1985
DOI: 10.1007/bf00701330
|View full text |Cite
|
Sign up to set email alerts
|

The Omenn's syndrome: Histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100+/T-6+ Langerhans-like cells

Abstract: A 7 month old female infant was affected by a rapidly fatal familial disease highly reminiscent of Omenn's syndrome. She presented with widespread eczematous lesions, hepatosplenomegaly, superficial lymphadenopathy, peripheral blood lymphocytosis, eosinophilia and hyper-IgE. An axillary lymph node was involved by a marked proliferation of T-3 +/T-10-- lymphocytes admixed with S-100+/T-6+/Leu-3a+/Ia + reticular cells which lacked typical LC granules; cell suspension study revealed that 90%-96% of the lymph node… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
3
2

Citation Types

0
16
0

Year Published

1991
1991
2003
2003

Publication Types

Select...
4
1
1

Relationship

0
6

Authors

Journals

citations
Cited by 26 publications
(16 citation statements)
references
References 21 publications
0
16
0
Order By: Relevance
“…Omenn's syndrome is a genetic disorder with recessive autosomal inheritance (1)(2)(3)(4)(5)(6)(7)(8)(9), characterized by lymphocytic infiltration ofthe skin, gut, liver, and spleen, leading to erythroderma and protracted diarrhea with failure to thrive. Blood T lymphocyte and eosinophil numbers are high, but there is marked lymphocyte depletion in the thymus and lymphoid tissues (1)(2)(3)(4)(5)(6)(7)(8)(9).…”
Section: Discussionmentioning
confidence: 99%
See 4 more Smart Citations
“…Omenn's syndrome is a genetic disorder with recessive autosomal inheritance (1)(2)(3)(4)(5)(6)(7)(8)(9), characterized by lymphocytic infiltration ofthe skin, gut, liver, and spleen, leading to erythroderma and protracted diarrhea with failure to thrive. Blood T lymphocyte and eosinophil numbers are high, but there is marked lymphocyte depletion in the thymus and lymphoid tissues (1)(2)(3)(4)(5)(6)(7)(8)(9).…”
Section: Discussionmentioning
confidence: 99%
“…Blood T lymphocyte and eosinophil numbers are high, but there is marked lymphocyte depletion in the thymus and lymphoid tissues (1)(2)(3)(4)(5)(6)(7)(8)(9). The syndrome is clearly due to an intrinsic defect of the lymphocyte lineage since it is curable by bone marrow transplantation (22).…”
Section: Discussionmentioning
confidence: 99%
See 3 more Smart Citations