Peripheral T cell lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

The peripheral T-cell lymphomas (PTCLs) consists of a heterogeneous group of aggressive diseases that constitutes ~10%-15% of non Hodgkin lymphoma. The incidence has been estimated to be <1 case per 100,000 of ppl in United states. The most common type is Peripheral T cell lymphoma, not otherwise specified (PTCL, NOS), followed by Anaplastic large cell lymphoma, primary systemic type (ALCL) , Angioimmunoblastic T cell lymphoma (AITL) and Extranodal NK/T cell lymphoma, nasal type (PTCLs ), which is the rarest type. Although each type presents differently and has special histological features, they all have similar treatment . In addition, all the types have been associated with poor prognosis. The most common presentation is generalized lymphadenopathy with or without extra nodal manifestations.

Historical Perspective

• [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
• In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
• In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

clinical trials.

Classification

In 2008, WHO classified peripheral T cell lymphoma in 4 groups ( cutaneous, nodal, extra nodal and leukemic) based on their clinical manifestations .

In which the majority of aggressive T-cell lymphomas have been included in the nodal, extranodal, and leukemic groups.

The nodal lymphoma consists of 3 entities which include :PTCL, not otherwise specified (NOS) which is the most common subtype accounting for 25.9% of cases, anaplastic large cell lymphoma (ALCL), and angioimmunoblastic T-cell lymphoma (AITL).

Anaplastic large cell lymphoma (ALCS) has two recognized sub groups: ALK+ and ALK- lymphomas. Both subtypes are morphologially and immunophenotypically similar to each other however they are different molecularly. They have similar incidences (6.6% and 5.5% respectively)

Angioimmunoblastic T-cell lymphoma (AITL) comprises 18.5% of cases . One of its variants ,lymphoepithelioid cell variant is consists of CD8 T cells with epithelioid cells in the background which is different than other variants.

Less common entities have been described in extra nodal groups. Tissue tropism has been the characterized distinguishing factor.

First subtype which comprises around 1.4% of PTCL cases and almost always is reported in children and young adults is Hepatosplenic γδ T-cell lymphoma. In this tumor, immature or nonactivated γδ T cells infiltrate bone marrow sinusoides and spleen and Isochromosome 7q chromosomal abnormality is present.

In second subgroup, which is hepatosplenic T-cell lymphoma has often poor prognoses with median survival of below 2 years. Patients usually present with B-symptoms and decreased cell counts. The cells express CD2, CD3 and CD7 markers and lack CD4, CD5 . while CD8 and natural killer cell markers expression are variable .

The third sub-type , Enteropathy-associated T-cell lymphoma (EATL) is more common in populations with high incidence of celiac disease and accounts for 4.7% of cases of T-cell lymphoma. Pain, weight loss, and bowel perforation are the usual clinical presentations. Two morphological variants have been recognized ; pleomorphic and monomorphic. The former group is associated with celiac disease the cells usually express CD3 and CD7 and lack CD56 expression . Conversely, The latter group is often not associated with the celiac disease and express CD56. Gains at chromosome 9q33-q34 has been reported in up to 70% of cases.

Furthermore, the intestinal T- and NK-cell lymphomas are part of the nasal-type NK-/T-cell lymphoma which have been reported in Asian countries and are associated with Epstein-Barr virus (EBV) infection .

In addition, The mucocutaneous γδ T-cell lymphomas,the nasal type NK-/T-cell lymphomas, and even ALCLs all can present as an intestinal lymphoma.

Panniculitis-like T-cell lymphomas constitute only 0.9% of PTCLs which is more common in males. The characteristic clinical presentation is subcutaneous nodules that can become necrotic .The cells express CD3 and CD8 and are CD4- .

The last group,leukemia has 4 subtypes including adult T-cell lymphoma (ATL) associated with human T-lymphotropic virus type I (HTLV-1), T-cell chronic large granular lymphocytic (LGL) leukemia associated with neutropenia , aggressive NK-cell leukemia, and T-cell prolymphocytic leukemia. . NK-cell leukemia and ATL often have a poor outcome.

Other types which have been included in WHO classifications include: hydroa vacciniforme-like lymphoma, NK-cell lymphoma and systemic EBV-positive T-cell lymphoproliferative disease of childhood.

Pathophysiology

• The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
• The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
• On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Clinical Features

Depending on the subtypes, clinical manifestations may vary however, the majority of patients present with generalized lymphadenopathy with or without extranodal disease . Having nodal disease alone is seen in around 38% of patients, 49% present with both nodal and extranodal disease, while around 13% only present with extranodal disease . Hepatomegaly is seen in 17% and splenomegaly in 24% of patients. The bone marrow involvement is seen in 20%. Some patients present with eosinophilia, pruritus]. In 1/4 of cases, thrombocytopenia and anemia are seen.

Extranodal sites involved are mainly skin and gastrointestinal tract . Fever, night sweats and weight loss which are typical systemic B symptoms ,are reported in 35% of cases . 50% of patients have increased LDH level and 14% of cases are associated with hypergammaglobulinemia.

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The incidence of PTCL is < 1 case per 100 000 people in the United States. The most common subtypes are the nodal T-cell lymphomas, with PTCL-NOS (25.9%) followed by AITLs (18.5%) and the ALCLs (12%). NK-/T-cell lymphomas (10.4%), while enteropathy-associated T-cell lymphomas (4.7%), hepatosplenic T-cell lymphomas (1.4%), and panniculitis-like T-cell lymphomas (0.9%) .

Age

• Patients of all age groups may develop [disease name].

• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

• NK-cell nasal and nasal-type lymphomas is more common in males than in females.

Race

• PTCL-NOS is more common in North America and less common in the European and Asian countries; AITL is more common in Europe than in Asia or North America; ALK+ ALCL is more common in North America; ALK− ALCL is slightly more common in Europe; and the NK-/T-cell lymphomas and ATL are more common in Asia. The EBV-associated lymphoproliferative, T-cell, and NK-cell neoplasms are seen mainly in Japan, Korea, and Northern China, but also in Native American populations from Central and South America. NK-cell nasal and nasal-type lymphomas is more commonly reported in Asia and Latin America

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].