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

[Heidi Schaballie]

Dear Dr. Alvarez Cardona,

we found HLH in one SDS patient presenting with shock, probably caused by
CMV infection. The patient only improved after initiation of ganciclovir
treatment.
Maybe something to rule out as well?
Kind regards,

Heidi Schaballie,MD
Departement of pediatrics
University Hospitals Leuven




On Wed, Oct 9, 2013 at 7:16 PM, Aristoteles Alvarez Cardona <
dr.aristoteles at yahoo.com.mx> wrote:


> 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

>

>

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