What does this research mean for the field?

Ruxolitinib-based regimens are effective in treating hemophagocytic lymphohistiocytosis (HLH), with primary HLH demonstrating significantly higher overall response and short-term survival rates compared to secondary HLH. Novelty: ClaimNovelty.SYNTHESIS. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To quantitatively assess and compare the efficacy and safety of ruxolitinib-based regimens between primary and secondary hemophagocytic lymphohistiocytosis.

September 14, 2026Annals of HematologyOpen Access

Differential efficacy and safety of ruxolitinib in primary versus secondary hemophagocytic lymphohistiocytosis: a systematic review and meta-analysis

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Authors

ZDZirui DingChongqing Medical University ZZZhiyong ZhangChina International Science and Technology Cooperation YDYing DouChina International Science and Technology Cooperation

Discussion

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Implication

Meta-analysis reveals higher short-term response and survival with ruxolitinib in primary versus secondary hemophagocytic lymphohistiocytosis, indicating subtype-dependent therapeutic efficacy.

Key Points

To quantitatively assess and compare the efficacy and safety of ruxolitinib-based regimens between primary and secondary hemophagocytic lymphohistiocytosis.
Systematic review and single-arm meta-analysis of 83 studies (27 single-arm trials or cohort studies and 56 case reports/series) comprising 542 patients.
Evaluated overall response rate, overall survival at 2, 6, and 12 months, and adverse events across disease subtypes (PROSPERO CRD42025639580).
The pooled overall response rate was 87.2% (95% CI, 80.8–92.7%; I² = 55.0%), with primary HLH achieving 100% versus 82.4% in secondary HLH (p = 0.005).
Overall survival across the cohort was 91.6% at 2 months (95% CI, 85.1–96.7%), 81.6% at 6 months (95% CI, 69.5–91.7%), and 73.9% at 1 year (95% CI, 56.3–88.9%), with primary HLH showing superior 2-month survival compared to secondary HLH (100% vs. 87.7%, p = 0.005).
Most frequent adverse events were infections (9.3%), neutropenia (7.5%), and hepatic dysfunction (7.0%), with primary HLH showing higher rates of pancreatic injury and severe hematological toxicities.

Cite This Study

Ding et al. (2026) studied this question.

synapsesocial.com/papers/6aa7b3aa0926e14a848b2c98 https://doi.org/10.1007/s00277-026-07274-9

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September 14, 2026Annals of HematologyOpen Access

Differential efficacy and safety of ruxolitinib in primary versus secondary hemophagocytic lymphohistiocytosis: a systematic review and meta-analysis

View Full Paper

Ask AI

Bookmark

Authors

ZDZirui DingChongqing Medical University ZZZhiyong ZhangChina International Science and Technology Cooperation YDYing DouChina International Science and Technology Cooperation

Discussion

Member takes

Implication

Meta-analysis reveals higher short-term response and survival with ruxolitinib in primary versus secondary hemophagocytic lymphohistiocytosis, indicating subtype-dependent therapeutic efficacy.

Key Points

To quantitatively assess and compare the efficacy and safety of ruxolitinib-based regimens between primary and secondary hemophagocytic lymphohistiocytosis.
Systematic review and single-arm meta-analysis of 83 studies (27 single-arm trials or cohort studies and 56 case reports/series) comprising 542 patients.
Evaluated overall response rate, overall survival at 2, 6, and 12 months, and adverse events across disease subtypes (PROSPERO CRD42025639580).
The pooled overall response rate was 87.2% (95% CI, 80.8–92.7%; I² = 55.0%), with primary HLH achieving 100% versus 82.4% in secondary HLH (p = 0.005).
Overall survival across the cohort was 91.6% at 2 months (95% CI, 85.1–96.7%), 81.6% at 6 months (95% CI, 69.5–91.7%), and 73.9% at 1 year (95% CI, 56.3–88.9%), with primary HLH showing superior 2-month survival compared to secondary HLH (100% vs. 87.7%, p = 0.005).
Most frequent adverse events were infections (9.3%), neutropenia (7.5%), and hepatic dysfunction (7.0%), with primary HLH showing higher rates of pancreatic injury and severe hematological toxicities.

Cite This Study

Ding et al. (2026) studied this question.

synapsesocial.com/papers/6aa7b3aa0926e14a848b2c98 https://doi.org/10.1007/s00277-026-07274-9

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