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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 case of a myasthenic patient with the association of unifocal
histiocytosis X
(eosinophilic granuloma) and lymphoid follicular hyperplasia of the thymus is presented. The combined diagnosis was made on histological grounds and supported by immunohistochemistry. Two aspects of this case are of interest: (1) it is the first reported case of the association of
histiocytosis X
and lymphoid follicular hyperplasia of the thymus in a myasthenic patient; and (2) the pathogenesis of this peculiar association does not seem to be fortuitous but rather might be related to the general derangement of the immune system present in
myasthenia gravis
.
...
PMID:Histiocytosis X and lymphoid follicular hyperplasia of the thymus in myasthenia gravis. 278 37
A patient with
myasthenia gravis
underwent thymectomy following the failure of medical treatment. The thymus was normal except for four nodules, which, on histologic and electron microscopic examination, were found to consist of proliferative clusters of Langerhans' cells characteristic of
histiocytosis X
. This patient therefore had two disease processes in which thymic abnormalities are frequently encountered, but their simultaneous occurrence in the same patient has not hitherto been documented. The case is briefly discussed in the context of current concepts of the pathogenesis of
histiocytosis X
and
myasthenia gravis
.
...
PMID:Histiocytosis X of the thymus in association with myasthenia gravis. 348 73
The pathogenesis of
Langerhans cell histiocytosis
(
LCH
) is unclear, but there is increasing evidence that an immunological dysfunction is present. We describe an infant who presented a
LCH
associated with
myasthenia gravis
. The thymus is known to play an important role in the pathophysiology of
myasthenia gravis
and is often involved in
LCH
. Infancy and signs of organ dysfunction are predictive of poor prognosis: immunotherapy is to be investigated (thymic hormone, cyclosporin, alpha interferon), as well as allogeneic bone marrow transplantation.
...
PMID:[Langerhans cell histiocytosis associated with myasthenia in an infant]. 805 36
The isolated appearance of
Langerhans cell histiocytosis
in the thymus is quite rare. We report the finding of multiple small Langerhans cell nodules in the thymus of an 11-month-old infant who had undergone thymectomy in the course of surgical correction of tetralogy of Fallot. The subtle appearance of some of these nodules and aggregates was unlike the pattern of massive thymic infiltration seen in 7 previously reported cases of thymic
Langerhans cell histiocytosis
in childhood. This difference led us to consider whether these aggregates might be common in the thymus but overlooked in thymus glands removed incidental to cardiac surgery. We examined histologic material from 22 sequential thymectomy specimens removed during cardiac surgery from infants and children. None (0/22) had cohesive aggregates of Langerhans cells, as were seen in the index patient's thymus. Our patient's small thymic nodules resemble more closely a pattern found in adult patients with
myasthenia gravis
who have been incidentally shown to have
Langerhans cell histiocytosis
.
...
PMID:Multiple Langerhans cell nodules in an incidental thymectomy. 1256 39
Langerhans cell histiocytosis
(
LCH
) is a neoplastic proliferation of Langerhans cells that occurs in a range of nodal and extranodal sites. Scattered reports of
LCH
within the thymus exist, typically among children within the setting of multifocal, multisystem disease. Rare cases of isolated
LCH
involving the thymus have occurred in adult patients with
myasthenia gravis
. We report a case of unifocal
LCH
involving the thymus in a middle-aged woman with a history of a resected leiomyosarcoma but no evidence of
myasthenia gravis
. Computed tomographic scans revealed an anterior mediastinal mass, which was excised and measured 9.0 cm. Histologic and immunophenotypic findings (CD1a, S100, and Fascin positive and CD68 negative) were consistent with
LCH
. To our knowledge, this is the first example of
LCH
occurring in a patient with a history of soft tissue sarcoma and one of the rare reported examples of
LCH
presenting as a large isolated lesion in the thymus of a nonmyasthenic adult.
...
PMID:Langerhans cell histiocytosis involving the thymus. A case report and review of the literature. 1282 60
Langerhans cell histiocytosis
(
LCH
) is an idiopathic group of reactive proliferative diseases linked to aberrant immunity, pathologically characterized by clonal proliferation of Langerhans cells.
LCH
rarely involves the thymus. We report a case of thymic
LCH
with diabetes insipidus as the first presentation, without evidence of
myasthenia gravis
and without evidenced involvement of the skin, liver, spleen, bones, lungs and superficial lymph nodes. This present case may have important clinical implications. In screening for
LCH
lesions, attention should be attached to rarely involved sites in addition to commonly involved organs. Follow-up and imageological examination are very important to a final diagnosis.
...
PMID:A case of thymic Langerhans cell histiocytosis with diabetes insipidus as the first presentation. 2327 40
