Treatment of immune thrombocytopenia (ITP) secondary to malignancy: a systematic review

Abstract

Immune thrombocytopenia (ITP) can be associated with lymphoproliferative diseases (LPD) or solid tumors. A systematic review of published literature was conducted to evaluate response to treatment of ITP secondary to malignancy. Primary outcome was overall response (complete response+response) to first-line treatments [steroids alone or in combination with intravenous immunoglobulins (IVIg)]. Among secondary outcomes, overall response to second-line treatments [splenectomy, rituximab or thrombopoietin receptor agonists (TPO-RA)] and death were evaluated. Of the retrieved 238 text articles, 108 were analyzable, for a total of 154 patients: 142 in 105 case reports and 12 in 3 observational studies. Thirty-nine patients had solid tumors, 114 LPD, and 1 both. The median follow up was 19 months (IQR, 9–40). The overall response was 50% (62% in solid tumors, 46% in LPD) after steroids and 47% (67% in solid tumors, 36% in LPD) after steroids+IVIg, which are lower than historical responses observed in primary ITP (≈80%). The overall responses to rituximab (used in LPD only), splenectomy and TPO-RA (70%, 73% and 92%, respectively) were similar to those observed in primary ITP. Seven patients (6%) died due to bleeding events. ITP secondary to malignancy appears to be associated with unsatisfactory response to first-line treatments.

Keywords:

• ITP
• malignancy
• secondary ITP

Authorship Contributions

Study concept and design: GMP, EF, SB. Acquisition of data: EF, SB, BR, MCDC. Analysis and interpretation of data: GMP, EF, SB, MC. Drafting of the manuscript: GMP, EF, MC. Critical revision of the manuscript for important intellectual content: GMP, EF, SB, BR, MCDC, GC, MC. Statistical analysis: GC.

Disclosure Statement

Gian Marco Podda, Elisa Fiorelli, Simone Birocchi, Benedetta Rambaldi, Maria Chiara Di Chio, Giovanni Casazza, Marco Cattaneo1declare no conflicts of interests.

Supplementary Material

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