Dear collegue,
by a fast review of your data (hematology blood counts are missing!) it
looks like a T cell immunodeficiency as the absolute numbers are far too low
for 9 month of age. Normal antibody production starts at 4-6 month. Bone
marrow failure seems to be secondary as T cells in primary failure are
normally not low
Serology will not help as antibody responses cannot be mounted. Only PCR
will help (antigen assays). Parvo B19 or adenoviruses or others. Liver
might be involved as a source of alternative hematopoiesis (if marrow is
empty and blood counts are normal it is quite obvious).
Marrow transplantation is impossible at the moment intravenous
immunoglobulins are the choice. If you know the virus you can look for
special batches with high titers against the virus. All transfusions must
be irradiated. I guess your pediatric collegues will know.
Sorry for my limited english.
Regards
Thomas Nebe
Dr.med. C. Thomas Nebe
Universitaetsklinikum Mannheim
Zentrallabor
Theodor-Kutzer-Ufer 1-3
D-68167 Mannheim
Tel. +49 621 383-3485
FAX +49 621 383-73 3485
+49 621 383-3819
e-mail: thomas.nebe@ikc.ma.uni-heidelberg.de
Bitte besuchen Sie unsere informativen Webseiten unter
http://www.ma.uni-heidelberg.de/inst/ikc/ (Internet)
http://pandora/inst/ikc/ (Intranet)
> -----Ursprüngliche Nachricht-----
> Von: gerhard nebe-von-caron [SMTP:Gerhard.Nebe-von-Caron@unilever.com]
> Gesendet am: Dienstag, 28. März 2000 17:00
> An: Nebe, Dr C.T.
> Betreff: FW: Advice on a patient
>
>
>
> -----Original Message-----
> From: Jason, Janine [SMTP:jmj1@CDC.GOV]
> Sent: Friday, March 24, 2000 4:51 PM
> To: Cytometry Mailing List
> Subject: FW: Advice on a patient
>
>
>
> > I wonder if I could get ideas on a patient.
> >
> > Patient is a 9 mo Indian girl presenting with 4 weeks of documented
> fevers
> > to 104, persisting through most of the day, without any obvious pattern.
> > Past history is remarkable only for a visit to India in January and
> > positive throat FA or culture (not clear which) for adenovirus on her
> and
> > for Group A strep on her 3 year old brother. At admission, the child
> > looked quite well, playing through her fevers, though she does not look
> > good now. PE was unremarkable, with no cough, clear lungs, no
> tenderness
> > or organomegaly or rash. Thick and thin smears X7 have been negative
> for
> > malaria. Also negative (bacteria, mycobacteria, etc.) are blood
> > cultures, stool cultures, urine cultures. Eye exam and cardiac echo
> were
> > negative. All serology was negative, including CMV, EBV, parvovirus,
> > febrile agglutinins, toxo. (Brucella, leishmania, HAV, HBV, HCV
> pending).
> > Albumin 2.8, total protein 7.2 ; IGG 1260, IGM 128; tetanus titers
> > pending (was vaccinated). Bone marrow aspirate and culture of aspirate
> > were negative for organisms, including salmonella and AFB and for viral
> > inclusions/multinucleated cells. Red cell precursors were absent and
> > there were minimal white cell precursors, except for a plethora of
> > eosinophils. Hematology read it as without hemophagocytosis and without
> > malignancy; pathology thought they saw rare hemophagocytizing cells.
> CT
> > of chest, abdomen and pelvis showed normal sized liver and spleen but
> > diffuse uptake in liver. Now we come to the bizarre parts. Liver
> biopsy
> > shows large areas of necrosis and inclusions strongly suggesting
> > adenovirus. (FAs are being done as I write.) A flow panel on
> > peripheral blood showed the following: WC 1.3 with 60% lymphocytes
> > (780). 11.3% CD3+, 5.2% CD4+, 1.2% CD8, 18.2% CD3-CD56+, CD3-CD16+
> > 19.2%, 64.6% CD19+.
> >
> > The adeno comes as quite a surprise. We had not even known about the
> > throat culture until today. It is odd that it is just in the liver.
> > Also, the LFTs were not really abnormal, at least 4 days ago, which is
> > also odd, given the amount of necrosis.
> >
> > We cannot help but suspect some underlying malignancy or
> > immunodeficiency, but there is no evidence of previous health problems.
> > Does anyone have any ideas re the possibility of underlying disease or
> > suggestions on how/whether to work this up further? Our therapeutic
> > options are poor, so any advice would be much appreciated.
> > Janine Jason, M.D.
> > Mailstop A25
> > Assistant Branch Chief, Clinical Studies
> > HIV Immunology and Diagnostic Serology Branch
> > Division of AIDS, STDs, and Tuberculosis Laboratory Research
> > National Center for Infectious Diseases
> > Centers for Disease Control and Prevention
> > Telephone 404-639-3919
> > FAX 404-639-2108
> > E-mail JMJ1@CDC.gov
> > << Datei: ATT14854.ATT >>
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