[PAGID] (no subject)

[Sullivan, John (Office of Research)]

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. 






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