells by preventing apoptosis. Point mutation of MYD88 leads to leucine to proline substitution in codon 265 (L265P) of MYD88 and produces constantly overactive protein causing proliferation of malignant cells that should normally undergo apoptosis.
- Monoclonal gammopathy of undetermined significance patients found to have MYD88 L265P mutation have significantly higher risk of progression to Waldenström macroglobulinemia or to other lymphoproliferative disorders.
- Less commonly (30-35%), nonsense or frameshift mutations in the C-X-C chemokine receptor type 4 (CXCR4) 5338X gene have also been reported in patients with Waldenström macroglobulinemia.[31]
- Patients with Waldenström's macroglobulinemia with co-existing mutation of MYD88 & CXCR4 are more likely to have hyper-viscosity syndrome and bone marrow involvement.[22]
- Somatic hypermutation in IGHV/IG gene rearrangement.[32]
- ARIDA mutations.
- A mutation of the MYD88 gene (L265P) has been found in more than 90% of patients with Waldenström macroglobulinemia, while it has rarely presented in patients with other types of mature B-cell tumors.[23][24][25][26][27][28][29][30]
Cytogenetics
- Many cytogenetic abnormalities were reported in Waldenström macroglobulinemia patients including:
- Deletion (genetics) of the long arm of chromosome 6q21-22.1 (most common,50%).[33]
- Deletion of long arm of chromosome 10, 12 0r 20.
- t(9;14)(p13;q32)(50%).[32]
- Trisomy 4(20%).[32]
- Trisomy 5.
- Monosomy 8.[34]
- Deletions of regions of 13q14 that include MIRN15A and MIRN16-1.[35]
- t(11;18)(q21;q21) involving API-malt1.[35]
- t(8;14).
- t(14;18).
Epigenetics
- Three most common epigenetic causes are DNA methylation, histone acetylation, and non-coding RNAs such as miRNAs.[36]
- Up-regulation of miRNAs 155, 184, 206, 363, 494, and 542-3p occurs in Waldenström macroglobulinemia; among which miRNA-155 has a crucial role in tumor cell growth and proliferation in Waldenström macroglobulinemia.
- Gene transcription through histone acetylation occurs following increased expression of miRNA-206 and reduced expression of miRNA-9.
Associated Conditions
Several studies showed an increased incidence of following second cancers in patients with Waldenström macroglobulinemia:[37]
- Diffuse large B-cell lymphoma
- Myelodysplastic syndrome/Acute myeloid leukemia
- Brain tumor
- Renal MALT lymphoma [38]
Microscopic Pathology
WM/LPL is a form of an indolent (slowly growing) non-hodgkin lymphoma. LPL is called so because the lymphoma cells have the characteristics of both lymphocytes and plasma cells. After a detailed clinicopathological assessment and review of the published literature, the following diagnostic criteria was proposed for WM:[39]
- IgM monoclonal gammopathy of any concentration.
- Bone marrow infiltration by:[40][41][42]
- Small lymphocytes with clumped chromatin, inconspicuous nucleoli, and sparse cytoplasm.
- Well-formed plasma cells.
- Plasmacytoid lymphocytes (have cytologic features intermediate between above 2 extremes), in following patterns:[43][44]
- Diffuse.
- Interstitial.
- Nodular.
- Paratrabecular.
- Nodular-interstitial.
- Mixed paratrabacular-nodular.
- Following lymphoid organs are involved in WM:
- Bone marrow.
- Lymph nodes(nodal involvement is characterized by paracortical and hilar infiltration with frequent sparing of the subscapular and marginal sinuses).
- Spleen.
- WM has two histologic subtypes:[44]
- Lymphoplasmacytoid (73%).
- Lymphoplasmacytic (27%).
- The cytologic composition and the degree of plasmacytic differentiation varies from case to case.
- The bone marrow contains variable numbers of pleomorphic lymphoid cells.
- Dutcher bodies may be seen in plasma cells, as intracytoplasmic inclusions positive for periodic acid Schiff.
- Mast cell hyperplasia is common and may stimulate tumor cell proliferation and monoclonal IgM secretion.
- Gene expression profiling has indicated that lymphoid cells of WM more closely resemble those of chronic lymphocytic leukemia than those of myeloma.[45]
Immunohistochemistry
Malignant cells in Waldenström macroglobulinemia have following immunophenotypic characteristics:[42] [22]
- Express pan B-cell antigens surface with following immunophenotype:[46][47][39]
- Variable expression of:
- Following aren't expressed:
- IgM positive (mostly).
- IgG positive (few).
- IgA (rare).
- IgD negative (lack).
Causes
Genetic Causes
- Waldenström Macroglobulinemia is most probably caused by a somatic mutation in the MYD88 gene (seen in 90% of cases) or CXR4 gene (seen in 30% of cases).[48][49]
Less Common Causes
Less common causes of Waldenström macroglobulinemia may include:[22][50]
- Chromosomal abnormalities: deletions of 6q23 and 13q14, and gains of 3q13-q28, 6p and 18q.
- Personal and family history of autoimmune diseases with autoantibodies and chronic immune stimulation leads to 2-3 fold higher risk of developing WM, especially elevated risks are associated with following:
- Hay fever.
- Environmental factors:
- According to some recent studies, exposure to following environmental factors seems to have association with the development of WM:[21][57]
- Occupational (Farming).
- Pesticides.
- Paint.
- Rubber dyes.
- Benzene.
- Coal dust.
- Leather manufacturing.
- Wood dust.
- Organic solvents.
- According to some recent studies, exposure to following environmental factors seems to have association with the development of WM:[21][57]
Differentiating Lymphoplasmacytic Lymphoma from Other B cell lymphoid neoplasms
Waldenström macroglobulinemia must be differentiated from other B cell lymphoid neoplasms including:
- Always express CD5
- Usually CD23 positive[58]
- Express CD138, CD38, CD79a, VS38c and CD56 (70%)
- Presence of plasmacytic cell infiltration of bone marrow, osteolytic lesions, and renal insufficiency
- Translocation involving chromosome 11 (t11;14)[63]
Epidemiology and Demographics
- Lymphoplasmacytic lymphoma is one of the rare subtypes of NHL accounting just 1-2% of it.
Prevalence
- The prevalence of Waldenström macroglobulinemia is estimated to be 1000-1,500 cases in United States annually.[68][69]
Incidence
- WM accounts for approximately 1% to 2% of hematologic cancers.[70][69]
- World-wide, the overall age-adjusted incidence of Waldenström macroglobulinemia is 0.38 cases per 100,000 persons annually, increasing with age to 2.85 in patients above 80 years (or 5 cases per 1 million persons per year).[71]
- The age-adjusted incidence rate for males is 0.92 per 100,000 person-years.[72]
- The age-adjusted incidence rate for females is 0.30 per 100,000 person-years.[72]
- Combined age and sex-adjusted incidence is 0.57 per 100,000 person-years.[72]
Age
- The incidence of Waldenström's macroglobulinemia increases after 50 years of age.[73]
Gender
- Men are twice more likely than women to develop WM(5.4 vs. 2.7 per million, respectively). [68][32][74][70]
Race
- Higher incidence in whites (4.1 per million per year) comparative to blacks (1.8 per million per year) and in past 20 years, incidence in whites has elevated.[68][32][75][70]
Epidemiology and demographics of Smoldering Waldenstrom macroglobulinemia
According to a recent study done in 2017, the following data was found out regarding epidemiology and demographics of SWM.[76]
Risk factors | Proportion of SWM |
---|---|
Sex | Males:27.72%, Females:28.31% |
Race | White, non-hispanic:28.97%, White, Hispanic:24.79%, Black:21.01%, Asian:20.41%, Other:26.08%. |
Age in years | 18-49:18.32%, 50-64:25.91%, 65-79:30.8%, ≥80 : 27.26% |
Risk Factors
Common risk factors in the development of Waldenström macroglobulinemia include:[73]
- Monoclonal gammopathy of undetermined significance (MGUS):
- Pre-existing monoclonal gammopathy of undetermined significance is the most common risk factor, associated with 40 times more likelihood of developing Waldenström's macroglobulinemia.
- Hepatitis C:
- Patients with chronic hepatitis C infection have an overall 20-30% increased risk for developing non-Hodgkin lymohoma and 3-fold increased risk for WM.[51][52][53]
- Autoimmune Diseases:
- Allergic conditions:
- Hay fever is also known to be associated with increased risk of WM.
Screening
There is insufficient evidence to recommend routine screening for [disease/malignancy].
OR
According to the [guideline name], screening for [disease name] is not recommended.
OR
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
Natural History, Complications, and Prognosis
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Diagnosis
Diagnostic Study of Choice
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
OR
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
OR
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
OR
There are no established criteria for the diagnosis of [disease name].
History and Symptoms
- Sometimes, Waldenstrom macroglobulinemia (WM) isn’t causing any symptoms when it’s first found. Instead, it’s found when the person has blood tests done for some other reason. WM found this way is sometimes called asymptomatic or smoldering WM.
- Hyperviscosity Syndrome:
The lymphoma cells make varying amounts of a protein called immunoglobulin M (IgM, or macroglobulin). Higher amounts of this protein than normal in blood tends to make it thick leading to hyperviscosity syndrome (when blood becomes thick, it is harder for blood to flow through small blood vessels), and when this occurs, the condition is termed as Waldenstrom macroglobulinemia. This excess amount of IgM antibodies can be ultimately associated with circulatory problems leading to less blood flow to the brain, the eyes or other organs, which may cause any of the following:
- Symptoms resembling those of stroke.
- Dizziness.
- Headache.
- Blurred vision or loss of vision.
- Shortness of breath.
- Numbness and tingling of the fingers or toes (called peripheral neuropathy).
- Muscle weakness.
- Confusion.
- Amyloidosis (due to IgM deposits in kidney and heart tissues lwading to problems with these organs).
- Cryoglobulinemia and vasculitis (inflammation of small blood vessels occurs when IgM antibody forms immune complexes with cold exposure (called cryoglobulin) in cooler parts of body like tip of nose, ears, fingers and toes. This leads to pain and other problems in these body parts upon exposure to cold temperature).
- Not all people with WM develop hyperviscosity, cryoglobulins, or amyloidosis.
Common symptoms of WM
- Mucous membrane bleeding (oronasal).
- Fatigue
- Weakness (due to anemia associated with IgM binding to RBCs).
- Loss of appetite.
- B symptoms as seen in other types of NHL:
- Unexplained fever.
- Drenching night sweats.
- Unexplained weight loss.
- Enlarged lymph nodes.
- Numbness and tingling (painful pins and needle sensation) of the fingers or toes (called peripheral neuropathy).
Less common signs and symptoms of WM
- Enlarged lymph nodes (appearing as 1-2 inches sized lumps under the skin in neck, groin or the armpits).
- Swollen belly/abdomen (due to hepatosplenomegaly).
- Problems with circulation system leading to:
- Headache.
- Confusion.
- Dizziness.
- Symptoms resembling stroke like slurred speech or weakness on one side of body (such patients are advised to consult from their doctor right away).
- Abnormal mucous membrane bleeding (epistaxis, bleeding gums).
- Vision problems (blurred vision or blind spots).
- Kidney problems (leading to weakness, trouble breathing and fluid buildup in body tissues associated with accumulation of excess salt, fluid and waste products in blood secondary to amyloidosis).
- Heart problems (Secondary to amyloidosis, build up of M protein in heart affects its pumping ability, and also the heart has to work harder to pump the thick blood ultimately leading to CHF with following symptoms).
- Palpitations.
- Feeling of tiredness and weakness.
- Cough.
- Shortness of breath.
- Rapid weight gain.
- Swelling of feet and legs.
- Infections (high levels of abnormal antibody in WM slows down the production of normal antibodies).
- Digestive problems (due to M protein build up in intestines):
- Diarrhea.
- Poor absorption of vitamins.
- GIT bleeding (blood in stools/dark stools).
- Sensitivity to cold (due to cryoglobulinemia which is associated with reduced blood flow leading to pain, itching, bluish discoloration or sores in following body parts).
- Tip of nose.
- Ears.
- Fingers.
- Toes.
Read more: http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/more-types-of-nhl/lymphoplasmacytic-lymphoma/?region=on#ixzz5eavoTzUH The majority of patients with [disease name] are asymptomatic.
OR
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
Physical Examination
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
OR
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
OR
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
Laboratory Findings
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
OR
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
OR
[Test] is usually normal among patients with [disease name].
OR
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
OR
There are no diagnostic laboratory findings associated with [disease name].
Electrocardiogram
There are no ECG findings associated with [disease name].
OR
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
X-ray
There are no x-ray findings associated with [disease name].
OR
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with [disease name].
OR
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
CT scan
There are no CT scan findings associated with [disease name].
OR
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
MRI
There are no MRI findings associated with [disease name].
OR
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Other Imaging Findings
There are no other imaging findings associated with [disease name].
OR
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
There are no other diagnostic studies associated with [disease name].
OR
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
Treatment
One or more of the following treatments can be given for lymphoplasmacytic lymphoma.
Watchful waiting
Watchful waiting (also called active surveillance) may be offered for lymphoplasmacytic lymphoma because it develops slowly and may not need to be treated right away. The healthcare team will carefully monitor the person with lymphoplasmacytic lymphoma and start treatment when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.
Chemotherapy
- People with lymphoplasmacytic lymphoma who have symptoms or hyperviscosity syndrome are usually given chemotherapy. Chemotherapy drugs that may be used with or without prednisone include:
- Chlorambucil (Leukeran)
- Fludarabine (Fludara)
- Bendamustine (Treanda)
- Cyclophosphamide (Cytoxan, Procytox)
- Combinations of chemotherapy drugs that may be used include:
- DRC – dexamethasone (Decadron, Dexasone), rituximab (Rituxan) and cyclophosphamide
- BRD – bortezomib (Velcade) and rituximab, with or without dexamethasone
- CVP – cyclophosphamide, vincristine (Oncovin) and prednisone
- R-CVP – CVP with rituximab
- Thalidomide (Thalomid) and rituximab
Targeted therapy
- Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
- Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).
Immunotherapy
- Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
- Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.
Radiation therapy
External beam radiation therapy may be used to treat lymphoplasmacytic lymphoma that develops outside of the lymphatic system (called extralymphatic disease), but this is rare.
Stem cell transplant
- Some people with lymphoplasmacytic lymphoma may be offered a stem cell transplant.
- It may be used if the lymphoma comes back (recurs) after treatment or doesn’t respond to other treatments (called refractory disease).
- Many people with lymphoplasmacytic lymphoma are older or may not be in good health, so a stem cell transplant may not be a good treatment option for them.
Medical Therapy
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
OR
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
OR
The majority of cases of [disease name] are self-limited and require only supportive care.
OR
[Disease name] is a medical emergency and requires prompt treatment.
OR
The mainstay of treatment for [disease name] is [therapy].
OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
OR
[Therapy] is recommended among all patients who develop [disease name].
OR
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
OR
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
OR
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
OR
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Surgery
Surgical intervention is not recommended for the management of [disease name].
OR
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
OR
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
OR
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
OR
Surgery is the mainstay of treatment for [disease or malignancy].
Primary Prevention
There are no established measures for the primary prevention of [disease name].
OR
There are no available vaccines against [disease name].
OR
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
OR
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].
Secondary Prevention
There are no established measures for the secondary prevention of [disease name].
OR
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].
References
- ↑ Waldenström, Jan (2009). "Incipient myelomatosis or «essential« hyperglobulinemia with fibrinogenopenia - a new syndrome?". Acta Medica Scandinavica. 117 (3–4): 216–247. doi:10.1111/j.0954-6820.1944.tb03955.x. ISSN 0001-6101.
- ↑ 2.0 2.1 2.2 Konoplev, Sergej; Medeiros, L. Jeffrey; Bueso-Ramos, Carlos E.; Jorgensen, Jeffrey L.; Lin, Pei (2005). "Immunophenotypic Profile of Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia". American Journal of Clinical Pathology. 124 (3): 414–420. doi:10.1309/3G1XDX0DVHBNVKB4. ISSN 0002-9173.
- ↑ MASSARI R, FINE JM, METAIS R (1962). "Waldenstrom's macroglobulinaemia observed in two brothers". Nature. 196: 176–8. PMID 13933388.
- ↑ Altieri A, Bermejo JL, Hemminki K (2005). "Familial aggregation of lymphoplasmacytic lymphoma with non-Hodgkin lymphoma and other neoplasms". Leukemia. 19 (12): 2342–3. doi:10.1038/sj.leu.2403991. PMID 16224483.
- ↑ Blattner WA, Garber JE, Mann DL, McKeen EA, Henson R, McGuire DB; et al. (1980). "Waldenström's macroglobulinemia and autoimmune disease in a family". Ann Intern Med. 93 (6): 830–2. PMID 6778280.
- ↑ Fine JM, Lambin P, Massari M, Leroux P (1982). "Malignant evolution of asymptomatic monoclonal IgM after seven and fifteen years in two siblings of a patient with Waldenström's macroglobulinemia". Acta Med Scand. 211 (3): 237–9. PMID 6805257.
- ↑ Fine JM, Muller JY, Rochu D, Marneux M, Gorin NC, Fine A; et al. (1986). "Waldenström's macroglobulinemia in monozygotic twins". Acta Med Scand. 220 (4): 369–73. PMID 3099545.
- ↑ Gétaz EP, Staples WG (1977). "Familial Waldenström's macroglobulinaemia: a case report". S Afr Med J. 51 (24): 891–2. PMID 408931.
- ↑ Linet MS, Humphrey RL, Mehl ES, Brown LM, Pottern LM, Bias WB; et al. (1993). "A case-control and family study of Waldenstrom's macroglobulinemia". Leukemia. 7 (9): 1363–9. PMID 8371587.
- ↑ Ogmundsdóttir HM, Jóhannesson GM, Sveinsdóttir S, Einarsdóttir S, Hegeman A, Jensson O; et al. (1994). "Familial macroglobulinaemia: hyperactive B-cells but normal natural killer function". Scand J Immunol. 40 (2): 195–200. PMID 8047841.
- ↑ Seligmann M, Danon F, Mihaesco C, Fudenberg HH (1967). "Immunoglobulin abnormalities in families of patients with Waldenström's macroglobulinemia". Am J Med. 43 (1): 66–83. PMID 4143650.
- ↑ Taleb N, Tohme A, Abi Jirgiss D, Kattan J, Salloum E (1991). "Familial macroglobulinemia in a Lebanese family with two sisters presenting Waldenström's disease". Acta Oncol. 30 (6): 703–5. PMID 1958390.
- ↑ 13.0 13.1 13.2 Treon SP, Hunter ZR, Aggarwal A, Ewen EP, Masota S, Lee C; et al. (2006). "Characterization of familial Waldenstrom's macroglobulinemia". Ann Oncol. 17 (3): 488–94. doi:10.1093/annonc/mdj111. PMID 16357024.
- ↑ Youinou P, le Goff P, Saleun JP, Rivat L, Morin JF, Fauchier C; et al. (1978). "Familial occurrence of monoclonal gammapathies". Biomedicine. 28 (4): 226–32. PMID 104746.
- ↑ Renier G, Ifrah N, Chevailler A, Saint-Andre JP, Boasson M, Hurez D (1989). "Four brothers with Waldenstrom's macroglobulinemia". Cancer. 64 (7): 1554–9. PMID 2505923.
- ↑ Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J; et al. (1999). "World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997". J Clin Oncol. 17 (12): 3835–49. doi:10.1200/JCO.1999.17.12.3835. PMID 10577857.
- ↑ Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML; et al. (1994). "A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group". Blood. 84 (5): 1361–92. PMID 8068936.
- ↑ 18.0 18.1 Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP (2005). "Diagnosis and management of Waldenstrom's macroglobulinemia". J Clin Oncol. 23 (7): 1564–77. doi:10.1200/JCO.2005.03.144. PMID 15735132.
- ↑ Abdallah AO, Atrash S, Muzaffar J, Abdallah M, Kumar M, Van Rhee F; et al. (2013). "Successful treatment of Bing-Neel syndrome using intrathecal chemotherapy and systemic combination chemotherapy followed by BEAM auto-transplant: a case report and review of literature". Clin Lymphoma Myeloma Leuk. 13 (4): 502–6. doi:10.1016/j.clml.2013.03.002. PMID 23747080.
- ↑ Kyle RA, Treon SP, Alexanian R, Barlogie B, Björkholm M, Dhodapkar M; et al. (2003). "Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia". Semin Oncol. 30 (2): 116–20. doi:10.1053/sonc.2003.50038. PMID 12720119.
- ↑ 21.0 21.1 Royer RH, Koshiol J, Giambarresi TR, Vasquez LG, Pfeiffer RM, McMaster ML (2010). "Differential characteristics of Waldenström macroglobulinemia according to patterns of familial aggregation". Blood. 115 (22): 4464–71. doi:10.1182/blood-2009-10-247973. PMC 2881498. PMID 20308603.
- ↑ 22.0 22.1 22.2 22.3 Ngo VN, Young RM, Schmitz R, Jhavar S, Xiao W, Lim KH, Kohlhammer H, Xu W, Yang Y, Zhao H, Shaffer AL, Romesser P, Wright G, Powell J, Rosenwald A, Muller-Hermelink HK, Ott G, Gascoyne RD, Connors JM, Rimsza LM, Campo E, Jaffe ES, Delabie J, Smeland EB, Fisher RI, Braziel RM, Tubbs RR, Cook JR, Weisenburger DD, Chan WC, Staudt LM (2011). "Oncogenically active MYD88 mutations in human lymphoma". Nature. 470 (7332): 115–9. doi:10.1038/nature09671. PMID 21179087.
- ↑ Treon, Steven P.; Xu, Lian; Yang, Guang; Zhou, Yangsheng; Liu, Xia; Cao, Yang; Sheehy, Patricia; Manning, Robert J.; Patterson, Christopher J.; Tripsas, Christina; Arcaini, Luca; Pinkus, Geraldine S.; Rodig, Scott J.; Sohani, Aliyah R.; Harris, Nancy Lee; Laramie, Jason M.; Skifter, Donald A.; Lincoln, Stephen E.; Hunter, Zachary R. (2012). "MYD88 L265P Somatic Mutation in Waldenström's Macroglobulinemia". New England Journal of Medicine. 367 (9): 826–833. doi:10.1056/NEJMoa1200710. ISSN 0028-4793.
- ↑ Varettoni M, Arcaini L, Zibellini S, Boveri E, Rattotti S, Riboni R; et al. (2013). "Prevalence and clinical significance of the MYD88 (L265P) somatic mutation in Waldenstrom's macroglobulinemia and related lymphoid neoplasms". Blood. 121 (13): 2522–8. doi:10.1182/blood-2012-09-457101. PMID 23355535.
- ↑ Shi M, Spurgeon S, Press R, Olson S, Fan G (2015). "MYD88 mutation analysis of a rare composite chronic lymphocyte leukemia and lymphoplasmacytic lymphoma by flow cytometry cell sorting". Ann Hematol. 94 (11): 1941–4. doi:10.1007/s00277-015-2460-6. PMID 26231802.
- ↑ Yang G, Zhou Y, Liu X, Xu L, Cao Y, Manning RJ; et al. (2013). "A mutation in MYD88 (L265P) supports the survival of lymphoplasmacytic cells by activation of Bruton tyrosine kinase in Waldenström macroglobulinemia". Blood. 122 (7): 1222–32. doi:10.1182/blood-2012-12-475111. PMID 23836557.
- ↑ Ngo VN, Young RM, Schmitz R, Jhavar S, Xiao W, Lim KH; et al. (2011). "Oncogenically active MYD88 mutations in human lymphoma". Nature. 470 (7332): 115–9. doi:10.1038/nature09671. PMC 5024568. PMID 21179087.
- ↑ Mori N, Ohwashi M, Yoshinaga K, Mitsuhashi K, Tanaka N, Teramura M; et al. (2013). "L265P mutation of the MYD88 gene is frequent in Waldenström's macroglobulinemia and its absence in myeloma". PLoS One. 8 (11): e80088. doi:10.1371/journal.pone.0080088. PMC 3818242. PMID 24224040.
- ↑ Abeykoon JP, Paludo J, King RL, Ansell SM, Gertz MA, LaPlant BR; et al. (2018). "MYD88 mutation status does not impact overall survival in Waldenström macroglobulinemia". Am J Hematol. 93 (2): 187–194. doi:10.1002/ajh.24955. PMID 29080258.
- ↑ Steven P. Treon, Lian Xu, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Patricia Sheehy, Robert J. Manning, Christopher J. Patterson, Christina Tripsas, Luca Arcaini, Geraldine S. Pinkus, Scott J. Rodig, Aliyah R. Sohani, Nancy Lee Harris, Jason M. Laramie, Donald A. Skifter, Stephen E. Lincoln & Zachary R. Hunter (2012). "MYD88 L265P somatic mutation in Waldenstrom's macroglobulinemia". The New England journal of medicine. 367 (9): 826–833. doi:10.1056/NEJMoa1200710. PMID 22931316. Unknown parameter
|month=
ignored (help) - ↑ Zachary R. Hunter, Lian Xu, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Robert J. Manning, Christina Tripsas, Christopher J. Patterson, Patricia Sheehy & Steven P. Treon (2014). "The genomic landscape of Waldenstrom macroglobulinemia is characterized by highly recurring MYD88 and WHIM-like CXCR4 mutations, and small somatic deletions associated with B-cell lymphomagenesis". Blood. 123 (11): 1637–1646. doi:10.1182/blood-2013-09-525808. PMID 24366360. Unknown parameter
|month=
ignored (help) - ↑ 32.0 32.1 32.2 32.3 32.4 32.5 Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L; et al. (2017). "Waldenström Macroglobulinemia: Review of Pathogenesis and Management". Clin Lymphoma Myeloma Leuk. 17 (5): 252–262. doi:10.1016/j.clml.2017.02.028. PMC 5413391. PMID 28366781.
- ↑ Treon, S. P.; Hunter, Z. R.; Aggarwal, A.; Ewen, E. P.; Masota, S.; Lee, C.; Santos, D. Ditzel; Hatjiharissi, E.; Xu, L.; Leleu, X.; Tournilhac, O.; Patterson, C. J.; Manning, R.; Branagan, A. R.; Morton, C. C. (2006). "Characterization of familial Waldenström's macroglobulinemia". Annals of Oncology. 17 (3): 488–494. doi:10.1093/annonc/mdj111. ISSN 1569-8041.
- ↑ Roelandt F. J. Schop, W. Michael Kuehl, Scott A. Van Wier, Gregory J. Ahmann, Tammy Price-Troska, Richard J. Bailey, Syed M. Jalal, Ying Qi, Robert A. Kyle, Philip R. Greipp & Rafael Fonseca (2002). "Waldenstrom macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions". Blood. 100 (8): 2996–3001. doi:10.1182/blood.V100.8.2996. PMID 12351413. Unknown parameter
|month=
ignored (help) - ↑ 35.0 35.1 Braggio E, Keats JJ, Leleu X, Van Wier S, Jimenez-Zepeda VH, Valdez R; et al. (2009). "Identification of copy number abnormalities and inactivating mutations in two negative regulators of nuclear factor-kappaB signaling pathways in Waldenstrom's macroglobulinemia". Cancer Res. 69 (8): 3579–88. doi:10.1158/0008-5472.CAN-08-3701. PMC 2782932. PMID 19351844.
- ↑ Waldenström macroglobulinemia. International Waldenström Macroglobulinemia foundation (2015)http://www.iwmf.com/sites/default/files/docs/WM_Review_Ghobrial_Jan2014.pdf Accessed on November 12, 2015
- ↑ Morra E, Varettoni M, Tedeschi A, Arcaini L, Ricci F, Pascutto C, Rattotti S, Vismara E, Paris L, Cazzola M (2013). "Associated cancers in Waldenström macroglobulinemia: clues for common genetic predisposition". Clin Lymphoma Myeloma Leuk. 13 (6): 700–3. doi:10.1016/j.clml.2013.05.008. PMID 24070824.
- ↑ Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT (2014). "Renal MALT lymphoma associated with Waldenström macroglobulinemia". J. Formos. Med. Assoc. 113 (4): 255–7. doi:10.1016/j.jfma.2011.02.007. PMID 24685302.
- ↑ 39.0 39.1 Owen RG (2003). "Developing diagnostic criteria in Waldenstrom's macroglobulinemia". Semin Oncol. 30 (2): 196–200. doi:10.1053/sonc.2003.50069. PMID 12720135.
- ↑ Morice WG, Chen D, Kurtin PJ, Hanson CA, McPhail ED (2009). "Novel immunophenotypic features of marrow lymphoplasmacytic lymphoma and correlation with Waldenström's macroglobulinemia". Mod Pathol. 22 (6): 807–16. doi:10.1038/modpathol.2009.34. PMID 19287458.
- ↑ Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML; et al. (2003). "Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia". Semin Oncol. 30 (2): 110–5. doi:10.1053/sonc.2003.50082. PMID 12720118.
- ↑ 42.0 42.1 Ansell, Stephen M.; Kyle, Robert A.; Reeder, Craig B.; Fonseca, Rafael; Mikhael, Joseph R.; Morice, William G.; Bergsagel, P. Leif; Buadi, Francis K.; Colgan, Joseph P.; Dingli, David; Dispenzieri, Angela; Greipp, Philip R.; Habermann, Thomas M.; Hayman, Suzanne R.; Inwards, David J.; Johnston, Patrick B.; Kumar, Shaji K.; Lacy, Martha Q.; Lust, John A.; Markovic, Svetomir N.; Micallef, Ivana N.M.; Nowakowski, Grzegorz S.; Porrata, Luis F.; Roy, Vivek; Russell, Stephen J.; Short, Kristen E. Detweiler; Stewart, A. Keith; Thompson, Carrie A.; Witzig, Thomas E.; Zeldenrust, Steven R.; Dalton, Robert J.; Rajkumar, S. Vincent; Gertz, Morie A. (2010). "Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines". Mayo Clinic Proceedings. 85 (9): 824–833. doi:10.4065/mcp.2010.0304. ISSN 0025-6196.
- ↑ Owen RG, Barrans SL, Richards SJ, O'Connor SJ, Child JA, Parapia LA; et al. (2001). "Waldenström macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors". Am J Clin Pathol. 116 (3): 420–8. doi:10.1309/4LCN-JMPG-5U71-UWQB. PMID 11554171.
- ↑ 44.0 44.1 Andriko JA, Aguilera NS, Chu WS, Nandedkar MA, Cotelingam JD (1997). "Waldenström's macroglobulinemia: a clinicopathologic study of 22 cases". Cancer. 80 (10): 1926–35. PMID 9366295.
- ↑ Chng WJ, Schop RF, Price-Troska T, Ghobrial I, Kay N, Jelinek DF; et al. (2006). "Gene-expression profiling of Waldenstrom macroglobulinemia reveals a phenotype more similar to chronic lymphocytic leukemia than multiple myeloma". Blood. 108 (8): 2755–63. doi:10.1182/blood-2006-02-005488. PMC 1895596. PMID 16804116.
- ↑ Dimopoulos MA, Gertz MA, Kastritis E, Garcia-Sanz R, Kimby EK, Leblond V; et al. (2009). "Update on treatment recommendations from the Fourth International Workshop on Waldenstrom's Macroglobulinemia". J Clin Oncol. 27 (1): 120–6. doi:10.1200/JCO.2008.17.7865. PMID 19047284.
- ↑ Vijay A, Gertz MA (2007). "Waldenström macroglobulinemia". Blood. 109 (12): 5096–103. doi:10.1182/blood-2006-11-055012. PMID 17303694.
- ↑ Steven P. Treon, Lian Xu, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Patricia Sheehy, Robert J. Manning, Christopher J. Patterson, Christina Tripsas, Luca Arcaini, Geraldine S. Pinkus, Scott J. Rodig, Aliyah R. Sohani, Nancy Lee Harris, Jason M. Laramie, Donald A. Skifter, Stephen E. Lincoln & Zachary R. Hunter (2012). "MYD88 L265P somatic mutation in Waldenstrom's macroglobulinemia". The New England journal of medicine. 367 (9): 826–833. doi:10.1056/NEJMoa1200710. PMID 22931316. Unknown parameter
|month=
ignored (help) - ↑ Zachary R. Hunter, Lian Xu, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Robert J. Manning, Christina Tripsas, Christopher J. Patterson, Patricia Sheehy & Steven P. Treon (2014). "The genomic landscape of Waldenstrom macroglobulinemia is characterized by highly recurring MYD88 and WHIM-like CXCR4 mutations, and small somatic deletions associated with B-cell lymphomagenesis". Blood. 123 (11): 1637–1646. doi:10.1182/blood-2013-09-525808. PMID 24366360. Unknown parameter
|month=
ignored (help) - ↑ Roelandt F. J. Schop, W. Michael Kuehl, Scott A. Van Wier, Gregory J. Ahmann, Tammy Price-Troska, Richard J. Bailey, Syed M. Jalal, Ying Qi, Robert A. Kyle, Philip R. Greipp & Rafael Fonseca (2002). "Waldenstrom macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions". Blood. 100 (8): 2996–3001. doi:10.1182/blood.V100.8.2996. PMID 12351413. Unknown parameter
|month=
ignored (help) - ↑ 51.0 51.1 51.2 Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O (2008). "Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden". Blood. 112 (8): 3052–6. doi:10.1182/blood-2008-06-162768. PMC 2569164. PMID 18703425.
- ↑ 52.0 52.1 52.2 52.3 52.4 52.5 Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O (2008). "Chronic immune stimulation and subsequent Waldenström macroglobulinemia". Arch Intern Med. 168 (17): 1903–9. doi:10.1001/archinternmed.2008.4. PMC 2670401. PMID 18809818.
- ↑ 53.0 53.1 de Sanjose S, Benavente Y, Vajdic CM, Engels EA, Morton LM, Bracci PM; et al. (2008). "Hepatitis C and non-Hodgkin lymphoma among 4784 cases and 6269 controls from the International Lymphoma Epidemiology Consortium". Clin Gastroenterol Hepatol. 6 (4): 451–8. doi:10.1016/j.cgh.2008.02.011. PMC 3962672. PMID 18387498.
- ↑ 54.0 54.1 54.2 54.3 Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I; et al. (2010). "Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia". J Natl Cancer Inst. 102 (8): 557–67. doi:10.1093/jnci/djq043. PMC 2857799. PMID 20181958.
- ↑ 55.0 55.1 55.2 55.3 55.4 55.5 Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J; et al. (2008). "Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium". Blood. 111 (8): 4029–38. doi:10.1182/blood-2007-10-119974. PMC 2288717. PMID 18263783.
- ↑ 56.0 56.1 Landgren O, Engels EA, Pfeiffer RM, Gridley G, Mellemkjaer L, Olsen JH; et al. (2006). "Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia". J Natl Cancer Inst. 98 (18): 1321–30. doi:10.1093/jnci/djj361. PMID 16985251.
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- Bone marrow infiltration of small, cleaved cells that are usually paratrabecular
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- ↑ 68.0 68.1 68.2 68.3 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.
- ↑ 69.0 69.1 Groves FD, Travis LB, Devesa SS, Ries LA, Fraumeni JF (1998). "Waldenström's macroglobulinemia: incidence patterns in the United States, 1988-1994". Cancer. 82 (6): 1078–81. PMID 9506352.
- ↑ 70.0 70.1 70.2 70.3 Herrinton LJ, Weiss NS (1993). "Incidence of Waldenström's macroglobulinemia". Blood. 82 (10): 3148–50. PMID 8219203.
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- ↑ 72.0 72.1 72.2 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.
- ↑ 73.0 73.1 Waldenström's macroglobulinemia. American Cancer Society (2015)http://www.cancer.org/cancer/waldenstrommacroglobulinemia/detailedguide/waldenstrom-macroglobulinemia-risk-factors Accessed on November 6, 2015
- ↑ Giordano TP, Henderson L, Landgren O, Chiao EY, Kramer JR, El-Serag H; et al. (2007). "Risk of non-Hodgkin lymphoma and lymphoproliferative precursor diseases in US veterans with hepatitis C virus". JAMA. 297 (18): 2010–7. doi:10.1001/jama.297.18.2010. PMID 17488966.
- ↑ Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS (2006). "Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001". Blood. 107 (1): 265–76. doi:10.1182/blood-2005-06-2508. PMC 1895348. PMID 16150940.
- ↑ Pophali, Priyanka Avinash; Bartley, Adam C.; Kapoor, Prashant; Gonsalves, Wilson I.; Ashrani, Aneel A.; Marshall, Ariela L.; Siddiqui, Mustaqeem Ahmad; Go, Ronald S. (2017). "Smoldering Waldenström's macroglobulinemia (SWM): Analysis from the National Cancer Database (NCDB)". Journal of Clinical Oncology. 35 (15_suppl): 1573–1573. doi:10.1200/JCO.2017.35.15_suppl.1573. ISSN 0732-183X.
- ↑ McMaster ML, Csako G, Giambarresi TR, Vasquez L, Berg M, Saddlemire S; et al. (2007). "Long-term evaluation of three multiple-case Waldenstrom macroglobulinemia families". Clin Cancer Res. 13 (17): 5063–9. doi:10.1158/1078-0432.CCR-07-0299. PMID 17785558.