Related Experiment Video
Updated: Jan 26, 2026

Establishing a Competing Risk Regression Nomogram Model for Survival Data
Published on: October 23, 2020
Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults
Elise Ouedraogo1, Jeremy Gottlieb1, Adèle de Masson2
1Assistance Publique-Hôpitaux de Paris, Département de Dermatologie, Hôpital Saint-Louis, Paris, France.
Background:
Paraneoplastic pemphigus (PNP) occurs more often in patients with hematologic malignancies (HMs) than in patients with solid cancer. Lung bronchiolitis obliterans (BO) is a severe complication of PNP.
Objective:
To determine the precise clinical and biologic features of HM-associated PNP and identify factors associated with mortality and survival.
Methods:
Systematic review of previously described cases of PNP associated with HMs.
Results:
A total of 144 patients were included. The HMs were non-Hodgkin lymphoma (52.78%), chronic lymphocytic leukemia (22.92%), Castleman disease (18.60%), and other underlying hematologic malignancy (5.70%). The mortality rate was 57%, and most deaths occurred within the first year after the diagnosis of PNP. Multivariate analysis showed that (1) the presence of antienvoplakin antibodies and BO were significantly associated with death, and (2) a toxic epidermal necrolysis-like clinical pattern, bullous pemphigoid-like clinical pattern, and BO were significantly associated with decreased survival.
Limitation:
Only case reports with sufficient mortality data were included.
Conclusion:
PNP associated with HM has a high mortality rate. The toxic epidermal necrolysis-like and BO-associated forms are independent survival factors in PNP associated with HMs.
Related Concept Videos
Factors Affecting the Risk of Infection
The integrity and count of the white blood cells help the body resist pathogens and fight infection. When impaired, it reduces the body's resistance to pathogens. The acidic pH levels of the gastrointestinal, genitourinary tracts, and skin...
Survival Tree
Building a Survival Tree
Constructing a...
Survival Curves
The Kaplan-Meier estimator is the most common method for constructing survival curves. This...
Relative Risk
Introduction To Survival Analysis
The primary goal of survival analysis is to estimate survival time—the time...
Comparing the Survival Analysis of Two or More Groups

