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

[Soheil Chegini]

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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