Primary cutaneous follicle centre lymphoma classification

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

Overview

Based on the growth pattern, primary cutaneous follicle centre lymphoma may be classified into follicular, diffuse, and mixed pattern.

Classification

Based on the growth pattern, primary cutaneous follicle centre lymphoma may be classified into:

• Follicular
• Diffuse
• Mixed

A. Updated WHO classification (2016)

• Non-Hodgkin lymphoma may be classified according to updated WHO classification into 2 groups:

• B-cell neoplasms
• T-cell and putative NK-cell neoplasms

Updated WHO classification of Non-Hodgkin lymphoma

B-cell neoplasms	T-cell and putative NK-cell neoplasms
Precursor B-cell neoplasm	Precursor T-cell neoplasm
Precursor B-acute lymphoblastic leukemia / lymphoblastic lymphoma (LBL)	Precursor T-acute lymphoblastic leukemia / lymphoblastic lymphoma (LBL)
Mature B-cell neoplasms	Peripheral T-cell and NK-cell neoplasms
Chronic lymphocytic leukemia / small lymphocytic lymphoma	T-cell prolymphocytic leukemia
Monoclonal B-cell lymphocytosis	T-cell granular lymphocytic leukemia
B-cell prolymphocytic leukemia	Chronic lymphoproliferative disorder of NK cells
Splenic marginal zone lymphoma (± villous lymphocytes) Splenic B-cell lymphoma/leukemia, unclassifiable 1. Splenic diffuse red pulp small B-cell lymphoma 2. Hairy cell leukemia-variant	Aggressive NK-cell leukemia
Hairy cell leukemia	Systemic EBV positive T-cell lymphoma of childhood
Lymphoplasmacytic lymphoma Waldenström's macroglubulinemia	Hydroa vacciniforme like lymphoproliferative disorder
Monoclonal gammopathy of undetermined significance (MGUS), IgM IgG/A	Adult T-cell leukemia/lymphoma
Heavy chain disease µ heavy-chain disease ɣ heavy-chain disease α heavy-chain disease	Extranodal T/NK-cell lymphoma, nasal type
Plasma cell myeloma (multiple myloma)	Enteropathy associated intestinal T-cell lymphoma
Solitary plasmacytoma of bone	Monomorphic epitheliotropic intestinal T-cell lymphoma
Extraosseous plasmacytoma	Indolent T-cell lymphoproliferative disorder of the GI tract
Monoclonal immunoglobulin deposition diseases	Hepatosplenic T-cell lymphoma
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)	Subcutaneous panniculitis-like T-cell lymphoma
Nodal marginal zone B-cell lymphoma (± monocytoid B-cells) pediatric Nodal marginal zone B-cell lymphoma	Mycosis fungoides
Follicular lymphoma In situ follicular neoplasia Duodenal-type follicular lymphoma Pediatric-type follicular lymphoma	Sézary syndrome
Large B-cell lymphoma with IRF4 rearrangement	Primary cutaneous CD30 T-cell lymphoproliferative disorders Lymphomatoid papulosis Primary cutaneous anaplastic large cell lymphoma
Primary cutaneous follicle center lymphoma	Primary cutaneous gamma delta T-cell lymphoma Primary cutaneous CD8 aggressive epidermotropic cytotoxic T-cell lymphoma Primary cutaneous acral CD8 T-cell lymphoma Primary cutaneous CD4 small/medium T-cell lymphoproliferative disorder
Mantle cell lymphoma In situ mantle cell neoplasia	Peripheral T-cell lymphoma, NOS * Peripheral T-cell lymphoma, not otherwise characterized
Diffuse large B-cell lymphoma (DLBCL), NOS Germinal center B-cell type Activated B-cell type Primary DLBCL of the central nervous system (CNS) Primary cutaneous DLBCL, leg type DLBCL associated with chronic inflammation HHV81 DLBCL, NOS	Angioimmunoblastic T-cell lymphoma
T-cell/histiocyte-rich large B-cell lymphoma	Follicular T-cell lymphoma
EBV1 DLBCL, NOS EBV1 mucocutaneous ulcer	Nodal peripheral T-cell lymphoma with TFH phenotype
Lymphomatoid granulomatosis	Anaplastic large cell lymphoma ALK positive ALK negitive
Primary mediastinal (thymic) large B-cell lymphoma	Breast implant associated anaplastic large-cell lymphoma
Intravascular large B-cell lymphoma
ALK1 large B-cell lymphoma
Plasmablastic lymphoma
Primary effusion lymphoma
Burkitt lymphoma Burkitt-like lymphoma with 11q aberration
High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements High-grade B-cell lymphoma, NOS*
B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma

B. Classification based on rate of growth

• Non-Hodgkin lymphoma may be classified based on rate of growth into 2 groups:

• Low-grade or Indolent lymphoma
• High-grade or Aggressive lymphoma

Non-Hodgkin lymphoma classification

Grade	Description
Low-grade or Indolent lymphoma	Tend to grow very slowly Tend to be widespread by the time they are diagnosed Often involving the bone marrow and spleen Often treated only when symptoms appear Can shrink or seem to disappear with treatment, but they tend to come back Can change into more aggressive lymphomas Have a fairly good prognosis
High-grade or Aggressive lymphoma	Grow quickly and tend to spread to lymph nodes or other organs throughout the body Cause symptoms and need treatment right away Can frequently be successfully treated with intensive chemotherapy treatment

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