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

[CIS-PIDD]

This patient would be an excellent IG study patient. Unfortunately, I am
not aware of any studies ongoing near Exton or Philadelphia. There are some
studies enrolling patients now. If he was willing to drive to the Cleveland
or DC area once a month, that could be a possibility. In order to qualify
he would need three months of stable IVIG treatment. If you can identify a
study site that would take him, you could appeal to one or more of the
manufacturers for compassionate "bridging" IG, treat him for three months
and send him to a study center. Check clinicaltrials.gov.
Good luck,
Richard Wasserman
Dallas

On Fri, Oct 20, 2017 at 1:17 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Hello everybody,
>
> I would like to get the group's input about a case that I floated on this
> forum just about 4 years ago. On a positive note, finally his Ig
> supplementation was approved after I sent hip to U Penn for a 2nd opinion
> that supported my assessment and management plan. He received IgSC
> (Hizentra) until the end of August, when he could no longer afford his
> health insurance premiums. He used to work as a maintenance man for an
> independent long term care facility, until a large corporation took over
> and he was laid off. He works as a carpenter/handy man and makes enough to
> live on, but not enough to keep up with the rising cost of health
> insurance coverage. Also, he makes more than could qualify him for
> Medicaid. The winter is fast approaching and I am again concerned that he
> may run into trouble with infections.
>
> I know that I am asking a logistical question and nothing that is uncommon
> or even interesting, but I am stuck again and need your help to continue
> the care that this patient needs. I hope that you can share your experience
> with cases similar to this. I would very much appreciate the group's advice
> and guidance.
>
> Best regards,
> Soheil
>
>
> ------------------------------
> *From:* Soheil Chegini <schegini at yahoo.com>
> *To:* CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> *Sent:* Wednesday, October 30, 2013 10:23 AM
> *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 <(888)%20671-5274>
>
>
> 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.
> <https://maps.google.com/?q=656+West+Lincoln+Hwy.+%0D+Exton,+PA+19341&entry=gmail&source=g>
> Exton, PA 19341
> Phone: (610) 269-3066
> Fax: (610) 269-8615
>
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-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
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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