“…We report on the occurrence of progressive splenomegaly after two or more years from diagnosis, in five PPBL cases. This observation is intriguing, since only two PBBL cases with a concomitant or subsequent malignant lymphoma have been reported out of a hundred described (de Haan & Pouwels, 2006) since the first report (Gordon et al , 1982), but none of them documented a correlation between the two conditions (Roy et al , 1998), supporting the notion of the benign course of this condition. More recently, in the largest series of PPBL reporting a long‐term follow‐up (median 4·4 years, range: 0·5–29) (Cornet et al , 2008), only three of 111 patients developed a high‐ or low‐grade lymphoma.…”