Analysis of histiocytosis deaths in the US and recommendations for incidence tracking.

Shilpa Jain, Norma F Kanarek, Robert J. Arceci

Research output: Contribution to journalArticle


We determined the frequency of deaths associated with histiocytosis in the United States (US) for which incidence data are lacking and could be potentially important in understanding outcomes for patients with these disorders. National death data collected by the US Vital Statistics Reporting System and aggregated using were analyzed for underlying cause of death due to malignant histiocytosis (MH), Langerhans cell histiocytosis (LCH) and Letterer-Siwe disease (LS, a form of LCH) for 3 periods: 1979-1988, 1989-1998, and 1999-2006. To capture histiocytosis, International Classification of Diseases (ICD)-9 codes 202.3, 202.5, and 277.8 and ICD-10 codes C96.1, C96.0, and D76.0-76.1 were used. Deaths were calculated for US residents stratified according to sex, race, region, and age. Other listed contributing causes of death with a histiocytosis diagnosis were also examined. A total of 2,416 deaths primarily due to histiocytosis as underlying cause occurred between 1979 and 2006. On comparison of the underlying and contributory cause for the period 1999-2006, histiocytosis mentioned on the death certificate as a contributory cause (N=562) occurs nearly as often as does underlying cause alone (N=648). The age-adjusted (year 2000) death rate was highest for MH (2.62 deaths per 10 million, 95% CI: 2.40-2.83) and for LCH and LS disease (2.17, 95% CI: 1.98-2.36) during the period 1979-1988. Death rates of each type of histiocytosis dropped significantly from 1979 to 1988 to 1999-2006 (p-value 54 years (p-value

Original languageEnglish (US)
Pages (from-to)156-162
Number of pages7
JournalJournal of registry management
Issue number4
Publication statusPublished - Dec 2010
Externally publishedYes


ASJC Scopus subject areas

  • Medicine(all)

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