Starting from 1980, 182 cases of primary extranodal non-Hodgkin’s lymphoma were diagnosed in our Division.

Gastrointestinal NHL were the most frequent: 66 patients (36%), followed by head and neck (42 = 23%), skin(23 =13%), urogenital tract (16 = 9%), CNS (10 =5%), chest (9 = 5%), spleen (7 = 4%), bone (5 = 3%) soft tissues (4 = 2%).

Median age was 55,4 (range 20–91); median age was lower in chest, bone and intestinal NHL.

77 patients had stage I disease (48,8%), 74 stage II (46,5%) and 8 patients stage III (5%).

In the whole group, 134/182 (73.6%) patients achieved complete remission (CR); CRs were 81.8% in gastroenteric NHL, 75% in urogenital, 69.5% in skin, 66.6% in head and neck.

Overall survival in the two most important groups are as follows: head and neck: 61% at 50 months and 55% at 100 months; gastrointestinal tract: at 50 months was 80%, at 100 months 75%.

When prognostic factors were considered for the whole group, stage and modified I.P.I. (MIPI) were significantly associated with rate of complete remission and overall survival.

Stage I pts had a significantly better rate of complete remission 83.1%, vs. 68.9% for stage II and 37.5% for stage III. Overall survival of stage I pts was significantly better than for patients with stage II or stage III disease (80% vs. 72.5% at 50 months; 80.1% vs. 62.7% at 100 months).

Modified International Prognostic Index, MIPI is a score ranging from 0 to 5 according to age (>60), LDH (over normal values), ECOG P.S. (>1), Ann Arbor stage (II or III), extranodal sites (>1). Thirty six patients had a MIPI score of 0, 80 pts MIPI 1, 50 pts MIPI 2 and 16 had a MIPI score of 3 or more. A significant correlation between MIPI and achievement of CR was shown with 88.8% of pts with MIPI 0 achieving CR, 78.5% in MIPI 1, 28% in MIPI 2 and 25% in MIPI 3 or more. Overall survival for the four groups was as follows: 96%, 75%, 62%, 61% at 50 months; 96%, 70%, 54%, 24% at 100 months.

Topics:
extranodal disease, lymphoma, non-hodgkin, prognostic factors, brachial plexus neuritis, complete remission, craniosynostosis, cytokine release syndrome, electrocorticogram, lung compliance, head and neck

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2005, The American Society of Hematology
2004
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