Cytophagia, particularly erythrophagia, was observed in bone marrow specimens of the patients cited above, but no bone marrow involvement by lymphoma was demonstrated in any of these cases. These findings are in agreement with the literature, extracutaneous involvement by SPTL having only rarely been described.9,16 In addition, autopsy studies2,16,29 of patients who died of CHP or SPTL have rarely been published. Autopsy examination performed in case 1 of our series revealed the presence of hemophagocytic histiocytosis without evidence of lymphoma in multiple organ sites, as previously reported.29 In the 4 cases in which histopathologic findings were consistent with subcutaneous lymphoma, immunohistochemical studies showed a CD2+, CD3+, and CD45RO+ T-cell phenotype of the neoplastic lymphocytes, with evidence of T-cell antigen loss (CD5− and/or CD7−). Two of these cases (patients 6 and 7) proved to be α/β T-cell lymphomas expressing the T-suppressor/cytotoxic cell antigen CD8, which is usually related to aggressive clinical behavior.30,31 The other 2 cases (patients 4 and 5) were γ/δ T-cell lymphomas and expressed the antigen CD56. The CD56 antigen marks the cutaneous NK and NK-like T-cell lymphomas, recently described as a rare subset of aggressive cutaneous lymphomas.32 Interestingly, in all cases the atypical lymphocytes were strongly labeled with the monoclonal antibody directed against TIA-1/GMP-17 (a granule membrane protein of the cytotoxic lymphocytes).33 Expression of this monoclonal antibody has recently been described in several lymphomas regarded as being derived from lymphocytes with cytotoxic potential and thus characterized by aggressive clinical behavior.34 In this regard, this immunohistochemical finding and the others mentioned above could help explain the rapidly fatal course of SPTL. In the other 2 cases (patients 2 and 3), characterized by histopathologic aspects of CHP and absence of clonality, the patients experienced a chronic course and were still alive 6 and 41 years after the disease onset, respectively. These patients lacked all of the clinical features typical of the fatal form of CHP and also presented without pancytopenia. However, such a long duration does not permit the certain conclusion that this is a nonfatal disease, since anaplastic transformation could occur with time, as previously described.24,35 This possibility may also be supported by the observation that both these cases and 2 of those classified as SPTL (patients 6 and 7) initially presented with a spectrum of systemic symptoms, including fever, polyarthritis, and pericarditis, that mimicked an autoimmune disorder. Thus, a lupus-like clinical presentation seems to represent an initial disease stage marking an immunological dysfunction that could be one of the factors promoting the progression to lymphoma.