[PAGID] (no subject)

[Christine Seroogy]

Have you considered systemic JRA in this patient?  Chris


On Mar 29, 2006, at 8:37 AM, Sullivan, John (Office of Research) wrote:

> Colleagues; I need your help with a difficult case; Patient  
> presented one year ago as a 2yo female from India; admitted with  
> fever, failure to thrive[ht and wt <3%], diffuse lymphadenopathy,  
> splenomegaly and arthralgias and history of intermittent rashes;  
> laboratory studies: WBC 17.4, Hgb 7.5, HCT 23, MCV 58, MCH 19.2,  
> MCHC33, Platelets 54K, Differential, Neutophils 67%,Lymphocytes  
> 28%,Monocytes 4%,Eosinophils 1%; ESR 48; IgG1780, normal IgA, IgM,  
> IgD, IgE; Total Hemolytic Complement normal; AST 74, ALT, 21, LDH  
> 1192, Tot Protein 6.2, Albumin 2.1, normal renal function; elevated  
> FDP and D- dimer, normal AT, compatible with active fibrinolysis  
> and early DIC; HIV serology negative; Bone Marrow, cellular marrow,  
> mild dyserythropoiesis, no evidence of malignancy, normal  
> karyotype, normal T and B cells; Lymph Node Biopsy, reactive lymph  
> node with changes consistent with chronic stimulation and  
> involution; Peripheral Blood, CD T Cells 78%, CD 4 T Cells 35%, CD  
> 8 TCells 45%, CD 4/CD 8 0.78.
>
> Diagnoses: Immune Activation Syndrome secondary to ? viral  
> infection; Alpha Thalassemia Trait
>
> Treatment: Begun in March 05 on Prednisone 2mg/kg/day with slow but  
> progressive improvement till al symptoms resolved except continued  
> HT and Wt <3%; tapered to 1mg/kg/day in August 05; then to QOD  
> prednisone; we were tapering off QOD regimen when over past two  
> weeks at 9mg QOD patient again started fevers, elevated ESR and  
> CRP, LFTs, albumin 2.8, Total protein 6.9; currently back on  
> Prednisone 1mg/kg/day. Parents frustrated as are Dr and Nurse; not  
> sure what we are treating at this point; any help will be  
> appreciated; thanks, John.
>
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> John L Sullivan MD
> Professor of Pediatrics
> University of Massachusetts Medical School
> 55 Lake Avenue North
> Worcester MA 01655
> Phone 508-856-1572
> Fax 508-856-5004
> email john.sullivan at umassmed.edu
>
>