Primary cutaneous follicle center lymphoma is a type of
lymphoma.
It was recognized as a distinct disease entity in the 2008 WHO classification.
PCFCL had been previously conceived as a variant of
follicular lymphoma
Follicular lymphoma (FL) is a cancer that involves certain types of white blood cells known as lymphocytes. The cancer originates from the uncontrolled division of specific types of B-cells known as centrocytes and centroblasts. These cells normal ...
(FL).
Cause
Unlike FL, PCFCL is not typically associated with t(14;18)
translocation
Translocation may refer to:
* Chromosomal translocation, a chromosome abnormality caused by rearrangement of parts
** Robertsonian translocation, a chromosomal rearrangement in pairs 13, 14, 15, 21, and 22
** Nonreciprocal translocation, transfer ...
although presence of that translocation does not exclude PCFCL.
It is usually not associated with overexpressed
Bcl-2.
PCFCL represents about 55% to 60% of primary cutaneous B-cell lymphomas (PCBCL);
and
diffuse large B-cell cell lymphoma, leg type
Diffusion is the net movement of anything (for example, atoms, ions, molecules, energy) generally from a region of higher concentration to a region of lower concentration. Diffusion is driven by a gradient in Gibbs free energy or chemical p ...
are the other primary cutaneous B-cell lymphomas.
The cause of PCFCL is unknown.
Treatment
Surgical removal and/or
radiotherapy
Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
is given for localized disease.
Radiation using multiple radiation fields is given if the disease has wider extent with grouped lesions.
For the less common situation of more extensive disease (still confined to skin),
rituximab without chemotherapy is used.
Intralesional
interferon alpha (IFN-α)
and intralesional rituximab have been used.
Approximately one-third of PCFCL relapse, usually in the skin; treatment is similar to initial management
and overall survival remains excellent.
Prognosis
Spread from the skin is unusual, and the
prognosis
Prognosis (Greek: πρόγνωσις "fore-knowing, foreseeing") is a medical term for predicting the likely or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) or remain stabl ...
is excellent
with a 5-year survival of over 97%.
The International Extranodal Lymphoma Study Group identified elevated
LDH, more than two skin lesions, and nodular lesions as three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is
prognostic of disease-free status.
See also
*
Cutaneous B-cell lymphoma
Cutaneous B-cell lymphomas constitute a group of diseases that occur less commonly than cutaneous T-cell lymphoma, and are characterized histologically by B-cells that appear similar to those normally found in germinal centers of lymph nodes. Cond ...
*
Primary cutaneous large B-cell lymphoma
Primary or primaries may refer to:
Arts, entertainment, and media Music Groups and labels
* Primary (band), from Australia
* Primary (musician), hip hop musician and record producer from South Korea
* Primary Music, Israeli record label
Works
...
*
Follicular lymphoma
Follicular lymphoma (FL) is a cancer that involves certain types of white blood cells known as lymphocytes. The cancer originates from the uncontrolled division of specific types of B-cells known as centrocytes and centroblasts. These cells normal ...
References
{{reflist, 2, refs=
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[{{cite journal , vauthors=Suárez AL, Pulitzer M, Horwitz S, etal , title=Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification , journal=J. Am. Acad. Dermatol. , volume=69 , issue=3 , pages=329.e1–13; quiz 341–2 , year=2013 , pmid=23957984 , doi=10.1016/j.jaad.2013.06.012 ]
[{{cite journal , vauthors=Suárez AL, Querfeld C, Horwitz S, etal , title=Primary cutaneous B-cell lymphomas: part II. Therapy and future directions , journal=J. Am. Acad. Dermatol. , volume=69 , issue=3 , pages=343.e1–11; quiz 355–6 , year=2013 , pmid=23957985 , doi=10.1016/j.jaad.2013.06.011 ]
[{{cite journal , vauthors=Senff NJ, Noordijk EM, Kim YH, etal , title=European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas , journal=Blood , volume=112 , issue=5 , pages=1600–9 , year=2008 , pmid=18567836 , doi=10.1182/blood-2008-04-152850 , doi-access=free ]
[{{cite book , first1=Steven H. , last1=Swerdlow , author2=International Agency for Research on Cancer , author3=World Health Organization , title=WHO classification of tumours of haematopoietic and lymphoid tissues , url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002 , archive-url=https://web.archive.org/web/20131012173547/http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002 , url-status=dead , archive-date=October 12, 2013 , year=2008 , publisher=International Agency for Research on Cancer , edition=4th , volume=2 , series=World Health Organization classification of tumours , isbn=9789283224310]
[{{cite journal , vauthors=Wilcox RA , title=Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management , journal=Am. J. Hematol. , volume=90 , issue=1 , pages=73–6 , year=2015 , pmid=25535037 , doi=10.1002/ajh.23863 , s2cid=10733762 , url=https://deepblue.lib.umich.edu/bitstream/2027.42/95353/1/23360_ftp.pdf, doi-access=free ]
Lymphoma
Lymphoid-related cutaneous conditions