t cell lymphoma surviva | DermNet New Zealand Cutaneous T cells lymphoma

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Cutaneous T cells lymphoma



What are T-cell cutaneous lymphomas?
Lymphomas are tumors of the lymph nodes and the lymphatic system. Extraganglionnaires lymphomas are tumors that appear in organs or tissues outside the lymphatic system. When lymphomas occur in the skin with no signs 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 can be divided into two categories, T-cell cutaneous lymphomas and B-cell cutaneous lymphomas. Of all primary cutaneous lymphomas, 65% are of the T-cell type.

Cutaneous T-cell lymphomas (CLO) refer to a serious but rare skin condition in which there is an abnormal neoplastic proliferation of lymphocytes with a Sous-type'T ' (thymus derived). The diagnosis is done by skin biopsy.

Classification of T-cell cutaneous lymphomas
Recently, the World Health Organization (WHO) and the European Organization for the research and Treatment of Cancer classification (EORTC) have achieved a consensus classification for cutaneous lymphoma [1] and revised by WHO in 2008 [2]. Cutaneous T lymphomas are divided into the following categories.

indolent Clinical behaviour (low grade/slow growth)

Aggressive Clinical behaviour

Mycosis Fungoides (MF)
MF variants and subtypes
Folliculotrope MF
Reticulosis Pagetoid
Granulomatous Skin Release
Primary cutaneous CD30 + lymphoproliferative disorders
Anaplastic primary cutaneous lymphoma with large cells
Papulosis lymphomatoid
Subcutaneous panniculitis-like T-cell lymphoma (lymphadenitis histiocytosis panniculitis), in which there is no involvement of the superficial parts of the skin (epidermis. dermis). T cells generally have αβ CD3 + phenotype, CD4. Patients have nodules on the lower limbs and trunk and have systemic symptoms such as fever and discomfort. Hemophagocytosis can be seen on histology (blood cells are found in phagocytes).
Primary cutaneous CD4 + small T/mean cell lymphoma pleomorphic, which usually has a single lesion and progresses slowly.
Sezary's syndrome
Adult T cells leukemia/lymphoma
Extraganglionnaire NK/T cell lymphoma, nasal type; It is associated with Epstein-Barr virus and is aggressive
Peripheral T-Cell primary cutaneous lymphoma, without precision
Primary cutaneous CD8 + T aggressive cell lymphoma
γ/δ cutaneous T-cell lymphoma
Mycosis Fungoides is the most common type of CLO and accounts for nearly 50% of all primary cutaneous lymphomas. The second most common group of CLO is CD30 + lymphoproliferative primary skin disorders.

Mycosis Fungoides
Mycosis Fungoides is a condition in which the skin is infiltrated by patches or pieces composed of white blood 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 allergic contact dermatitis or an infection by a retrovirus.

The Mycosis Fungoides has an indolent (low-grade) clinical evolution, which means it can persist in a stage or over the years or decades sometimes, slowly evolve to another stage (thick-plate patches and possibly Tumors).

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

Patch Stage
In the stage mycosis fungoides patch, skin lesions are flat. Most often, there are oval or ring-shaped (annular) pink pityriasis on the covered skin. They may disappear spontaneously, remain the same size, or grow slowly. The skin can 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.

Mycosis Fungoides Stadium Patch

Poikiloderma s Vascular

Poikiloderma of vascular is an unusual variant in which the skin shows thinning areas, pigment change and capillary dilation (telangiectasia).

Poikiloderma Atrophicans Vasculare

Stage Plate

In the stage Mycosis fungoides plate, the stains become thickened and may resemble psoriasis. They are usually itchy.

Stage Mycosis Fungoides Plate

The tumor Stage

In the stage fungoides mycosis tumor, large irregular pieces develop from the plates, or de novo. They can ulcerate. At this stage, the spread to other organs is more likely than in the early stages. The type of tumor can turn into a large cell lymphoma.

Stage Fungoides mycosis tumor

MF variants and subtypes

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

Folliculotrope MF (follicular cell lymphoma)

A localized form of T-cell cutaneous lymphoma in which there is a solitary patch extending slowly. Plaque or tumor in which the biopsy shows the characteristic lymphomateuse change around the hair follicles.

Most often found in the area of the head and neck.

Skin lesions are often associated with alopecia, and sometimes with Mucinorrhea (see Alopecia mucinous).

Patches or plaques localized with intra-epidermal growth of neoplastic T cells.

Presents as a patch or psoriasis in plaques or type hyperkeratosis solitaire. Usually on the ends.

Granulomatous Skin Release

Extremely rare subtype characterized by a slow development of the folds of the skin released in the large folds of the skin.

Skin creases show a granulomatosis infiltration with clonal T cells.

Occurs most often in the groin and axillary regions.

Primary cutaneous CD30 + lymphoproliferative disorders

This is the second most common group of CLO and represents about 30% of all CLO cases. This group includes primary lymphoma with large anaplastic skin cells and lymphomatoid papulosis.

Anaplastic primary cutaneous lymphoma with large cells

Usually solitary or localized nodules or tumors, often with ulceration

Lesions observed in multifocal about 20% of patients

Lesions may appear partial or complete spontaneous regression

Regional lymph nodes can participate in 10% of patients

The prognosis is generally favorable

Chronic. Recurrent, papulonecrotic skin disease or self-healing Papulonodulaire

Lesions occur mainly on the trunk and limbs

Clear lesions spontaneously within the 3-12 weeks, and may leave superficial scars

Recurrences occur throughout the duration of the disease which may be from several months to over 40 years

CD30 + lymphoproliferative Disorders

Anaplastic lymphoma with large cells

Sezary's syndrome

Sezary syndrome is the name given when T lymphoma affects the skin of the whole body. It is also known as the Red Man syndrome because the skin is bright red. The skin is also thickened, dry or scaly and generally very itchy. The examination usually reveals the presence of neoplastic T cells (sezary cells) in the skin, in the lymph nodes and in peripheral blood. The prognosis of Sezary syndrome is generally poor with a median of survival between 2 and 4 years. Most patients die from opportunistic infections that are due to immunosuppression.

Sezary's syndrome

Adult T cells leukemia/lymphoma

Adult T cells leukemia/lymphoma (ATLL), is a serious blood disease in which there are a large number of atypical circulating cells. It is caused by a retroviral infection with the human T-lymphotropic virus (HTLV-I). The state can be divided into acute and chronic categories. Acute ATLL is characterized by skin lesions similar to those found in Mycosis fungoides or sezary syndrome, lymph glands, high levels of calcium in the blood and bone lesions. The prognosis is bad for this type of survival from 2 weeks to more than one year. ATLL presents chronic with only skin lesions and have a more clinical course and survival, but this can turn into an acute phase with an aggressive course.

What investigations should be done in T-cutaneous to lymphoma cells?

The diagnosis of T-cell cutaneous lymphoma is clinically and confirmed by a dermatopathologiste. There are some microscopic changes observed on the skin biopsy. Diagnosis is often delayed for months or years and may require several biopsies, as early cutaneous T-cell lymphoma can be difficult to distinguish from other skin diseases, especially eczema.

Lymph nodes can also be biopsied. Cutaneous T-cell lymphoma can cause harmless swelling, known as nom'reactive ' or lymphadénopathie'dermopathic ', or lead to malignant growth in the lymph nodes.

Blood counts are normal in most patients with T-cell cutaneous lymphoma, but high white blood cell counts are characteristic of Sezary syndrome. Some patients may undergo bone puncture or biopsy.

Patients with advanced T-skin lymphoma may have CT or MRI to determine if the disease affects internal organs.

What is the treatment of T-cell cutaneous lymphoma?

The treatment of individual patients varies and depends on the stage, local expertise and available medications and equipment. The following can be helpful.

Prognosis

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

Unlike other lymphomas, the outlook is generally good. Symptoms can usually be controlled with treatment. However the treatment is non-curative.