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

[Richard Wasserman]

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
Cell (214) 697-7211

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