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)

A 43 year old man with diabetes insipidus who showed panhypopituitarism and marked hypergammaglobulinemia due to histiocytosis X is reported. His low basal plasma adrenocorticotropin (ACTH) and growth hormone (GH) failed to respond to insulin-induced hypoglycemia. His basal serum thyroid hormone level was below normal and normal basal plasma thyrotropin (TSH) showed a delayed response with normal peak value to TSH-releasing hormone (TRH). Normal basal plasma pituitary gonadotropin also showed a delayed response with normal peak value to luteinizing hormone-releasing hormone (LH-RH). Suppression of plasma prolactin (PRL) by levodopa (l-dopa) was impaired and elevation of basal plasma PRL was noted at the second admission. These results, combined with diabetes insipidus, suggested that the panhypopituitarism in these patients was hypothalamic in origin. The polyclonal hypergammaglobulinemia was characterized by elevated serum IgG and IgE levels which returned to normal after corticosteroid treatment with concomitant clinical improvement. Elevated serum IgE levels, tissue and peripheral eosinophilia, and the effectiveness of corticosteroid therapy support the hypothesis that some allergic mechanism may be involved in the pathogenesis of this disease.
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PMID:A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E. 22 67

Histiocytosis X is a disease of histiocyte proliferation in response to some unknown etiology. Thyroid involvement is extremely rare in the literature. In this paper, we present an 18-year-old female with histiocytosis X with thyroid involvement. This patient had had a goiter with normal thyroid function since 12 years of age. A thyroidectomy was done under the suspicion of thyroid cancer. Pathology revealed histiocytosis X. Hypoparathyroidism and hypothyroidism were noted after the operation and were treated with thyroid hormone, vitamin D and calcium carbonate. This patient also had lesions on the left side of mandible, in the suprasellar region and possibly in the right mastoid. A curettage biopsy of her mandibular lesion was also compatible with histiocytosis X. She was proven to have hypothalamic and pituitary dysfunction including hypogonadism and hypoadrenalism. Her thyroid lesion did not recur after the thyroidectomy. The toothache that she had also experienced subsided after the curettage biopsy of the mandibular lesion. Hypothalamic and pituitary dysfunction were controlled by hormone replacement. Because her disease had been running a benign clinical course, no chemotherapy was given.
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PMID:Histiocytosis X with thyroid involvement: report of a case. 136 23