Mushroom Mycosis: Causes, Symptoms, Diagnosis, Treatment

Mushroom mycosis is a rare chronic T-cell lymphoma, initially affecting the skin and sometimes internal organs.

[1], [2], [3], [4], [5], [6]

Symptoms of the mushroom mycosis

Mushroom mycosis is less common than Hodgkin's lymphoma and other types of non-Hodgkin's lymphomas. Mushroom mycosis has a latent origin, often manifesting as a chronic itchy rash, difficult to diagnose. Starting locally, it can spread, affecting most of the skin. Places of damage are similar to plaques, but can manifest as nodules or sores. Subsequently, systemic damage to the lymph nodes, liver, spleen, lungs develops, systemic clinical manifestations are added that include fever, night sweats, unexplained weight loss.

Diagnostics of the mushroom mycosis

The diagnosis is based on the results of a study of skin biopsy specimens, but a histological picture at an early stage may be questionable due to an insufficient number of lymphoma cells. Malignant cells are mature T cells (T4, T11, T12). Characteristic are microabscesses, which can appear in the epidermis. In some cases, a leukemia phase is identified, called the Cesary syndrome, characterized by the appearance of malignant T cells with sinuous cores in the peripheral blood.

Staging of fungal mycosis is performed using CT scan and bone marrow biopsy examination to assess the extent of the lesion. PET can be performed if suspicion of involvement of visceral organs is suspected.

[7], [8], [9], [10]

Treatment of the mushroom mycosis

Radiation therapy with accelerated electrons is highly effective, at which energy is absorbed in external 5-10 mm tissue, and local treatment with nitrogen mustard. To influence the plaques, phototherapy and topical glucocorticoids can be used. Systemic therapy with alkylating agents and antagonists of folic acid leads to a temporary regression of the tumor, but these methods are used in the ineffectiveness of other therapies, after relapse, or in patients with documented extranodal and / or subcutaneous lesions. Extracorporeal phototherapy in combination with chemosensitizers demonstrates moderate efficacy. Promising in terms of effectiveness are adenosine deaminase inhibitors fludarabine and 2-chlorodeoxyadenosine.

Forecast

In most patients, the diagnosis is set at the age of over 50 years. The average life expectancy after diagnosis is about 7-10 years, even without treatment. Survival of patients depends on the stage at the time of detection of the disease. Patients who received therapy at the IA stage of the disease have a life expectancy similar to those corresponding to age, sex and race to people who do not have fungal mycosis. In patients who received treatment for stage IIB disease, the survival rate is about 3 years. In patients treated at stage III of the disease, the average survival is 4-6 years, and in the IVA or IVB stage (extranodal lesions), the survival rate does not exceed 1.5 years.