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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{Infobox_Disease |
{{Infobox_Disease |
   Name          = {{PAGENAME}} |
   Name          = {{PAGENAME}} |
   Image          = Lymphoma ExEx.jpg |
   Image          = Lymphoma ExEx.jpg |
   Caption        = Lymph node: Malignant Lymphoma Large Cell Type: Gross natural color excellent view of cut mesentery showing massively enlarged mesenteric nodes with focal hemorrhages case diagnosed several years ago as reticulum cell sarcoma excellent demonstration of nodes with lymphoma. <br>[http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]|
   Caption        = Lymph node: Malignant Lymphoma Large Cell Type: Gross natural color excellent view of cut mesentery showing massively enlarged mesenteric nodes with focal hemorrhages case diagnosed several years ago as reticulum cell sarcoma excellent demonstration of nodes with lymphoma. <br>[http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]|
  DiseasesDB    = |
  ICD10          = {{ICD10|C|81||c|81}}-{{ICD10|C|96||c|81}} |
  ICD9          = |
  ICDO          = 9590-9999 |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshID        = D008223 |
}}
}}
{{SI}}
{{Lymphoma}}
{{CMG}}


'''Associate Editor-in-Chief:''' Ross Bonander
{{CMG}}; {{AE}} Ross Bonander, {{ADG}}
__NOTOC__
{{Editor Help}}


==Overview==
==Overview==
'''Lymphoma''' is  a type of [[cancer]] that originates in [[lymphocyte]]s (a type of white blood cell in the vertebrate immune system). There are many types of lymphoma. Lymphomas are part of the broad group of diseases called [[Hematological malignancy|hematological neoplasms]].
'''Lymphoma''' is  a type of [[cancer]] that originates in [[lymphocyte]]s (a type of white blood cell in the vertebrate immune system). There are many types of lymphoma. Lymphomas are part of the broad group of diseases called [[Hematological malignancy|hematological neoplasms]]. It was discovered by [[Thomas Hodgkin]] in 1832 and was called [[Hodgkin's Disease]] throughout the 19th and 20th centuries. Colloquially, lymphoma is broadly categorized as [[Hodgkin's lymphoma]] or [[Non-Hodgkin lymphoma|non-Hodgkin lymphoma]] (all other types of lymphoma). Scientific classification of the types of lymphoma is more detailed. Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B, T or NK cell lineage. Histiocytic malignancies are rare and are classified as sarcomas.<ref name="isbn92-832-2411-6">{{cite book |author= |title=Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.) |publisher=Oxford Univ Pr |location= |year= |pages= |isbn=92-832-2411-6 |oclc= |doi=}}</ref>
 
In the 19th and 20th centuries the affliction was called [[Hodgkin's Disease]], as it was discovered by [[Thomas Hodgkin]] in 1832. Colloquially, lymphoma is broadly categorized as [[Hodgkin's lymphoma]] and [[Non-Hodgkin lymphoma|non-Hodgkin lymphoma]] (all other types of lymphoma). Scientific classification of the types of lymphoma is more detailed.
 
Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B, T or NK cell lineage. Histiocytic malignancies are rare and are classified as sarcomas.<ref name="isbn92-832-2411-6">{{cite book |author= |title=Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.) |publisher=Oxford Univ Pr |location= |year= |pages= |isbn=92-832-2411-6 |oclc= |doi=}}</ref>
 
==Prevalence==
According to the U.S. [[National Institutes of Health]], lymphomas account for about five percent of all cases of cancer in the United States, and Hodgkin's lymphoma in particular accounts for less than one percent of all cases of cancer in the United States.
 
Because the lymphatic system is part of the body's immune system, patients with a weakened immune system, such as from [[HIV]] infection or from certain drugs or medication, also have a higher incidence of lymphoma.
 
==Classification==
===WHO classification===
 
The '''WHO Classification''' is the latest classification of lymphoma, published by the [[World Health Organization]] in 2001.<ref name="isbn92-832-2411-6">{{cite book |author= |title=Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.) |publisher=Oxford Univ Pr |location= |year= |pages= |isbn=92-832-2411-6 |oclc= |doi=}}</ref> It was based upon the "Revised European-American Lymphoma classification" (REAL).


This classification attempts to classify lymphomas by cell type, i.e. the normal cell type that most closely resembles the tumor. They are classified in three large groups: the [[B cell]] tumors, the [[T cell]] and [[natural killer cell]] tumors, [[Hodgkin lymphoma]], and other minor groups: ([[ICD-O]] codes are provided where available)
== Classification ==
{{family tree/start}}
{{family tree | | | | | | | | | | | | | A01 | | | | | | | | | | | | |A01=Lymphoma }}
{{family tree | | | | | | | | | | | | | |!| | | | | | | | | | | | | |}}
{{family tree | | | | | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| | | |}}
{{family tree | | | | | | B01 | | | | | | | | | | | | B02 | | | |B01=[[Non-Hodgkin lymphoma]]|B02= [[Hodgkin's Lymphoma]]}}
{{family tree | | | | | | |!| | |,|-|-|-|v|-|-|-|v|-|-|^|v|-|-|-|v|-|-|-|.|}}
{{family tree | | | | | | |!| | E01 | | E02 | | E03 | | E04 | | E05 | | E06|E01=Classical|E02=Nodular sclerosis|E03=Lymphocyte rich|E04=Mixed cellularity|E05=Lymphocyte depleted|E06=Nodular lymphocyte}}
{{family tree | | | | | | |!| | | | | | | | | | | | | | | | | | |}}
{{family tree | | | | | | |)|-|-|-|-|-|-|-|-|-|.| | | | | | | | | | | | | }}
{{family tree | | | | | | C01 | | | | | | | | C02 | | | | | | | | | | | | | | | | | |C01=B-cell Lymphoma|C02=T cell Lymphoma }}
{{family tree | | | | | | |!| | | | | | | | | |!| | | | | | | |}}
{{family tree | | |,|-|-|-|^|-|-|-|.| | | | |,|^|-|-|-|-|-|-|.| | | |}}
{{family tree | | D01 | | | | | | D02 | | | D03 | | | | | | D04 | | | | |D01=Precursor B cell |D02=Peripheral B cell| D03=Precursor B cell |D04=Peripheral B cell}}
{{family tree | | |!| | | | | | | |!| | | | |!| | | | | | | |!| | | }}
{{family tree | | C01 | | | | | | C02 | | | C03 | | | | | | C04 | | | |C01=[[Acute Lymphobalstic lymphoma]]|C02=Small Lymphocytic lymphoma (SLL)<br> [[Chronic lymphocytic leukemia]]<br>[[Mantle cell lymphoma]]<br> Follicular Lymphoma<br> Marginal zone lymphoma <br> Diffuse large B cell lymphoma <br> [[Burkitt lymphoma]]| C03= [[Acute Lymphobalstic lymphoma]]|C04=Anaplastic large cell T lymphoma<br>Peripheral T cell lymphoma<br> Mycosis fungoidies}}
{{family tree/end}}


====Mature B cell neoplasms====
== Differentiating Lymphoma From Other Diseases ==
{| class="wikitable"
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating diagnosis of Lymphoma
! colspan="5" style="background:#4479BA; color: #FFFFFF;" align="center" + |Symptoms
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Signs
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Additional Findings
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Fever
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Rash
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diarrhea
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Abdominal pain
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Weight loss
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Painful lymphadenopathy
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Hepatosplenomegaly
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Arthritis
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Lab Findings
|-
| style="background:#DCDCDC;" align="center" + |[[Lymphoma]]
| +
|–
|–
| +
| +
|–
| +
|–
|Increase [[ESR]], increased [[LDH]]
|[[Night sweats]], constant fatigue
|-
| style="background:#DCDCDC;" align="center" + |[[Brucellosis]]
| +
| +
|–
| +
| +
| +
| +
| +
|[[Lymphocytosis|Relative lymphocytosis]]
|[[Night sweats]], often with characteristic smell, likened to wet hay
|-
| style="background:#DCDCDC;" align="center" + |[[Typhoid fever]]
| +
| +
|–
| +
|–
|–
| +
| +
|Decreased [[hemoglobin]]
|Incremental increase in temperature initially and than sustained [[fever]] as high as 40°C (104°F)
|-
| style="background:#DCDCDC;" align="center" + |[[Malaria]]
| +
|–
| +
| +
|–
|–
| +
| +
|Microcytosis,
elevated [[LDH]]
|"Tertian" fever: paroxysms occur every second day
|-
| style="background:#DCDCDC;" align="center" + |[[Tuberculosis]]
| +
| +
|–
| +
| +
| +
|–
| +
|Mild normocytic [[anemia]], [[hyponatremia]], and
[[hypercalcemia]]
|[[Night sweats]], constant fatigue
|-
| style="background:#DCDCDC;" align="center" + |[[Mumps]]
| +
|–
|–
|–
|–
| +
|–
|–
|[[Lymphocytosis|Relative lymphocytosis]], serum [[amylase]]<nowiki/>elevated
|[[Parotid gland|Parotid]]<nowiki/>swelling/tenderness
|-
| style="background:#DCDCDC;" align="center" + |[[Rheumatoid arthritis]]
|–
| +
|–
|–
|–
|–
|–
| +
|[[ESR]] and [[CRP]] elevated, positive [[rheumatoid factor]]
|Morning stiffness
|-
| style="background:#DCDCDC;" align="center" + |[[SLE]]
|–
| +
|–
| +
| +
|–
|–
| +
|[[ESR]] and [[CRP]] elevated, positive [[ANA]]
|[[Fatigue]]
|-
| style="background:#DCDCDC;" align="center" + |[[Human Immunodeficiency Virus|HIV]]
|–
|–
|–
| +
| +
| +
|–
| +
|Leukopenia
|Constant fatigue
|}


**[[Small cell lymphoma|Chronic lymphocytic leukemia/small lymphocytic lymphoma]]
CNS lymphoma must be differentiated from other causes of seizures, headache, and fever in immunocompromised patients such as disseminated tuberculosis and disseminated aspergillosis.
**[[B-cell prolymphocytic leukemia]]
{| class="wikitable"
**Lymphoplasmacytic lymphoma
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Disease
**Splenic marginal zone B-cell lymphoma (with or without villous lymphocytes)
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating signs and symptoms
**[[Hairy cell leukemia]]
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating tests
**Splenic lymphoma/leukemia, unclassifiable
|-
**[[Plasma cell myeloma/plasmacytoma]]
| style="background:#DCDCDC;" align="center" + |[[Lymphoma|CNS lymphoma]]<ref name="pmid20212226">{{cite journal |vauthors=Gerstner ER, Batchelor TT |title=Primary central nervous system lymphoma |journal=Arch. Neurol. |volume=67 |issue=3 |pages=291–7 |year=2010 |pmid=20212226 |doi=10.1001/archneurol.2010.3 |url=}}</ref>
**[[Heavy chain diseases]]
|
**Extranodal marginal zone B-cell lymphoma of MALT type ([[MALT lymphoma]])
* Patient is [[immunocompetent]]
**Nodal marginal zone B-cell lymphoma (with or without monocytoid B cells)
* Focal symptoms indicative of a mass [[lesion]]
**[[Follicular lymphoma]]
* [[Seizure]]
**Primary cutaneous follicle center lymphoma
|
**[[Mantle cell lymphoma]]
*Single solitary ring enhancing [[lesion]] on [[CT]] or [[MRI]]
**[[Diffuse large B-cell lymphoma]] (DLBCL)
|-
***Diffuse large B-cell lymphoma, not otherwise specified
| style="background:#DCDCDC;" align="center" + |[[Disseminated tuberculosis]]<ref name="pmid21740673">{{cite journal |vauthors=von Reyn CF, Kimambo S, Mtei L, Arbeit RD, Maro I, Bakari M, Matee M, Lahey T, Adams LV, Black W, Mackenzie T, Lyimo J, Tvaroha S, Waddell R, Kreiswirth B, Horsburgh CR, Pallangyo K |title=Disseminated tuberculosis in human immunodeficiency virus infection: ineffective immunity, polyclonal disease and high mortality |journal=Int. J. Tuberc. Lung Dis. |volume=15 |issue=8 |pages=1087–92 |year=2011 |pmid=21740673 |doi=10.5588/ijtld.10.0517 |url=}}</ref>
***T-cell /histiocyte rich large B-cell lymphoma
|
***DLBCL associated with chronic inflammation
* Prior history of residence in an [[Endemic (epidemiology)|endemic]] area
***EBV positive DLBCL of the elderly
* Chronic [[cough]], [[weight loss]], [[hemoptysis]]
***Lymphomatoid granulomatosis
|
***Primary mediastinal (thymic) large B-cell lymphoma
* [[PCR]] of [[CSF]] for [[tuberculosis]]
***Intravascular large B-cell lymphoma
* Mycobacterial culture of [[CSF]]
***Primary cutaneous DLBCL, leg type
* [[Brain]] biopsy for [[acid-fast bacilli]] staining
***ALK positive DLBCL
* Culture and acid stain positive for [[acid-fast bacilli]]
***Plasmablastic lymphoma
* CXR shows [[Cavitation|cavitations]]
***Primary effusion lymphoma
|-
***Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease
| style="background:#DCDCDC;" align="center" + |[[Aspergillosis]]<ref name="pmid10194462">{{cite journal |vauthors=Latgé JP |title=Aspergillus fumigatus and aspergillosis |journal=Clin. Microbiol. Rev. |volume=12 |issue=2 |pages=310–50 |year=1999 |pmid=10194462 |pmc=88920 |doi= |url=}}</ref>
**[[Burkitt lymphoma/Burkitt cell leukemia]]
|
**B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
* [[Pulmonary]] [[lesions]] in addition to [[CNS]] [[lesions]]
**B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma
* Symptoms may include [[cough]], [[chest pain]], and [[hemoptysis]]
|
*[[CSF]] fungal culture, [[galactomannan]]
|-
| style="background:#DCDCDC;" align="center" + |[[Cryptococcosis]]
|
*Symptoms include [[cough]], [[chest pain]], and [[hemoptysis]]
|
*[[Cryptococcal infection|Cryptococcal]] [[antigen]] from [[CSF]] and [[serum]]
*[[CSF]] fungal culture
|-
| style="background:#DCDCDC;" align="center" + |[[Chagas disease]]<ref name="pmid20399979">{{cite journal |vauthors=Rassi A, Rassi A, Marin-Neto JA |title=Chagas disease |journal=Lancet |volume=375 |issue=9723 |pages=1388–402 |year=2010 |pmid=20399979 |doi=10.1016/S0140-6736(10)60061-X |url=}}</ref>
|
*History of residence in Central or South America
*Acute infection is rarely symptomatic
*[[Encephalitis]] or focal [[brain]] [[lesions]]
*[[Myocarditis]]
*[[Chronic]] [[infections]] in [[immunocompromised]] patients develop into [[encephalitis]] with [[necrotic]] [[brain]] lesions causing a [[mass effect]]
|
*[[Trypanosoma cruzi]] in [[blood]], [[Tissue (biology)|tissue]], or [[CSF]], [[PCR]] of [[Tissue (biology)|tissue]] or [[body fluids]], and [[Serological testing|serologic tests]]
|-
| style="background:#DCDCDC;" align="center" + |[[Cytomegalovirus infection|CMV infection]]<ref name="pmid11215290">{{cite journal |vauthors=Emery VC |title=Investigation of CMV disease in immunocompromised patients |journal=J. Clin. Pathol. |volume=54 |issue=2 |pages=84–8 |year=2001 |pmid=11215290 |pmc=1731357 |doi= |url=}}</ref>
|
*Most common [[CNS]] [[opportunistic infection]] in [[AIDS]] patients
*Presents with [[encephalitis]], [[retinitis]], progressive [[myelitis]], or [[polyradiculitis]]
*In [[disseminated disease]], it involves both the [[liver]] and kidneys
|
*[[Brain]] [[CT]]/[[MRI]]/[[biopsy]]: location of [[lesions]] is usually near the [[brain stem]] or periventricular areas
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic
*[[Brain biopsy]] with + [[staining]] for [[CMV]] or evidence of owl's eyes is also diagnostic, but it is rarely performed because of the location of [[brain]] lesions
|-
| style="background:#DCDCDC;" align="center" + |[[HSV|HSV infection]]<ref name="pmid1919640">{{cite journal |vauthors=Bustamante CI, Wade JC |title=Herpes simplex virus infection in the immunocompromised cancer patient |journal=J. Clin. Oncol. |volume=9 |issue=10 |pages=1903–15 |year=1991 |pmid=1919640 |doi=10.1200/JCO.1991.9.10.1903 |url=}}</ref>
|
*[[Seizures]], [[headache]], [[confusion]] and/or [[urinary retention]] can be seen in [[disseminated disease]], which usually affects only the [[immunocompromised]] or acute [[infections]]
*In [[pregnant]] women, it may be associated with concurrent [[genital]]/[[oral]] [[lesions]]; can be spread to the [[neonate]] during acute infection in the mother, or via [[viral shedding]] in the [[birth canal]]
*[[Neonatal]] [[Herpes simplex virus|HSV]] can range from localized [[Skin and soft-tissue infections|skin infections]] to [[encephalitis]], [[pneumonitis]], and [[disseminated disease]]
|
*[[Brain]] [[CT]]/[[MRI]]/[[biopsy]]: location of [[lesions]] is usually the [[medial]] [[temporal lobe]] or the [[Orbital cavity|orbital]] surface of the [[frontal lobe]].
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic
|-
| style="background:#DCDCDC;" align="center" + |[[Chickenpox|Varicella Zoster infection]]<ref name="pmid15864101">{{cite journal |vauthors=Hambleton S |title=Chickenpox |journal=Curr. Opin. Infect. Dis. |volume=18 |issue=3 |pages=235–40 |year=2005 |pmid=15864101 |doi= |url=}}</ref>
|
*Multifocal involvement has subacute course, usually only in [[immunosuppressed]], with [[headache]], [[fever]], focal deficits, and [[seizures]].
*Unifocal involvement is more typically seen in [[immunocompetent]] hosts, occurring after [[contralateral]] [[cranial nerve]] [[herpes zoster]], with [[Altered mental status|mental status changes]], [[TIA|TIAs]], and [[stroke]]
*[[Disseminated disease|Disseminated]] [[varicella zoster virus]] can occur in adults during primary [[infection]], presenting with [[pneumonitis]] and/or [[hepatitis]]
*Disease is a [[Vasculitis|vasculopathy]] with [[hemorrhage]] and [[stroke]]
|
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic
|-
| style="background:#DCDCDC;" align="center" + |[[Brain abscess]]<ref name="pmid24174804">{{cite journal |vauthors=Alvis Miranda H, Castellar-Leones SM, Elzain MA, Moscote-Salazar LR |title=Brain abscess: Current management |journal=J Neurosci Rural Pract |volume=4 |issue=Suppl 1 |pages=S67–81 |year=2013 |pmid=24174804 |pmc=3808066 |doi=10.4103/0976-3147.116472 |url=}}</ref><ref name="pmid25360205">{{cite journal |vauthors=Patel K, Clifford DB |title=Bacterial brain abscess |journal=Neurohospitalist |volume=4 |issue=4 |pages=196–204 |year=2014 |pmid=25360205 |pmc=4212419 |doi=10.1177/1941874414540684 |url=}}</ref>
|
*Associated with [[sinusitis]] (abutting the sinuses) or with [[bacteremia]]
*Signs and symptoms includes [[fever]] and [[necrotizing]] [[brain]] [[lesions]] with [[mass effect]]
|
*[[CSF]] culture or culture of [[brain abscess]]
|-
| style="background:#DCDCDC;" align="center" + |[[Progressive multifocal leukoencephalopathy]]<ref name="pmid20298966">{{cite journal |vauthors=Tan CS, Koralnik IJ |title=Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis |journal=Lancet Neurol |volume=9 |issue=4 |pages=425–37 |year=2010 |pmid=20298966 |pmc=2880524 |doi=10.1016/S1474-4422(10)70040-5 |url=}}</ref>
|
*Symptoms are often more insidious in onset and progress over months. Symptoms include progressive [[weakness]], poor [[coordination]], with gradual slowing of [[mental]] function. Only seen in the [[immunosuppressed]]. Rarely associated with [[fever]] or other systemic symptoms


====B-cell proliferations of uncertain malignant potential====
|
*[[Polymerase chain reaction|PCR]] of [[CSF]] for [[JC virus]]
*[[Biopsy]] reveals [[white matter]] [[lesions]] and not well-circumscribed [[lesions]].
|}


**[[Lymphomatoid granulomatosis]]
== Epidemiology and Demographics ==
**Post-transplant lymphoproliferative disorder
* According to the U.S. [[National Institutes of Health]], lymphomas account for about five percent of all cases of cancer in the United States.
 
* Hodgkin's lymphoma in particular accounts for less than one percent of all cases of cancer in the United States
[[Image:lymphoma_microarray.jpg|500px|thumb|left|DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. Colors indicate levels of expression; green indicates genes that are overexpressed in normal cells compared to lymphoma cells and red indicates genes that are overexpressed in lymphoma cells compared to normal cells.]]
<br clear="left"/>
 
====Mature T cell and natural killer (NK) cell neoplasms====
Leukemic/disseminated
*[[T-cell prolymphocytic leukemia|T cell prolymphocytic leukemia]]
*[[T-cell large granular lymphocyte leukemia|T cell large granular lymphocytic leukemia]]
*Aggressive NK cell leukemia
*[[Adult T-cell leukemia|Adult T-cell leukemia/lymphoma (ATLL)]]
Cutaneous
*[[Mycosis fungoides]]
*[[Sezary syndrome]]
*Primary cutaneous CD30-positive T cell lymphoproliferative disorders
**Primary cutaneous anaplastic large cell lymphoma
**[[Lymphomatoid papulosis]]
Other extranodal
*Extranodal NK/T cell lymphoma, nasal type
*Enteropathy-type T cell lymphoma
*Hepatosplenic T cell lymphoma
*Subcutaneous pannicultis-like T cell lymphoma
Nodal
*Peripheral T cell lymphoma, NOS (not otherwise specified)
*Angioimmunoblastic T cell lymphoma
*Primary systemic anaplastic large cell lymphoma
Neoplasms of uncertain lineage, stage of differentiation
*Blastic NK cell lymphoma
 
====Hodgkin Lymphoma====
**Nodular lymphocyte-predominant Hodgkin lymphoma
**Classical Hodgkin lymphoma
***Nodular sclerosis
***Mixed cellularity
***Lymphocyte-rich
***Lymphocyte depleted or not depleted
 
====Immunodeficiency-associated lymphoproliferative disorders====
**Associated with a primary immune disorder
**Associated with the Human Immunodeficiency Virus ([[HIV]])
**Post-transplant
**Associated with Methotrexate therapy
 
===Working formulation===
The '''Working Formulation''', published in 1982, is primarily descriptive.  It is still occasionally used, but has been superseded by the WHO classification, above.
 
====Low grade====
*Malignant Lymphoma, small lymphocytic ([[chronic lymphocytic leukemia]])
*Malignant Lymphoma, [[lymph follicle|follicular]], predominantly small cleaved cell
*Malignant Lymphoma, follicular, mixed (small cleaved and [[large cell]])
 
====High grade====
*Malignant Lymphoma, large cell, immunoblastic
*Malignant Lymphoma, [[lymphoblastic]]
*Malignant Lymphoma, small non-cleaved cells ([[Burkitt's lymphoma]])
 
====Miscellaneous====
*Composite
*[[Histiocytic]]
*[[Extramedullary plasmacytoma]]
*Unclassifiable
 
===Other classification systems===
* [[ICD-O]] (codes 9590-9999, details at [http://web.archive.org/web/20040627090029/http://www.cog.ufl.edu/publ/apps/icdo/icdo_morph.txt])
* [[ICD|ICD-10]] (codes C81-C96, details at [http://www3.who.int/icd/vol1htm2003/fr-icd.htm?gc81.htm+])
 
== For diagnosis, etiology, staging, prognosis, and treatment ==
Please see separate links to [[Hodgkin's lymphoma]] and [[non-Hodgkin's lymphoma]].
 
== Genetics ==
Enteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of [[Triticeae glutens]]. In gluten sensitive individuals with EATL 68% are homozygotes of the DQB1*02 subtype at the HLA-DQB1 locus (serotype DQ2).<ref name="pmid17470479">{{cite journal |author=Al-Toma A, Verbeek WH, Hadithi M, von Blomberg BM, Mulder CJ |title=Survival in Refractory Coeliac Disease and Enteropathy associated T cell Lymphoma: Retrospective evaluation of single centre experience |journal= |volume= |issue= |pages= |year=2007 |pmid=17470479 |doi=10.1136/gut.2006.114512}}</ref> (See [[Coeliac Disease]], [[HLA-DQ]], [[HLA DR3-DQ2]])
 
==See also==
* [[Hodgkin's lymphoma]]
* [[Non-Hodgkin's lymphoma]]
* [[Follicular lymphoma]]
* [[Diffuse large B cell lymphoma]]
* [[Gastric lymphoma]]
* [[BCP-1 cells]]
* [[Ann Arbor staging]]
* [[International Prognostic Index]]


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


==External links==
{{WikiDoc Help Menu}}
 
{{WikiDoc Sources}}
===Research Foundations and Working Groups===
*[http://www.lymphoma.org Lymphoma Research Foundation]
*[http://www.leukemia-research.org Leukemia Research Foundation]
*[http://epi.grants.cancer.gov/InterLymph International Lymphoma Epidemiology Consortium]
*[http://www.lymphome.de/en/index.jsp Competence Network Malignant Lymphomas (German Lymphoma Study Groups)]
 
===Information===
*[http://www.nlm.nih.gov/medlineplus/lymphoma.html MedlinePlus: Lymphoma]
*[http://www.cancer.gov/cancertopics/types/non-hodgkin National Cancer Institute: Non-Hodgkin Lymphomas]
*[http://www.lymphomainfo.net Lymphoma Information Network]
*[http://www3.who.int/icd/vol1htm2003/fr-icd.htm?gc81.htm+ ICD10 classification of lymphoma]
*[http://jco.ascopubs.org/cgi/content/full/17/4/1244 Report of an International Workshop to Standardize Response Criteria for NHLs]
 
===Societies and Support Groups===
*[http://www.leukemia-lymphoma.org/all_page?item_id=7030 The Leukemia & Lymphoma Society]
*[http://www.supportgroups.com/support-groups/cancer-support-groups Online Lymphoma Support Groups]
*[http://www.lymphomas.org.uk/ The Lymphoma Association (UK)]
*[http://www.lymphoma.ie Lymphoma Support Ireland]
*[http://www.coventry-lymphoma.com Coventry Lymphoma Association Support Group (UK)]
 
===Statistics===
*[http://seer.cancer.gov/statfacts/html/lymph.html US Lymphoma SEER Fact Sheet from the National Cancer Institute]
*[http://info.cancerresearchuk.org/cancerstats/types/hodgkinslymphoma/?a=5441 UK Hodgkin Lymphoma Statistics]
*[http://info.cancerresearchuk.org/cancerstats/types/nhl/?a=5441 UK Non-Hodgkin Lymphoma Statistics]
*[http://www.lymphomation.org/aboutNHL.htm About Lymphomas - Patients Against Lymphoma]
*[http://www.lymphomainfo.net/hodgkins/timeline.html Timeline of discovery and treatment of Hodgkin's Lymphoma]
* {{cite news | first= | last=National Cancer Institute | coauthors= | title=Risk of Lymphoma Increases with Hepatitis C Virus Infection | date=May 2007 | publisher= | url =http://www.cancer.gov/newscenter/pressreleases/HepCLymphomaRelease | work = | pages = | accessdate = | language = }}
 
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Latest revision as of 21:06, 9 January 2019

For patient information click here

Lymphoma
Lymph node: Malignant Lymphoma Large Cell Type: Gross natural color excellent view of cut mesentery showing massively enlarged mesenteric nodes with focal hemorrhages case diagnosed several years ago as reticulum cell sarcoma excellent demonstration of nodes with lymphoma.
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Lymphoma Main Page

Home

Patient Information

Overview

Classification

Non-Hodgkin lymphoma
Hodgkin lymphoma

Differentiating Lymphoma from other Diseases

Epidemiology and Demographics

Case Studies

Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ross Bonander, Aditya Ganti M.B.B.S. [2]

Overview

Lymphoma is a type of cancer that originates in lymphocytes (a type of white blood cell in the vertebrate immune system). There are many types of lymphoma. Lymphomas are part of the broad group of diseases called hematological neoplasms. It was discovered by Thomas Hodgkin in 1832 and was called Hodgkin's Disease throughout the 19th and 20th centuries. Colloquially, lymphoma is broadly categorized as Hodgkin's lymphoma or non-Hodgkin lymphoma (all other types of lymphoma). Scientific classification of the types of lymphoma is more detailed. Although older classifications referred to histiocytic lymphomas, these are recognized in newer classifications as of B, T or NK cell lineage. Histiocytic malignancies are rare and are classified as sarcomas.[1]

Classification

 
 
 
 
 
 
 
 
 
 
 
 
Lymphoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Non-Hodgkin lymphoma
 
 
 
 
 
 
 
 
 
 
 
Hodgkin's Lymphoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Classical
 
Nodular sclerosis
 
Lymphocyte rich
 
Mixed cellularity
 
Lymphocyte depleted
 
Nodular lymphocyte
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
B-cell Lymphoma
 
 
 
 
 
 
 
T cell Lymphoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Precursor B cell
 
 
 
 
 
Peripheral B cell
 
 
Precursor B cell
 
 
 
 
 
Peripheral B cell
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acute Lymphobalstic lymphoma
 
 
 
 
 
Small Lymphocytic lymphoma (SLL)
Chronic lymphocytic leukemia
Mantle cell lymphoma
Follicular Lymphoma
Marginal zone lymphoma
Diffuse large B cell lymphoma
Burkitt lymphoma
 
 
Acute Lymphobalstic lymphoma
 
 
 
 
 
Anaplastic large cell T lymphoma
Peripheral T cell lymphoma
Mycosis fungoidies
 
 
 

Differentiating Lymphoma From Other Diseases

Differentiating diagnosis of Lymphoma Symptoms Signs Diagnosis Additional Findings
Fever Rash Diarrhea Abdominal pain Weight loss Painful lymphadenopathy Hepatosplenomegaly Arthritis Lab Findings
Lymphoma + – – + + – + – Increase ESR, increased LDH Night sweats, constant fatigue
Brucellosis + + – + + + + + Relative lymphocytosis Night sweats, often with characteristic smell, likened to wet hay
Typhoid fever + + – + – – + + Decreased hemoglobin Incremental increase in temperature initially and than sustained fever as high as 40°C (104°F)
Malaria + – + + – – + + Microcytosis,

elevated LDH

"Tertian" fever: paroxysms occur every second day
Tuberculosis + + – + + + – + Mild normocytic anemia, hyponatremia, and

hypercalcemia

Night sweats, constant fatigue
Mumps + – – – – + – – Relative lymphocytosis, serum amylaseelevated Parotidswelling/tenderness
Rheumatoid arthritis – + – – – – – + ESR and CRP elevated, positive rheumatoid factor Morning stiffness
SLE – + – + + – – + ESR and CRP elevated, positive ANA Fatigue
HIV – – – + + + – + Leukopenia Constant fatigue

CNS lymphoma must be differentiated from other causes of seizures, headache, and fever in immunocompromised patients such as disseminated tuberculosis and disseminated aspergillosis.

Disease Differentiating signs and symptoms Differentiating tests
CNS lymphoma[2]
Disseminated tuberculosis[3]
Aspergillosis[4]
Cryptococcosis
Chagas disease[5]
CMV infection[6]
HSV infection[7]
Varicella Zoster infection[8]
Brain abscess[9][10]
Progressive multifocal leukoencephalopathy[11]
  • Symptoms are often more insidious in onset and progress over months. Symptoms include progressive weakness, poor coordination, with gradual slowing of mental function. Only seen in the immunosuppressed. Rarely associated with fever or other systemic symptoms

Epidemiology and Demographics

  • According to the U.S. National Institutes of Health, lymphomas account for about five percent of all cases of cancer in the United States.
  • Hodgkin's lymphoma in particular accounts for less than one percent of all cases of cancer in the United States

References

  1. ↑ Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6.
  2. ↑ Gerstner ER, Batchelor TT (2010). "Primary central nervous system lymphoma". Arch. Neurol. 67 (3): 291–7. doi:10.1001/archneurol.2010.3. PMID 20212226.
  3. ↑ von Reyn CF, Kimambo S, Mtei L, Arbeit RD, Maro I, Bakari M, Matee M, Lahey T, Adams LV, Black W, Mackenzie T, Lyimo J, Tvaroha S, Waddell R, Kreiswirth B, Horsburgh CR, Pallangyo K (2011). "Disseminated tuberculosis in human immunodeficiency virus infection: ineffective immunity, polyclonal disease and high mortality". Int. J. Tuberc. Lung Dis. 15 (8): 1087–92. doi:10.5588/ijtld.10.0517. PMID 21740673.
  4. ↑ Latgé JP (1999). "Aspergillus fumigatus and aspergillosis". Clin. Microbiol. Rev. 12 (2): 310–50. PMC 88920. PMID 10194462.
  5. ↑ Rassi A, Rassi A, Marin-Neto JA (2010). "Chagas disease". Lancet. 375 (9723): 1388–402. doi:10.1016/S0140-6736(10)60061-X. PMID 20399979.
  6. ↑ Emery VC (2001). "Investigation of CMV disease in immunocompromised patients". J. Clin. Pathol. 54 (2): 84–8. PMC 1731357. PMID 11215290.
  7. ↑ Bustamante CI, Wade JC (1991). "Herpes simplex virus infection in the immunocompromised cancer patient". J. Clin. Oncol. 9 (10): 1903–15. doi:10.1200/JCO.1991.9.10.1903. PMID 1919640.
  8. ↑ Hambleton S (2005). "Chickenpox". Curr. Opin. Infect. Dis. 18 (3): 235–40. PMID 15864101.
  9. ↑ Alvis Miranda H, Castellar-Leones SM, Elzain MA, Moscote-Salazar LR (2013). "Brain abscess: Current management". J Neurosci Rural Pract. 4 (Suppl 1): S67–81. doi:10.4103/0976-3147.116472. PMC 3808066. PMID 24174804.
  10. ↑ Patel K, Clifford DB (2014). "Bacterial brain abscess". Neurohospitalist. 4 (4): 196–204. doi:10.1177/1941874414540684. PMC 4212419. PMID 25360205.
  11. ↑ Tan CS, Koralnik IJ (2010). "Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis". Lancet Neurol. 9 (4): 425–37. doi:10.1016/S1474-4422(10)70040-5. PMC 2880524. PMID 20298966.


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