[CIS PIDD] [cis-pidd] HLH & Renal failure. Background immunosuppresion?

[Nacho Gonzalez]

Dear all,

I would like to hear from your experience in HLH patients with renal
failure. We have an 8yo girl with HLH (supposedly secondary to SoJIA
(no infection,normal perforin expresion, CD107a degranulation assay is
pending)  and a renal failure since she developed a multiorganic
failure in this episode (creatinine -one week after hemofiltration was
stopped- between 1.4-1.6 mg/dL)
However, after 3 weeks with HLH-04 protocol (two etoposide doses
missed due to severe neutropenia)on treatment with
dexametasone+etoposide+IVIG plus anakinra since May 27 (2mg/kg qd,
today changed to bid). she has still her HLH active and we feel that
she needs something instead of CsA as background immunosuppresion.

Any input will be appreciated.
Best regards

Luis Ignacio Gonzalez-Granado
Immunodeficiencies.Pediatrics. Hospital 12 octubre
Madrid. Spain

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