[CIS PIDD] [cis-pidd] CGD with ITP, treatment options

[Karin Chen]

Dear All:


I'd appreciate your thoughts on treatment options for persistent immune thrombocytopenia for an 18yr old male with autosomal recessive chronic granulomatous disease (NCF1, p47phox) and limited scleroderma/CREST.
--Rituxan?
--Splenectomy was considered but I am concerned about permanent increased risk of infection.

Current history:
10/11/12 Admitted for severe thrombocytopenia/petechiae, treated with IVIG 1 g/kg without signficant response
10/16/12-10/21/12  Admitted again for severe thrombocytopenia/petechiae, treated with IVIG 2g/kg, then WinRho (RhoD Immune globulin) x2, no response. Prednisone 1.5-2mg/kg started. Discharged with platelet count of 4000.
10/29/12 Platelet count 68000. Prednisone decreased to 1mg/kg/day.
11/5/12 Platelets 5000.
11/12/12 Platelets 10000.

Medications:
Nifedipine
pantoprazole
Bactrim
Itraconazole liquid
Prednisone


Past Medical History:

8 yrs- possible leishmaniasis,

          -selective IgA deficiency

9 yrs- chronic ITP with + direct antiplatelet antibody

11 yrs- left lobe pneumonia/empyema

17 yrs-

2/2012 New diagnosis of limited scleroderma/CREST with digital vasospasm/early digital ischemia

            ANA + 1:2560, antiCentromere antibody positive 108 (0-40), + Rheumatoid factor 31 (<15)
            MI-2 antibody moderate positive, U1 RNP antibody positive,   RNP antibody strongly positive,

           SSA52 KD IgG antibody moderate positive.

   ---> multiple other autoantibodies checked and are negative.

8/2012 Developed indolent pneumonia/pulmonary lesions and adenopathy, treated.

10/2012 Chronic granulomatous disease diagnosed, autosomal recessive, p47phox mutations confirmed

-Serratia skin infection



Thanks for your thoughts,



Karin





Karin Chen, MD
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
University of Utah
karin.chen at hsc.utah.edu

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