[CIS PIDD] [cis-pidd] 80y and combined immunodeficiency

[Jason W. Caldwell]

I would consider Good syndrome and check for Thymoma.


Jason W Caldwell, DO
Assistant Professor Internal Medicine & Pediatrics
[Wake Forest School of Medicine]
Section Pulmonary, Critical Care, Allergic, and Immunological Diseases
Medical Center Boulevard  \  Winston-Salem, NC 27157
p 336.716.5166  \  f 336.716.4743  \  pager 336.806.8330
jcaldwel at wakehealth.edu  \  WakeHealth.edu



From: Daniel E. Pleguezuelo [mailto:pleguezuelo at live.com]
Sent: Wednesday, July 03, 2013 2:55 PM
To: CIS-PIDD
Subject: [cis-pidd] 80y and combined immunodeficiency

Dear all,

It's my first email to the cis-pidd list. Thank you so much in advance!

I would like to present the case of an old man, born and raised in Spain, from a non-consanguineous family, who worked as a car mechanic and who developed (or was detected) at the age of 65 a severe lymphocytopenia and IgG lambda MGUS. Total IgG was found reduced to 635mg/dL and IgM and IgA were progressively going down. Lymphocyte distribution showed these numbers: total lymphocyte count: 300 per microliter. CD19+: 2% of lymphocytes; CD4+: 7.5% of lymphocytes; CD4+/CD8+: 0,1. NK: 41%. Our colleagues performed invitro stimulation studies with PMA and anti-CD3 and observed no increase in expression of CD25+ on T cells. CD95 was found in high intensity only on CD4+ cells. Idiopathic CD4+ lymphocytopenia and stable MGUS were diagnosed and trimetoprim-sulfametoxazol prophylaxis was initiated.

After 15 years of follow he is almost 80 and neither had mayor infections until this year (continuous upper respiratory tract infections and 1 pneumonia) nor progressed to multiple myeloma. Now MGUS IgG is 433mg/dL and polyclonal IgG is 211mg/dL with IgM at 10,4mg/dL and IgA at 30mg/dL. Bone marrow aspiration studies are negative for malignancy by cytology and by flow cytometry CD19-CD138+CD38++ represents 2,5% of celularity. No anemia or thrombocytopenia. Lymphocytes are at 200 cells per microliter. CD4/CD8=0,2; T cells are 46% of lymphocytes (92/uL) B cells  are 0,3% of lymphocytes (<1cell/uL). Vaccination responses results are still pending. HIV is negative by p24 PCR.

He is now under IVIG substitution therapy at 500mg/kg and cotrimoxazol. Other comorbilities are Arterial hypertension, diabetes mellitus with insulin therapy, dislipemia, coronary ateromatosis and left ventricle hypertrophy. Tonsils were removed at chilhood. No adenopathies are observed.

With CD4+ lymphocytopenia, B cell deficiency, T cell lymphocytopenia, hypogammaglobulinemia and IgG stable MGUS, what would you suggest as a global diagnosis?

Thanks,

Daniel E. Pleguezuelo
pleguezuelo at salud.madrid.org<mailto:pleguezuelo at salud.madrid.org>
Immunology Resident.
Hospital Universitario Ramón y Cajal
Madrid, Spain.

________________________________

Date: Wed, 3 Jul 2013 14:06:06 -0300
From: dmvascon at usp.br<mailto:dmvascon at usp.br>
To: cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>
Subject: Re: [cis-pidd] Yellow Fever Vaccine in sIgAD

Dear Yeşim and all, good morning

Here in São Paulo the frequency of IgA deficient patients in a cohort of blood donors is approximately 1:1000 (Carneiro-Sampaio et al, 1989 Frequency of selective IgA deficiency among Brazilian blood donors and healthy pregnant women. Allergol Immunopathol (Madr). 1989 Jul-Aug;17(4):213-6).

I am not aware of any case of yellow fever vaccine adverse reaction among IgA deficient patients (I will ask to the epidemiologic surveillance center to know if they have any data on this subject).

Brazilian data on yellow fever vaccine adverse reactions says that ther frequency of the reactions are approximately:
Severe anaphylactic reactions: 1: 131,000 doses;
Encephalitis: 1: 150,000 - 250,000 doses (most among infants younger than 6 months old);
Multisystemic reactions: 1: 200,000 - 300,000 doses (higher among individuals over 60 years old - 1: 40,000 - 50,000 doses).

I don´t think that this patient has IgA deficiency. Could be interesting to ask about the therapy for Graves' disease, as propylthiouracil as well as methimazole are immunosuppressive drugs and might be associated to these mild decrease of IgA and IgM. As YF vaccine severe reactions are associated to cellular immunodeficiency, if you want to know more about the risk could be interesting to test lymphoproliferative response to T cell mitogens and recall antigens - I don´t know if any lab does it as a routine.
Approximately ten years ago there were three severe reactions to YF vaccine here in Brazil and we standardized a lymphoproliferation assay to YF vaccine virus at that time (not doing it nowadays but we can send the data on standardization if you want).

Best regards,

Dewton


Yeşim Yılmaz Demirdağ wrote:
Dear all,

I would like to bring the "Yellow Fever vaccine and IgA deficiency" topic back to the group discussion. Last discussion concluded that YF vaccine is not contraindicated in IgA deficiency.

I received an e-mail today from a colleague. His patient, a 25 yo woman with history of Grave's disease, is planning to visit Ghana and she needs Yellow Fever vaccination. She had slightly low IgA and IgM 3 years ago (checked during Grave's work-up). She does not have recurrent infections and she is otherwise healthy. She and her father contacted CDC and were told that YF vaccine is contraindicated in selective IgA deficiency and they were given this reference  http://www.cdc.gov/mmwr/pdf/rr/rr6002.pdf  (Table 13 on page 48). I was not aware of this publication, so I wanted to let you all know about it and ask your opinion.

Back to the patient: Her recent IgA level is 49 mg/dl (70-312), IgM is 43 mg/dl (56-352) and IgG is 913 mg/dl (639-1349).  I am not impressed by these values. But the family is reluctant. What would you recommend for this patient? Does she have selective IgA deficiency? What about slightly low IgM? Do you think this combination should be a contraindication for the vaccine?

Dewton, you mentioned that YF vaccine is widely used in inland Brazil. Do you have any data on the frequency of IgA deficiency in patients who developed severe reaction to this vaccine?

Thank you,

Yesim



Yesim Yilmaz Demirdag, MD
Assistant Professor of Pediatrics
Division of Allergy and Immunology
Columbia University Medical Center
New York







On Wed, Jun 19, 2013 at 1:13 PM, Church, Joseph <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:
Colleagues:

A 20+ year old patient is traveling to Africa and is recommended to receive Yellow Fever Vaccine.

She has selective IgA deficiency.  Assuming she does not have an antibody deficiency is there a contraindication to giving  her the YF vaccine?

Joe Church
Children's hospital Los Angeles

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