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Incidence of hematologic malignancies in Europe by morphologic subtype: results of the HAEMACARE project

Milena Sant, Claudia Allemani, Carmen Tereanu, Roberta De Angelis, Riccardo Capocaccia, Otto Visser, Rafael Marcos-Gragera, Marc Maynadié, Arianna Simonetti, Jean-Michel Lutz, Franco Berrino and and the HAEMACARE Working Group
This article has an Erratum 117(12):3477

Data supplements

  • Supplemental materials for: Sant et al

    HAEMACARE Working Group
    Austria: Hackl M (Austrian National Cancer Registry)
    Czech Republic: Holub J (West Bohemia Cancer Registry)
    France: Maynadie M (Cote d�Or Haematological Malignancies Cancer Registry)
    Germany: Holleczek B (Saarland Cancer Registry)
    Iceland: Tryggvadottir L (Icelandic Cancer Registry)
    Ireland: Comber H (National Cancer Registry of Ireland)
    Italy: Bellù F (Alto Adige Cancer Registry), Giacomin A (Biella Cancer Registry), Ferretti S (Ferrara Cancer Registry), Crocetti E (Firenze Cancer Registry), Serraino D (Friuli Cancer Registry), Vercelli M (Genova Cancer Registry), Federico M (Modena Cancer Registry), Fusco M (Napoli Cancer Registry), Michiara M (Parma Cancer Registry), Tumino R (Ragusa Cancer Registry), Mangone L (Reggio Emilia Cancer Registry), Falcini F (Romagna Cancer Registry), Iannelli A (Salerno Cancer Registry), Budroni M (Sassari Cancer Registry), Zanetti R (Torino Cancer Registry), Piffer S (Trento Cancer Registry), La Rosa F (Umbria Cancer Registry), Zambon P (Venetian Cancer Registry), Sant M (Project Leader), Allemani C, Berrino F, Sowe S, Tereanu C (Fondazione IRCCS �Istituto Nazionale dei Tumori,� Milan), Capocaccia R, De Angelis R, Simonetti A (Centro Nazionale di Epidemiologia, Istituto Superiore di Sanità, Rome)
    Malta: England K (Malta National Cancer Registry)
    Norway: Langmark F (The Cancer Registry of Norway)
    Poland: Rachtan J (Cracow Cancer Registry), Mezyk R (Kielce Cancer Registry), Zwierko M (Warsaw Cancer Registry)
    Slovakia: Ondrusova M (National Cancer Registry of the Slovak Republic)
    Slovenia: Primic-�akelj M (National Cancer Registry of Slovenia)
    Spain: Marcos-Gragera R (Girona Cancer Registry)
    Sweden: Khan S (National Cancer Registry of Sweden)
    Switzerland: Jundt G (Basel Cancer Registry), Usel M (Geneva Cancer Registry), Ess SM (St. Gall Cancer Registry), Bordoni A (Ticino Cancer Registry)
    The Netherlands: Visser O (Amsterdam Cancer Registry), Otter R (CCC-Groningen, North Netherlands), Coebergh JW (Eindhoven Cancer Registry), Siesling S (CCC-Stedendriehoek Twente)
    UK-England: Greenberg D (Eastern Cancer Registration and Information Centre), Easey N (Northern and Yorkshire Cancer Registry), Roche M (Oxford Cancer Intelligence Unit), Lawrence G (West-Midlands Cancer Intelligence Unit)
    UK-Northern Ireland: Gavin A (Northern Ireland Cancer Registry)
    UK-Scotland: Brewster DH (Scottish Cancer Registry)
    UK-Wales: Steward J (Welsh Cancer Intelligence & Surveillance Unit)

    Files in this Data Supplement:

Article Figures & Data

Figures

  • Figure 1

    Age-specific incidence rates (per 100 000) for HMs diagnosed in 2000-2002 and archived by 44 European CRs by age class and morphologic type (HAEMACARE groupings). (A) Lymphoid malignancies. (B) Myeloid malignancies.

  • Figure 2

    Age-standardized incidence rates (per 100 000) for HMs diagnosed in 2000-2002 and archived by 44 European CRs by European region and morphologic type (HAEMACARE groupings). (A) Lymphoid malignancies. (B) Myeloid malignancies.

Tables

  • Table 1

    Percentage of national coverage, coverage period, reference population, number of cases, mean and median age at diagnosis, and data quality indicators for 48 European CRs with incidence data in 2000-2002 for hematologic malignancies

    European region/countryCancer registryNational coverage,* percentageCases diagnosed in 2000-2002
    Years coveredAverage population per yearTotal casesMean age, yMedian age, yDeath certificate only/autopsy, percentageVerified microscopically, percentageUnknown morphology, percentage
    Northern Europe16 83765701.599.025.3
        IcelandIceland100.02000-2002282 99524361670.4100.00.8
        NorwayNorway100.02000-20024 502 000522965691.097.016.6
        SwedenSweden100.02000-20028 884 44911 36566701.799.929.8
    United Kingdom and Ireland39 57565692.990.215.2
        IrelandIreland100.02000-20023 836 871430362672.895.721.0
        EnglandUnited Kingdom East Anglia5.42000-20022 749 513397266701.495.116.2
    United Kingdom Northern and Yorkshire13.32000-20026 563 034896266701.195.814.0
    United Kingdom Oxford5.42000-20022 732 200289763670.5100.016.1
    United Kingdom West Midlands10.72000-20025 284 832628165696.484.012.5
        Northern IrelandUnited Kingdom Northern Ireland100.02000-20021 689 635169463671.376.129.7
        ScotlandUnited Kingdom Scotland100.02000-20025 060 647762366700.595.511.1
        WalesUnited Kingdom Wales100.02000-20022 913 4983843667010.864.315.9
    Central Europe16 58763685.293.217.0
        AustriaAustria100.02000-20028 029 4266998646910.386.832.3
        FranceCote d'Or (Cancer Registry of Haematological Malignancies)0.92000-2002508 22274166710.0100.03.5
        GermanySaarland1.32000-20021 067 443126964674.591.07.1
        SwitzerlandBasel6.12000-2001433 02727864683.299.65.8
    Geneva5.62000-2002417 09653264680.698.117.3
    St Gallen7.22000-2002519 58362163681.3100.06.3
    Ticino4.32000-2002311 54941363672.794.96.5
        The NetherlandsAmsterdam17.62000-20022 872 613268561650.699.12.9
    Eindhoven6.12000-2001989 68058160640.097.98.5
    North Netherlands12.92000-20012 070 146138062662.398.55.4
    Twente7.22000-20021 156 162108962670.799.05.6
    Southern Europe17 97264681.192.417.4
        ItalyAlto Adige0.82000-2002465 93850464680.099.05.0
    Biella0.32000-2002187 98337066700.395.73.2
    Ferrara0.62000-2002347 15661167710.898.77.9
    Firenze2.02000-20021 162 973194164680.669.021.5
    Friuli Venezia Giulia2.12000-20021 192 711191865691.699.629.1
    Genova1.62000900 73557967700.979.37.8
    Modena1.12000-2002638 743104563680.399.42.6
    Napoli0.92000546 39919251571.076.623.6
    Parma0.72000-2002402 78876966700.0100.015.9
    Ragusa0.52000-2002295 49645364691.695.44.9
    Reggio Emilia0.82000-2002462 46973465690.197.350.7
    Romagna1.72000-2002991 045171366702.397.511.7
    Salerno1.92000-20011 082 71070061662.793.422.7
    Sassari0.82000-2002469 20057662660.396.75.6
    Torino1.62000-2001900 40888764671.095.422.0
    Trento0.82000477 85920066700.596.59.0
    Umbria1.52000-2002833 506112564690.777.638.8
    Veneto3.520002 015 29094464681.992.614.5
        MaltaMalta100.02000-2002388 75237860650.098.417.5
        SloveniaSlovenia100.02000-20021 990 625165260660.9100.08.8
        SpainGirona1.32000-2002558 64968164703.495.55.7
    Eastern Europe655060659.891.414.5
        Czech RepublicWest Bohemia8.32000-2002854 58383961657.091.912.8
        PolandCracow1.92000-2002731 16249760658.582.934.2
    Kielce3.12000-20021 325 26097260660.089.223.5
    Warsaw4.22000-20021 673 830113061660.097.213.6
        SlovakiaSlovakia100.02000-20025 385 4643112586417.391.29.3
    Total97 52164693.292.717.6
    • * Proportion of national population covered by each registry in 1995-1999.

    • Unknown morphology (NOS) includes the following ICD-O-3 codes: 9590, 9591, 9800, 9801, 9805, 9820, 9832, and 9860.

    • CR was excluded from the final analysis because NOS cases exceeded 30%.

  • Table 2

    Number of cases and crude incidence rates (IR) per 100 000 for lymphoid malignancies by sex and morphologic type diagnosed in 2000-2002 and archived in 44 European CRs

    HAEMACARE groupingsICD-O-3 codeICD-O-3 descriptionNo. of casesAllMalesFemales
    IR95% CIIR95% CIIR95% CI
    HL55712.49(2.42-2.55)2.81(2.71-2.91)2.18(2.09-2.26)
        HL, nodular lymphocyte predominance9659HL, nodular lymphocyte predominance1950.09(0.08-0.10)0.12(0.10-0.15)0.05(0.04-0.07)
        Classic HL53762.40(2.34-2.47)2.69(2.59-2.79)2.12(2.04-2.21)
    9650HL, NOS9960.44(0.42-0.47)0.52(0.48-0.57)0.37(0.34-0.41)
    9661Hodgkin granuloma (obsolete)
    9662Hodgkin sarcoma (obsolete)
    9651HL, lymphocyte rich2170.10(0.08-0.11)0.13(0.11-0.15)0.07(0.05-0.08)
    9663HL, nodular sclerosis, NOS31651.41(1.36-1.46)1.45(1.38-1.53)1.37(1.31-1.44)
    9664HL, nodular sclerosis cellular phase
    9665HL, nodular sclerosis grade 1
    9667HL, nodular sclerosis grade 2
    9652HL, mixed cellularity, NOS9090.41(0.38-0.43)0.53(0.49-0.58)0.28(0.25-0.32)
    9653HL, lymphocyte depletion, NOS890.04(0.03-0.05)0.05(0.04-0.07)0.03(0.02-0.04)
    9654HL, lymphocyte depletion, diffuse fibrosis
    9655HL, lymphocyte depletion, reticular
    Mature B-cell neoplasms42 85519.14(18.96-19.32)21.30(21.03-21.57)17.07(16.83-17.31)
        SBLL/CLL9670Malignant lymphoma, small B-cell lymphocytic, NOS11 0194.92(4.83-5.01)5.87(5.73-6.02)4.01(3.90-4.13)
    9823B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma
        Immunoproliferative diseases9671Malignant lymphoma, lymphoplasmacytic18590.83(0.79-0.87)1.00(0.94-1.06)0.67(0.63-0.72)
    9760Immunoproliferative disease, NOS
    9761Waldenström macroglobulinemia
    9762Heavy chain disease, NOS
        Mantle cell/centrocytic lymphoma9673Mantle cell lymphoma10120.45(0.42-0.48)0.64(0.60-0.69)0.27(0.24-0.30)
        Follicular B-cell lymphoma9690Follicular lymphoma, NOS48812.18(2.12-2.24)2.10(2.01-2.19)2.26(2.17-2.35)
    9691Follicular lymphoma, grade 2
    9695Follicular lymphoma, grade 1
    9698Follicular lymphoma, grade 3
        Diffuse B-cell lymphoma9675Malignant lymphoma, mixed small and large cell, diffuse (obsolete)85383.81(3.73-3.89)4.06(3.95-4.19)3.57(3.46-3.68)
    9678Primary effusion lymphoma
    9679Mediastinal large B-cell lymphoma
    9680Malignant lymphoma, large B-cell, diffuse, NOS
    9684Malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS
        Burkitt lymphoma/leukemia9687Burkitt lymphoma, NOS4880.22(0.20-0.24)0.31(0.27-0.34)0.13(0.11-0.16)
    9826Burkitt cell leukemia
        Marginal zone lymphoma9689Splenic marginal zone B-cell lymphoma9500.42(0.40-0.45)0.40(0.36-0.44)0.45(0.41-0.49)
    9699Marginal zone B-cell lymphoma, NOS/mucosa-associated lymphoid tissue lymphoma
    9764Immunoproliferative small intestinal disease (Mediterranean lymphoma)
        Mature B-cell leukemia9833Prolymphocytic leukemia, B-cell type6520.29(0.27-0.31)0.46(0.42-0.50)0.13(0.11-0.15)
    9940Hairy cell leukemia
        Plasma cell neoplasms13 4566.01(5.91-6.11)6.46(6.31-6.61)5.58(5.44-5.72)
    9732Multiple myeloma12 1925.44(5.35-5.54)5.85(5.70-5.99)5.06(4.93-5.19)
    9733Plasma cell leukemia920.04(0.03-0.05)0.04(0.03-0.05)0.05(0.03-0.06)
    9731Plasmacytoma, NOS11720.52(0.49-0.55)0.58(0.53-0.62)0.47(0.43-0.51)
    9734Plasmacytoma, extramedullary
    Mature T-cell and NK-cell neoplasms25271.13(1.08-1.17)1.41(1.34-1.48)0.86(0.81-0.92)
        Cutaneous T-cell lymphoma9700Mycosis fungoides12080.54(0.51-0.57)0.68(0.64-0.73)0.40(0.37-0.44)
    9701Sézary syndrome
    9708Subcutaneous T panniculitis-like T-cell lymphoma
    9709Cutaneous T-cell lymphoma, NOS
    9718Primary cutaneous CD30+ T-cell lymphoproliferative disorder
        Other T-cell lymphomas9702Mature T-cell lymphoma, NOS13190.59(0.56-0.62)0.72(0.67-0.78)0.46(0.42-0.50)
    9705Angioimmunoblastic T-cell lymphoma
    9714Anaplastic large cell lymphoma, T-cell and null cell type
    9716Hepatosplenic γδ cell lymphoma
    9717Intestinal T-cell lymphoma
    9948Aggressive NK-cell leukemia
    9719NK/T-cell lymphoma, nasal and nasal-type
    9827Adult T-cell leukemia/lymphoma (HTLV-1 positive)
    9831T-cell large granular lymphocytic leukemia
    9834Prolymphocytic leukemia, T-cell type
    Lymphoblastic lymphoma/acute (precursor cell) lymphatic leukemia28631.28(1.23-1.33)1.44(1.37-1.51)1.12(1.06-1.19)
        B-cell9728Precursor B-cell lymphoblastic lymphoma1900.08(0.07-0.10)0.09(0.07-0.11)0.08(0.06-0.10)
    9836Precursor B-cell lymphoblastic leukemia
        T-cell9729Precursor T-cell lymphoblastic lymphoma640.03(0.02-0.04)0.04(0.03-0.06)0.01(0.01-0.02)
    9837Precursor T-cell lymphoblastic leukemia
        NOS9727Precursor cell lymphoblastic lymphoma, NOS26091.17(1.12-1.21)1.31(1.24-1.38)1.03(0.97-1.09)
    9835Precursor cell lymphoblastic leukemia, NOS
    Unknown lymphoid neoplasms12 5475.60(5.51-5.70)5.87(5.72-6.01)5.35(5.22-5.49)
        Lymphoma, NOS9590Malignant lymphoma, NOS48032.14(2.08-2.21)2.21(2.12-2.30)2.09(2.00-2.17)
        NHL, NOS9591Malignant lymphoma, NHL, NOS74503.33(3.25-3.40)3.51(3.40-3.62)3.15(3.05-3.25)
        Lymphatic leukemia, NOS9820Lymphoid leukemia, NOS2940.13(0.12-0.15)0.15(0.13-0.17)0.12(0.10-0.14)
    9832Prolymphocytic leukemia, NOS
    All lymphoid malignancies*66 37129.64(29.41-29.86)32.83(32.49-33.17)26.59(26.29-26.89)
    • * Eight cases of composite HL and NHL (ICD-O-3 code 9596) not shown in Table 2 have been included in the totals.

  • Table 3

    Number of cases and crude incidence rate (IR) per 100 000 for myeloid malignancies diagnosed in 2000-2002 archived in 44 European CRs by sex and morphologic type

    HAEMACARE groupingsICD-O-3 codeICD-O-3 descriptionNo. of casesTotalMalesFemales
    IR95% CIIR95% CIIR95% CI
    Acute myeloid leukemia*81073.62(3.54-3.70)3.90(3.78-4.02)3.35(3.25-3.46)
        Subgroup 19840Acute erythroid leukemia75453.37(3.29-3.45)3.64(3.53-3.76)3.11(3.01-3.21)
    9861AML, NOS
    9867Acute myelomonocytic leukemia
    9870Acute basophilic leukemia
    9872AML, minimal differentiation
    9873AML without maturation
    9874AML with maturation
    9891Acute monocytic leukemia
    9910Acute megakaryoblastic leukemia
    9930Myeloid sarcoma
        Subgroup 29866Acute promyelocytic leukemia t(15; 17) (q22; q11-12)3110.14(0.12-0.16)0.13(0.11-0.15)0.15(0.13-0.17)
    9871AML with abnormal marrow eosinophils
    9896AML, t(8,21) (q22,q22)
    9897AML, 11q23 abnormalities
        Subgroup 39895AML, with multilineage dysplasia1370.06(0.05-0.07)0.07(0.06-0.09)0.05(0.04-0.07)
    9984Refractory anemia with excess blasts in transformation (obsolete)
        Subgroup 49931Acute panmyelosis with myelofibrosis1060.05(0.04-0.06)0.05(0.04-0.07)0.04(0.03-0.05)
    Myeloproliferative neoplasms74743.34(3.26-3.41)3.50(3.39-3.62)3.18(3.08-3.28)
        CML9863CML, NOS24681.10(1.06-1.15)1.23(1.17-1.30)0.98(0.92-1.04)
    9875Chronic myelogenous leukemia, BCR/ABL positive
        Other myeloproliferative neoplasms50062.24(2.17-2.30)2.27(2.19-2.36)2.20(2.11-2.29)
            Subgroup 19950Polycythemia vera34311.53(1.48-1.58)1.57(1.49-1.64)1.50(1.43-1.57)
    9961Myelosclerosis with myeloid metaplasia
    9962Essential thrombocythemia
    9963Chronic neutrophilic leukemia
    9964Hypereosinophilic syndrome
            Subgroup 29960Chronic myeloproliferative disease, NOS15460.69(0.66-0.73)0.69(0.64-0.74)0.69(0.64-0.74)
    Myelodysplastic syndrome40741.82(1.76-1.88)2.03(1.95-2.12)1.62(1.54-1.69)
    9980Refractory anemia
    9982Refractory anemia with sideroblasts
    9983Refractory anemia with excess blasts
    9985Refractory cytopenia with multilineage dysplasia
    9986Myelodysplastic syndrome 5q deletion
    9989Myelodysplastic syndrome, NOS
    Myelodysplastic/myeloproliferative neoplasms7760.35(0.32-0.37)0.42(0.38-0.46)0.28(0.25-0.31)
    9945Chronic myelomonocytic leukemia
    9876Atypical CML, BCR/ABL-1 negative
    9946Juvenile myelomonocytic leukemia
    9975Myelodysplastic/myeloproliferative neoplasm, unclassifiable
    Unknown myeloid neoplasms13650.61(0.58-0.64)0.66(0.61-0.71)0.56(0.52-0.61)
        Leukemia, NOS9800Leukemia, NOS10100.45(0.42-0.48)0.48(0.44-0.53)0.42(0.38-0.46)
    9801Acute leukemia, NOS
    9805Acute leukemia, ambiguous lineage
        Myeloid leukemia, NOS9860Myeloid leukemia, NOS3550.16(0.14-0.18)0.17(0.15-0.20)0.14(0.12-0.17)
    All myeloid malignancies21 7969.73(9.60-9.86)10.51(10.32-10.70)8.99(8.82-9.17)
    • * Eight cases of therapy-related AML, NOS, and therapy-related myelodysplastic syndrome, NOS (ICD-O-3 codes 9920 and 9987, respectively) not shown in Table 3 were included with acute myeloid leukemia.

    • Twenty-nine cases of mastocytoma NOS/mast cell sarcoma, malignant mastocytosis, and mast cell leukemia (ICD-O-3 codes 9740, 9741, and 9742, respectively) not shown in Table 3 were included with other myeloproliferative neoplasms.