Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report an autopsy case of true malignant histiocytosis that developed during chemotherapy for mediastinal immature teratoma. The patient was a 14-year-old boy who exhibited hepatosplenomegaly while receiving chemotherapy for a mediastinal immature teratoma that had been resected 11 months before. The spleen and liver of the excisional biopsy displayed infiltration of multinucleated giant atypical cells with prominent erythrophagia in massive aggregations. These atypical cells expressed CD68, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, and vimentin, suggesting that the tumor cells may have been derived from macrophages. Immunocytochemistry showed p53 expression in the tumor cells of the malignant histiocytosis, as well as in the elements of the immature teratoma. Direct sequence analysis showed the p53 mutation in the tumor cells of the immature teratoma to be a mutation at codon 175 (exon 5), whereas the mutation in the malignant histiocytosis occurred at codon 285 (exon 8), ie, polyclonality was exhibited and these features suggested that the malignant histiocytosis arose independently from the immature teratoma during the chemotherapy.
 ...
PMID:True malignant histiocytosis developed during chemotherapy for mediastinal immature teratoma. 889 99

In a retrospective study, the authors examined the occurrence of hydrops in foetal and neonatal cases in a five-year period. During this time, the clinical and pathologic diagnosis of hydrops was established in 28 cases. In three cases, the hydrops was caused by Rh incompatibility, and in 25 cases non-immune hydrops was discovered by clinical and pathologic examination. The cause of hydrops was recognised in 25 cases and no underlying disease was discernible in three. In 12 cases, the pregnancy was spontaneously terminated. Artificial abortion was performed in four cases. In this series, four hydropic newborns died on the first week of their life. The average age in artificial abortion was 16-20 gestational week and 24 in spontaneous abortion or stillbirth. Pathologic examination revealed increased weight, oedema, ascites, and hydrothorax in all cases. In half of the cases, there was also hydropericardium. Hepatosplenomegaly, cardiomegaly, pulmonary hypoplasia, increased extramedullary hemopoiesis, and placenta oedema were seen in all the cases. Causes of the non-immune hydrops were cardiac malformation in 4 cases, chromosome anomaly in 3, cystic hygroma in 2, skeletal anomaly in 1, foeto-foetal transfusion in 3, infection in 7 and sacrococcygeal teratoma in 1 case. In two cases, the underlying disease was reported to be of maternal origin. Comparing the clinical and pathologic findings there were only three cases with absolute agreement. The underlying disease revealed by pathologic examination was regarded to be significant from respect of inheritance, therapeutic approach, and further management in 10 cases. The authors emphasise the importance of pathologic examinations in foetal hydrops and point out the significance of clinico-pathological collaboration in the decision about further management of hydropic foetuses.
 ...
PMID:[Hydrops fetalis--a retrospective study]. 1735 Sep 12