How is malignant histiocytosis manifested and treated in children?

Malignant histiocytosis is a rare disease that occurs in connection with the proliferation of histiocytes. It is characterized by high lethality. Is manifested by lymphadenopathy, weakness, fever. Patients often lose weight. During the examination, characteristic malignant processes with bone marrow involvement are revealed.

Classification of malignant histiocytosis

Now cases of malignant histiocytosis are often classified as positive anaplastic large-cell lymphomas Ki-1, more often not truly histiocytic, but of T-cell origin. For the treatment of pathology, a program is used, designed for malignant lymphomas, according to the individual type of tumor, respectively.

Complex diagnosis of detection of malignant histiocytosis includes:

• submicroscopic method;
• cytology;
• histology;
• immunohistochemical method;
• cytochemistry.

Tumor cells are characterized by polymorphism. It is accepted to distinguish tumor cells of three types:

• cells with vacuolization and basophilic cytoplasm;
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• cells with an eccentrically located nucleus, having an elongated shape;
• large cells with phagocytosed particles with erythrophagocytosis.

Clinic of the disease

There is a disease in different age groups, from two months to 90 years of age. At the age of up to 15 years, girls and boys can get sick with the same probability. Adult young and middle-aged men are 2.2 times more likely to suffer from this disease than women.

The onset of the disease is acute. Symptoms:

• progressive fast weight loss;
• high temperature;
• growing weakness;
• jaundice;
• shortness of breath;
• diarrhea;
• generalized lymphadenopathy;
• splenomegaly and hepatomegaly.

In many patients, the first lesions are detected in the lymph nodes. The second most frequent place of occurrence of the first lesions is the bone system. On the roentgenogram near the growth zones in tubular and flat bones, small-focal destruction is detected. Large-focal destruction has a draining character, is found on the bone throughout its extent, or, like myeloma, in the form of multiple foci.

On the skin, pathology occurs in 10-15% of cases. This pathology is similar to that seen with histiocytosis X, but with malignant formation, deep layers of the skin are affected more clearly, like subcutaneous tissue. Nodular or plaque-like violet or brown infiltrates are formed, which often ulcerate and disintegrate in the center, especially during generalization. Perhaps the appearance of papular rashes with the same characteristics.

Diagnostic Complexities

It is very difficult to diagnose malignant histiocytosis. In half of the cases, verification of the diagnosis is possible through bone marrow puncture, in which total or partial infiltration is detected by immature atypical histiocytes. A biopsy of the liver, skin, and spleen is used. Affected tissues contain proliferates, in the skin appear focal infiltrates, located perivascular and perifollicular.

In different tissues, tumor cells of different sizes are found, with polymorphic nuclei, broad eosinophilic protoplasm, a nucleus often of a bean-shaped form, an edge position in the cell. The cytoplasm may contain phagocytosed material. Cages with ugly, lobed nuclei can be detected, and giant multinucleated ones also occur.

The figures of cell division are different, including atypical ones. Infiltrates can contain eosinophils, neutrophils, lymphocytes, plasma cells. Often there are necrosis. Subcutaneous fat is affected by panniculitis with characteristic focal lipogranulomas.

Peripheral blood may be characterized by the presence of pancytopenia and atypical histiomonocytic cells, usually in the terminal stage.

Treatment of this rare disease is very complicated, with little chance of success. The lethality of malignant histiocytosis is the highest. Combined intensive chemotherapy is used. It allows to increase the life expectancy of the patient up to 46 months.