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Cutaneous T-cell lymphoma

Author: Vanessa Ngan, Staff Writer, 2006.

Cutaneous T-cell lymphoma — codes and concepts

What are cutaneous T-cell lymphomas?

Lymphomas are tumours of the lymph nodes and lymphatic system. Extranodal lymphomas are tumours that occur in organs or tissues outside of the lymphatic system. When lymphomas occur in the skin with no evidence of disease anywhere else at the time of diagnosis, they are called ‘primary’ cutaneous lymphomas. There are many different types of primary cutaneous lymphomas but they can be broadly divided into two categories, cutaneous T-cell lymphomas and cutaneous B-cell lymphomas. Of all primary cutaneous lymphomas, 65% are of the T-cell type.

Cutaneous T-cell lymphomas (CTCL) refer to a serious but uncommon skin condition in which there is an abnormal neoplastic proliferation of lymphocytes with a ´T´ subtype (thymus-derived). The diagnosis is made by skin biopsy.

Classification of cutaneous T-cell lymphomas

Recently the World Health Organisation (WHO) and European Organization for Research and Treatment of Cancer Classification (EORTC) reached a consensus classification for cutaneous lymphomas [1] and revised by WHO in 2008 [2]. Cutaneous T-cell lymphomas are broken down into the following classifications.

Indolent (low-grade/slow growing) clinical behaviour Aggressive clinical behaviour
  • Mycosis fungoides (MF)
  • MF variants and subtypes
    • Folliculotropic MF
    • Pagetoid reticulosis
    • Granulomatous slack skin
  • Primary cutaneous CD30+ lymphoproliferative disorders
    • Primary cutaneous anaplastic large cell lymphoma
    • Lymphomatoid papulosis
  • Subcutaneous panniculitis-like T-cell lymphoma (histiocytic cytophagic panniculitis), in which there is no involvement of superficial parts of the skin (epidermis, dermis). T cells usually have αβ phenotype, CD3+, CD4-. Patients present with nodules on lower extremities and trunk and with systemic symptoms such as fever and malaise. Haemophagocytosis may be seen on histology (blood cells are found within phagocytes).
  • Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma, which usually presents with a single lesion and progresses slowly.
  • Sézary syndrome
  • Adult T-cell leukaemia/lymphoma
  • Extranodal NK/T-cell lymphoma, nasal type; this is associated with Epstein Barr virus
  • Primary cutaneous peripheral T-cell lymphoma, unspecified
    • Primary cutaneous aggressive CD8+ T-cell lymphoma
    • Cutaneous γ/δ T-cell lymphoma

Mycosis fungoides is the most common type of CTCL and accounts for almost 50% of all primary cutaneous lymphomas. The second most common group of CTCL is primary cutaneous CD30+ lymphoproliferative disorders.

Mycosis fungoides

Mycosis fungoides is a condition in which the skin is infiltrated by patches or lumps composed of white cells called lymphocytes. It is more common in men than women and is very rare in children. Its cause is unknown but in some patients, it is associated with a pre-existing contact allergic dermatitis or infection with a retrovirus.

Mycosis fungoides has an indolent (low-grade) clinical course, which means that it may persist in one stage or over years or sometimes decades, slowly progress to another stage (from patches to thicker plaques and eventually to tumours).

The name mycosis fungoides is historical and confusing: cutaneous T-cell lymphoma has nothing to do with fungal infection.

Patch stage

In patch stage mycosis fungoides, the skin lesions are flat. Most often there are oval or ring-shaped (annular) pink dry patches on covered skin. They may spontaneously disappear, remain the same size, or slowly enlarge. The skin may be atrophic (thinned), and may or may not itch. The patch stage of mycosis fungoides can be difficult to distinguish from psoriasis, discoid eczema or parapsoriasis.

Patch stage mycosis fungoides

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Poikiloderma atrophicans vasculare

Poikiloderma atrophicans vasculare is an unusual variant in which the skin shows areas of thinning, pigmentary change and dilation of the capillaries (telangiectasia).

Poikiloderma vasculare atrophicans

Plaque stage

In plaque stage mycosis fungoides, the patches become thickened and may resemble psoriasis. They are usually itchy.

Plaque stage mycosis fungoides

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Tumour stage

In tumour stage mycosis fungoides, large irregular lumps develop from plaques, or de novo. They may ulcerate. At this stage, spread to other organs is more likely than in earlier stages. The tumour type may transform into a large cell lymphoma.

Tumour stage mycosis fungoides

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Mycosis fungoides variants and subtypes

There are a number of variants and subtypes of mycosis fungoides (MF). Most follow the same clinical and pathological features as mycosis fungoides but some have distinctive features as described below.

Variant/subtype Clinical/pathological features
Folliculotropic MF (follicular cell lymphoma)
  • The localised form of cutaneous T-cell lymphoma in which there is a slowly enlarging solitary patch, plaque or a tumour in which biopsy shows characteristic lymphomatous change around hair follicles.
  • Most commonly found in the head and neck area.
  • Skin lesions are often associated with alopecia, and sometimes with mucinorrhea (see alopecia mucinosa).
Pagetoid reticulosis
  • Localised patches or plaques with an intraepidermal growth of neoplastic T cells.
  • Presents as a solitary psoriasis-like or hyperkeratotic patch or plaque, usually on the extremities.
Granulomatous slack skin
  • Extremely rare subtype characterised by the slow development of folds of lax skin in the major skin folds.
  • Skin folds show a granulomatous infiltrate with clonal T cells.
  • Occurs most commonly in the groin and underarm regions.
Mycosis fungoides palmaris et plantaris
  • Confined to palms and soles.

Primary cutaneous CD30+ lymphoproliferative disorders

This is the second most common group of CTCL and accounts for about 30% of all CTCL cases. This group includes primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis.

Primary cutaneous anaplastic large cell lymphoma Lymphomatoid papulosis
  • Usually solitary or localised nodules or tumours, often with ulceration
  • Multifocal lesions affect about 20% of patients
  • Lesions may show partial or complete spontaneous regression
  • Regional lymph nodes may become involved in 10% of patients
  • Prognosis is generally favourable
  • Chronic, recurrent, self-healing papulonecrotic or papulonodular skin disease
  • Lesions occur predominantly on the trunk and limbs
  • Lesions clear spontaneously within 3-12 weeks and may leave behind superficial scars
  • Recurrences occur throughout the disease duration which may be from several months to more than 40 years

CD30+ lymphoproliferative disorders

Sézary syndrome

Sézary syndrome is the name given when T-cell lymphoma affects the skin of the entire body. The skin is bright red, thickened, dry or scaly and usually very itchy. Examination usually reveals the presence of neoplastic T cells (Sézary cells) in the skin, in enlarged lymph nodes, and in peripheral blood. The prognosis of Sézary syndrome is generally poor with a median survival between 2 and 4 years. Most patients die of opportunistic infections that are due to immunosuppression.

Sézary syndrome

Adult T-cell leukaemia/lymphoma

Adult T-cell leukaemia/lymphoma (ATLL), is a serious blood disease in which there are large numbers of circulating atypical cells. It is caused by a retrovirus infection with human T-lymphotropic virus (HTLV I). The condition can be divided into acute and chronic types. Acute ATLL is characterised by skin lesions similar to those found in mycosis fungoides or Sézary syndrome, enlarged lymph glands, high levels of calcium in the blood and bone lesions. Prognosis is poor for this type with survival ranging from 2 weeks to more than 1 year. Chronic ATLL present with skin lesions only and have a longer clinical course and survival, however, this may transform into an acute phase with an aggressive course.

What investigations should be done in cutaneous T-cell lymphoma?

The diagnosis of cutaneous T-cell lymphoma is made clinically and confirmed by a dermatopathologist (see mycosis fungoides pathology). Multiple biopsies are often required to detect the characteristic histopathology changes, especially in the early patch stages. There are characteristic microscopic changes seen on skin biopsy. The diagnosis is often delayed for months or years as early cutaneous T-cell lymphoma can be difficult to tell apart from other skin conditions, particularly eczema.

Enlarged lymph nodes may also be biopsied. Cutaneous T-cell lymphoma can cause harmless swelling, known as ´reactive´ or ´dermopathic´ lymphadenopathy, or result in malignant growth in the lymph nodes.

The blood count is normal in most patients with cutaneous T-cell lymphoma, but an elevated white cell count is characteristic of Sézary syndrome. Some patients may undergo bone marrow aspirate or biopsy.

Patients with advanced cutaneous T-cell lymphoma may have CT or MRI scans to determine whether the disease affects internal organs.

What is the treatment for cutaneous T-cell lymphoma?

Treatment of individual patients varies and depends on the stage, local expertise and available drugs and equipment. The following may be useful.

What is the prognosis of T-cell lymphoma?

Cutaneous T-cell lymphoma may remain confined to the skin for many years, but the abnormal cells may eventually infiltrate other tissues including blood, lymph nodes, lungs, heart, liver, and spleen.

Unlike some other lymphomas, the outlook is generally good. Symptoms can usually be controlled with treatment. However, treatment is not curative.

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