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Malignant histiocytosis in children Clinical features


Students of malignant disease,[link widoczny dla zalogowanych], clinical manifestations varied. Early easily misdiagnosed. We report a case admitted to our hospital in recent years. And reviewing literature to discuss its clinical characteristics: a clinical 1.1 General Information October 1992 ~ June 1997 were treated in our hospital 11 cases of malignant histiocytosis: Male 3 down. 8 females down. Age: 1 year and 3 months to 3 years in 4 cases, 6 - 13 years 7 down. The shortest onset to treatment time of 3 days, maximum of 3 years: 1.2 clinical manifestations in 11 cases had fever, hepatosplenomegaly, anemia. Lymph nodes in 9 cases, bleeding in 6 cases. 3 cases of rash, jaundice 3 down. 2 cases of pleural effusion, ascites in 2 cases, 1 case of hemiplegia, 1 case of parotid swelling 1.3 13.1 Haka laboratory blood of three lines are reduced in 6 cases, 2 cases with platelet reduction. Leukocyte reduction in 2 cases, 1 case of leukocyte. Concentration in peripheral blood smears in which a back macrophages found in blood cells and abnormal tissue cells: bone ■ 1.3.2 l0 patients were normal bone marrow biopsy. 7 down myeloproliferative active low 3 hyperplasia and abnormal tissue cells than down 3% - 26.5%. 6 of them were down 3 times more than the parts of the bone marrow biopsy after the diagnosis. 1.3.3 pathological lymph node biopsy taken in 4 cases, 1 case of lymph node biopsy were found after 2 malignant cells. 3 back to the autopsy, the results of extensive invasion of malignant cells worship heart, liver, spleen, kidney,[link widoczny dla zalogowanych], lung, intestine and other organs. 1 case to take pleural effusion, ascites smear after centrifugation did find abnormal cells. 1.3.4 Other tests GPT increased in 3 cases, 5 cases of hypoproteinemia, CRP increased in 4 cases, ESR increase of 2 cases l1 Chest x-ray films were suggestive of interstitial lung changes. 2 to discuss the cause of malignant histiocytosis is not clear, but clinical disease is not uncommon for major violations of the lymphoid tissue, liver, spleen, lung, bone and other organs. King due to different organs have different clinical manifestations, lack of specificity, early misdiagnosis. 1O cases in this group that had been misdiagnosed, the longest 1 was misdiagnosed, asked for 3 years. Misdiagnosed diseases are hemolytic anemia, splenomegaly disease, subacute necrotizing lymphadenitis, infectious mononucleosis syndrome, connective tissue disease, malignant lymph bridle, mumps, hemophagocytic j (total 605) 29 cell histiocytosis . Age: According to reports in the literature the minimum age of onset of the disease 2 months, less than 2 years of age accounted for 33%. 59% less than n years old, the youngest age group of 1 year and 3 months of clinical characteristics: ① malignant cells more acute onset of disease, mostly associated with fever in this group were reported down ¨ fever, irregular fever type fever, intermittent fever and remittent fever. ② liver, spleen, lymph nodes, and often splenomegaly based. The patients had liver and spleen. 7 down to the main splenomegaly (63%). Lymph nodes in 9 cases (82%), including 4 cases of systemic lymph nodes, local lymph nodes (neck, flutter down) 5 down. ③ anemia and (or) bleeding was mainly due to a large number of body cells by the grab Lan, cytotoxicity tangled role of phagocytic cells and hypersplenism caused by other factors. All this group had anemia, of which mild to moderate anemia in 6 cases. Severe in 3 cases. Very severe in 2 cases. ④ also highlighted the performance of multiple organ damage. The liver, the most common renal dysfunction, liver damage can occur jaundice, hypoproteinemia, pleural effusion and ascites was followed: literature chest, abdomen, pericardium, and the cranial cavity can be expressed as serositis,[link widoczny dla zalogowanych], often bloody and serous fibrin exudates. 2 cases of pleural effusion in this group, 2 cases of malignant ascites are bloody exudates nerve tissue cells can be violations of headache,[link widoczny dla zalogowanych], vomiting, convulsions and other performances, the group 1 patients with hemiplegia is also in this category. Another one in this group ever appeared in the parotid gland enlargement, parotid gland may be affected by King, the literature has a similar report. The group 3 patients were autopsy confirmed that Si cells have been widely invaded the internal organs and central nervous system to reduce the misdiagnosis of this disease, we understand: ① malignant histiocytosis short duration, rapid, if not most of the 6 October after treatment in death. Therefore, fever, anemia, liver and spleen and lymph nodes of unknown diagnosis, rapid progression of the disease should take into account the disease. But there are also longer course,[link widoczny dla zalogowanych], where this group of 3 patients the course more than 6 months, 1 case from the onset to death for up to 3 years. ② morphological change is the basis for diagnosis of this disease. So. For suspected cases could not be confirmed to be several times more area to do bone marrow biopsy. If conditions do biopsy pathology, but that the positive rate of less than the former. ⑤ For the chest, abdomen bearing specimens, take centrifugation and sedimentation cell smears, can improve the diagnostic yield. 2 cases in this group by this method is found in malignant pleural effusion cells and confirmed. (Received 1998-09-03 Revised 1998-07-14) [Lvpei Bin braided hair]
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