[CIS PIDD] [cis-pidd] Schwachman Diamond Patient-septic shock

[Church, Joseph]

Although SDS patients often have specific (polysaccharide) antibody deficiency, I don't know if that would be sufficient to predispose to septic shock.

If the patient has SDS, I suggest looking for of myleodysplastic syndrome or  tumor.  I had an adult with SDS who died of complications of ovarian tubular carcinoma.

Joe Church
Children's Hospital Los Angeles



From: Aristoteles Alvarez Cardona [mailto:dr.aristoteles at yahoo.com.mx]
Sent: Wednesday, October 09, 2013 10:16 AM
To: CIS-PIDD
Subject: [cis-pidd] Schwachman Diamond Patient-septic shock

Dear Professors:

I have been called to evaluate a 25 year old man with Schwachman Diamond Syndrome (SDS) who is currently in a ICU with septic shock.

The SDS was made clinically with the following:
-History of pancreatic insufficiency, failure to thrive and chronic diarrhea during childhood that improved with age and oral therapy.
-Low bone density in humeral metaphysis
-Pancytopenia

Molecular diagnosis pending

He was admitted last month, when the diagnosis was suspected, at that moment he had pancytopenia, splenomegaly and pneumonia that improved with antibiotics along with GM-SCF, on CT the pancreas had mild fatty infiltration with normal enzyme levels, normal Immunoglobulin levels (IgG 875mg/dl) and malignancy was ruled out (Bone Marrow exam)

He was admitted yesterday with severe bleeding and pancytopenia, an emergency splenectomy was performed with dramatic increase on WBC and platelet counts, currently receiving antibiotics and ICU care but still in shock. The spleen shows only infarctions without malignancy and the surgeon comments findings suggestive of miliary TB on peritoneum (millet seeds) we are waiting for the pathology results. He is receiving brad spectrum antibiotics, will initiate anti TB drugs and IVIG was indicated.

Now, I will be very  gratefull if you could suggest treatment options or practical management of such a patient.

Kind regards.


Aristóteles Álvarez Cardona

Universidad Autónoma de Aguascalientes
Profesor Investigador Asociado. Centro de Ciencias de la Salud.
Avenida Universidad. Ciudad Universitaria
Edificio 107 Aguascalientes, Ags. México CP 20131.
www.uaa.mx<http://www.uaa.mx>



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