Splenectomy Outcomes in Relapsed or Refractory Immune Thrombocytopenia according to First-Line Intravenous Immunoglobulin Response

Abstract

Introduction: Although splenectomy has long been second-line option for immune thrombocytopenia (ITP) patients, an indicator that reliably predicts the efficacy of splenectomy is still being explored. We investigated the treatment outcomes of splenectomy as a second-line therapy for relapsed/refractory ITP according to first-line intravenous immunoglobulin (IVIG) responses. Methods: Fifty-two adult patients treated with splenectomy as second-line therapy for ITP between 2009 and 2019 were included, and they were classified according to first-line IVIG responses (no response to IVIG: non-responders; only transient IVIG response shorter than 4 weeks: poor responders; IVIG response for a longer period; stable responders). The efficacy of splenectomy was analyzed in the three subgroups. Results: Of the 52 patients, 10 were IVIG non-responders, 34 were poor responders, and the remaining eight were stable responders. Response to splenectomy was observed in 50.0% of IVIG non-responders, 94.1% of poor responders, and 100% of stable responders (p = 0.0030). Among the 45 patients who responded to splenectomy, 51.1% relapsed subsequently, and a significantly lower relapse rate was noted in the stable IVIG responders (12.5%, p = 0.0220) than in non-responders (60.0%) and poor responders (59.4%). Conclusions: First-line IVIG response is indicated as a useful predictive factor for response to splenectomy.

Is splenectomy a good strategy for refractory immune thrombocytopenia in adults?

“…More than 20 years ago, a Canadian study showed a strong positive association between response to IVIg and response to splenectomy 40 . This was confirmed in a recent study showing that with response to IVIg, the probability of response to splenectomy was extremely high, but in this study, the negative predictive value was not poor because 50% of the patients with failure of IVIg responded to splenectomy 41 . In our experience, we did not find a clear association between response to IVIg or splenectomy 42 .…”

“…40 This was confirmed in a recent study showing that with response to IVIg, the probability of response to splenectomy was extremely high, but in this study, the negative predictive value was not poor because 50% of the patients with failure of IVIg responded to splenectomy. 41 In our experience, we did not find a clear association between response to IVIg or splenectomy. 42 Therefore, splenectomy should not reasonably be contraindicated in a patient who does not respond to IVIg.…”

Markers of refractory primary immune thrombocytopenia

“…Previous response to IVIg was associated with splenectomy response in three of seven case series 6 . In a recent monocentric study in Korea including 54 ITP patients splenectomized for ITP between 2009 and 2019, the response rate (≥30 × 10 9 /L and doubling of baseline value) after splenectomy was 50% in non‐responders to IVIg ( n = 10) and 95.2% in patients who previously achieved response with IVIg ( n = 42) 10 . Previous responses to corticosteroids, IVIg, TPO‐RAs, rituximab and dapsone were not associated individually with the achievement of sustained response after splenectomy in the recent study by Mageau et al 8 …”

“…6 In a recent monocentric study in Korea including 54 ITP patients splenectomized for ITP between 2009 and 2019, the response rate (≥30 × 10 9 /L and doubling of baseline value) after splenectomy was 50% in non-responders to IVIg (n = 10) and 95.2% in patients who previously achieved response with IVIg (n = 42). 10 Previous responses to corticosteroids, IVIg, TPO-RAs, rituximab and dapsone were not associated individually with the achievement of sustained response after splenectomy in the recent study by Mageau et al 8 Having been treated with ≥2 drugs for ITP before splenectomy was associated with a lower rate of response (platelet count >50 × 10 9 /L at 1 month post-splenectomy) and with refractoriness (platelet count <30 × 10 9 /L at last follow-up) in the study by Vianelli et al in both univariable and multivariable models. 7 Previous treatment with ≥4 drugs was associated with a decreased probability of sustained response after splenectomy in the French multicentre study conducted between 2011 and 2020, with 79.8% of patients exposed to ≥2 drugs for ITP before splenectomy.…”

Prediction of Splenectomy Outcomes in Relapsed or Refractory Immune Thrombocytopenia

“…

Dear Editor, Kwag et al [1] describe splenectomy outcomes in 45 patients with relapsed or refractory immune thrombocytopenia (ITP) according to first-line response to intravenous immunoglobulins (IVIG). Both stable responders and poor responders to IVIG had high initial responses to splenectomy.

…”