T-cell large granular lymphocytic leukemia when ciclosporin fails to control cytopenias
In T-cell large granular lymphocytic leukemia (T-cell LGLL), ciclosporin is used as a next-line immunosuppressive agent with the aim of achieving haematological response. When cytopenias do not respond — or a response is gained and then lost — further treatment escalation is required. This protocol addresses that specific situation.
Previous treatment — failure condition
Ciclosporin (next-line immunosuppression) did not achieve the treatment goal: amelioration of cytopenias (haematological response). Median expected time to response is 3–4 months. Non-achievement of this endpoint defines the indication for escalation to this protocol.
Next-line approach — partial overview
For T-cell LGLL where cytopenias have failed standard immunosuppression including ciclosporin, the protocol draws on a range of further options — including targeted therapies, chemotherapy-class agents, biological approaches, and procedural interventions. The complete regimen details, selection criteria, and sequencing are available in the structured protocol.
References
DOI: 10.1002/hon.70076
- In many patients however, the cytopenias do not respond to these standard immunosuppressive agents, or the response is lost with time.
- A plethora of other agents have been investigated, often just in a single case or in small retrospective case series as summarized in Table 3.
- JAK inhibitors have more recently been proven to have activity in this condition.
- Ruxolitinib was investigated in 23 patients, and in the 20 patients that were evaluable at the time of reporting the ORR was 55%, with the presence of a STAT3 mutation associated with response.
- 25 patients given alemtuzumab Iv 10 mg for 10 days ORR 56%
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