04 June 2021
: Case report
Xanthomatous Inflammatory Infiltrate Involving the Spleen: An Unusual Presentation of Erdheim-Chester Disease and Review of the Literature
Rare disease
Gioia Di Stefano12ABDEF*, Massimo Granai3BCDEF, Francesco Giudici4B, Giuliana Roselli5BCE, Stefano Lazzi1AE, Raffaella Santi2ABDEFDOI: 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. | ||||||






