[CIS PIDD] [MARKETING] [cis-pidd] hemophagocytic lymphohistiocytosis after HSCT

Fri Aug 7 07:41:10 EDT 2015

Thank you Mona! We will try Anakinra. 

Best 

Ele 

*******************************************************************
 Eleonora Gambineri, MD
 Researcher/Assistant Professor

 Department of "NEUROFARBA": Section of Child's Health
 University of Florence 

 Department of Haematology-Oncology: BMT Unit
 Department of Fetal and Neonatal Medicine: Rare Diseases,
 "Anna Meyer" Children's Hospital

 Viale Gaetano Pieraccini,24
 50139 FIRENZE
 ITALY
 Tel +39 055 5662405 (office)/055 5662738(BMT ward)
 Fax +39 055 4221012
 e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it
 ******************************************************************** 

Il Mercoledì 05/08/2015 23:03 Mona Riskalla ha scritto: 

> Eleonora, 
> 
> You could consider anakinra in this setting. We have used it in cases of HLH ( secondary to other diagnoses) where complex immune suppression or prolonged corticosteroids is a concern. Because it is fast acting, and offset is quick, if it does not work to improve your patient's laboratory testing in 6-7 days you can stop it easily. The dosing is quite broad., I am happy to apply off line if you like to know more about dosing this medication. 
> 
> Mona 
> 
> ___________________________________
> Mona M. Riskalla, MD, MS
> Rheumatology, Children's of Minnesota 
> Garden View Medical Building Suite 504, Room 5007
> 347 North Smith Avenue, 
> St. Paul, MN 55102
> Pager: 651-629-1314 
> Desk phone 651-220-6137
> Office : 651-220-5862 
> FAX: 651-220-7233
>>>> Eleonora Gambineri <eleonora.gambineri at unifi.it> 8/5/2015 7:10 AM >>> 
> 
> Dear all, 
> 
> I would like to ask your advice for a patient affected by Thalassemia who underwent haploidentical HSCT (PT-Cyclophosphamide) about one year ago. She didn't suffer from classical GvHD, but she experienced cytopenias (leucopenia and neutropenia, thrombocytopenia, anemia) that we initially thought to be immune mediated, therefore she was treated with steroids and Rituximab with partial response. At a recent BM aspirate many histiocytes were found in the bone marrow. She is presumably suffering from hemophagocytic lymphohistiocytosis after transplantation (she also have high ferritin and triglicerid levels as well as CRP). She was treated with Desametasone (10mg/mq) with partial response (on red cells and platelets, but neutropenia persisted) and she relapsed when the steroid was tapered. She is at the moment on CsA. 
> 
> Does anyone has experience on that? Any advice on further treatment? Vincristine? VP-16? Re-transplant? Prognosis? 
> 
> Thanks a lot for your help! 
> 
> Best wishes,
> Eleonora 
> 
> *******************************************************************
> Eleonora Gambineri, MD
> Researcher/Assistant Professor
> 
> Department of "NEUROFARBA": Section of Child's Health
> University of Florence 
> 
> Department of Haematology-Oncology: BMT Unit
> Department of Fetal and Neonatal Medicine: Rare Diseases,
> "Anna Meyer" Children's Hospital
> 
> Viale Gaetano Pieraccini,24
> 50139 FIRENZE
> ITALY
> Tel +39 055 5662405 (office)/055 5662738(BMT ward)
> Fax +39 055 4221012
> e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it
> ******************************************************************** 
> 
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