Hodgkins Lymphoma

The first case of definite Hodgkin's disease (HD) developing in a patient with primary SS has been reported in 1990 [136], This association had been mentioned previously [10, 14, 29], but it had not been completely documented nor had the histological features been entirely conclusive of HD. Vivancos et al. [96] described in 1992 two additional cases of histologically proven HD occurring in patients with a previous clinical and histological diagnosis of primary SS. These two patients had different histological types of HD: lymphocytic depletion and mixed-cellularity

Year Author Ref. Type

1990 Martin-Santos et al. [136] Lymphocytic predominance

1992 Vivancos et al. [96] Lymphocytic depletion

1992 Vivancos et al. [96] Mixed-cellularity

1993 Nagai et al. [37] Mixed-cellularity

1998 Ramos-Casals et al. [19] Mixed-cellularity 1998 Ramos-Casals et al. [19] Lymphocytic depletion

NA: not available.

types, and a lymphocytic predominance type was found in the first published case [136]. Other studies reported additional cases of SS and HD (Table 5) [19, 137, 138],

It is more difficult to give a convincing explanation about a pathogenetic relationship between primary SS and HD. In fact, the cause of HD is still unknown. Recent studies strongly support a B-cell origin for Reed-Sternberg cells, and one could argue that in patients with SS there might be a pathogenetic predisposition to the development of HD similar to that described for B-cell lymphoma. In contrast to this speculation, however, we must bear in mind that the incidence of NHL in patients with SS is much greater than that expected in the general population, whereas the incidence of HD in this disorder appears to be very low. Therefore, this might be only a casual relationship. Nevertheless, it seems reasonable to include HD in the clinical spectrum of the lymphoproliferative disorders that may occur in the course of primary SS.

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