Malignant histiocytosis - a rare disease that occurs due to the proliferation of histiocytes.It features a high mortality rate.Manifested lymphadenopathy, malaise, fever.Patients often lose weight.During examination revealed typical malignant processes of bone marrow lesions.
classification of malignant histiocytosis
Now cases of malignant histiocytosis are often classified as positive anaplastic large cell lymphoma Ki-1, often not true histiocytic and T-cell origin.For the treatment of disease using a program designed for malignant lymphomas, respectively individually set the type of tumor.
Integrated diagnostics detect malignant histiocytosis includes:
- submicroscopic method;
- immunohistochemical method;
Tumor cells are characterized by polymorphism.Accepted provide three types of tumor cells:
- cells with vacuolization and basophilic cytoplasm;
- cells with eccentrically located nucleus, having an elongated shape;
- large cells with phagocytosed particlesto erythrophagocytosis.
disease occurs disease in different age groups, from two months to 90 years of age.At the age of 15, girls and boys can develop them with equal probability.Adults, young and middle-aged men are 2.2 times more likely to suffer from this disease than women.
Onset of the disease manifests itself acutely.Symptoms:
- progressing fast weight loss;
- high temperature;
- increasing weakness;
- shortness of breath;
- generalized lymphadenopathy;
- splenomegaly and hepatomegaly.
Many patients with early lesions are detected in the lymph nodes.The second scene of the first frequency of the lesions is the skeletal system.On radiographs near the sprout zones in tubular and flat bones revealed small focal destruction.Macrofocal destruction is flush character is found in the bones around it stretch, or, like multiple myeloma, as multiple lesions.
The skin abnormality occurs in 10-15% of cases.This pathology is similar to that seen with histiocytosis X, but malignant tumors deep layers of skin are affected more clearly how and subcutaneous tissue.Formed knotted or blyashkovidnye purple or brown infiltrates, which are often at the center of ulcerate and disintegrate, especially in the generalization.You may experience papular rash with the same characteristics.
diagnose malignant histiocytosis is very difficult.In half the cases the diagnosis can be verified by a bone marrow puncture, in which found total or partial infiltration of immature atypical histiocytes.Used liver biopsy, skin, spleen.The affected tissues contain proliferative in the skin appear patchy infiltrates located perivascular and perifollicular.
in different tissues detected tumor cells of different sizes, with polymorphic nuclei and broad eosinophilic cytoplasm, nucleus often bean-shaped, the edge position in the cell.The cytoplasm may contain phagocytosed material.Can be found cells with ugly, lobed nuclei, there are giant multinucleated.Figures
different dividing cells including atypical.Infiltrates can include eosinophils, neutrophils, lymphocytes, plasma cells.Often there are necrosis.Subcutaneous tissue is affected panniculitis with characteristic focal lipogranulemami.
peripheral blood can be characterized by the presence of pancytopenia and atypical gistiomonotsitarnyh cells, usually in the terminal stage.
treatment of this rare disease is very complex, with little chance of success.Mortality of malignant histiocytosis - the highest.Used combined intensive chemotherapy.It allows you to prolong the patient's life up to 46 months.