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

[Richard Wasserman]

The letter doesn't really matter at all. You need to contact your BCBS
provider representative and request a peer to peer conference. That will
usually be with a non-A/I medical director. It is much more effective to
speak to another doctor than write letters. Often the person you are
speaking to will say that they can't approve and then you request an appeal
to the BCBS A/I consultant. It is very likely that you don't need a lot of
research citations to convince that person although, years ago I spoke with
an A/I consultant who told me that he know all about light and dark chains
but wouldn't approve IgG therapy. If the BCBS A/I consultant refuses, you
then have the right to request an opinion from an immunologist who doesn't
work for BCBS.

This is a lot of work and a lot of time but, in 31 years, it has only been
unsuccessful once. Your first step is to start talking to other doctors.
Good luck,
Richard Wasserman
Dallas


On Wed, Oct 30, 2013 at 9:41 AM, Forbes, Lisa R. <Lisa.Forbes at bcm.edu>wrote:


> Dear Soheil,

> Your letter is quite strong and convincing.  I find it helpful to site

> several studies from the literature including the comprehensive studies

> from Dr. Chapel and Dr. Cunningham-Rundles.  In my experience, citing the

> literature along with the strength of your letter makes it more difficult

> to deny.

>

> Infection outcomes in patients with common variable immunodeficiency

> disorders: relationship to immunoglobulin therapy over 22 years.<http://www-ncbi-nlm-nih-gov.ezproxyhost.library.tmc.edu/pubmed/20471071>

>

> Lucas M, Lee M, Lortan J, Lopez-Granados E, Misbah S, *Chapel* H.

>

> J Allergy Clin Immunol. 2010 Jun;125(6):1354-1360.e4. doi:

> 10.1016/j.jaci.2010.02.040. Epub 2010 May 14.

> PMID: 20471071 [PubMed - indexed for MEDLINE]

>

> The EUROclass trial: defining subgroups in common variable

> immunodeficiency.<http://www-ncbi-nlm-nih-gov.ezproxyhost.library.tmc.edu/pubmed/17898316>

>

> Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E, Vlkova M,

> Hernandez M, Detkova D, Bos PR, Poerksen G, von Bernuth H, Baumann U,

> Goldacker S, Gutenberger S, Schlesier M, Bergeron-van der Cruyssen F, Le

> Garff M, Debré P, Jacobs R, Jones J, Bateman E, Litzman J, van Hagen PM,

> Plebani A, Schmidt RE, Thon V, Quinti I, Espanol T, Webster AD, *Chapel* H,

> Vihinen M, Oksenhendler E, Peter HH, Warnatz K.

>

> Blood. 2008 Jan 1;111(1):77-85. Epub 2007 Sep 26.

> PMID: 17898316 [PubMed - indexed for MEDLINE]

>

> Morbidity and mortality in common variable immune deficiency over 4

> decades.<http://www-ncbi-nlm-nih-gov.ezproxyhost.library.tmc.edu/pubmed/22180439>

>

> *Resnick ES*, Moshier EL, Godbold JH, Cunningham-Rundles C.

>

> *Blood*. 2012 Feb 16;119(7):1650-7. doi: 10.1182/*blood*-2011-09-377945.

> Epub 2011 Dec 16.

> PMID: 22180439 [PubMed - indexed for MEDLINE]

>

> The many faces of the clinical picture of common variable immune

> deficiency.<http://www-ncbi-nlm-nih-gov.ezproxyhost.library.tmc.edu/pubmed/23026770>

>

> *Resnick ES*, Cunningham-Rundles C.

>

> Curr Opin Allergy Clin Immunol. 2012 Dec;12(6):595-601. doi:

> 10.1097/ACI.0b013e32835914b9. Review.

> PMID: 23026770 [PubMed - indexed for MEDLINE]

>

> Best of luck!

> Lisa

>

> Lisa Forbes, MD

> Assistant Professor

> Baylor College of Medicine, Texas Children's Hospital

> Section of Immunology, Allergy, and Rheumatology

> Center for Human Immunobiology, Clinical Director

> 1102 Bates FC330

> Houston, Tx 77030

> 832-824-1319

>

>

>

>

>

> From: Soheil Chegini <schegini at yahoo.com>

> Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>

> Date: Wednesday, October 30, 2013 9:20 AM

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

>

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

> not to treat and how?

>

> Thank you all for your comments and suggestions. I am still struggling to

> provide my patient with what I believe to be appropriate care and want to

> ask you again for your input.

>

> Unfortunately, Independence Blue Cross sees no urgency to act on this case

> and my appeal has fallen on deaf ears. I have no way of knowing whether the

> insurance physician, Dr. Karen White, who deemed this patient is not

> eligible for IgG supplementation is even qualified to make that

> determination. The clinical appeals coordinator is a nurse without any

> immunology background and I have already done what I could to convince her

> of the necessity. I would like to ask whether I can refer this patient to

> any entity such as IDF or Jeffrey Modell Foundation that could offer him

> legal counseling and advice or represent him by flexing its legal muscle to

> help him get IgG approved. Thank you again for your help and advice.

>

> Here is an update on the results of his follow-up lab results:

> IgG 136

> IgA <4

> IgM 2

> ABO group: A; reverse type failed to show anti-B

> Isohemagglutinins (A1, A2 and B) <1:2

> Lymphocyte mitogen and Ag stimulation was normal to PHA, ConA and PWM as

> well as candida and tetanus.

>

> Here is the note I sent to IBC:

>

> Kelli Kobb, RN****

> Clinical Appeals Coordinator****

> Independence Blue Cross****

> P.O Box 41820****

> Philadelphia, PA 19101-1820****

> Fax: 888-671-5274****

> ** **

> ** **

> Dear Ms. Kobb,****

> ** **

> I am writing to appeal your denial of appropriate care to one of my

> patients, Mr.  , who has profound panhypogammaglobulinemia and requires

> prophylactic immunoglobulin replacement.****

> ** **

> I am very concerned about his risk of a catastrophic infection and for

> that reason applied for authorization to start appropriate treatment with

> subcutaneous prophylactic immunoglobulin replacement. Unfortunately, this

> was denied IBC because he has not had a significant infectious history. I

> have appealed IBC’s initial denial, but I have not received a final

> decision about my appeal yet. I wonder whether you would have this patient

> obtain a second opinion by a specialist treating immunodeficiency

> conditions or provide any ideas about how to proceed in the care if this

> patient to avert a potentially disastrous outcome that would undoubtedly

> result in a costly litigation.****

>  ****

> ** **

> Thank you very much in advance for helping my patient and allowing him to

> receive state of the art care for this kind of humoral immunodeficiency.**

> **

> ** **

> ** **

> Sincerely,****

> ** **

>

> Soheil Chegini, M.D.****

>

>

>   On Monday, October 14, 2013 2:11 AM, Carla Gianelli <

> gianellicarla at gmail.com> wrote:

>  Dear Soheil,

>  I agree with Mikko that this case looks like IDVC.

> Did you rule out Celiac Disease? The serologic test could be negative with

> IgA isotope so take in count to do it with IgG (anti-tissue

> transglutaminase antibodies (tTGA) or anti-endomysium antibodies (EMA). Or

> if it it still suspect biopsy of the small intestine could be performed to

> confirm the diagnosis.

> Kind regards,

> Carla Gianelli

> Clinical Immunology

> Hospital Ramón y Cajal.

> Madrid. Spain.

>

>

> 2013/10/14 Seppänen Mikko <Mikko.Seppanen at hus.fi>

>

> **

> Dear Soheil,

>

> I of course agree with all previous comments, and to me Your case looks in

> all ways like a very typical CVID (or Good's, like said), even by looking

> at tetanus and diphteria (latter is low) responses.

>

> I would as well order (if not yet done, in my opinion should be part of

> initial workup even before vaccine responses checked): electrophoresis of

> urine and serum, B12-TC2, (S-MetMal), fS-A-vit, TSH, T4.

>

> There is some data on *infection risk* with this low Igs, check Quinti I

> et al (the Italian cohort) as well as Oxford cohort (Chapel H and Misbah

> S among authors), both of which found that IgG around 400-500 is the

> threshold after which the risk of infection skyrockets...I have, after 17

> years of practice, yet to see the patient who - with that low levels - woud

> not develop a life-threatening infection or die w/o treatment, given a

> reasonable follow up period. Not all agree with therapy so one "gets" to

> follow up what happens... I guess most of us share this opinion?

>

> And about* valproic acid*: it could be one of the factors leading to

> this, but since the patient has already a full-blown CVID phenotype, I am

> not aware of any reports describing a recovered Ig-production after it has

> been stopped? is anyone else? IgAD+IgG2D can recover though, see for

> example Hammastrom L et al for review.

> Personally, I think (IMHO) that if person develops CVID-like after

> valproic acid/gold/SSP or other old antirheumatic or anticonvulsant, they

> probably have genetic predisposing factors (would not include them to

> primary CVID studies though)?

>

> Yours

>

> Mikko Seppänen

> MD PhD Assoc prof

> Immunodeficiency Unit, Helsinki Finland

>

>

> ------------------------------

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

> *Lähetetty:* 11. lokakuuta 2013 23:49

> *Vastaanottaja:* CIS-PIDD

> *Aihe:* [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
Cell (214) 697-7211

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