[CIS PIDD] [cis-pidd] Profound panhypogammaglobulinemia - To treat or not to treat and how?

[dmvascon at usp.br]

Dear Soheil, 

I also agree with all previous comments and would try to begin the replacement of immunoglobulin (SC or IV). 
As Nacho said, IgM (and frequently IgA) are normal in PLE, due to its higher molecular weight. 

Moreover it is important to rule out thymoma, which can associate with hypogammaglobulinemia in Good's syndrome. 
I've seen thymoma preceding, being concomitant and succeeding the appearance of the immunodeficiency. In Good's syndrome usually B cells are very low (almost absent) and the association of anaeosinophilia and anabasophilia are common. 
Despite low levels of immunoglobulins sometimes we are surprised with autoimmune manifestations (AIHA, ITP, myasthenia gravis etc.) 

All the best, 

Dewton 

Dewton de Moraes Vasconcelos, MD, PhD 
University of Sao Paulo School of Medicine 
Sao Paulo, Brazil 
----- Mensagem original -----


> De: "Nacho Gonzalez" <nachgonzalez at gmail.com>

> Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>

> Enviadas: Sábado, 12 de Outubro de 2013 9:33:27

> Assunto: Re: [cis-pidd] Profound panhypogammaglobulinemia - To treat

> or not to treat and how?



> Dear all,

> Agree with previous comments. Note that in PLE IgM is usually within

> normal levels despite very low IgG. Many patients needing Ig

> replacement therapy have normal antibody after tetanus vaccine.

> Agree that with such levels in high risk of whatever:

> meningitis,arthritis, complicated pneumonia...

> Regards

> Luis Ignacio Gonzalez-Granado

> Immunodeficiencies Unit.

> Hospital 12 octubre. Madrid. Spain

> El 12/10/2013 11:16, "Richard Wasserman" < drrichwasserman at gmail.com

> > escribió:



> > The risk is that the next infection, if he survives, will result in

> > bronchiectasis. This patient needs to be treated. The next step is

> > to request an appeal with a BCBS AI specialist. If that is not

> > successful, request an appeal to an outside AI. In 30 years I have

> > failed only once to get approval.

> 

> > Richard Wasserman

> 

> > Dallas

> 



> > On Fri, Oct 11, 2013 at 11:05 PM, John Ziegler <

> > j.ziegler at unsw.edu.au > wrote:

> 



> > > Dear Soheil

> > 

> 



> > > Presumably this insurance company would not support a car being

> > > fitted with airbags until it had been involved in an accident.

> > 

> 



> > > SCIG is prophylaxis, not treatment for infection.

> > 

> 



> > > However it would be good to show that his Igs are not low because

> > > of

> > > losses. The normal albumin suggests not but stool a1AT would be

> > > useful.

> > 

> 



> > > John

> > 

> 



> > > Professor John B. Ziegler

> > 

> 

> > > Department of Immunology & Infectious Diseases

> > 

> 

> > > Sydney Children's Hospital

> > 

> 

> > > High St., Randwick NSW 2031

> > 

> 

> > > Australia

> > 

> 

> > > T: (02) 93821515

> > 

> 

> > > F: + 61 + 2 93821580

> > 

> 

> > > E: j.ziegler at unsw.edu.au

> > 

> 



> > > From: Soheil Chegini [mailto: schegini at yahoo.com ]

> > 

> 

> > > Sent: Saturday, 12 October 2013 7:49 AM

> > 

> 

> > > To: CIS-PIDD

> > 

> 

> > > Subject: [cis-pidd] Profound panhypogammaglobulinemia - To treat

> > > or

> > > not to treat and how?

> > 

> 



> > > Dear Colleagues,

> > 

> 



> > > I am writing to ask for your advice in the management of a 52

> > > y.o.

> > > man, whom I was consulted to evaluate for

> > > panhypogammaglobulinemia.

> > > I was concerned about his risk of a catastrophis infection and

> > > applied for authorization to start his treatment with SCIg, which

> > > was denied by his insurance IBC because he has not had a

> > > significant

> > > infectious history. This decision has been appealed, but I would

> > > appreciate your advice until a final decision is made.

> > 

> 



> > > His most recent IgG was 136, IgA <4, IgM 2 on Sept 13, 2013.

> > > After

> > > vaccination, he boosted his tetanus and diphtheria titers from

> > > 0.30

> > > to 1.54, and from 0.02 to 0.22, respectively, but failed to

> > > respond

> > > to Pneumovax with all 14 tested serotypes <0.3 pre- and post

> > > vaccination. He has no detectable hemagglutinins. FACS analysis

> > > of

> > > his lymphocytes showed CD3 of 785 (75%) and CD4 428 (40%) that

> > > are

> > > just below the expected levels, but otherwise unremarkable. The

> > > lab

> > > (Quest) could not properly set up mitogen and antigen

> > > proliferation

> > > studies and ABO blood typing and yet I will have to send him back

> > > to

> > > the lab to have that done.

> > 

> 



> > > He presented in April 2013 with anemia and prolonged diarrhea

> > > that

> > > had persisted since March 2012 to his gastroenterologist, who

> > > then

> > > referred him to me. Over that period he had lost a significant

> > > amount of his body weight, from 266 lbs. down to 189 lbs.

> > > Subsequently, he had an extensive workup that identified

> > > giardiasis,

> > > which was appropriately treated with metronidazole and resolved

> > > completely after completion of the course with corresponding

> > > weight

> > > gain of about 10 lbs. In this process he was discovered to be

> > > IgA-deficient on May 10, 2013 among several other pertinent

> > > abnormal

> > > findings. Further investigation revealed very low gamma

> > > globulins,

> > > and immunoglobulin levels across the board. Mesenteric and sub

> > > mandibular lymphadenopathy was noted on his abdominal and neck CT

> > > scans, but hematology/oncology evaluation and bone marrow and

> > > inguinal lymph node biopsy ruled out hematologic malignancies. In

> > > addition, biopsies from his small bowel polyps did not show

> > > pathologic changes consistent with lymphoma. He had significant

> > > respiratory infection in March 2013 that he describes as a flu

> > > with

> > > fevers and chills, cough and shortness of breath, for which he

> > > was

> > > seen by his primary physician. At that time, he did not have any

> > > radiographic imaging of his chest and was empirically treated

> > > with

> > > an antibiotic for 10 days. He was sick for six days, but

> > > gradually

> > > recovered without any complications. He was diagnosed with

> > > bipolar

> > > disorder in 2005 and was placed on Depakote that has been

> > > effective

> > > in stabilizing his mood. Otherwise his PMH is unremarkable.

> > 

> 



> > > Here is a synopsis of his lab data before I first saw him on Jul

> > > 31,

> > > 2013; hemoglobin: 13.7 g/dL; hematocrit: 40.6; RBC count: 4.42;

> > > normal RBC Indices, WBC 9.7 and platelet count 203. Differential

> > > was

> > > remarkable for neutrophilia, absolute neutrophil count (ANC) of

> > > 8200

> > > and with 84% neutrophils and lymphocytopenia with 9% lymphocytes

> > > and

> > > absolute lymphocyte count (ALC) of 873; otherwise normal. CMP)

> > > revealed low total protein at 5.2 with normal albumin at 3.7

> > > (globulin level 1.5 g/dL); IgG was 110, IgM was 7 and IgA < 7

> > > mg/mL

> > > and IgE< 1U/mL; SPEP revealed low beta and gamma globulins.

> > 

> 



> > > Chest CT scan on May 18, 2013 was within normal limits, but CT of

> > > his

> > > neck demonstrated a prominent left submandibular lymph node that

> > > measured less than 1 cm in each dimension. Abdominal CT scan at

> > > that

> > > time and again on July 13, 2013 revealed mesenteric

> > > lymphadenopathy

> > > without any significant change, and multiple areas of small bowel

> > > intussusception without evidence of obstruction.

> > 

> 



> > > I felt that the diagnosis of CVID) cannot be established despite

> > > profound panhypogammaglobulinemia until other defined causes of

> > > hypogammaglobulinemia have been excluded. He has had persistent

> > > unexplained enteropathy after eradication of giardia, but no

> > > history

> > > of recurrent infections or non-infectious complications such as

> > > auto-immune cytopenia, polyclonal lymphocytic proliferation. I

> > > suspected that his treatment with an anticonvulsant, valproic

> > > acid

> > > (Depakote) since 2005 for bipolar disorder could induce secondary

> > > hypogammaglobulinemia. I instructed the patient to see his

> > > psychiatrist, who in the interim has stopped Depakote 3 weeks

> > > ago.

> > > Even if that is the culprit, I would need to be very optimistic

> > > to

> > > expect a rapid resolution of secondary hypogammaglobulinemia. I

> > > have

> > > not repeated his lab workup yet, but am expecting another set of

> > > immunoglobulins in about 2 weeks.

> > 

> 



> > > Thank you very much for your help and guidance.

> > 

> 



> > > Soheil Chegini, M.D.

> > 

> 

> > > Exton Allergy & Asthma Associates

> > 

> 

> > > 656 West Lincoln Hwy.

> > 

> 

> > > Exton, PA 19341

> > 

> 

> > > Phone: (610) 269-3066

> > 

> 

> > > Fax: (610) 269-8615

> > 

> 

> > > ---

> > 

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> 

> > --

> 

> > Richard L. Wasserman, MD, PhD

> 

> > DallasAllergyImmunology

> 

> > 7777 Forest Lane, Suite B-332

> 

> > Dallas, Texas 75230

> 

> > Office (972) 566-7788

> 

> > Fax (972) 566-8837

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