[CIS PIDD] Post-Fontan hypogam & PLE

[Bodo Grimbacher]

Given this limited clinical information,
please consider MALT lymphoma or "paraneoplastic" to lung cancer
recurrence.
Thanks, Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher
Scientific Director
CCI - Centre of Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Engesserstraße 4, 79108 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
http://www.uniklinik-freiburg.de/cci









Am 23.08.12 18:24 schrieb "Boyce, Thomas G., M.D." unter
<Boyce.Thomas at mayo.edu>:


>Doubt PLE given NL or low stool A1AT.

>

>

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>

>On Aug 22, 2012, at 4:57 PM, "Stan Ress" <Stan.Ress at uct.ac.za> wrote:

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>I wondered if PLE could account for isolated IgG deficiency in 67

>year-old lady with preserved serum albumin. She has a complex history of

>surgery & irradiation for lung ca in 1997 & 2008. Recently presented with

>weight loss, diarrhoea, & food intolerance. Found to have IgG 6.04 g/L &

>5.3 more recently, IgA 1.33 g/L, IgM 0.60g/L. No paraprotein serum peak

>or urine Bence Jones protein detectable. No evidence of malabsorbtion.

>Serum Albumin 42 g/L (37-52), low serum gamma-globulin of 5.7 (8-13g/L).

> 

>Could she still have PLE, despite normal serum albumin & low stool

>alpha-1-antitrypsin <0.18 mg/g (0.43-1.47), with normal serum

>alpha-1-antitrypsin level?

> 

>I would appreciate any comments/suggestions.

> 

>Thanks.

> 

>Stan Ress

>

> 

>--

>Stanley Ress

>Associate Professor of Medicine

>Head: Division of Clinical Immunology

>Department of Medicine

>H47 Old Main Building-room 26

>Groote Schuur Hospital and UCT

>Observatory 7925

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>email: stan.ress at uct.ac.za

>>>> "Boyce, Thomas G., M.D." <Boyce.Thomas at mayo.edu> 8/22/2012 3:40 AM >>>

>

>Agree usually albumin is low in PLE but would check a stool alpha 1

>antitrypsin to see if losing protein in stool. Also very hard to replete

>IgG in pts with PLE.

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>

>

>

>On Aug 21, 2012, at 8:29 PM, "Church, Joseph" <JChurch at chla.usc.edu>

>wrote:

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>

>

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>

>Colleagues:

>

>I follow an 8yo boy with the following

>            - Protein losing enteropathy (history of)

>            - Fontan procedure for hypoplastic left heart

>            - IgG 334 IgA 50 IgM58

>            - CD3+ 26% (308), CD19 42% (478)

>            - Serum albumin 4.7 mg/dL.

>

>Has any one seen this pattern of immunodeficiency in the face of a very

>normal serum albumin level?

>

>Thank you for your thoughts.

>

>Joe Church

>Children's Hospital Los Angeles

>

>

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