Table 1

Epidemiologic data (n = 303)

CharacteristicsRecords, no.Clinical data
Sex 302 169 females, 133 males 
Year of birth (± SD) 300 1957 (± 17) 
Age at onset, y (± SD) 156 27 (± 17) 
Age at diagnosis 289 35 (± 16) 
Splenomegaly, % 284 40.5 
Lymphadenopathy, % 260 26.2 
Granulomatous disease, % 303 11.6 
Autoimmune phenomena, % 286 20.3 
Autoimmune cytopenia, % 213 20.2 
CharacteristicsRecords, no.Clinical data
Sex 302 169 females, 133 males 
Year of birth (± SD) 300 1957 (± 17) 
Age at onset, y (± SD) 156 27 (± 17) 
Age at diagnosis 289 35 (± 16) 
Splenomegaly, % 284 40.5 
Lymphadenopathy, % 260 26.2 
Granulomatous disease, % 303 11.6 
Autoimmune phenomena, % 286 20.3 
Autoimmune cytopenia, % 213 20.2 

Description of the European cohort, including sex, age, age of onset, age of diagnosis, and associated clinical phenomena of the 303 patients included in this trial. Splenomegaly was defined by ultrasound or CT scan to be larger than 4.7 × 11 cm. Lymphadenopathy was defined by clinical examination or ultrasound or CT scan. Granulomatous disease was judged positive only in histologically proven cases. All others were assumed negative. Autoimmune phenomena other than cytopenia included autoimmune thyroiditis, vitiligo, and pernicious anemia. Autoimmune cytopenia refers to AIHA and AITP.

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