Lymphoplasmacytic lymphoma differential diagnosis: Difference between revisions
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'''For the WikiDoc page for this topic, click [[Lymphoplasmacytic lymphoma|here]]''' | '''For the WikiDoc page for this topic, click [[Lymphoplasmacytic lymphoma|here]]''' | ||
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==Overview== | ==Overview== | ||
Lymphoplasmacytic lymphoma must be differentiated from [[multiple myeloma]], [[chronic lymphocytic leukemia]]/[[small lymphocytic lymphoma]], [[b-cell prolymphocytic leukemia]], [[follicular lymphoma]], [[mantle cell lymphoma]], and [[marginal zone lymphoma]]. | [[Lymphoplasmacytic lymphoma]] must be differentiated from [[multiple myeloma]], [[chronic lymphocytic leukemia]]/[[small lymphocytic lymphoma]], [[b-cell prolymphocytic leukemia]], [[follicular lymphoma]], [[mantle cell lymphoma]], and [[marginal zone lymphoma]]. | ||
==Differentiating Lymphoplasmacytic lymphoma from other Diseases== | ==Differentiating Lymphoplasmacytic lymphoma from other Diseases== | ||
[[Lymphoplasmacytic lymphoma]] must be differentiated from other B cell lymphoid neoplasms including: | [[Lymphoplasmacytic lymphoma]] must be differentiated from other [[B cell]] [[lymphoid]] [[neoplasms]] including: | ||
*[[Chronic lymphocytic leukemia]]/[[small lymphocytic lymphoma]]: | *[[Chronic lymphocytic leukemia]]/[[small lymphocytic lymphoma]]: | ||
:*Always express CD5 | :*Always express [[CD5]]. | ||
:*Usually CD23 positive<ref name="CLL">{{cite journal |vauthors=Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating MJ, Montserrat E, Rai KR, Kipps TJ |title=Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines |journal=Blood |volume=111 |issue=12 |pages=5446–56 |year=2008 |pmid=18216293 |pmc=2972576 |doi=10.1182/blood-2007-06-093906 |url=}}</ref> | :*Usually [[CD23]] positive.<ref name="CLL">{{cite journal |vauthors=Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating MJ, Montserrat E, Rai KR, Kipps TJ |title=Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines |journal=Blood |volume=111 |issue=12 |pages=5446–56 |year=2008 |pmid=18216293 |pmc=2972576 |doi=10.1182/blood-2007-06-093906 |url=}}</ref> | ||
*[[B-cell prolymphocytic leukemia]]: | *[[B-cell prolymphocytic leukemia]]: | ||
:*Express bright surface [[Immunoglobulin M|IgM]], [[CD20]] and other B-cell antigens ([[CD19]], [[CD22]], [[CD79a]], [[FMC7]])<ref name=",m">{{cite journal |vauthors=Del Giudice I, Davis Z, Matutes E, Osuji N, Parry-Jones N, Morilla A, Brito-Babapulle V, Oscier D, Catovsky D |title=IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL) |journal=Leukemia |volume=20 |issue=7 |pages=1231–7 |year=2006 |pmid=16642047 |doi=10.1038/sj.leu.2404238 |url=}}</ref><ref name="njl">{{cite journal |vauthors=Ravandi F, O'Brien S |title=Chronic lymphoid leukemias other than chronic lymphocytic leukemia: diagnosis and treatment |journal=Mayo Clin. Proc. |volume=80 |issue=12 |pages=1660–74 |year=2005 |pmid=16342661 |doi=10.4065/80.12.1660 |url=}}</ref> | :*Express bright surface [[Immunoglobulin M|IgM]], [[CD20]] and other [[B-cell]] [[antigens]] ([[CD19]], [[CD22]], [[CD79a]], [[FMC7]]).<ref name=",m">{{cite journal |vauthors=Del Giudice I, Davis Z, Matutes E, Osuji N, Parry-Jones N, Morilla A, Brito-Babapulle V, Oscier D, Catovsky D |title=IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL) |journal=Leukemia |volume=20 |issue=7 |pages=1231–7 |year=2006 |pmid=16642047 |doi=10.1038/sj.leu.2404238 |url=}}</ref><ref name="njl">{{cite journal |vauthors=Ravandi F, O'Brien S |title=Chronic lymphoid leukemias other than chronic lymphocytic leukemia: diagnosis and treatment |journal=Mayo Clin. Proc. |volume=80 |issue=12 |pages=1660–74 |year=2005 |pmid=16342661 |doi=10.4065/80.12.1660 |url=}}</ref> | ||
*[[Follicular lymphoma]]: | *[[Follicular lymphoma]]: | ||
:*Express [[CD10]], [[HLA-DR]], pan B-cell antigens (CD19, CD20, CD79a), CD21, and surface IgM, [[Immunoglobulin G|IgG]], or [[IgA]] | :*Express [[CD10]], [[HLA-DR]], pan [[B-cell]] [[antigens]] ([[CD19]], [[CD20]], [[CD79a]]), [[CD21]], and surface [[IgM]], [[Immunoglobulin G|IgG]], or [[IgA]]. | ||
:*Rearrangement of Bcl-2<ref name="FL">{{cite journal |vauthors=Karube K, Guo Y, Suzumiya J, Sugita Y, Nomura Y, Yamamoto K, Shimizu K, Yoshida S, Komatani H, Takeshita M, Kikuchi M, Nakamura N, Takasu O, Arakawa F, Tagawa H, Seto M, Ohshima K |title=CD10-MUM1+ follicular lymphoma lacks BCL2 gene translocation and shows characteristic biologic and clinical features |journal=Blood |volume=109 |issue=7 |pages=3076–9 |year=2007 |pmid=17138820 |doi=10.1182/blood-2006-09-045989 |url=}}</ref><ref name="FL1">{{cite journal |vauthors=Anderson KC, Bates MP, Slaughenhoupt BL, Pinkus GS, Schlossman SF, Nadler LM |title=Expression of human B cell-associated antigens on leukemias and lymphomas: a model of human B cell differentiation |journal=Blood |volume=63 |issue=6 |pages=1424–33 |year=1984 |pmid=6609729 |doi= |url=}} | :*Rearrangement of [[Bcl-2|Bcl-2.]]<ref name="FL">{{cite journal |vauthors=Karube K, Guo Y, Suzumiya J, Sugita Y, Nomura Y, Yamamoto K, Shimizu K, Yoshida S, Komatani H, Takeshita M, Kikuchi M, Nakamura N, Takasu O, Arakawa F, Tagawa H, Seto M, Ohshima K |title=CD10-MUM1+ follicular lymphoma lacks BCL2 gene translocation and shows characteristic biologic and clinical features |journal=Blood |volume=109 |issue=7 |pages=3076–9 |year=2007 |pmid=17138820 |doi=10.1182/blood-2006-09-045989 |url=}}</ref><ref name="FL1">{{cite journal |vauthors=Anderson KC, Bates MP, Slaughenhoupt BL, Pinkus GS, Schlossman SF, Nadler LM |title=Expression of human B cell-associated antigens on leukemias and lymphomas: a model of human B cell differentiation |journal=Blood |volume=63 |issue=6 |pages=1424–33 |year=1984 |pmid=6609729 |doi= |url=}} | ||
:*Bone marrow infiltration of small, cleaved cells that are usually paratrabecular | :*Bone marrow infiltration of small, cleaved cells that are usually paratrabecular | ||
</ref> | </ref> | ||
*[[Multiple myeloma]]: | *[[Multiple myeloma]]: | ||
:*Express CD138, CD38, CD79a, VS38c and CD56 (70%) | :*Express [[CD138]], [[CD38]], [[CD79a]], VS38c and [[CD56]] (70%) | ||
:*Presence of plasmacytic cell infiltration of bone marrow, osteolytic lesions, and [[renal insufficiency]] | :*Presence of plasmacytic cell infiltration of [[bone marrow]], osteolytic lesions, and [[renal insufficiency]] | ||
:*Translocation involving chromosome 11 (t11;14)<ref name="UTD">{{cite journal |vauthors=Pangalis GA, Kyrtsonis MC, Kontopidou FN, Vassilakopoulos TP, Siakantaris MP, Dimopoulou MN, Kittas C, Angelopoulou MK |title=Differential diagnosis of Waldenstrom's macroglobulinemia from other low-grade B-cell lymphoproliferative disorders |journal=Semin. Oncol. |volume=30 |issue=2 |pages=201–5 |year=2003 |pmid=12720136 |doi=10.1053/sonc.2003.50046 |url=}}</ref> | :*[[Translocation]] involving [[chromosome 11]] (t11;14)<ref name="UTD">{{cite journal |vauthors=Pangalis GA, Kyrtsonis MC, Kontopidou FN, Vassilakopoulos TP, Siakantaris MP, Dimopoulou MN, Kittas C, Angelopoulou MK |title=Differential diagnosis of Waldenstrom's macroglobulinemia from other low-grade B-cell lymphoproliferative disorders |journal=Semin. Oncol. |volume=30 |issue=2 |pages=201–5 |year=2003 |pmid=12720136 |doi=10.1053/sonc.2003.50046 |url=}}</ref> | ||
*[[Mantle cell lymphoma]]: | *[[Mantle cell lymphoma]]: | ||
:* Expresses CD5+ and CD23+ | :* Expresses [[CD5]]+ and [[CD23]]+ | ||
:* Expresses surface IgM, IgD, and cyclin D1 in majority of cases | :* Expresses surface [[IgM]], [[IgD]], and [[cyclin D1]] in majority of cases | ||
:*Infiltration of bone marrow by monomorphous small lymphoid cells with irregular nuclei<ref name="MCL">{{cite journal |vauthors=Dorfman DM, Pinkus GS |title=Distinction between small lymphocytic and mantle cell lymphoma by immunoreactivity for CD23 |journal=Mod. Pathol. |volume=7 |issue=3 |pages=326–31 |year=1994 |pmid=8058704 |doi= |url=}}</ref><ref name="MCL1">{{cite journal |vauthors=DiRaimondo F, Albitar M, Huh Y, O'Brien S, Montillo M, Tedeschi A, Kantarjian H, Lerner S, Giustolisi R, Keating M |title=The clinical and diagnostic relevance of CD23 expression in the chronic lymphoproliferative disease |journal=Cancer |volume=94 |issue=6 |pages=1721–30 |year=2002 |pmid=11920534 |doi= |url=}}</ref> | :*Infiltration of [[bone marrow]] by monomorphous small [[lymphoid]] cells with irregular [[nuclei]]<ref name="MCL">{{cite journal |vauthors=Dorfman DM, Pinkus GS |title=Distinction between small lymphocytic and mantle cell lymphoma by immunoreactivity for CD23 |journal=Mod. Pathol. |volume=7 |issue=3 |pages=326–31 |year=1994 |pmid=8058704 |doi= |url=}}</ref><ref name="MCL1">{{cite journal |vauthors=DiRaimondo F, Albitar M, Huh Y, O'Brien S, Montillo M, Tedeschi A, Kantarjian H, Lerner S, Giustolisi R, Keating M |title=The clinical and diagnostic relevance of CD23 expression in the chronic lymphoproliferative disease |journal=Cancer |volume=94 |issue=6 |pages=1721–30 |year=2002 |pmid=11920534 |doi= |url=}}</ref> | ||
*[[Marginal zone lymphoma]]: | *[[Marginal zone lymphoma]]: | ||
:*Expresses B cell markers CD19, CD20, and CD22 | :*Expresses [[B cell]] markers [[CD19]], [[CD20]], and [[CD22]]. | ||
:*Infiltrates the bone marrow with a characteristic intertrabecular and intrasinusoidal pattern | :*Infiltrates the [[bone marrow]] with a characteristic intertrabecular and intrasinusoidal pattern | ||
:*Most common cytogenetic abnormalities are loss of 7q (19%) along with +3q (19%) and +5q (10% )<ref name="add">{{cite journal |vauthors=Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD |title=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 |journal=J. Clin. Oncol. |volume=17 |issue=12 |pages=3835–49 |year=1999 |pmid=10577857 |doi= |url=}}</ref><ref name="asdf">{{cite journal |vauthors=Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC |title=A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group |journal=Blood |volume=84 |issue=5 |pages=1361–92 |year=1994 |pmid=8068936 |doi= |url=}}</ref> | :*Most common [[cytogenetic]] abnormalities are loss of 7q (19%) along with +3q (19%) and +5q (10% )<ref name="add">{{cite journal |vauthors=Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD |title=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 |journal=J. Clin. Oncol. |volume=17 |issue=12 |pages=3835–49 |year=1999 |pmid=10577857 |doi= |url=}}</ref><ref name="asdf">{{cite journal |vauthors=Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC |title=A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group |journal=Blood |volume=84 |issue=5 |pages=1361–92 |year=1994 |pmid=8068936 |doi= |url=}}</ref> | ||
{| class="wikitable" | {| class="wikitable" | ||
|+ '''Histopathology, immunophenotype, and genetic features of differential diagnosis of lymphoplasmacytic lymphoma''' | |+ '''[[Histopathology]], [[immunophenotype]], and [[genetic]] features of [[differential diagnosis]] of [[lymphoplasmacytic lymphoma]]''' | ||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Disease entity | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Disease entity | ||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Histopathology | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Histopathology | ||
| Line 46: | Line 46: | ||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Genetic or other features | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Genetic or other features | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''Lymphoplasmacytic lymphoma''' | | style="background:#DCDCDC;" align="center" + |'''[[Lymphoplasmacytic lymphoma]]''' | ||
| style="background:#F5F5F5;" align="center" + |≥10 percent infiltration by small lymphocytes, plasmacytoid lymphocytes, and plasma cells, with variable numbers of admixed immunoblasts. Has a characteristic (but not pathognomonic) hyperplasia of mast cells in marrow. Lymph nodes are usually diffusely effaced. Absence of proliferation centers and marginal zone type differentiation. | | style="background:#F5F5F5;" align="center" + |≥10 percent infiltration by small [[lymphocytes]], plasmacytoid [[lymphocytes]], and [[plasma cells]], with variable numbers of admixed immunoblasts. Has a characteristic (but not pathognomonic) [[hyperplasia]] of [[mast cells]] in marrow. [[Lymph nodes]] are usually diffusely effaced. Absence of [[proliferation]] centers and [[marginal zone]] type [[differentiation]]. | ||
| style="background:#F5F5F5;" align="center" + |Expression of pan B-cell antigens (CD19, CD20, CD22, CD79a), failure to express CD5 in mostly cases and variable expression of CD11c, CD43, CD25. Mostly cases have IgM expression with only fewer expressing IgG or IgA. There's no CD10 and cyclin D1 expression. | | style="background:#F5F5F5;" align="center" + |Expression of pan [[B-cell]] [[antigens]] ([[CD19]], [[CD20]], [[CD22]], [[CD79a]]), failure to express [[CD5]] in mostly cases and variable expression of [[CD11c]], [[CD43]], [[CD25]]. Mostly cases have [[IgM]] expression with only fewer expressing [[IgG]] or [[IgA]]. There's no [[CD10]] and [[cyclin D1]] [[expression]]. | ||
| style="background:#F5F5F5;" align="center" + |Majority have a monoclonal IgM paraprotein. No specific chromosomal abnormalities. | | style="background:#F5F5F5;" align="center" + |Majority have a monoclonal [[IgM]] [[paraprotein]]. No specific [[chromosomal abnormalities]]. | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''Chronic lymphocytic leukemia/small lymphocytic lymphoma''' | | style="background:#DCDCDC;" align="center" + |'''[[Chronic lymphocytic leukemia]]/[[small lymphocytic lymphoma]]''' | ||
| style="background:#F5F5F5;" align="center" + |"Typical" CLL/SLL cells are small mature appearing lymphocytes with a dense nucleus, partially aggregated chromatin, no discernible nucleoli, and a narrow border of clear to slightly basophilic cytoplasm. | | style="background:#F5F5F5;" align="center" + |"Typical" [[CLL]]/[[SLL]] cells are small mature appearing [[lymphocytes]] with a dense [[nucleus]], partially aggregated [[chromatin]], no discernible [[nucleoli]], and a narrow border of clear to slightly [[basophilic]] [[cytoplasm]]. | ||
| style="background:#F5F5F5;" align="center" + |Always express CD5, usually CD23 positive with a dim expression of CD20 and surface Ig. | | style="background:#F5F5F5;" align="center" + |Always express [[CD5]], usually [[CD23]] positive with a dim [[expression]] of [[CD20]] and surface Ig. | ||
| style="background:#F5F5F5;" align="center" + |Del13q, del 11q, del17p, trisomy 12 | | style="background:#F5F5F5;" align="center" + |Del13q, del 11q, del17p, [[trisomy]] 12 | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''B-cell prolymphocytic leukemia''' | | style="background:#DCDCDC;" align="center" + |'''[[B-cell prolymphocytic leukemia]]''' | ||
| style="background:#F5F5F5;" align="center" + |Prolymphocytes comprise >55 percent of the neoplastic cells. Bone marrow has interstitial pattern of infiltration. Lymph nodes may show vague nodularity, but proliferation centers are absent. | | style="background:#F5F5F5;" align="center" + |[[Prolymphocytes]] comprise >55 percent of the [[neoplastic]] cells. [[Bone marrow]] has [[interstitial]] pattern of [[Infiltration (medical)|infiltration]]. [[Lymph nodes]] may show vague nodularity, but [[proliferation]] centers are absent. | ||
| style="background:#F5F5F5;" align="center" + |Express bright surface IgM +/- IgD and bright CD20 as well as other B-cell antigens (CD19, CD22, CD79a, FMC7). | | style="background:#F5F5F5;" align="center" + |Express bright surface [[IgM]] +/- [[IgD]] and bright [[CD20]] as well as other [[B-cell]] [[antigens]] ([[CD19]], [[CD22]], [[CD79a]], [[FMC7]]). | ||
| style="background:#F5F5F5;" align="center" + |t(11;14) must be excluded. There's no associated paraproteinemia. | | style="background:#F5F5F5;" align="center" + |t(11;14) must be excluded. There's no associated [[paraproteinemia]]. | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''Follicular lymphoma''' | | style="background:#DCDCDC;" align="center" + |'''[[Follicular lymphoma]]''' | ||
| style="background:#F5F5F5;" align="center" + |Nodular growth pattern of follicle center cells (centrocytes and centroblasts). | | style="background:#F5F5F5;" align="center" + |[[Nodular]] growth pattern of [[follicle]] center cells (centrocytes and centroblasts). | ||
| style="background:#F5F5F5;" align="center" + |Typically express CD10, HLA-DR, pan B-cell antigens (CD19, CD20, CD79a), CD21, and surface IgM, IgG, or IgA. | | style="background:#F5F5F5;" align="center" + |Typically express [[CD10]], [[HLA-DR]], pan [[B-cell]] [[antigens]] ([[CD19]], [[CD20]], [[CD79a]]), [[CD21]], and surface [[IgM]], [[IgG]], or [[IgA]]. | ||
| style="background:#F5F5F5;" align="center" + |t(14;18) | | style="background:#F5F5F5;" align="center" + |t(14;18) | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''Multiple myeloma''' | | style="background:#DCDCDC;" align="center" + |'''[[Multiple myeloma]]''' | ||
| style="background:#F5F5F5;" align="center" + |Infiltration of plasma cells in the bone marrow. | | style="background:#F5F5F5;" align="center" + |[[Infiltration (medical)|Infiltration]] of [[plasma cells]] in the [[bone marrow]]. | ||
| style="background:#F5F5F5;" align="center" + |Surface Ig is absent. Express CD138, CD38, CD79a, and VS38c. Infrequently express CD19. Approximately 70 percent of myeloma cells will express CD56. | | style="background:#F5F5F5;" align="center" + |Surface Ig is absent. Express [[CD138]], [[CD38]], [[CD79a]], and VS38c. Infrequently express [[CD19]]. Approximately 70 percent of myeloma cells will express [[CD56]]. | ||
| style="background:#F5F5F5;" align="center" + |Cytogenetics usually abnormal, although there is no specific cytogenetic abnormality. | | style="background:#F5F5F5;" align="center" + |[[Cytogenetics]] usually abnormal, although there is no specific [[cytogenetic]] abnormality. | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''Mantle cell lymphoma''' | | style="background:#DCDCDC;" align="center" + |'''[[Mantle cell lymphoma]]''' | ||
| style="background:#F5F5F5;" align="center" + |Monomorphous small to medium-sized B lymphocytes with irregular nuclei. | | style="background:#F5F5F5;" align="center" + |Monomorphous small to medium-sized [[B lymphocytes]] with irregular [[nuclei]]. | ||
| style="background:#F5F5F5;" align="center" + |CD5+ and CD23-; typically co-express surface IgM and IgD; the vast majority over-express cyclin D1. | | style="background:#F5F5F5;" align="center" + |[[CD5]]+ and [[CD23]]-; typically co-express surface [[IgM]] and [[IgD]]; the vast majority over-express [[cyclin D1]]. | ||
| style="background:#F5F5F5;" align="center" + |t(11;14) | | style="background:#F5F5F5;" align="center" + |t(11;14) | ||
|- | |- | ||
| style="background:#DCDCDC;" align="center" + |'''Marginal zone lymphoma''' | | style="background:#DCDCDC;" align="center" + |'''[[Marginal zone lymphoma]]''' | ||
| style="background:#F5F5F5;" align="center" + |Polymorphous infiltrate of small cells with paler-appearing marginal zone-type differentiation in lymph nodes. | | style="background:#F5F5F5;" align="center" + |Polymorphous infiltrate of small cells with paler-appearing marginal zone-type [[differentiation]] in [[lymph nodes]]. | ||
| style="background:#F5F5F5;" align="center" + |Expresses B cell markers CD19, CD20, and CD22, and not CD5, CD10, and CD23. | | style="background:#F5F5F5;" align="center" + |Expresses [[B cell]] markers [[CD19]], [[CD20]], and [[CD22]], and not [[CD5]], [[CD10]], and [[CD23]]. | ||
| style="background:#F5F5F5;" align="center" + |Chromosomal abnormalities, usually trisomy 3 or t(11;18), are found in most cases. May demonstrate mixed cryoglobulinemia +/- hepatitis C infection. | | style="background:#F5F5F5;" align="center" + |[[Chromosomal abnormalities]], usually [[trisomy]] 3 or t(11;18), are found in most cases. May demonstrate mixed [[cryoglobulinemia]] +/- [[hepatitis C]] [[infection]]. | ||
|} | |} | ||
Revision as of 15:34, 19 February 2019
For the WikiDoc page for this topic, click here
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Overview
Lymphoplasmacytic lymphoma must be differentiated from multiple myeloma, chronic lymphocytic leukemia/small lymphocytic lymphoma, b-cell prolymphocytic leukemia, follicular lymphoma, mantle cell lymphoma, and marginal zone lymphoma.
Differentiating Lymphoplasmacytic lymphoma from other Diseases
Lymphoplasmacytic lymphoma must be differentiated from other B cell lymphoid neoplasms including:
- 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)[6]
- Expresses B cell markers CD19, CD20, and CD22.
- Infiltrates the bone marrow with a characteristic intertrabecular and intrasinusoidal pattern
- Most common cytogenetic abnormalities are loss of 7q (19%) along with +3q (19%) and +5q (10% )[9][10]
| Disease entity | Histopathology | Immunophenotype | Genetic or other features |
|---|---|---|---|
| Lymphoplasmacytic lymphoma | ≥10 percent infiltration by small lymphocytes, plasmacytoid lymphocytes, and plasma cells, with variable numbers of admixed immunoblasts. Has a characteristic (but not pathognomonic) hyperplasia of mast cells in marrow. Lymph nodes are usually diffusely effaced. Absence of proliferation centers and marginal zone type differentiation. | Expression of pan B-cell antigens (CD19, CD20, CD22, CD79a), failure to express CD5 in mostly cases and variable expression of CD11c, CD43, CD25. Mostly cases have IgM expression with only fewer expressing IgG or IgA. There's no CD10 and cyclin D1 expression. | Majority have a monoclonal IgM paraprotein. No specific chromosomal abnormalities. |
| Chronic lymphocytic leukemia/small lymphocytic lymphoma | "Typical" CLL/SLL cells are small mature appearing lymphocytes with a dense nucleus, partially aggregated chromatin, no discernible nucleoli, and a narrow border of clear to slightly basophilic cytoplasm. | Always express CD5, usually CD23 positive with a dim expression of CD20 and surface Ig. | Del13q, del 11q, del17p, trisomy 12 |
| B-cell prolymphocytic leukemia | Prolymphocytes comprise >55 percent of the neoplastic cells. Bone marrow has interstitial pattern of infiltration. Lymph nodes may show vague nodularity, but proliferation centers are absent. | Express bright surface IgM +/- IgD and bright CD20 as well as other B-cell antigens (CD19, CD22, CD79a, FMC7). | t(11;14) must be excluded. There's no associated paraproteinemia. |
| Follicular lymphoma | Nodular growth pattern of follicle center cells (centrocytes and centroblasts). | Typically express CD10, HLA-DR, pan B-cell antigens (CD19, CD20, CD79a), CD21, and surface IgM, IgG, or IgA. | t(14;18) |
| Multiple myeloma | Infiltration of plasma cells in the bone marrow. | Surface Ig is absent. Express CD138, CD38, CD79a, and VS38c. Infrequently express CD19. Approximately 70 percent of myeloma cells will express CD56. | Cytogenetics usually abnormal, although there is no specific cytogenetic abnormality. |
| Mantle cell lymphoma | Monomorphous small to medium-sized B lymphocytes with irregular nuclei. | CD5+ and CD23-; typically co-express surface IgM and IgD; the vast majority over-express cyclin D1. | t(11;14) |
| Marginal zone lymphoma | Polymorphous infiltrate of small cells with paler-appearing marginal zone-type differentiation in lymph nodes. | Expresses B cell markers CD19, CD20, and CD22, and not CD5, CD10, and CD23. | Chromosomal abnormalities, usually trisomy 3 or t(11;18), are found in most cases. May demonstrate mixed cryoglobulinemia +/- hepatitis C infection. |
 |
 |
References
- ↑ Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating MJ, Montserrat E, Rai KR, Kipps TJ (2008). "Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines". Blood. 111 (12): 5446–56. doi:10.1182/blood-2007-06-093906. PMC 2972576. PMID 18216293.
- ↑ Del Giudice I, Davis Z, Matutes E, Osuji N, Parry-Jones N, Morilla A, Brito-Babapulle V, Oscier D, Catovsky D (2006). "IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL)". Leukemia. 20 (7): 1231–7. doi:10.1038/sj.leu.2404238. PMID 16642047.
- ↑ Ravandi F, O'Brien S (2005). "Chronic lymphoid leukemias other than chronic lymphocytic leukemia: diagnosis and treatment". Mayo Clin. Proc. 80 (12): 1660–74. doi:10.4065/80.12.1660. PMID 16342661.
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- Bone marrow infiltration of small, cleaved cells that are usually paratrabecular
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