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Updated: Sep 3, 2026
In Situ Detection of Autoreactive CD4 T Cells in Brain and Heart Using Major Histocompatibility Complex Class II Dextramers
Published on: August 1, 2014
[Severe combined immunodefficiency disease, 4 autopsy case reports]
Objective:
To study the pathological changes of severe combined immunodefficiency disease (SCID).
Methods:
4 cases with SCID proven by autopsy examination.
Results:
All patients were male with an average age of 5 moths. The major clinical manifestations included severe repeated infection with bacteria, virus, fungi and pneumocystis carinii. Autopsy examination revealed hypoplasia of thymus, spleen and lymph nodes. 1 case died from pneumocystis carinii pneumonia and BCG vaccine disease, 2 cases died of severe general infection and 1 case died from large cell lymphoma.
Conclusions:
The main diagnosis criteria for SCID are hypoplasia of both T and B lymphocytes, the pathologic changes of SCID are very complex.
Insights
This study details the complex pathological changes in severe combined immunodeficiency (SCID) in four male infants. Key findings include immune system hypoplasia and varied causes of mortality, highlighting diagnostic challenges.
Area of Science:
- Immunology
- Pathology
- Pediatrics
Context:
- Severe combined immunodeficiency (SCID) is a group of rare genetic disorders characterized by profound defects in both T and B lymphocytes.
- Early diagnosis and treatment are crucial for survival in SCID patients.
Purpose:
- To investigate the pathological alterations observed in four cases of severe combined immunodeficiency (SCID) confirmed by autopsy.
- To correlate clinical manifestations with autopsy findings in SCID.
Summary:
- Autopsy examination of four male infants (average age 5 months) with SCID revealed significant hypoplasia of the thymus, spleen, and lymph nodes.
- Clinical presentations included recurrent severe infections from bacteria, viruses, fungi, and Pneumocystis carinii.
- Mortality causes varied, including Pneumocystis pneumonia, BCG vaccine disease, generalized infection, and large cell lymphoma.
Impact:
- This study underscores the complex pathology of SCID and the importance of recognizing its diverse clinical and pathological features.
- Findings contribute to a better understanding of SCID, aiding in diagnosis and management strategies for this severe primary immunodeficiency.
