Classification of Diseases – Oncology, 3 rd edition (ICD-O-3). We included all the patients with the codes 9823/3 (B-cell chronic lym- phocytic leukemia/small lymphocytic lymphoma) and 9670/3 (ma- lignant lymphoma, small B lymphocytic NOS). Results: A total of 8480 new cases of malignant hemopathies was recorded between 1997 and 2007. The 1170 new cases of CLL account for 13.25% of all the incident cancer cases recorded during the study period. The world-standardized incidence rate per 100,000 individuals (WSR) was 3.5 (4.7 male, 2.6 female): it regularly increases with age up to 85 years in both genders: 4.0 in 45-54 years, 13.2 in 55-64 years, 24.0 in 65-74 years and 35.9/100.000 in 75-84 years, then slightly decreases to 30.5 in  85 years. All medical records were reviewed: CLL diag- nosis was confirmed by the determination of the Royal Marsden Hospital (RMH) scoring system, clonality of the circulating B-lym- phocytes and typical blood cytology in 809 cases. In 361 cases, the diagnosis was based on absolute blood lymphocytosis ( 4 10 9 /L) for duration of at least 3 months and typical blood lymphocyte mor- phology. There were 677 male patients (58%) and 493 female pa- tients (42%). The mean age at CLL diagnosis was 70.5 years (26-96). 324 patients (27.7%) were  65 years, 371 (31.7%) patients were aged between 66 and 75 and 475 (40.6%)  75 years. RMH was respectively: 5 in 531 cases (65.5%) cases, 4 in 220 (27.2%), 3 in 39 (5%), 2 in 8 (1%) and 1 in 11 (1.3%). We did not observe any significant difference in the incidence of CLL depending on the department. When defined by the presence of fewer than 5000 B- lymphocytes per L, MBL was diagnosed in 156/738 cases (21.13%) (86 male, 70 female; mean age 68 years: 41-91), with a mean of monoclonal B cells of 2.625 (0.072-3.991). MBL was diag- nosed in 1/2 cases in patients with an age 20-29 years, 0/3 for 30-39, 9/37 (24%) for 40-49, 33/115 (21%) for 50-59, 25/181 (14%) for 60-69, 64/284 (23%) for 70-79, 22/105 (21%) for 80-89 and 2/11 (18%) for age above 90 years. Conclusion: These results are useful for the organization and follow-up of medical care. High-quality collected data remains necessary for continuous surveillance and re- search on patients with CLL and MBL. 3.9 Familial CLL in Norway Viggo Jønsson, 1 Bernt Ly, 2 Tom B. Johannesen, 2 Geir E. Tjønnfjord 1 1 Department of Hematology, Oslo University Hospital, Rikshospital, Oslo, Norway; 2 Norwegian Cancer Registry, Oslo, Norway A total of 388 cases of CLL (234 males and 154 females) were recorded by the National Norwegian CLL Registry during a period of 27 months from Oct. 1 st 2007 to Dec. 31 st 2009. This is a 100 per cent statutory registration of new cases based on flow cytometric documentation according to consensus diagnostic criteria of CLL. Cross-check of all 388 cases with the Norwegian Cancer Registry revealed 42 CLL patients (11%, 26 males and 16 females) who had 1 (30) or more than 1 (12) family members with malignant hemato- logical disease (MHD) in 3 or 4 generations related to the CLL proband. Self-reported pedigrees, available in 236 (61%) of the 388 cases, were used to identify older family members who died in the first part of the nineteenth century before the civil registration num- ber came into use. A total of 60 relatives to the 42 CLL probands had MHD: lymphoproliferative disease (49) and myeloproliferative dis- ease (11) in the following combinations: CLL proband – Parents (n = 17): CLL-CLL 6, CLL-NHL IN 3, CLL-NHL AG 1, CLL-NHL NOS 1, CLL-HL 2, CLL-HCL 1, CLL-MM 2, CLL- AML 1. CLL proband – Grandparents (n = 8): CLL-CLL 3, CLL-MM 1, CLL- leukemia NOS 2, CLL-AML 1, CLL-CML 1. CLL proband – Chil- dren (n = 1): CLL-NHL AG 1. CLL proband – Grandchildren (n = 1): CLL-NHL AG 1. CLL proband – Aunt, uncle, cousin or nephew, etc. (n = 25): CLL-CLL 4, CLL-NHL IN 3, CLL-NHL AG 2, CLL-NHL NOS 1, CLL- Tcell-NHL 1, CLL-ALL 2, CLL-HL 2, CLL-MM 2, CLL-AML 4, CLL-CML 2, CLL-myeloid leukemia NOS 1, CLL-leukemia NOS 1. Sib concordance, viz. more affected sibs (n = 8): CLL-CLL 3, CLL-NHL IN 1, CLL-NHL AG 2, CLL-MM 1, CLL-AML 1. Abbreviations: ALL acute lymphoblastic leukemia, AML acute myeloid leukemia, CLL chronic lymphocytic leukemia, CML chronic myeloid leukemia, HL Hodgkin’s lymphoma, HCL hairy cell leukemia, MM multiple myeloma, NHL non-Hodgkin lymphoma, NOS not otherwise specified, IN indolent, AG aggres- sive. Based on detailed pedigrees from each of the 42 CLL probands, the position of affected family members in either the father’s or mother’s line could be determined in 36 cases (19 patrilineal, 17 matrilineal) while in 6 cases both lines were involved. Haldane- Smith’s test for birth order effect 1 was used for calculation of the mean rank by age in the sibship of each CLL proband. The birth order of the CLL probands differed between patrilineal and matri- lineal lines in terms of Haldane-Smith adjusted mean (‘6A’) and its 95% confidence interval: Patrilineal (n = 19): 288 (192 - 282), p = 0.02, mean sibship size = 2.9. In patrilineal lines, the CLL proband is nearly never first in the sibship having a higher birth order range with healthy older sibs. Matrilineal (n = 17): 216 (186-278), p = 0.7, mean sibship size = 2.9. In matrilineal lines, the CLL probands occur randomly in the sibship. Conclusion: Familial CLL (11%, 42 of 388 cases) is related to a wide pleiotypic repertoire of both lym- phoproliferative and myeloproliferative disorders. In the 42 families with 42 CLL probands in addition to 60 affected relatives with MHD, the mean number of patients is 2.4 per family. CLL-CLL and CLL- B-cell NHL kindred are the most frequent combinations, both seen in 16 (27%) of the 60 combinations, respectively. CLL-my- eloproliferative disease is seen in 11 (18%) of the 60 combinations predominated by AML (7 of 11 cases). This cross-checked Norwe- gian material confirms a patrilineal birth order effect in CLL while no such birth order could be seen in matrilineal transmissions. Epige- netic segregation of the MHD-susceptibility is one possible explana- tion of this difference. References 1. Emery AEH. Parental age and birth order. In: Emery AEH (ed.) Methodology in medical genetics. 2 nd Edition, Edinburgh, London, Churchill Livingstone 1986, p. 140-53. Abstracts Clinical Lymphoma, Myeloma & Leukemia Supplement October 2011 S201