Xanthoma disseminatum (XD) is one of cutaneous nonhistiocytosis X, and characterized by multiple, widely distributed red, brown, yellowish papules and nodules that tend to coalesce. We present a case of XD with characteristic clinical findings and treated with the combination therapy of COz laser vaporization and high dose steroid.
Histiocytosis, Non-Langerhans-Cell* ; Laser Therapy ; Lasers, Gas* ; Volatilization* ; Xanthomatosis*

By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2-3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive.
Clone Cells ; Histiocytosis, Non-Langerhans-Cell/*blood ; Histiocytosis, Non-Langerhans-Cell/therapy ; Macrophages/*pathology ; gamma-Globulins/therapeutic use

Child, Preschool ; Female ; Histiocytosis, Non-Langerhans-Cell ; diagnosis ; etiology ; therapy ; Humans

By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2-3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive.
Child ; Child, Preschool ; Clone Cells ; Histiocytosis, Non-Langerhans-Cell ; blood ; therapy ; Humans ; Infant ; Macrophages ; pathology ; gamma-Globulins ; therapeutic use

The effect of radiation therapy, either alone or combined with surgery or chemotherapy is accepted well in the treatment of metastatic carcinoma, multiple myeloma, reticulum cell sarcoma and Ewing's sarcoma. But its effect on osteosarcoma and chondrosarcoma is less clear. The authors reviewed 90 patients treated with radiation therapy, 62 cases with primary bone tumor and 28 cases with multiple myeloma, from 1969 to 1988. There were 20 Ewing's sarcoma, 12 osteosarcoma, 12 chondrosarcoma, 9 Histiocytosis-X and 3 reticulum cell sarcoma among 62 primary bone tumors. And 40 patients with more than three months follow-up were analyzed for the primary response of tumor three months after radiation therapy and the long term effect of the radiation therapy. When the radiation therapy was done alone, the primary response was poor in osteogenic sarcoma, chondrosarcoma, Ewing's sarcoma and reticulum cell sarcoma. But with the multimodal therapy, the primary response was rslatively good in reticulum cell sarcoma and chondrosarcoma. In 15 patients, more than one year follow-up was done. The status of these patients at the last follow-up was poor in all cases trearted with radiation therapy alone than the multimodal therapy except Histiocytosis-X. In multiple myeloma, the effect of radiation therapy for the relief of pain was analyzed. There were complete relief of pain in 14.3%, partial relief in 71.4% and no relief in 10.7%.
Chondrosarcoma ; Drug Therapy ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell ; Humans ; Lymphoma, Non-Hodgkin ; Multiple Myeloma ; Osteosarcoma ; Sarcoma, Ewing

Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal disorder of children, affecting predominantly the mononuclear phagocytic system. Previous reports indicate that Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) can also be fatal in many cases, although the prognosis for EBV-HLH is better than for the familial form of hemophagocytic lymphohistiocytosis. We treated four patients with EBV-HLH using immunochemotherapy including steroid, etoposide (VP-16), and cyclosporin, according to the HLH-94 protocol. All patients experienced persistent fever, cytopenia, and hypertriglyceridemia. Serological testing for EBV showed reactivated EBV infections in all patients. EBV DNA detected by PCR and EBV-encoded small RNA measured by in situ hybridization were confirmed in the patients' bone marrow specimens. Hemophagocytosis was shown in bone marrow aspirates and liver biopsy specimen. Complete remission was achieved in all patients after induction and continuation therapy for 4-10 months (median, 7 months) and was maintained for 15-27 months (median, 19 months) without the need for bone marrow transplantation. These results suggest that EBV-HLH can be effectively controlled by immunochemotherapy using the HLH-94 protocol.
Adolescent ; Bone Marrow Transplantation ; Child, Preschool ; Cyclosporine/administration & dosage ; Dexamethasone/administration & dosage ; Drug Therapy, Combination ; Epstein-Barr Virus Infections/*drug therapy ; Etoposide/administration & dosage ; Female ; Histiocytosis, Non-Langerhans-Cell/*drug therapy ; Humans ; Male ; Research Support, Non-U.S. Gov't

Blood Cell Count ; Bone Marrow Cells ; pathology ; Child ; Cytokines ; analysis ; Fever ; Histiocytosis, Non-Langerhans-Cell ; metabolism ; pathology ; therapy ; Humans ; Interleukin-2 ; analysis ; Interleukin-6 ; analysis ; Receptors, Interleukin-1 ; analysis

We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase. Computed tomography (CT) of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly. CT-guided adrenal gland biopsy showed numerous epithelioid cells and infiltration with caseous necrosis consistent with tuberculosis. Bone marrow aspiration and biopsy showed significant hemophagocytosis without evidence of malignancy, hence HPS associated with bilateral adrenal tuberculosis was diagnosed. During anti-tuberculosis treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency, and we added corticosteroid treatment. But pancytopenia, especially thrombocytopenia, persisted and repeated bone marrow aspiration showed continued hemophagocytosis. On treatment day 41 multiple organ failure occurred in the patient during anti-tuberculous treatment and steroid replacement.
Adrenal Gland Diseases/*complications/drug therapy/radiography ; Antitubercular Agents/therapeutic use ; Histiocytosis, Non-Langerhans-Cell/*etiology/pathology ; Human ; Isoniazid/therapeutic use ; Male ; Middle Aged ; Tomography, X-Ray Computed ; Tuberculosis, Endocrine/*complications/drug therapy/radiography

PURPOSE: We assessed the nutritional status and the alterations of oral diets during anti-cancer chemotherapy in pediatric oncology patients. METHODS: Twenty children with malignancy were evaluated from day 0 until day 21 of post-chemotherapy. Nutritional status was assessed by body weight and biochemical parameters. The amount and calories of oral diets were assessed and food preference before and during chemotherapy were analysed by questionnelle. RESULTS: 1) The underlying diseases of 20 patients were 11 acute lymphoblastic lekemia, 2 non-Hodgkin's lymphoma, 2 Langerhans cell histiocytosis, 2 Wilm's tumor, 2 brain tumor, 1 rhabdomyosarcoma. 2) There were weight loss during chemotherapy in 8 patients (40.0%), weigt gain in 5 patients (25.0%), and no significant changes in 7 patients (35.0%). 3) Biochemical parameters showed no significant interval changes during chemotherapy except elevation of serum ALT level. 4) The daily caloric intakes of oral diets during chemotherapy were 310~600 Kcal which was much lower than average of daily recommended calory for Korean children. 5) The most favorate food was altered by chemotherapy, from meats to carbonated beverages and unfavorate food was not altered as vegetables. CONCLUSION: The periodic assessment of nutritional status and dietary supplements according to preferred foods of patients will be required for the optimal nutrition care in cancer patients.
Body Weight ; Brain Neoplasms ; Carbonated Beverages ; Child ; Diet ; Dietary Supplements ; Drug Therapy* ; Food Preferences ; Histiocytosis, Langerhans-Cell ; Humans ; Lymphoma, Non-Hodgkin ; Meat ; Nutrition Assessment* ; Nutritional Status ; Rhabdomyosarcoma ; Vegetables ; Weight Loss ; Wilms Tumor

Defective Viruses ; metabolism ; Epstein-Barr Virus Infections ; diagnosis ; prevention & control ; therapy ; Herpesvirus 4, Human ; isolation & purification ; Histiocytosis, Non-Langerhans-Cell ; diagnosis ; Humans ; Infectious Mononucleosis ; diagnosis ; Leukoplakia, Hairy ; diagnosis ; Lymphoproliferative Disorders ; diagnosis ; Vaccination ; Virus Latency