T-cellular lymphoma of skin

Description:

T-cellular malignant lymphoma of skin represent heterogeneous group of diseases which basis of development makes T-limfoproliferativny process which begins in skin, eventually progresses, vovlekayalimfatichesky nodes, peripheral blood and internals.

Symptoms of the T-cellular lymphoma of skin:

The clinical picture is defined by degree of a differentiation of tumor cells. Usually it is mature T lymphocytes with T-helperov properties. They possess the expressed epidermotropizm. Infiltrate lies preferential in a papillary layer of a derma and is shown by spots, plaques with unsharp borders, small knots, an erythrosis. Rashes are followed moknutiy and an itch, leave behind a superficial atrophy of skin.

When progressing process the epidermotroiizm of T lymphocytes decreases, their migratory activity amplifies. As a result infiltrate spreads from a papillary layer of a derma in mesh that leads to emergence of plaques with sharp borders and the nodes inclined to an ulceration.

With increase of weight of process at TKLK in blood of patients the monocytosis, a leukopenia are noted. At the TKLK I erythrodermic form (a pre-Sezara syndrome) in blood are noted a leukocytosis with a neutrocytosis, an eosinophilia, the IgE level is sharply increased; at leukemic option of TKLK II (Cesaria's syndrome) the leukocytosis can reach 30000, is more rare 60000, and sometimes 200000.

TKLK I is clinically shown:

    * nodular,
    * blyashechny
    * erythrodermic forms.

Uzelkovayaforma TKLK 1 (primary reticulosis of skin, nodular form) at a high-quality current proceeds 15-20 years, small knots are located in the form of small accumulations, do not exceed the size of prosyany grain, have the flat form, flesh-colored or lilac color, a smooth and brilliant surface, spontaneously are exposed to regress.

At a malignant current small knots lose a tendency to group, become similar to halves of cherries by the size, a form, coloring, are often transformed to plaques or ulcerate.

In 2-5 years patients die of metastasises.

TKLK 1 Blyashechny form (skin reticulosis primary, blyashechny form; parapsoriasis atrophic). At a high-quality current on site of the slaboinfiltrirovanny, unsharply delimited flesh-colored plaques the size to a palm and more the superficial atrophy of skin with a hyperpegmentation and teleangiectasias develops.

At a process malignancy clinical evolution proceeds as fungoid mycosis or a red chromophytosis of Gebra.

TKLK I erythrodermic form (skin reticulosis primary, erythrodermic form; the pre-Sezara syndrome) develops in 10-15 years against the background of a periodic recurrence of "eczema", "neurodermatitis".

Skin is edematous, hyperemic, plentifully covered with macrolaminar white thin scales. The generalized lymphadenopathy, a hair loss, change of nails on dystrophic type, "lymphoblastoid papules" and infiltrates are observed by the platform; the dermographism becomes white.

The painful itch, the exhausting fever disturb. 3-4 months later process is exposed to full regress.

At a long current in several years there comes death from a cachexia, consecutive infection or process gets a typical clinical picture of a syndrome of Cesaria.

TKLK II is clinically shown by melkouzelkovy, blyashechny, infiltrative and tumoral and erythrodermic forms. Morphologically represents a lymphocytic lymphoma with an epithelioid component.

TKLK II Melkouzelkovy form (skin reticulosis primary, nodular form; mycosis fungoid) meets a follicular mucinosis seldom. Damage of skin develops from follicular small knots. Being grouped in the centers of the wrong outlines, small knots form plaques with unsharp borders, a psoriasiform peeling on a surface. Rashes have no favourite localization. Appearing on some certain site, they quickly accept widespread character, is frequent as an erythrosis. Free are shins and feet, axillary and popliteal spaces. The affected skin hair drop out that leads to a focal or total alopecia.

On such background large small knots which are exposed to a necrosis in the center form and leave stamped scars. Evolution comes to an end with spontaneous traceless regress of one plaques and transformation in flat, a pasty consistence nodes of others.

At widespread process sharp increase in all groups of hypodermic lymph nodes is noted. The weak or moderate itch is subjectively noted.

The TKLK II Blyashechny form (mycosis fungoid Alibera) makes 26% of all lymphoma of skin. The disease begins aged from 30 up to 60 years.

This clinical form most defiantly reflects staging of process.

On the first - an erythematic stage on skin the most various rashes develop. Most often it the edematous, bright pink, shelled spots, sometimes with microvesiculation.

On the second - a blyashechny stage on site of spots there are likhenifitsirovanny plaques the size to a palm, with the erased borders, stagnant-red color. Their surface is deprived of hair, covered with scales or cheshuyko-crusts. quite often with sites a moknutiya. Growth of the centers peripheral, uneven.

In the third - a tumoral stage on site of plaques and on not affected skin flat nodes the size from plum to orange, a soft pasty consistence with a necrosis or an ulceration in the center form. Regional lymph nodes increase. The itch, sometimes painful, especially in the first two stages is subjectively noted.

The TKLK II infiltrative and tumoral form (a reticulosis of skin of Potekayev infiltrative and tumoral) meets extremely seldom, is almost exclusive at men.

Death is caused by the accruing cachexia.

The TKLK II erythrodermic form (Cesaria a syndrome) makes more than 7% of all lymphoma of skin.

TKLK III is shown by nodes from "'the lymphoblasts which are not twisted". Proceeds as fungoid mycosis of Vidalya-Brock or Gottron's reticulum cell sarcomatosis.

At fungoid mycosis of Vidalya-Brock survival makes 2-2,5 years.

The disease begins with development of one or several sharply delimited dense bright pink plaques, within a year which are transformed to nodes which then are cleared away with formation of the ulcers surrounded with the dense roller. The regional lymphadenopathy develops.

Subjectively sick are disturbed by a periodic itch, in process of process generalization - weakness, fever.

At Gottron's reticulum cell sarcomatosis survival less than 2 years.

The disease begins with emergence of one, and then the numerous densely located spots size to coins of different advantage, bright pink coloring. Within 3-4 months against the background of spots plaques and dense nodes of cyanotic-pink color with a brownish shade, petechias, teleangiectasias and lack of growth of hair on a smooth surface form.

Sometimes damage of skin is originally limited solitary очагот, but then quickly enough disseminates. With spread of rashes the regional lymphadenopathy joins. Subjectively sick weakness, fever disturb.

Reasons of the T-cellular lymphoma of skin:

Malignant lymphoma of skin arise preferential at persons of advanced and senile age (from 50 to 80 years) that corresponds to a tendency of development of tumoral diseases at such age. At the same time some authors note more frequent registration of ZLK at persons of young age, even children in recent years. Studying in comparative aspect of average age of initially registered sick ZLK in the Moscow region for the 5-year period (from 1978 to 1983 and from 1993 to 1998) showed that it respectively made 56,7 years and 60,1 years, that is rejuvenation  of this category of patients is noted in recent years.

Treatment of the T-cellular lymphoma of skin:

    Treatment of lymphoma:

    * at TKLK I and the II nodular, blyashechny, infiltrative and tumoral forms appoint photochemotherapy - 32-36 sessions to a course,
    * at an erythrodermic form - Prednisolonum with Chlorbutinum.
    * In places of large folds it is recommended to apply occlusive bandages with corticosteroid ointments limited plaques.
    * At TKLK III and VKLK I of a knotty form the most effectively electron beam treatment by a krupnopolny method with total focal doses of 30-40 Gr.
    * At VKLK II and III carrying out polychemotherapy according to the program is reasonable
          o TsOP - Cyclophosphanum, Oncovinum (Vincristinum), Prednisolonum or
          o TsAOP - Cyclophosphanum, adriamycin, Oncovinum, Prednisolonum (2 cycles with a Z weeks interval).