[PAGID] (no subject)

[Kathleen E.Sullivan]

HLH?

On Mar 29, 2006, at 9:37 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
>
>
Kathleen E. Sullivan MD PhD
Chief, Division of Allergy and Immunology
Associate Professor of Pediatrics
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 215-590-3044