Table 1 Diagnoses.
From: Inheritance of Susceptibility to Malignant Blood Disorders
Diagnoses | Familial Diagnoses Observed | Cancer Registries | ||||||
|---|---|---|---|---|---|---|---|---|
ICD-10 code numbers | The Faroe Islands | Norway and Denmark | Norway and Denmark | |||||
Lymphoproliferative Disorders | Pc | Pc | All cases recorded | |||||
Total | (males, females) | % | Total | (males, females) | % | % (mean) | ||
Hodgkin’s lymphoma HL C81 | 19 | (12, 7) | 8.7 | 11 | (8, 3) | 4.0 | 6 | |
Follicular lymphoma FL C82 | 14 | (9, 5) | 6.4 | 19 | (13, 6) | 6.9 | 12 | |
Mantle cell lymphoma MCL C82.7 | 0 | 4 | (4, 0) | 1.4 | <1 | |||
Diffuse Non-Hodgkin’s lymphoma DLBCL C83.3 | 34 | (21, 13) | 15.6 | 16 | (9,7) | 5.8 | 25 | |
Peripheral T-cell lymphoma TNHL C84 | 6 | (2, 4) | 2.7 | 2 | (2, 0) | 0.7 | 2 | |
Monocytoid B-cell lymphoma MONOC C85.7 | 0 | 4 | (1, 3) | 1.4 | 2 | |||
Non-Hodgkin lymphoma NOS NHL NOS C85.9 | 25 | (14, 11) | 11.4 | 9 | (4, 5) | 3.3 | 6 | |
Waldenström’s disease WA C88 | 2 | (1, 1) | 0.9 | 8 | (5, 3) | 2.9 | 3 | |
Multiple myeloma MM C90 | 50 | (31, 19) | 22.8 | 10 | (6, 4) | 3.6 | 14 | |
Acute lymphoblastic leukemia ALL C91.0 | 16 | (10, 6) | 7.3 | 4 | (2, 2) | 1.4 | 4 | |
Chronic lymphocytic leukemia CLL C91.1 | 50 | (29, 21) | 22.8 | 181 | (98, 83) | 65.6 | 22 | |
Prolymphocytic leukemia PLL C91.3 | 2 | (1, 1) | 0.9 | 2 | (0, 2) | 0.7 | 1 | |
Hairy cell leukemia HCL C91.4 | 1 | (1, 0) | 0.5 | 1 | (0, 1) | 0.4 | 1 | |
Large granular T-cell leukemia LGTCL C91.7 | 0 | 3 | (2, 1) | 1.1 | <1 | |||
Monoclonal gammopathy MGUS D47.2 | 0 | 2 | (1, 1) | 0.7 | 2 | |||
Lymphoproliferative disease LPD total | 219 | (131, 88) | 100 | 276 | (155, 121) | 99.9 | 100 | |
Male/Female ratio | 1.5 | 1.3 | ||||||
Age at onset of disease Years (median) | 61 | (58, 64) | 67 | (65, 69) | ||||
Birth order effect | NO | Patrilineal CLL, only | ||||||
Myeloproliferative Disorders | ||||||||
Acute myeloblastic leukaemia AML C92.0 and C92.2-9 | 44 | (28, 16) | 50.6 | 9 | (6, 3) | 37.6 | 70 | |
Chronic myeloid leukemia CML C92.1 | 14 | (9, 5) | 16.1 | 3 | (1, 2) | 12.5 | 15 | |
Polycythemia vera PV D45 | 4 | (2, 2) | 4.6 | 6 | (4, 2) | 25.0 | 5 | |
Myelodysplasia MDS D46 | 2 | (0, 2) | 2.3 | 2 | (1, 1) | 8.3 | 5 | |
Myelofibrosis MF D47.1 | 8 | (4, 4) | 9.2 | 1 | (0, 1) | 4.2 | 5 | |
Essential thrombocytosis ET D47.3 | 0 | 3 | (1, 2) | 12.5 | <1 | |||
Myeloid leukemia, uncertain ML NOS C92.9 | 15 | (9, 6) | 17.2 | 0 | <1 | |||
Myeloproliferative disease MDP total | 87 | (52, 35) | 100 | 24 | (13, 11) | 99.9 | 100 | |
Male/Female ratio | 1.5 | 1.2 | ||||||
Age at onset of disease Years (median) | 54 | (49, 60) | 63 | (59, 68) | ||||
Birth order effect | NO | NO | ||||||
Other Malignant Disorders | ||||||||
Leukemia, uncertain L NOS C95.9 | 7 | (2, 5) | 1 | (1, 0) | ||||
Malignant histiocytosis MH C96.1 | 2 | (0, 2) | 0 | |||||
