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Malignant Histiocytosis

What is Malignant Histiocytosis?

Malignant histiocytosis is a very rare malignant process associated with the proliferation of histiocytes. Malignant histiocytosis in childhood) is a rare variant, characterized by high mortality, usually affects children under the age of 3 years, more often than boys.

Causes of Malignant Histiocytosis

The etiology and pathogenesis of malignant histiocytosis are not well understood. There are prerequisites for a discussion about the viral nature of malignant histiocytosis (Epstein-Barr DNA is sometimes found in tumor cells). Many authors consider it a systemic progressive disease of the mononuclear-histiocytic system with neoplastic proliferation of atypical histiocytes and other mononuclear phagocytes. According to the histological classification of malignant neoplasms of the hematopoietic and lymphoid tissues, WHO refers to systemic diseases such as acute leukemia.

Symptoms of Malignant Histiocytosis

Clinically, the disease is manifested by lymphadenopathy and common symptoms such as weakness, recurrent fever, weight loss.

In addition to multiple systemic changes in the lymph nodes, internal organs and bones of the skeleton, characteristic skin lesions are observed – erythmatoid rashes and large brown flaky papules, which quickly ulcerate.

Death occurs in a few months, years (the average life expectancy from the onset of the disease is about 6 months).

Diagnosis of Malignant Histiocytosis

When examining such patients, a lesion of the bone marrow is revealed with characteristic signs of a malignant process. From modern diagnostic positions (immunohistochemically), many cases of malignant histiocytosis are now classified as Ki-1 positive large cell anaplastic lymphomas, predominantly of T-cell origin (i.e., not truly histiocytic origin). In any case, this pathology is treated according to a program for high-grade lymphomas in accordance with the established type of tumor.