Cancer incidence in persons with Fanconi anemia

Philip S. Rosenberg, Mark H. Greene, Blanche P. Alter

Research output: Contribution to journalArticle

Abstract

Fanconi anemia (FA) is an autosomal recessive condition associated with congenital abnormalities, progressive pancytopenia, and a predisposition to leukemia and solid tumors. We studied a retrospective cohort of North American patients with FA. We calculated relative risks of cancer compared to the general population and cause-specific hazards of the first major adverse outcomes of FA: bone marrow transplantation (BMT) for marrow complications, acute myeloid leukemia (AML), solid tumors, or death from bone marrow failure. We also estimated the cumulative incidence of each adverse event in the presence of the competing risks. Among 145 patients with FA, 9 developed leukemia and 14 developed a total of 18 solid tumors. The ratio of observed to expected cancers (O/E ratio) was 50 for all cancers, 48 for all solid tumors, and 785 for leukemia; these increased risks were statistically significant. The highest solid tumor O/E ratios were 4317 for vulvar cancer, 2362 for esophageal cancer, and 706 for head and neck cancer. Cause-specific hazards of both death and AML peaked at 1%/y in teenage years; the hazard of BMT peaked at 4%/y at age 7. In contrast, the hazard of a solid tumor approached 8%/y by age 40 years. The cumulative incidence to age 48 was 10% for leukemia, 11% for death from marrow failure, 29% for a solid tumor, and 43% for BMT. The risk of a solid tumor may become even higher as death from aplastic anemia is reduced and as patients survive longer after BMT.

Original languageEnglish (US)
Pages (from-to)822-826
Number of pages5
JournalBlood
Volume101
Issue number3
DOIs
StatePublished - Feb 1 2003
Externally publishedYes

Fingerprint

Fanconi Anemia
Tumors
Incidence
Bone
Neoplasms
Hazards
Bone Marrow Transplantation
Leukemia
Bone Marrow
Head and Neck Neoplasms
Acute Myeloid Leukemia
Vulvar Neoplasms
Pancytopenia
Aplastic Anemia
Esophageal Neoplasms

ASJC Scopus subject areas

  • Hematology

Cite this

Rosenberg, P. S., Greene, M. H., & Alter, B. P. (2003). Cancer incidence in persons with Fanconi anemia. Blood, 101(3), 822-826. https://doi.org/10.1182/blood-2002-05-1498

Cancer incidence in persons with Fanconi anemia. / Rosenberg, Philip S.; Greene, Mark H.; Alter, Blanche P.

In: Blood, Vol. 101, No. 3, 01.02.2003, p. 822-826.

Research output: Contribution to journalArticle

Rosenberg, PS, Greene, MH & Alter, BP 2003, 'Cancer incidence in persons with Fanconi anemia', Blood, vol. 101, no. 3, pp. 822-826. https://doi.org/10.1182/blood-2002-05-1498
Rosenberg PS, Greene MH, Alter BP. Cancer incidence in persons with Fanconi anemia. Blood. 2003 Feb 1;101(3):822-826. https://doi.org/10.1182/blood-2002-05-1498
Rosenberg, Philip S. ; Greene, Mark H. ; Alter, Blanche P. / Cancer incidence in persons with Fanconi anemia. In: Blood. 2003 ; Vol. 101, No. 3. pp. 822-826.
@article{cb898a0ce23643249d9b3c1bbc94daa7,
title = "Cancer incidence in persons with Fanconi anemia",
abstract = "Fanconi anemia (FA) is an autosomal recessive condition associated with congenital abnormalities, progressive pancytopenia, and a predisposition to leukemia and solid tumors. We studied a retrospective cohort of North American patients with FA. We calculated relative risks of cancer compared to the general population and cause-specific hazards of the first major adverse outcomes of FA: bone marrow transplantation (BMT) for marrow complications, acute myeloid leukemia (AML), solid tumors, or death from bone marrow failure. We also estimated the cumulative incidence of each adverse event in the presence of the competing risks. Among 145 patients with FA, 9 developed leukemia and 14 developed a total of 18 solid tumors. The ratio of observed to expected cancers (O/E ratio) was 50 for all cancers, 48 for all solid tumors, and 785 for leukemia; these increased risks were statistically significant. The highest solid tumor O/E ratios were 4317 for vulvar cancer, 2362 for esophageal cancer, and 706 for head and neck cancer. Cause-specific hazards of both death and AML peaked at 1{\%}/y in teenage years; the hazard of BMT peaked at 4{\%}/y at age 7. In contrast, the hazard of a solid tumor approached 8{\%}/y by age 40 years. The cumulative incidence to age 48 was 10{\%} for leukemia, 11{\%} for death from marrow failure, 29{\%} for a solid tumor, and 43{\%} for BMT. The risk of a solid tumor may become even higher as death from aplastic anemia is reduced and as patients survive longer after BMT.",
author = "Rosenberg, {Philip S.} and Greene, {Mark H.} and Alter, {Blanche P.}",
year = "2003",
month = "2",
day = "1",
doi = "10.1182/blood-2002-05-1498",
language = "English (US)",
volume = "101",
pages = "822--826",
journal = "Blood",
issn = "0006-4971",
publisher = "American Society of Hematology",
number = "3",

}

TY - JOUR

T1 - Cancer incidence in persons with Fanconi anemia

AU - Rosenberg, Philip S.

AU - Greene, Mark H.

AU - Alter, Blanche P.

PY - 2003/2/1

Y1 - 2003/2/1

N2 - Fanconi anemia (FA) is an autosomal recessive condition associated with congenital abnormalities, progressive pancytopenia, and a predisposition to leukemia and solid tumors. We studied a retrospective cohort of North American patients with FA. We calculated relative risks of cancer compared to the general population and cause-specific hazards of the first major adverse outcomes of FA: bone marrow transplantation (BMT) for marrow complications, acute myeloid leukemia (AML), solid tumors, or death from bone marrow failure. We also estimated the cumulative incidence of each adverse event in the presence of the competing risks. Among 145 patients with FA, 9 developed leukemia and 14 developed a total of 18 solid tumors. The ratio of observed to expected cancers (O/E ratio) was 50 for all cancers, 48 for all solid tumors, and 785 for leukemia; these increased risks were statistically significant. The highest solid tumor O/E ratios were 4317 for vulvar cancer, 2362 for esophageal cancer, and 706 for head and neck cancer. Cause-specific hazards of both death and AML peaked at 1%/y in teenage years; the hazard of BMT peaked at 4%/y at age 7. In contrast, the hazard of a solid tumor approached 8%/y by age 40 years. The cumulative incidence to age 48 was 10% for leukemia, 11% for death from marrow failure, 29% for a solid tumor, and 43% for BMT. The risk of a solid tumor may become even higher as death from aplastic anemia is reduced and as patients survive longer after BMT.

AB - Fanconi anemia (FA) is an autosomal recessive condition associated with congenital abnormalities, progressive pancytopenia, and a predisposition to leukemia and solid tumors. We studied a retrospective cohort of North American patients with FA. We calculated relative risks of cancer compared to the general population and cause-specific hazards of the first major adverse outcomes of FA: bone marrow transplantation (BMT) for marrow complications, acute myeloid leukemia (AML), solid tumors, or death from bone marrow failure. We also estimated the cumulative incidence of each adverse event in the presence of the competing risks. Among 145 patients with FA, 9 developed leukemia and 14 developed a total of 18 solid tumors. The ratio of observed to expected cancers (O/E ratio) was 50 for all cancers, 48 for all solid tumors, and 785 for leukemia; these increased risks were statistically significant. The highest solid tumor O/E ratios were 4317 for vulvar cancer, 2362 for esophageal cancer, and 706 for head and neck cancer. Cause-specific hazards of both death and AML peaked at 1%/y in teenage years; the hazard of BMT peaked at 4%/y at age 7. In contrast, the hazard of a solid tumor approached 8%/y by age 40 years. The cumulative incidence to age 48 was 10% for leukemia, 11% for death from marrow failure, 29% for a solid tumor, and 43% for BMT. The risk of a solid tumor may become even higher as death from aplastic anemia is reduced and as patients survive longer after BMT.

UR - http://www.scopus.com/inward/record.url?scp=0037306904&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0037306904&partnerID=8YFLogxK

U2 - 10.1182/blood-2002-05-1498

DO - 10.1182/blood-2002-05-1498

M3 - Article

C2 - 12393424

AN - SCOPUS:0037306904

VL - 101

SP - 822

EP - 826

JO - Blood

JF - Blood

SN - 0006-4971

IS - 3

ER -