Malignant histiocytosis?

summary

Malignant histiocytosis is a systemic malignant disease with abnormal proliferation of histiocytes in monocyte macrophage system. The clinical manifestations were fever, emaciation, pancytopenia, enlargement of liver, spleen and lymph nodes. Most of them are young people with acute onset and poor prognosis. Malignant histiocytosis? Let's talk about it

Malignant histiocytosis?

Anaplastic large cell lymphoma (ALCL). According to the classification of lymphocyte markers, most of the malignant cells in this disease are T-cell markers, a few are B-cell markers, and some are non-T-cell and non-B-cell markers, which are called naked cells. A few of them are double labeled with T cells and B cells. All ALCL malignant cells generally express CD30.

Malignant B-cell lymphoma. Malignant B-cell lymphoma is a malignant clonal hyperplasia of large B cells. It is characterized by fever, hepatosplenomegaly, hemophagocytosis and some laboratory abnormalities. However, there is no lymphadenopathy, skin damage and central nervous system invasion.

Histiocytic lymphoma (THL). In recent years, it has been confirmed that THL is a rare lymphoma. The morphology of tumor cells accorded with the characteristics of histocytes; It has malignant characteristics and can be differentiated from reactive histiocytes

matters needing attention

Virus infection is common in patients and immunosuppressants should be used with caution If bacterial infection can be cured, drug sensitivity test should be adjusted in time. Dexamethasone was used daily for 8 weeks. If it was secondary HPS, it could be stopped if the treatment improved. For familial HPS, dexamethasone was injected every 2 weeks for 1 year.