Xanthomatous Inflammatory Infiltrate Involving the Spleen: An Unusual Presentation of Erdheim-Chester Disease and Review of the Literature

04 June 2021: Articles

Rare disease

Gioia Di Stefano ^** , Massimo Granai ^* , Francesco Giudici ^B , Giuliana Roselli ^B , Stefano Lazzi ^A , Raffaella Santi ^A

DOI: 10.12659/AJCR.931060

Am J Case Rep 2021; 22:e931060

Table 2. Cases of ECD with spleen localization.

Authors	Sex, age	Clinical presentation	Radiologic investigations	Multisystemic	HP*	Skeletal involvement
Rao et al. (2005)	M, 48	Dry cough and pleuritic chest pain	CT: increased interstitial septal markings, bilateral ground glass infiltrates, and a small right pleural effusion	Yes	Present	–
Busemann et al. (2007)	M, 49	Abdominal distension, weight loss, night sweats and fever, and hepatosplenomegaly	CT: large retroperitoneal mass	Yes	Present	+
Dickson et al. (2008)	F, 77	Progressive dyspnea and peripheral edema, after she presented with pericardial effusion and constrictive pericarditis	CT: mediastinal and lung changes consistent with metastatic carcinoma and multiple suspicious liver lesions	Yes	Not described	–
Gaspar et al. (2017)	F, 24	During puerperium presented with fever, rash, and jaundice	CT: hepatosplenomegaly	Yes	Present	–
HP – hemophagocytosis.