Objective:

To describe a newly identified 'sponge-like' splenic microtexture pattern in patients with inborn errors of immunity (IEIs) and hematologic immune dysregulation.

Approach:

Key Findings:

• In the index case, a 'sponge-like' pattern was observed using high-frequency ultrasound, characterized by multiple hypoechoic round foci.
• In the IEI cohort, 46.2% had splenomegaly, and 30.8% exhibited a persistent sponge-like pattern without infections or malignancies.
• Only one control patient showed similar features transiently during acute EBV infection.
• Immunomodulation treatments resulted in significant improvement or normalization of the sponge-like pattern.

Interpretation:

Remove unsupported conclusions about histopathological substrate.

Limitations:

• The study is preliminary and requires further validation.
• Limited data availability on splenic microtexture in patients with IEIs due to the infrequent use of high-frequency ultrasound.

Conclusion:

Revise to eliminate implications about clinical practice and focus on findings.

Attribution Notice

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