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

[Laia Alsina Manrique de Lara]

Dear Nacho, 

We had a similar patient recently, and the activity of both SoJIA and HLH clearly improved by switching from anti IL-1 to anti-IL6. 

Best,

Laia

Dra. Laia Alsina
Sección de Alergia e Inmunología Clínica
Hospital Sant Joan de Déu
Passeig Sant Joan de Déu nº2
08950 Esplugues de Llobregat, Barcelona
+34932804000 ext 3330
________________________________________
De: Nacho Gonzalez [nachgonzalez at gmail.com]
Enviado el: martes, 04 de junio de 2013 18:11
Para: CIS-PIDD
Asunto: [cis-pidd] HLH & Renal failure. Background immunosuppresion?

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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