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LCH Adenitis - Rosai Dorfman Disease
Joseph Junewick, MD FACR
over 6 years ago
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Chronic Granulomatous Disease - Thymus

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: 11 months ago
Updated: 11 months ago
Tags:
Modality/Study Types: CT PET
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History

2 year old male with known CGD


Case Images


Diagnosis

Chronic granulomatous disease – Thymus

Clinical Notes

Liver abscess diagnosed at 1 year of age.

Image-guided thymus sampling produced 12 mL brown fluid; cultures negative.

Findings

CT – Abdomen images show hepatic abscess and left upper quadrant bowel/mesentery inflammation. Chest images show enlarged, heterogeneous thymus.

PET – Central thymus hypometabolic regions corresponding to fluid on CT.

Discussion

Phagocytosis leads to induction of NADPH and activated oxygen compounds. In CGD, phagocytosis is normal although interruption of NADPH prevents killing of ingested bacteria. CGD is secondary to X-linked (~70%) or autosomal recessive (~30%) defects. Patients experience multiple infections and abscesses in skin, bone, lungs, lymph nodes, and gastrointestinal and genitourinary tracts. Typically, bacteria are catalase producing organisms like S. aureus, E. coli, Serratia, Klebsiella, Pseudomonas. Aspergillus leading cause of death; Serratia infection in a child should prompt the diagnosis of CGD. Thymus involvement may be related to antigen presentation to the thymus during T-cell maturation and involvement of the thymus epithelium.

Reference

Godoy MCB, Vos PM, Cooperberg PL, et al. Chest Radiographic and CT Manifestations of Chronic Granulomatous Disease in Adults. AJR (2008); 191:1570-1575.



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