ALK− anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project

Mucosal CD30-positive T-cell lymphoproliferations of the head and neck show a clinicopathologic spectrum similar to cutaneous CD30-positive T-cell lymphoproliferative disorders

“…This mortality rate in patients with secondary mucosal lesions is similar to that reported in secondary cutaneous involvement by systemic anaplastic large cell lymphoma 4 and also reflects the generally poor prognosis of systemic ALK-negative anaplastic large cell lymphoma (49% 5-year overall survival) compared with either primary cutaneous anaplastic large cell lymphoma (90% 5-year overall survival) or systemic ALK-positive anaplastic large cell lymphoma (70% 5-year overall survival). 5 Skin is a recognized secondary site of involvement by systemic ALKpositive and ALK-negative anaplastic large cell lymphomas, 2,34 but secondary mucosal involvement is less well documented. Two systemic ALK-positive anaplastic large cell lymphomas have been reported that presented clinically with symptoms referable to involvement of the conjunctiva and orbit, respectively.…”

“…Primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis require only local therapy and have 5-year overall survival rates of 90% or higher. 4,5 Secondary cutaneous involvement by systemic anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma has overlapping morphologic and phenotypic features but is treated with systemic combination chemotherapy and has a 5-year overall survival rate of only 49%. 5 CD30-positive T-cell lymphoproliferations also may present at mucosal sites in the head and neck, particularly the oral mucosa, but no stratification system analogous to that used for cutaneous involvement exists to classify these lesions.…”

“…4,5 Secondary cutaneous involvement by systemic anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma has overlapping morphologic and phenotypic features but is treated with systemic combination chemotherapy and has a 5-year overall survival rate of only 49%. 5 CD30-positive T-cell lymphoproliferations also may present at mucosal sites in the head and neck, particularly the oral mucosa, but no stratification system analogous to that used for cutaneous involvement exists to classify these lesions. Terminology to describe mucosal lesions with overlapping morphologic and phenotypic features has ranged from reactive (eg, traumatic eosinophilic granuloma or traumatic ulcerative granuloma with stromal eosinophilia) to malignant (including ALK-negative anaplastic large cell lymphoma).…”

The aggressive peripheral T‐cell lymphomas: 2013

“…The five-year overall survival (70% vs. 49%) and five-year failure-free survival (60% vs. 36%) both favor patients with ALK positive anaplastic Usually Indolent With Extra-nodal Presentation Mycosis fungoides and variants Cutaneous CD30 positive cutaneous lymphoproliferative disorders Lymphomatoid papulosis Cutaneous anaplastic large cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Primary cutaneous CD4-postive small/medium T-cell lymphoma Usually Aggressive-Typically Nodal Presentation Angioimmunoblastic T-cell lymphoma Anaplastic large cell lymphoma ALK positive ALK negative Peripheral T-cell lymphoma -not otherwise specified Adult T-cell lymphoma/leukemia Usually Aggressive-Typically Extra-nodal Presentation Extra-nodal NK/T cell lymphoma, nasal type Enteropathy type intestinal T-cell lymphoma Hepatosplenic T-cell lymphoma EBV positive lymphoproliferative disorders of childhood Primary cutaneous gamma-delta T-cell lymphoma Primary cutaneous CD-8 positive aggressive epidermotropic cytotoxic T-cell lymphoma Typically Leukemic Presentation T-cell prolymphocytic leukemia T-cell large granular lymphocytic leukemia Chronic lymhoproliferative disorders of NK cells Aggressive NK-cell leukemia a Bolded lymphomas are those discussed in this paper annual clinical updates in hematological malignancies: a continuing medical education series large cell lymphoma [25]. However, age was a powerful predictor of survival in patients with ALK positive anaplastic large cell lymphoma and in patients over 40 years of age (i.e., few patients with ALK negative anaplastic large cell lymphoma are younger than 40 years of age) there was no difference in survival between the two subtypes.…”

“…1 and 2) Anaplastic large-cell lymphoma Anaplastic large cell lymphoma is the only aggressive PTCL where a significant group of patients can be cured utilizing CHOP-like regimens. However, CHOP is not an optimal treatment for these patients with 40% of patients with ALK-positive disease and over 60% of patients with ALK-negative disease failing to be cured using this approach [25]. German investigators have reported an improved failure-free survival when patients receive CHOP plus etoposide [40].…”