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Primary cutaneous follicle centre lymphoma is a slow-growing lymphoma of germinal-centre B cells that stays in the skin, usually as lumps on the head or trunk. Its outlook is excellent, with about 95 in 100 people alive at five years, and radiotherapy or excision is usually all that is needed; the important thing is not to mistake it for the aggressive leg-type large B-cell lymphoma.
WHO-HAEM5 lists primary cutaneous follicle centre lymphoma among the cutaneous B-cell lymphomas as a distinct entity from follicular lymphoma, defined by follicle centre cells in the skin with no extracutaneous disease at staging (Alaggio 2022). It has a five-year overall survival of about 95 percent against about 50 percent for primary cutaneous large B-cell lymphoma, leg type, and the two are separated in practice by IgM staining, present in all 40 leg-type cases and only 5 of 53 follicle centre cases in the defining study (Am J Surg Pathol 2010). Its molecular landscape differs from classic follicular lymphoma: 27 percent lack CD10 but all express MEF2B and HGAL, TNFRSF14 is the most commonly mutated gene (40 percent, with a further 10 percent carrying 1p36 deletions), followed by CREBBP, TNFAIP3, KMT2D, SOCS1, EP300, STAT6 and FOXO1, and BCL2 rearrangements are usually absent (Human Pathology 2020).
How it differs from its parent: it is skin-confined, rarely carries the t(14;18) that defines nodal follicular lymphoma, is not graded or staged as follicular lymphoma is, and is cured locally in most cases; the parent's chemoimmunotherapy is reserved for the rare disseminated case.
How common: no registry share in the sources read.
Treatment: local radiotherapy or excision for solitary or localised lesions; rituximab for multifocal skin disease; the parent's systemic pathways only for extracutaneous spread; skin relapses are common and treated locally again (Am J Surg Pathol 2010 for the survival figures).
Rare; the largest series in the sources read hold 53 patients (Am J Surg Pathol 2010) and 22 (Human Pathology 2020). No registry share is in the sources read.
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
Rituximab for multifocal skin disease; the parent's systemic pathways for the rare extracutaneous spread.
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Query for this cancer: (TITLE:"Primary cutaneous follicle centre lymphoma" OR ABSTRACT:"Primary cutaneous follicle centre lymphoma" OR TITLE:"Primary cutaneous follicular lymphoma" OR ABSTRACT:"Primary cutaneous follicular lymphoma" OR TITLE:"Primary cutaneous follicle center lymphoma" OR ABSTRACT:"Primary cutaneous follicle center lymphoma" OR TITLE:"PCFCL" OR ABSTRACT:"PCFCL" OR TITLE:"Crosti lymphoma" OR ABSTRACT:"Crosti lymphoma" OR TITLE:"Reticulohistiocytoma of the dorsum" OR ABSTRACT:"Reticulohistiocytoma of the dorsum") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Primary cutaneous follicle centre lymphoma, not a curated reading list.
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A shortage of antibodies because treatment has wiped out the B cells or plasma cells that make them, as CD19 CAR-T, CD20 antibodies and BCMA therapies deliberately do. It leaves patients prone to infections, sometimes for years, and is managed with immunoglobulin infusions.
Reactions around the moment a drug is given: chills, fever or breathlessness from antibodies (infusion reactions), true allergy (hypersensitivity, rarely anaphylaxis), and leakage of a damaging drug into tissue around the vein (extravasation).
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