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

[Mona Riskalla]

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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