Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Sep 19;134(12):960-969.
doi: 10.1182/blood.2019001362. Epub 2019 Aug 8.

Analysis of 153 115 patients with hematological malignancies refines the spectrum of familial risk

Amit Sud  1   2 , Subhayan Chattopadhyay  2   3 , Hauke Thomsen  2 , Kristina Sundquist  4   5   6 , Jan Sundquist  4   5   6 , Richard S Houlston  1   7 , Kari Hemminki  2   4
Affiliations

Analysis of 153 115 patients with hematological malignancies refines the spectrum of familial risk

Amit Sud et al. Blood. .

Abstract

Estimating familial cancer risks is clinically important in being able to discriminate between individuals in the population at differing risk for malignancy. To gain insight into the familial risk for the different hematological malignancies and their possible inter-relationship, we analyzed data on more than 16 million individuals from the Swedish Family-Cancer Database. After identifying 153 115 patients diagnosed with a primary hematological malignancy, we quantified familial relative risks (FRRs) by calculating standardized incident ratios (SIRs) in 391 131 of their first-degree relatives. The majority of hematological malignancies showed increased FRRs for the same tumor type, with the highest FRRs being observed for mixed cellularity Hodgkin lymphoma (SIR, 16.7), lymphoplasmacytic lymphoma (SIR, 15.8), and mantle cell lymphoma (SIR, 13.3). There was evidence for pleiotropic relationships; notably, chronic lymphocytic leukemia was associated with an elevated familial risk for other B-cell tumors and myeloproliferative neoplasms. Collectively, these data provide evidence for shared etiological factors for many hematological malignancies and provide information for identifying individuals at increased risk, as well as informing future gene discovery initiatives.

PubMed Disclaimer

Conflict of interest statement

Conflict-of-interest disclosure: The authors declare no competing financial interests.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
The inter-relationship between familial relative risks for different hematological malignancies. The color corresponds to the magnitude of the familial risk as indicated. White indicates a familial risk that crosses unity (nonsignificant). HL comprises nodular sclerosis HL and mixed cellularity HL. NHL comprises DLBCL, FL, LPL, MCL, small lymphocytic lymphoma, hairy cell leukemia, and the T-cell leukemias/lymphomas. Myeloid malignancies comprise PV, ET, myelofibrosis, MPN-NOS, MDS, CML, and AML. Myeloproliferative neoplasms comprise PV, ET, myelofibrosis, MPN-NOS. Hematological malignancies with no significant familial associations are not shown.
Figure 2.
Figure 2.
Lifetime cumulative risk for all hematological malignancies, NHL, and CLL. (A) All hematological malignancies, (B) NHL, and (C) CLL.
See this image and copyright information in PMC

References

    1. Swerdlow SH, Campo E, Pileri SA, et al. . The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375-2390. - PMC - PubMed
    1. Arber DA, Orazi A, Hasserjian R, et al. . The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-2405. - PubMed
    1. Sant M, Allemani C, Tereanu C, et al. ; HAEMACARE Working Group . Incidence of hematologic malignancies in Europe by morphologic subtype: results of the HAEMACARE project. Blood. 2010;116(19):3724-3734. - PubMed
    1. Hjalgrim H, Askling J, Rostgaard K, et al. . Characteristics of Hodgkin’s lymphoma after infectious mononucleosis. N Engl J Med. 2003;349(14):1324-1332. - PubMed
    1. Epstein MA, Achong BG, Barr YM. Virus particles in cultured lymphoblasts from Burkitt’s lymphoma. Lancet. 1964;1(7335):702-703. - PubMed

Publication types