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CHAPTER 18 - Mature T- and NK-cell neoplasms
Chapter 18. MATURE T- AND NK-CELL NEOPLASMS
Mature T- and natural killer (NK)-cell neoplasms are a heterogeneous group of disorders arising from neopla- stic proliferation of lymphoid cells with mature T or NK phenotype, possibly involving the peripheral blood, bone marrow, lymph nodes, liver, spleen, and skin. A detailed morphological, immunophenotypical and cytogenetic/ molecular examination will enable the correct identification of the clinicopathological entity (Tables 1-3). Mature T- and NK-cell neoplasms include chronic lymphoid leukemias and lymphomas.
Unlike mature B-cell neoplasms, mature T-cell neoplasms often show irregular-shaped nuclei and focal cyto- plasmic reactivity for acid hydrolases; there is also frequent skin involvement.
All small T-cell lymphoid neoplasms may have a leukemic presentation. They have post thymic phenotype; however, their antigenic profile often differs from that of normal mature T lymphocytes. Because immunophe- notypic methods cannot define clonality in T-cell processes, molecular studies of DNA should be performed in problematic cases.
Table 1.  01   orld Health  rganiza on classi ca on of mature T  and natural  iller (  ) cell neoplasms (Swerdlow et al., 2016).
   T cell prolymphocy c leu emia
   T cell large granular lymphocy c leu emia
    hronic lymphoprolifera ve disorder of    cells 
    ggressive    cell leu emia
   Systemic  pstein  arr virus (   ) posi ve T cell and    cell lymphoprolifera ve diseases of childhood
Systemic EB -posi ve T-cell lymphoma of childhood
Chronic ac ve EB  infec on of T- and NK-cell type, systemic form Hydroa vacciniforme-like lymphoprolifera ve disorder
Severe mosquito bite allergy
    dult T cell leu emia/lymphoma
    xtranodal   /T cell lymphoma  nasal type
   Intes nal T cell lymphoma
Enteropathy-associated T-cell lymphoma
Monomorphic epitheliotropic intes nal T-cell lymphoma
Intes nal T-cell lymphoma, not otherwise speci ed (NOS)
Indolent T-cell lymphoprolifera ve disorder of the gastrointes nal (GI) tract
   Hepatosplenic T cell lymphoma
   Subcutaneous panniculi s li e T cell lymphoma
   Mycosis fungoides
   S zary syndrome
   Primary cutaneous  D 0 posi ve T cell lymphoprolifera ve disorders
Lymphomatoid papulosis
Primary cutaneous anaplas c large cell lymphoma
   Primary cutaneous peripheral T cell lymphomas  rare subtypes
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 lymphoprolifera ve disorder
   Peripheral T cell lymphoma  not otherwise speci ed (  S)
    ngioimmunoblas c T cell lymphoma and other nodal lymphomas of T follicular helper cell origin
Angioimmunoblas c T-cell lymphoma
Follicular T-cell lymphoma
Nodal peripheral T-cell lymphoma with T-follicular helper phenotype
    naplas c large cell lymphoma   L  posi ve
    naplas c large cell lymphoma   L  nega ve
    reast implant associated anaplas c large cell lymphoma 
*Provisional en ty
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