Malignant histiocytosis is a malignant disease characterized by abnormal proliferation of histiocytes and their precursor cells. Clinically, it is primarily marked by high fever, splenomegaly, and pancytopenia.

bubble_chart Diagnosis

I. Medical History and Symptoms

1. Medical history inquiry: Pay attention to the onset (acute or gradual) and whether it is accompanied by fever, hepatosplenomegaly, lymphadenopathy, and progressive exhaustion.

2. Clinical symptoms: Dizziness, lack of strength, palpitation, irregular fever (mostly high fever); symptoms of tissue/organ infiltration such as jaundice, skin lesions, serous cavity effusion, etc.

II. Physical Examination Findings

Anemic appearance, possible skin petechiae, ecchymosis, nodules, papules, or ulcers; sternal tenderness; hepatosplenomegaly and lymphadenopathy; jaundice and corresponding signs of histiocytic infiltration into tissues/organs in advanced stages.

III. Auxiliary Examinations

1. Blood test: Progressive pancytopenia; smears may show a small number of abnormal histiocytes, occasionally with immature granulocytes or erythrocytes.

2. Bone marrow examination: Mostly hypercellular, with varying numbers of abnormal histiocytes (generally >10%). The detection of multinucleated giant histiocytes is the main diagnostic criterion. Megakaryocytes are often reduced.

3. Histopathological examination: Destruction of normal tissue structure and infiltration by various abnormal histiocytes confirm the diagnosis.

4. Cytochemical staining: Diffuse positivity for acid phosphatase; negative for Sudan black staining; significantly reduced neutrophil alkaline phosphatase.

IV. Differential Diagnosis

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bubble_chart Treatment Measures

1. Supportive treatment: Same as for acute leukemia.

2. Chemotherapy: CHOP regimen: Cyclophosphamide 600mg/m2, doxorubicin 30mg/m2, vincristine 1.4mg/m2, all administered intravenously on day 1; prednisone 40mg/m2, orally on days 1~5. COPP regimen: The administration method is the same as for lymphoma.

3. Bone marrow transplantation: Eligible patients should undergo allogeneic bone marrow transplantation, which may cure some patients.