Lymphoplasmacytic lymphoma diagnostic study of choice: Difference between revisions

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__NOTOC__
__NOTOC__
{{Lymphoplasmacytic lymphoma}}
{{Lymphoplasmacytic lymphoma}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}}{{S.M.}}
== Overview ==
== Overview ==
The [[diagnosis]] of [[lymphoplasmacytic lymphoma]]  is [[Based on Symptoms|based]] on [[bone marrow aspiration]] and [[biopsy]] and [[serum]] [[protein]] [[analysis]] [[Study design|studies]] such as [[immunohistochemistry|immunohistochemistry,]]  [[flow cytometry]] and [[cytogenetics]] to [[Differentiate|distinguish]] [[Lymphoplasmacytic lymphoma|LPL]] from other types of [[B-cell]] [[lymphomas]]. [[CSF]] [[flow cytometry]], [[protein electrophoresis]] and [[immunofixation]] is [[done]] for the [[diagnosis]] of [[Bing-Neel syndrome]] (a late, but severe, [[rare]] [[Complication (medicine)|complication]]).


== Diagnostic Study of Choice ==
== Diagnostic Study of Choice ==
*There is no single [[diagnostic study of choice]] for the [[diagnosis]] of [[lymphoplasmacytic lymphoma]] (LPL), but '''[[bone marrow aspiration]]''' and '''[[biopsy]]''' is considered to be [[Mandatory labelling|mandatory]] for the [[Assessment and Plan|assessment]] of [[patients]] with LPL and further [[Support|supported]] by [[monoclonal]] [[protein]]/[[Immunophenotyping|immunophenotypic studies]] such as [[immunohistochemistry]], [[flow cytometry]] and [[cytogenetics]] to distinguish LPL from other types of [[B-cell]] [[lymphomas]].<ref name="pmid15735132">{{cite journal| author=Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP| title=Diagnosis and management of Waldenstrom's macroglobulinemia. | journal=J Clin Oncol | year= 2005 | volume= 23 | issue= 7 | pages= 1564-77 | pmid=15735132 | doi=10.1200/JCO.2005.03.144 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15735132  }} </ref><ref name="pmid26980727">{{cite journal| author=Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R et al.| title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms. | journal=Blood | year= 2016 | volume= 127 | issue= 20 | pages= 2375-90 | pmid=26980727 | doi=10.1182/blood-2016-01-643569 | pmc=4874220 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26980727  }} </ref>
*Not all the [[diagnostic]] [[Test|tests]] mentioned are [[Performance status|performed]] in a [[patient]] with [[lymphoplasmacytic lymphoma]]. A [[Doctor of Medicine|doctor]] takes into account the following factors before choosing [[diagnostic]] [[Test|tests]] in a particular [[patient]]:
** Suspected type of [[cancer]]
** [[Signs and Symptoms|Signs]] and [[symptoms]]
** [[Age]]
** [[Medical condition]] of the [[patient]]
** [[Result|Results]] of earlier [[medical]] [[Test|tests]]


=== Study of choice ===
===Diagnostic Criteria:===
[Name of the investigation] is the gold standard test for the diagnosis of [disease name].
====Diagnostic criteria presented in second International Workshop, Greece, 2002====


OR
* In September 26-30, 2002, in Athens, Greece,the Second International Workshop was held in which a [[Diagnosis|diagnostic]] criteria for [[Waldenström's macroglobulinemia|Waldenstrom's Macroglobulinemia]] was [[Proposition|proposed]]. According to this [[criteria]], the following findings on [[Performance status|performing]] [[Bone marrow examination|bone marrow biopsy]] and [[serum]] [[protein]] analysis are [[Confirmatory factor analysis|confirmatory]] of [[Waldenström macroglobulinemia]] and [[Exclusion criteria|exclude]] other small [[B cell]] [[lymphoid]] [[neoplasms]] with plasmacytic [[differentiation]]:<ref name="pmid15735132" /><ref name="pmid12720118">{{cite journal| author=Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML et al.| title=Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. | journal=Semin Oncol | year= 2003 | volume= 30 | issue= 2 | pages= 110-5 | pmid=12720118 | doi=10.1053/sonc.2003.50082 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12720118  }}</ref>


The following result of [gold standard test] is confirmatory of [disease name]:
{| class="wikitable"
* [Result 1]
|+
* [Result 2]
! colspan="3" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Diagnostic criteria presented in second International Workshop, Greece, 2002}}
|-
| colspan="2" |'''1:'''[[Presenting symptom|Presence]] of [[Immunoglobulin M|IgM]] [[monoclonal gammopathy]] of any [[concentration]] on [[Serum protein electrophoresis|serum protein analysis]]
|'''[[Necessary and sufficient|Necessary]]''' [[criteria]]
|-
| rowspan="3" |'''2:'''A [[bone marrow]] [[biopsy]] demonstrating more than 10% [[Infiltration (medical)|infiltration]] by small [[lymphocytes]], [[plasmacytoid]] [[lymphocytes]], and [[plasma cells]], (with [[variable]] [[Number|numbers]] of admixed [[Immunoblast|immunoblasts]]), with an intertrabecular [[pattern]] consistent with [[lymphoplasmacytic lymphoma]]
|[[Proliferation]] centers ([[pathognomonic]] of [[CLL]]/[[SLL]]) and [[Paleness|paler]]-[[Appearance|appearing]] [[marginal zone]] type [[differentiation]] (seen in [[marginal zone lymphoma]]) are absent
| rowspan="3" |'''[[Necessary and sufficient|Necessary]]''' [[criteria]]
|-
|[[IgM]] [[concentration]] [[Wide and fast|widely]] [[Variable|varies]] in [[Waldenström's macroglobulinemia|WM]], and it is not [[Possibility theory|possible]] to define a [[concentration]] that reliably distinguishes [[Waldenström's macroglobulinemia|WM]] from other [[lymphoproliferative disorders]]. Hence, a [[diagnosis]] of [[Waldenström's macroglobulinemia|WM]] can be made irrespective of [[IgM]] [[concentration]] if there is an [[evidence]] of [[bone marrow]] [[Infiltration (medical)|infiltration]] by lymphoplasmacytoid [[lymphoma]] as defined by the Revised European-American [[Lymphoma]] [[classification]] and [[WHO]] [[criteria]]. This is a [[tumor]] of small [[lymphocytes]] showing [[evidence]] of [[plasmacytoid]] or [[plasma cell]] [[differentiation]].
|-
|A [[Recent changes|recent]] [[Study design|study]] found that, in 39% of [[patients]], the [[bone marrow]] [[aspirate]] contained a [[spectrum]] of small [[lymphocytes]], [[plasmacytoid]] [[lymphocytes]], and [[plasma cells]]; in 39% of [[patients]], there was a [[Predominance diagram|predominance]] of small [[lymphocytes]] with [[Fewmets|fewer]] [[plasmacytoid]] [[lymphocytes]] or [[plasma cells]], and 22% of the [[patients]] contained a [[mixture]] of small [[lymphocytes]] and [[plasma cells]], with [[rare]] [[plasmacytoid]] [[Cells (biology)|cells]]. [[Mast cells]] were increased in 26% of [[patients]].
|-
| colspan="2" |'''3:'''Intertrabecular [[pattern]] of [[bone marrow]] [[Infiltration (medical)|infiltration]]
|'''[[Support|Supportive]]''' [[criteria]]
|-
| colspan="2" |'''4:'''[[Immunophenotype]] of the [[Lymphoplasmacytic lymphoma|lymphoplasmacytic]] [[Infiltration (medical)|infiltrate]] consistent with [[Waldenström's macroglobulinemia|Waldenstrom's macroglobulinemia]]. This includes: [[IgM]]+, [[CD5]]-, [[CD10]]-, [[CD11c]]-, [[CD19]]+, [[CD20]]+, [[CD22]]+, [[CD23]]-, [[CD25]]+, [[CD27]]+, [[FMC7]]+, [[CD103]]- and [[CD138]]+
|'''[[Support|Supportive]]''' [[criteria]]
|}
====mSMART guidelines for diagnosis of Waldenstrom macroglobulinemia and associated disorders====


OR
* [[Mayo Clinic|Mayo]] [[Stratification]] of [[Macroglobulinemia]] and [[RiskMetrics|Risk]]-[[Adapted process|Adapted]] [[Therapy]] (mSMART) [[Medical guideline|Guidelines]] 2016 for [[diagnosis]] of [[Waldenstrom macroglobulinemia]] and [[Association (statistics)|associated]] [[disorders]] are as follows:<ref name="AnsellKyle2010">{{cite journal|last1=Ansell|first1=Stephen M.|last2=Kyle|first2=Robert A.|last3=Reeder|first3=Craig B.|last4=Fonseca|first4=Rafael|last5=Mikhael|first5=Joseph R.|last6=Morice|first6=William G.|last7=Bergsagel|first7=P. Leif|last8=Buadi|first8=Francis K.|last9=Colgan|first9=Joseph P.|last10=Dingli|first10=David|last11=Dispenzieri|first11=Angela|last12=Greipp|first12=Philip R.|last13=Habermann|first13=Thomas M.|last14=Hayman|first14=Suzanne R.|last15=Inwards|first15=David J.|last16=Johnston|first16=Patrick B.|last17=Kumar|first17=Shaji K.|last18=Lacy|first18=Martha Q.|last19=Lust|first19=John A.|last20=Markovic|first20=Svetomir N.|last21=Micallef|first21=Ivana N.M.|last22=Nowakowski|first22=Grzegorz S.|last23=Porrata|first23=Luis F.|last24=Roy|first24=Vivek|last25=Russell|first25=Stephen J.|last26=Short|first26=Kristen E. Detweiler|last27=Stewart|first27=A. Keith|last28=Thompson|first28=Carrie A.|last29=Witzig|first29=Thomas E.|last30=Zeldenrust|first30=Steven R.|last31=Dalton|first31=Robert J.|last32=Rajkumar|first32=S. Vincent|last33=Gertz|first33=Morie A.|title=Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines|journal=Mayo Clinic Proceedings|volume=85|issue=9|year=2010|pages=824–833|issn=00256196|doi=10.4065/mcp.2010.0304}}</ref>
 
{| class="wikitable"
[Name of the investigation] must be performed when:
|+
* The patient presents with [symptom/sign 1], [symptom/sign 2], and [symptom/sign 3].
! colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|mSMART guidelines 2016 for diagnosis of Waldenstrom macroglobulinemia and associated disorders}}
* A [name of test] is positive for [sign 1], [sign 2], and [sign 3] in the patient.
|-
 
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Waldenström macroglobulinemia]]
OR
|[[IgM]] [[monoclonal gammopathy]] (regardless of the [[Size consistency|size]] of the [[M protein]]) with >10% [[bone marrow]] lymphoplasmacytic [[Infiltration (medical)|infiltration]] (usually intertrabecular) by small [[lymphocytes]] that exhibit plasmacytoid or [[plasma cell]] [[differentiation]] and a [[Typical set|typical]] [[immunophenotype]] ([[Surface chemistry|surface]] [[IgM]]+, [[CD5]]–, [[CD10]]–, [[CD19]]+, [[CD20]]+, [[CD23]]–) that satisfactorily [[Exclusion criteria|excludes]] other [[lymphoproliferative disorders]], including [[chronic lymphocytic leukemia]] and [[mantle cell lymphoma]]
 
[Name of the investigation] is the gold standard test for the diagnosis of [disease name].
 
OR
 
The diagnostic study of choice for [disease name] is [name of the investigation].
 
OR
 
There is no single diagnostic study of choice for the diagnosis of [disease name].
 
OR
 
There is no single diagnostic study of choice for the diagnosis of [disease name], but [disease name] can be diagnosed based on [name of the investigation 1] and [name of the investigation 2].
 
OR
 
[Disease name] is primarily diagnosed based on the clinical presentation.
 
OR
 
Investigations:
* Among the patients who present with clinical signs of [disease name], the [investigation name] is the most specific test for the diagnosis.
* Among the patients who present with clinical signs of [disease name], the [investigation name] is the most sensitive test for diagnosis.
* Among the patients who present with clinical signs of [disease name], the [investigation name] is the most efficient test for diagnosis.
 
==== The comparison of various diagnostic studies for [disease name] ====
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" | Test
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sensitivity
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Specificity
|-
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 1
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[IgM]] [[MGUS]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|[[Serum]] [[IgM monoclonal protein]] level <3 g/dL, [[bone marrow]] lymphoplasmacytic [[Infiltration (medical)|infiltration]] <10%, and no [[evidence]] of [[anemia]], constitutional [[symptoms]], [[hyperviscosity]], [[lymphadenopathy]], or [[hepatosplenomegaly]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|-
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 2
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Smoldering [[Waldenström macroglobulinemia]] (indolent /[[asymptomatic]] [[Waldenström macroglobulinemia]])
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|[[Serum]] [[IgM monoclonal protein]] level ≥3 g/dL and/or [[bone marrow]] lymphoplasmacytic [[Infiltration (medical)|infiltration]] ≥10% and no [[evidence]] of [[End organ damage|end-organ damage]], such as [[anemia]], constitutional [[symptoms]], [[hyperviscosity]], [[lymphadenopathy]], or [[hepatosplenomegaly]], that can be [[Attribution (psychology)|attributed]] to a lymphoplasmacytic [[Proliferation|proliferative]] [[Disorder (medicine)|disorder]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|}
|}
<small> [Name of test with higher sensitivity and specificity] is the preferred investigation based on the sensitivity and specificity</small>


===== Diagnostic results =====
====Definitive Diagnostic Tests====
The following finding(s) on performing [investigation name] is(are) confirmatory for [disease name]:
*[[Genetic]] [[Testing]]:
* [Finding 1]
**ARIDA
* [Finding 2]
**[[Immunoglobulin|IG]] [[gene]] [[rearrangement]]
**[[CXCR4]] 5338X
**[[MYD88]] L265P
*[[Immunophenotyping]]
*[[Serum]] [[paraprotein]]


===== Sequence of Diagnostic Studies =====
===Bone Marrow Aspirate:===
The [name of investigation] must be performed when:
* A [[Bone marrow aspiration|bone marrow aspirate]] is [[Essential medicines|essential]] in the [[diagnosis]] of [[lymphoplasmacytic lymphoma]]
* The patient presented with symptoms/signs 1, 2, and 3 as the first step of diagnosis.
* Findings [[Suggestion|suggestive]] of [[lymphoplasmacytic lymphoma]] include:<ref name="pmid18555588">{{cite journal| author=Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J et al.| title=Waldenstrom macroglobulinemia. | journal=Cancer Lett | year= 2008 | volume= 270 | issue= 1 | pages= 95-107 | pmid=18555588 | doi=10.1016/j.canlet.2008.04.040 | pmc=3133633 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18555588  }} </ref>
* A positive [test] is detected in the patient, to confirm the diagnosis.
** Hypercellular [[bone marrow]] [[aspirate]]
**[[Lymphoplasmacytic lymphoma|Lymphoplasmacytic]] [[Infiltration (medical)|infiltrate]] with [[Characteristic function (probability theory)|characteristic]] [[immunophenotype]]


OR
{|
|
[[File:Wm bm aspirate.png|thumb|250px|none| [[Bone marrow]] [[aspirate]]. [[Lymphocytes]] with [[Lymphoplasmacytic lymphoma|lymphoplasmacytic]] appearance (arrows).[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3894394_br-48-300-g001&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=91 Source: D'Angelo G. et al, Laboratorio di Chimica-Clinica, Ematologia e Microbiologia (Ematologia/Coagulazione), Azienda Ospedaliera "S. Antonio Abate" di Gallarate, Varese, Italy.]]]
|}


The various investigations must be performed in the following order:
=== Bone Marrow Biopsy: ===
* [Initial investigation]
*A [[bone marrow biopsy]] may be [[Help desk|helpful]] in the [[diagnosis]] of [[lymphoplasmacytic lymphoma]] <ref name="pmid18555588">{{cite journal| author=Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J et al.| title=Waldenstrom macroglobulinemia. | journal=Cancer Lett | year= 2008 | volume= 270 | issue= 1 | pages= 95-107 | pmid=18555588 | doi=10.1016/j.canlet.2008.04.040 | pmc=3133633 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18555588  }} </ref>
* [2nd investigation]
*Findings on the [[biopsy]] [[Suggestion|suggestive]] of [[lymphoplasmacytic lymphoma]] include:<ref name="pmid18555588">{{cite journal| author=Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J et al.| title=Waldenstrom macroglobulinemia. | journal=Cancer Lett | year= 2008 | volume= 270 | issue= 1 | pages= 95-107 | pmid=18555588 | doi=10.1016/j.canlet.2008.04.040 | pmc=3133633 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18555588  }} </ref>
**Hypercellular and [[Infiltration (medical)|infiltrated]] with [[lymphoid]] and [[Plasmacytoid|plasmacytoid cells]]
**Dutcher [[Body|bodies]] ([[PAS stain|PAS]] positive intra-nuclear [[vacuoles]] containing [[IgM]] [[monoclonal]] [[protein]])
***[[Characteristic function (probability theory)|Characteristic]] [[Features (pattern recognition)|feature]] of [[lymphoplasmacytic lymphoma]]
*Three [[Pattern|patterns]] of [[Bone marrow|marrow]] involvement are described, as follows:
**Lymphoplasmacytoid [[Cells (biology)|cells]] (lymphoplasmacytic and small [[lymphocytes]]) in a [[nodular]] [[pattern]]
**[[Lymphoplasmacytic lymphoma|Lymphoplasmacytic]] [[Cells (biology)|cells]] (small [[lymphocytes]], mature [[plasma cells]], [[mast cells]]) in an [[interstitial]]/[[nodular]] [[pattern]]
**A polymorphous [[Infiltration (medical)|infiltrate]] (small [[lymphocytes]], [[plasma cells]], [[plasmacytoid]] [[Cells (biology)|cells]], [[Immunoblast|immunoblasts]] with [[mitotic]] figures)


=== Name of Diagnostic Criteria ===
===Electrophoresis and Immunofixation===
*[[Serum protein electrophoresis]] is important for the [[diagnosis]] of [[lymphoplasmacytic lymphoma]]
*Findings on an [[electrophoresis]] [[diagnostic]] of [[lymphoplasmacytic lymphoma]] include:<ref name="pmid1872571">{{cite journal| author=Riches PG, Sheldon J, Smith AM, Hobbs JR| title=Overestimation of monoclonal immunoglobulin by immunochemical methods. | journal=Ann Clin Biochem | year= 1991 | volume= 28 ( Pt 3) | issue=  | pages= 253-9 | pmid=1872571 | doi=10.1177/000456329102800310 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1872571  }} </ref>
**Sharp, narrow [[Spike sorting|spike]] of [[monoclonal]] [[IgM]] [[protein]]
**[[Dense]] band of [[monoclonal]] [[IgM]] [[protein]]
**The [[paraprotein]] can be of any [[Size consistency|size]]
*[[Serum]] [[immunofixation]] is important for the [[diagnosis]] of [[lymphoplasmacytic lymphoma]]. It helps in [[Confirmatory factor analysis|confirming]] the [[Presenting symptom|presence]] of a [[Monoclonal|monoclonal protein]], in [[Addition reaction|addition]] to determining its type<ref name="pmid1872571">{{cite journal| author=Riches PG, Sheldon J, Smith AM, Hobbs JR| title=Overestimation of monoclonal immunoglobulin by immunochemical methods. | journal=Ann Clin Biochem | year= 1991 | volume= 28 ( Pt 3) | issue=  | pages= 253-9 | pmid=1872571 | doi=10.1177/000456329102800310 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1872571  }} </ref>


'''It is recommended that you include the criteria in a table. Make sure you always cite the source of the content and whether the table has been adapted from another source.'''
{|
|
[[File:Serum immunofixation electrophoresis.png|thumb|250px|none| Serum [[immunofixation]] electrophoresis. (A) There is a slightly dense band with [[IgM]], kappa [[antisera]], suggestive of [[Monoclonal gammopathy|monoclonal]] gammopathy (B) After the treatment, a dense band with [[IgM]] was disappeared.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3102879_jkms-26-824-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=41 Source: Kim YL. et al, Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea.]]]
|}


[Disease name] is primarily diagnosed based on clinical presentation. There are no established criteria for the diagnosis of [disease name].
===CSF flow cytometry, protein electrophoresis and immunofixation for diagnosis of Bing-Neel syndrome:===
*For [[Diagnosis|diagnosing]] [[Bing-Neel syndrome]], after [[lumbar puncture]], [[CSF]] [[flow cytometry]] is [[done]] which shows a [[Lambda (anatomy)|lambda]] [[light chain]]-[[Restriction|restricted]] [[population]] of [[B-cells]] consistent with a [[CD5]]+ [[CD10]]+ [[B-cell lymphoma]]
*Furthermore, [[protein electrophoresis]] and [[immunofixation]] should be [[done]] for the [[Detection theory|detection]] and [[classification]] of a [[monoclonal]] [[protein]] as well as [[molecular]] [[diagnostic testing]] for [[immunoglobulin]] [[gene]] [[rearrangement]] and [[mutated]] [[MYD88]]<ref name="pmid30228918">{{cite journal| author=O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A| title=A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib. | journal=Case Rep Hematol | year= 2018 | volume= 2018 | issue=  | pages= 8573105 | pmid=30228918 | doi=10.1155/2018/8573105 | pmc=6136466 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30228918  }} </ref><ref name="pmid27758817">{{cite journal| author=Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ et al.| title=Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome. | journal=Haematologica | year= 2017 | volume= 102 | issue= 1 | pages= 43-51 | pmid=27758817 | doi=10.3324/haematol.2016.147728 | pmc=5210231 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27758817  }} </ref><ref name="pmid30279255">{{cite journal| author=Tallant A, Selig D, Wanko SO, Roswarski J| title=First-line ibrutinib for Bing-Neel syndrome. | journal=BMJ Case Rep | year= 2018 | volume= 2018 | issue=  | pages=  | pmid=30279255 | doi=10.1136/bcr-2018-226102 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30279255  }} </ref>


OR
{|
 
|
There is no single diagnostic study of choice for [disease name], though [disease name] may be diagnosed based on [name of criteria] established by [...].
[[File:Brain biopsy gif.gif|thumb|250px|none| Stereotactic brain [[biopsy]] showing diffuse infiltration of atypical plasmacytoid [[lymphocytes]] into the dural [[fibrous tissue]] (A) [[Hematoxylin and eosin stain|Hematoxylin]] & eosin (original magnification ×200); (B) Positive [[immunohistochemical]] staining for [[CD20]] (original magnification ×40). [https://openi.nlm.nih.gov/detailedresult.php?img=PMC2694623_jkms-22-1079-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=29 Source: Kim HD. et al, Department of Internal Medicine, Yeoungnam University College of Medicine, Daegu, Korea.]]]
 
|
OR
[[File:Csf plasmacytoid cells.png|thumb|250px|none| Plasmacytoid cells found on cytospin of the cerebrospinal fluid confirming cellular infiltration of the central nervous system.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC4837273_CRIHEM2016-3931709.004&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=71 Source: Halperin D. et al, Whipps Cross Hospital, London E11 1NR, UK.]]]
 
|}
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 includes [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
[Disease name] may be diagnosed at any time if one or more of the following criteria are met:  
* Criteria 1
* Criteria 2
* Criteria 3
 
OR
 
'''IF there are clear, established diagnostic criteria'''
 
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
 
'''IF there are no established diagnostic criteria'''
 
There are no established criteria for the diagnosis of [disease name].


==References==
==References==
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[[Category:Disease]]
[[Category:Blood]]
[[Category:Hematology]]

Latest revision as of 14:44, 29 October 2019

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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]

Overview

The diagnosis of lymphoplasmacytic lymphoma is based on bone marrow aspiration and biopsy and serum protein analysis studies such as immunohistochemistry, flow cytometry and cytogenetics to distinguish LPL from other types of B-cell lymphomas. CSF flow cytometry, protein electrophoresis and immunofixation is done for the diagnosis of Bing-Neel syndrome (a late, but severe, rare complication).

Diagnostic Study of Choice

Diagnostic Criteria:

Diagnostic criteria presented in second International Workshop, Greece, 2002

Diagnostic criteria presented in second International Workshop, Greece, 2002
1:Presence of IgM monoclonal gammopathy of any concentration on serum protein analysis Necessary criteria
2:A bone marrow biopsy demonstrating more than 10% infiltration by small lymphocytes, plasmacytoid lymphocytes, and plasma cells, (with variable numbers of admixed immunoblasts), with an intertrabecular pattern consistent with lymphoplasmacytic lymphoma Proliferation centers (pathognomonic of CLL/SLL) and paler-appearing marginal zone type differentiation (seen in marginal zone lymphoma) are absent Necessary criteria
IgM concentration widely varies in WM, and it is not possible to define a concentration that reliably distinguishes WM from other lymphoproliferative disorders. Hence, a diagnosis of WM can be made irrespective of IgM concentration if there is an evidence of bone marrow infiltration by lymphoplasmacytoid lymphoma as defined by the Revised European-American Lymphoma classification and WHO criteria. This is a tumor of small lymphocytes showing evidence of plasmacytoid or plasma cell differentiation.
A recent study found that, in 39% of patients, the bone marrow aspirate contained a spectrum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells; in 39% of patients, there was a predominance of small lymphocytes with fewer plasmacytoid lymphocytes or plasma cells, and 22% of the patients contained a mixture of small lymphocytes and plasma cells, with rare plasmacytoid cells. Mast cells were increased in 26% of patients.
3:Intertrabecular pattern of bone marrow infiltration Supportive criteria
4:Immunophenotype of the lymphoplasmacytic infiltrate consistent with Waldenstrom's macroglobulinemia. This includes: IgM+, CD5-, CD10-, CD11c-, CD19+, CD20+, CD22+, CD23-, CD25+, CD27+, FMC7+, CD103- and CD138+ Supportive criteria

mSMART guidelines for diagnosis of Waldenstrom macroglobulinemia and associated disorders

mSMART guidelines 2016 for diagnosis of Waldenstrom macroglobulinemia and associated disorders
Waldenström macroglobulinemia IgM monoclonal gammopathy (regardless of the size of the M protein) with >10% bone marrow lymphoplasmacytic infiltration (usually intertrabecular) by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation and a typical immunophenotype (surface IgM+, CD5–, CD10–, CD19+, CD20+, CD23–) that satisfactorily excludes other lymphoproliferative disorders, including chronic lymphocytic leukemia and mantle cell lymphoma
IgM MGUS Serum IgM monoclonal protein level <3 g/dL, bone marrow lymphoplasmacytic infiltration <10%, and no evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly
Smoldering Waldenström macroglobulinemia (indolent /asymptomatic Waldenström macroglobulinemia) Serum IgM monoclonal protein level ≥3 g/dL and/or bone marrow lymphoplasmacytic infiltration ≥10% and no evidence of end-organ damage, such as anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly, that can be attributed to a lymphoplasmacytic proliferative disorder

Definitive Diagnostic Tests

Bone Marrow Aspirate:

Bone marrow aspirate. Lymphocytes with lymphoplasmacytic appearance (arrows).[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3894394_br-48-300-g001&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=91 Source: D'Angelo G. et al, Laboratorio di Chimica-Clinica, Ematologia e Microbiologia (Ematologia/Coagulazione), Azienda Ospedaliera "S. Antonio Abate" di Gallarate, Varese, Italy.
]

Bone Marrow Biopsy:

Electrophoresis and Immunofixation

Serum immunofixation electrophoresis. (A) There is a slightly dense band with IgM, kappa antisera, suggestive of monoclonal gammopathy (B) After the treatment, a dense band with IgM was disappeared.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3102879_jkms-26-824-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=41 Source: Kim YL. et al, Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea.
]

CSF flow cytometry, protein electrophoresis and immunofixation for diagnosis of Bing-Neel syndrome:

Stereotactic brain biopsy showing diffuse infiltration of atypical plasmacytoid lymphocytes into the dural fibrous tissue (A) Hematoxylin & eosin (original magnification ×200); (B) Positive immunohistochemical staining for CD20 (original magnification ×40). [https://openi.nlm.nih.gov/detailedresult.php?img=PMC2694623_jkms-22-1079-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=29 Source: Kim HD. et al, Department of Internal Medicine, Yeoungnam University College of Medicine, Daegu, Korea.
]
Plasmacytoid cells found on cytospin of the cerebrospinal fluid confirming cellular infiltration of the central nervous system.Source: Halperin D. et al, Whipps Cross Hospital, London E11 1NR, UK.

References

  1. ↑ 1.0 1.1 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.
  2. ↑ Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R; et al. (2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 5.0 5.1 5.2 Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J; et al. (2008). "Waldenstrom macroglobulinemia". Cancer Lett. 270 (1): 95–107. doi:10.1016/j.canlet.2008.04.040. PMC 3133633. PMID 18555588.
  6. ↑ 6.0 6.1 Riches PG, Sheldon J, Smith AM, Hobbs JR (1991). "Overestimation of monoclonal immunoglobulin by immunochemical methods". Ann Clin Biochem. 28 ( Pt 3): 253–9. doi:10.1177/000456329102800310. PMID 1872571.
  7. ↑ O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A (2018). "A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib". Case Rep Hematol. 2018: 8573105. doi:10.1155/2018/8573105. PMC 6136466. PMID 30228918.
  8. ↑ Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ; et al. (2017). "Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome". Haematologica. 102 (1): 43–51. doi:10.3324/haematol.2016.147728. PMC 5210231. PMID 27758817.
  9. ↑ Tallant A, Selig D, Wanko SO, Roswarski J (2018). "First-line ibrutinib for Bing-Neel syndrome". BMJ Case Rep. 2018. doi:10.1136/bcr-2018-226102. PMID 30279255.

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