[CIS PIDD] Refractory TCP

[Dr. Carsten Speckmann]

Dear Rohan,

If the last shot of cyclophosphamide will not help, I would consider bortezumib. Since 4 doses of Rituximab and all other procedures did not help - the antibodies are probably produced by CD20 neg plasma cells. Good luck with this patient, Carsten

--
Sent from my phone - please excuse the brevity and possible typos

Dr. Carsten Speckmann 
Center for Chronic Immunodeficiency - CCI
and Center of Pediatrics
University of Freiburg
Germany
phone: +49 (0)761 270 43010
fax: +49 (0)761 270 45990
www.cci.uniklinik-freiburg.de

Am 09.07.2012 um 08:26 schrieb "Rohan Ameratunga (ADHB)" <RohanA at adhb.govt.nz>:


> Dear colleagues,

> 

> My haematology colleagues have been struggling with this young woman with refractory life-threatening TCP.  Any advice would be appreciated. I suggested plasma exchange which does not seem to have helped.  Will check she has been receiving IVIG after each exchange. 

> 

> Thanks

> 

> Rohan Ameratunga

> Adult and paediatric Immunologist

> Auckland 

> New Zealand

> ________________________________________

> From: Lucy Pemberton (Haematologist @ ADHB)

> Sent: Monday, July 09, 2012 5:17 PM

> To: Rohan Ameratunga (ADHB)

> Subject: 19 year old with refractory ITP

> 

> Dear Rohan,

> 

> Here is a quick summary on the 19 year old lady with refractory ITP as of 9/7/2012

> 

> 1.      Severe refractory immune thrombocytopaenia.

> -          Possible underlying SLE with ANA 1280, anti-Ro 94, positive ANA but normal double stranded DNA.

> -          First presented to Middlemore Hospital in Auckland on 15 May 2012 with mucosal bleeding and platelets <10.

> -          Treated at Middlemore Hospital with 1mg/Kg Prednisone, two separate doses of IVIG, Cyclosporin and nine days of Eltrombopag – no rise in platelet count over 3 weeks.

> -          Bone marrow biopsy consistent with ITP (this has been reviewed by several haematologists)

> 2.      Posterior fossa haemorrhage on 7 June 2012.

> -          Transferred from Middlemore Hospital to Auckland City Hospital.  At presentation, GCS 3/15 and pupils not reactive.

> -          Immediate 90 g IVIG and 1g IV Methylprednisolone given with four units of platelets running.  Platelet count peaked at 121, but <10 six hours later

> -          EVD inserted promptly. Haemorrhage not drained. Subsequent GCS 15/15 but persistent ataxia.

> -          Emergency splenectomy 8/6/2012 with platelet cover.

> -          Subsequent treatment with Rituximab 375mg/m2 weekly - four doses completed 28/6/2012

> -          Platelets twice daily since 8/6/2012 – mucosal and line site bleeding occurs without this - ongoing

> -          Vincristine 2 mg IV weekly for 2 doses – stopped 21/6/2012  due to peripheral neuropathy

> -          Cyclophosphamide 300 mg/m² weekly, dose 1 on 22/6/2012, dose 2 on 28/6/2012, dose 3 omitted due to plasma exchange

> -          Eltrombopag restarted 26/6/2012.

> -          Hydroxychloroquine 200mg BD started 21/6/2012.

> -          Daily plasma exchange started 5/7/2012 – no response to 5 days exchange - stopped

> 3.   MRSA colonisation.

> 

> Medications

> 1.        Prednisone 80 mg daily – to wean to 70mg daily from 7/7/2012

> 2.        Tranexamic Acid 1 g tds IV and tds mouthwash

> 3.        Omeprazole 40 mg daily

> 4.        Acyclovir 400 mg bd

> 5.        Cotrimoxazole 960 mg Mon/Wed/Fri

> 6.        Fluconazole 200 mg daily

> 7.        Cyclosporin 150 mg bd

> 8.        Hydroxychloroquine 200mg BD

> 9.        Eltrombopag 50mg OD restarted 26/6/2012

> 10.    Thrombin and adrenalin topically PRN for bleeding

> 11.    Norethisterone 5mg TDS started 5/7/2012 for PV bleeding

> 

> She is needing ongoing daily transfusions of 2 pools of platelets a day due to problems with bleeding at line sites, mucosal bleeding and petechiae.  Topical adrenaline and thrombin are occasionally needed to control line exit site bleeding.  Her coagulation is normal and there is no evidence of a MAHA.

> 

> Plasma exchange was the most recent trial of therapy – there has been no response to 5 days of plasma exchange – still getting line exit site bleeding, mucosal bleeding and petechiae.

> 

> A fragmented RBC scan is planned to exclude an accessory spleen.

> 

> Her prednisone is being slowly weaned as there has been no evidence of response.  There has been no response to full dose IVIG.

> 

> I plan on a further dose of cyclophosphamide this week.

> 

> Any advice very gratefully received.

> 

> Regards,

> 

> Lucy Pemberton

> Haematologist

> Auckland City Hospital

>