Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver biopsies were done on 20 patients with histiocytosis X (HX) as part of pretreatment evaluation prior to entry on two Childrens Cancer Study Group protocols. Seventeen patients had hepatomegaly, and seven had one or more abnormal laboratory parameters using Lahey's criteria for liver dysfunction. Nineteen of 20 specimens showed various abnormalities of the portal triads. A single biopsy revealed normal liver. Among the changes were triaditis, bile duct proliferation, variable fibrosis with histiocytic infiltrates, and cirrhosis. One patient had typical granulomas of HX within the liver parenchyma in addition to portal triaditis. Patients with larger livers and dysfunction tended to show more marked histologic abnormalities in the portal triads. However, correlations among liver size, function, and pathology showed considerable overlap. Early death among these patients was more likely to be associated with progressive HX in other sites and/or infection. Death from cirrhosis and liver failure per se occurred in one patient 4 years after initial biopsy, but five other children had evidence of cirrhosis on biopsy or at autopsy. The majority of patients with triaditis initially did not have clinical evidence of progressive liver disease although four expired with other manifestations of HX or infection. Conversely, patients showing fibrohistiocytic changes or cirrhosis initially were likely to have continuing or progressive liver disease. Although the liver histology was not diagnostic of HX, the types of portal changes usually predicted the subsequent course of liver disease.
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PMID:Pretreatment liver biopsy in 20 children with histiocytosis X: a clinicopathologic correlation. 240 53

Using a modified method of Con A, LCH or PHA-E affinity crossed-line immunoelectrophoresis, we studied AFP subfractions in 78 sera including 58 from patients with primary hepatoma, 11 from patients with hepatic metastasis of gastric cancer and 9 from patients with germ cell tumors of the gonads (yolk sac tumor, immature solid teratoma or mature solid teratoma). It was found that AFP in primary hepatoma, metastatic hepatoma or germ cell tumors of the gonads were differently glycosylated, and different patterns of AFP subfractions identified by Con A, LCH or PHA-E affinity crossed-line immunoelectrophoresis facilitated a differential diagnosis of such AFP related malignancies.
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PMID:[Serum AFP subfractions in patients with hepatic cancer or germ cell tumor of the gonads]. 241 63

We studied the peanut agglutinin (PNA) staining patterns of histiocytosis X (H-X) (three cases, including one case of Letterer-Siwe disease) and well-characterized cases of malignant histiocytosis (two cases) and monoblastic leukemia (one case) in paraffin-embedded specimens. H-X cells showed staining identical to that of interdigitating reticulum cells, with unique paranuclear and cell surface deposits of reaction products. Thus, H-X cells were easily distinguished from benign macrophage-histiocytes, which show diffuse cytoplasmic staining, and malignant cells of malignant histiocytosis and monoblastic leukemia, which did not stain with PNA, PNA staining proved more useful than Leu-M1 antibody for this purpose, since neither interdigitating reticulum cells in ten reactive lymph nodes nor Langerhans'-type cells in three cases of H-X were stained with Leu-M1 antibody. These observations support a close relationship between H-X cells and interdigitating reticulum cells, and demonstrate the usefulness of PNA for the identification of these cells in paraffin-embedded material.
Cancer 1986 Jan 15
PMID:Peanut agglutinin. A useful marker for histiocytosis X and interdigitating reticulum cells. 241 75

Using a modified method of concanavalin A (Con A), lentil lectin (LCH) or phytohemagglutinin-E (PHA-E) affinity crossed-line immunoelectrophoresis (ACIE), we studied alpha-fetoprotein (AFP) subfractions in 69 sera, including 58 from patients with primary liver cancer and 11 from patients with hepatic metastasis of gastric cancer. We found that Con A non-reactive subfraction (type b) or LCH weakly-reactive subfraction (type B) was more frequently detected in metastatic liver cancer, as compared with liver cancer hepatoma. The amount of Con A non-reactive subfraction (type b) or of PHA-E reactive subfraction (type X) was significantly higher in case of metastatic liver cancer than in primary liver cancer. Since different affinities between AFP and lectins are due to the microheterogeneity in AFP sugar chain, our findings suggest that AFP in primary liver cancer and metastatic liver cancer is glycosylated in a different manner. It is also indicated that different patterns of AFP subfractions identified by the combination of Con A, LCH or PHA-E ACIE facilitate a differential diagnosis of these hepatic malignancies.
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PMID:Serum alpha-fetoprotein subfractions in hepatic malignancies identified by different reactivities with concanavalin A, lentil lectin or phytohemagglutinin-E. 242 Oct 34

In this study fresh frozen tissue samples of benign osseous tumors (five non-osteogenic fibromas, one fibrous dysplasia, one chondromyxoidfibroma), tumors of uncertain biological behaviour (eight cases of histiocytosis X, two giant-cell tumors), and of malignant intraosseous tumors (two malignant fibrous histiocytomas, two malignant histiocytosis, four osteosarcomas, one chondrosarcoma and two Ewing sarcomas) were studied with a panel of monoclonal antibodies reactive with monocyte/macrophages and various types of dendritic cells. In addition, tumors were further defined with a broad spectrum of antibodies against filamentous proteins and lymphocyte differentiation antigens. The specimens were stained with a triple-layer immunoalkaline phosphatase protocol. Tumors stained with these antibodies could be roughly divided into two groups. The first group comprised tumors with one predominant cell population reactive with one particular monoclonal antibody. In this group, cases of histiocytosis X were found to be consistently labelled with CD-1 antibodies. The giant-cell tumors showed a very homogeneous staining with certain monocyte/macrophage antibodies (Ki-M8). Nevertheless, even in these tumors, heterogeneity was demonstrated by the occurrence of cells with monocytic differentiation in histiocytosis X and conversely by the occurrence of cells with differentiation antigens of the dendritic cell system in giant-cell tumors. An exception has to be made for the two cases of malignant histiocytosis examined. These tumors were selectively labelled with antibodies against monocyte/macrophages (Ki-M8, IOM-1). The second group comprised tumors showing a high degree of heterogeneity demonstrated by the varying amounts of tumor cells reacting with the applied markers of the monocyte/macrophage and dendritic cell systems. In most cases it was difficult to ascribe labelled cells to the tumor cell population as opposed to an "innocent bystander" inflammatory cell population. This distinction was especially difficult in malignant fibrous histiocytomas underlining the current concept that these tumors are of primitive mesenchymal rather than true histiocytic origin.
J Cancer Res Clin Oncol 1988
PMID:Histiocytic differentiation in benign and malignant bone tumors. 246 63

Alpha-fetoprotein (AFP) subfractions were studied in 38 sera including 34 patients with primary hepatoma and 4 from patients with hepatic metastasis of gastric cancer. Fractionation of AFP was carried out by concanavalin A (Con A) or lentil lectin (LCH) crossed-line affinity immunoelectrophoresis. With use of Con A, fetal-liver-originated subfraction (peak a) was commonly found in both primary hepatoma and metastatic liver cancer, while yolk-sac-originated subfraction (peak b) was detected in 7 of 34 (20.6%) primary hepatomas and 4 of 4 (100%) metastatic liver cancers. With use of LCH, fetal-liver-originated subfractions (peaks A and/or C) were commonly found in both primary hepatoma and hepatic metastasis of gastric cancer, while yolk-sac-originated subfraction (peak B) was found only in metastatic liver cancer. These findings suggest that glycosylation of AFP in primary hepatoma differs from that in hepatic metastasis of gastric cancer. It is also suggested that AFP synthesized in hepatic cancers and fetal liver are differently glycosylated and AFP synthesis of hepatic malignancies are not always retrogenetically expressed, as in case of the fetal liver. Clinically, different patterns of AFP subfraction identified by Con A or LCH crossed-line affinity immunoelectrophoresis facilitate a differential diagnosis of primary hepatoma and hepatic metastasis of gastric cancer, in cases of elevated serum AFP levels. In the current study, attention was also given to the retrogenetic expression of AFP synthesis in hepatic metastasis of gastric cancer.
Cancer 1985 Jan 01
PMID:Serum alpha-fetoprotein subfractions in patients with primary hepatoma or hepatic metastasis of gastric cancer. 257 79

Obstructive jaundice secondary to external compression of the extrahepatic bile duct caused by tumor of non-liver origin was found in 5 of 199 consecutive children with cancer between 1986 and 1988 at the Department of Pediatrics, National Taiwan University Hospital. Of the 5 patients, 2 had non-Hodgkin's lymphoma and the other 3 had acute promyelocytic leukemia, histiocytosis X and neuroblastoma, respectively. Extrahepatic biliary obstruction occurred as part of the initial presentation of malignancy in 3 cases, and later in the course of disease in the other 2 cases. In each instance, abdominal ultrasonography and computed tomography revealed dilatation of intrahepatic biliary trees due to mass compressing effects. A huge multilobulated tumor and multiple enlarged lymph nodes near the porta hepatis were found in all 3 patients who underwent an exploratory laparotomy. Wedge biopsy of the liver showed no cancer cell invasion. One case died before chemotherapy had commenced. The other 4 patients received chemotherapy and 3 of them received additional radiotherapy. Although jaundice and tumor regressed dramatically with this mode of treatments, subsequent recurrence of tumor without jaundice rapidly ensued in 3 patients. They all died, except 1 case, within 18 months from the occurrence of jaundice. This suggests that these patients were in an advanced stage of disease and should be diagnosed early and treated vigorously. Accordingly, cancer of non-liver origin, although rare, should be considered in the differential diagnosis of obstructive jaundice if survival is to be improved in these cancer children.
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PMID:Extrahepatic biliary obstruction caused by cancer of non-liver origin in children: report of 5 cases. 259 45

Histiocytosis X (HX) is a rare disorder of histiocytic proliferation characterized by a broad spectrum of clinicopathologic disease. An unusual case of Letterer-Siwe disease (LSD) or subacute disseminated HX in a 71-year-old woman is presented. The patient had a 3-year history of splenomegaly before skin lesions developed. She presented to our clinic at 1.5 years later and the diagnosis of HX was made by skin biopsy. Topical nitrogen mustard (NM) therapy resulted in complete clearing of cutaneous lesions. Her condition was stable over the next 10 months. However, she subsequently suffered a rapid and fatal dissemination of her disease. Systemic treatment with prednisone, vinblastine sulphate, and suppressin A (SA) (a calf thymus derived hormone preparation that specifically induces suppressor T-cells) was ineffective. Characteristic histopathologic, immunohistochemical, and electron microscopic findings of HX are illustrated. A review of the adult cases of LSD and treatment options for HX are presented and discussed.
Cancer 1989 Jan 01
PMID:Letterer-Siwe disease in adults. 264 29

We report on a 54-year-old woman with disseminated histiocytosis X who had a complete regression of all mucocutaneous lesions within 1 month from the completion of radiation therapy (4500 cGy) to the hypothalamic-hypophyseal (H-H) area. This response lasted 12 months, after which new cutaneous and bone lesions appeared.
Cancer 1989 Jul 01
PMID:Irradiation of the hypothalamic-hypophyseal area induces complete regression of mucocutaneous lesions in disseminated histiocytosis X. 278 48

Immunosuppressive acidic protein (IAP), a substance purified from cancer ascitic fluid, is released into the culture medium of macrophages and neutrophils, and is supposed to suppress an immune function of the host. Elevation of serum IAP in cancer patients has been reported. In the current study, presence of IAP in proliferating diseases of macrophage was examined. Macrophages in reactive proliferation, histiocytosis X, and neoplastic proliferation gave positive reaction for IAP. Diffuse cytoplasmic staining was usual, but dot-like or globular and surface staining also were observed. Proliferating cells in malignant fibrous histiocytoma were more sensitively stained by IAP than by other ordinary used markers for macrophage. These findings provide useful information to understand the mechanism of elevation of serum IAP in cancer patients.
Cancer 1987 Nov 15
PMID:Immunohistochemical determination of immunosuppressive acidic protein in reactive and neoplastic diseases of macrophage. 282 19


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