J ALLERGY CLIN IMMUNOL

VOLUME 133, NUMBER 2

Abstracts AB9

SATURDAY

31 A Case Of A CVID Patient and Rabies Exposure

Dr. Hassan Nasir, DO1, Dr. Shahnaz Fatteh2; 1Nova Southeastern

University College of Osteopathic Medicine, Davie, FL; Larkin Commu-

nity Hospital, Miami, FL, 2Larkin Community Hospital, SouthMiami, FL.

RATIONALE: It is rare to find patients with CVID who are exposed to

Rabies. A key problem that is faced by patients with CVID and other

similar patients, is that these patients cannot fully recognize vaccines due

to impaired B Cell Differentiation.

METHODS: A 57 year old male patient presents with a history of

Hemochromatosis and CVID presented with chief complaint of recent

exposure to bats. Patient proceeded to go to the ER for further evaluation

regarding post-exposure prophylaxis.

RESULTS: An Allergy and Immunology specialist was consulted in the

emergency department, and the patient was given both the Rabies vaccine

and Immunoglobulin immediately after exposure, and completed all 4

vaccines over 14 day period. The patient was subsequently followed up

out-patient by allergy and immunology specialist and was found to have

tolerated vaccinations with no consequences. The patient showed no signs

or symptoms consistent with rabies infection and admitted to having no

side effects from the rabies vaccines and immunoglobulin’s.

CONCLUSIONS: Due to the unknown probability of a response to the

rabies vaccine by CVID patients, giving a patient the rabies vaccine alone

mayormaynot be helpful.On the other handdue to the extreme fatalityof the

rabies virus, and due to the fact that a dead-inactivated vaccinewill likely not

harm a patient (Kopel, Oren et al. 2012), the current recommendation is that

the rabies vaccine should be given in the event that a patient is exposed to the

rabies virus in a CVID patient along with Immunoglobulin.

32 Common Variable Immunodeficiency In The Very OldDr. Katherine E. Gundling, MD; UCSF, San Francisco, CA.

RATIONALE: Common Variable Immundeficiency (CVID) is a

primary immunodeficiency that is diagnosed in people of all ages.

We evaluated and diagnosed a patient to have CVID at the age of 92,

which led us to realize that very little is known about CVID diagnosed

in the very old.

METHODS: We reviewed available data about the oldest patients known

to have CVID, and researched the literature for information about the

oldest age at diagnosis of CVID.

RESULTS: Our literature search revealed little information on how

CVID differs in the very old compared to younger patients, except

that, almost by definition, they have not had the severe, life-

threatening illnesses that shorten the lifespan of certain CVID

phenotypes. There is also little infomation available regarding age at

diagnosis in the later years.

CONCLUSIONS: Whether due to delay of diagnosis or due to milder

disease, CVID can be diagnosed in patients of very advanced age. Our

patient may be the oldest person ever diagnosed with CVID. Examination

of available databases revealed several patients in this age group, but

whether there are distinct differences in comparison to younger patients is

not clear. Our patient’s quality of life greatly improved after institution of

IVIg and we propose that advanced age is not a contraindication to

receiving immunoglobulin.

33 Clinical Predictors Of Granulomatous Disease In CommonVariable Immunodeficiency

Dr. Anna R. Wolfson, Dr. Anna Kovalszki; Beth Israel Deaconess

Medical Center, Boston, MA.

RATIONALE: 8-22% of patients with Common Variable

Immunodeficiency (CVID) will develop granulomatous lymphocytic

interstitial lung disease (GLILD). There are currently no screening

guidelines. Earlier detection of disease may result in improved morbidity

and mortality. We set out to detect markers predictive of GLILD to aid in

development of screening guidelines.

METHODS: Patients were identified using ICD-9 code 279.0 in

online medical records at Beth Israel Deaconess Medical Center

(BIDMC). 94 patients were selected based on IgG <700mg/dL or

subclass deficiency; IgA <60mg/dL or IgM <50mg/dL; if IgG>400mg/

dL, demonstration of poor vaccine response. 13.8% (13/94) of patients

met criteria (chest CT findings of GLILD or biopsy demonstrating

granulomas) for GLILD. This project was approved by the IRB at

BIDMC.

RESULTS: The following statistically significant differences were

found: Hemoglobin (CVID mean 13.16g/dL, SD 1.6; GLILD mean

11.79g/dL, SD 1.924; p50.00051); AST (CVID mean 28.17IU/L, SD

14.15; GLILD mean 47.15IU/L, SD 20.29; p5.00008); Alkaline

Phosphatase (CVID mean 81.42IU/L, SD 34.15; GLILD mean

200.5IU/L, SD 183.5; p50.000003); Total Bilirubin (CVID mean

0.4054mg/dL, SD 0.224; GLILD mean 0.758mg/dL, SD 0.729;

p50.0016); Albumin (CVID mean 4.29g/dL, SD 0.520; GLILD

mean 3.88g/dL, SD 0.649; p50.0229); CD8+ T-cells (CVID mean

851.38/uL, SD 210.11; GLILD mean 1045.43, SD 1668.04;

p50.0086); T-cell CD4/CD8 ratio (CVID mean 3.104, SD 2.28;

GLILD mean 1.14, SD 0.509; p50.029).

CONCLUSIONS: Certain biochemical markers are more likely to be

associated with GLILD in CVID patients. Future work is needed

to develop odds ratios to guide GLILD screening. We are

currently beginning this step using data from other Harvard-affiliated

hospitals.

34 Jacobsen Syndrome With Combined VariableImmunoddeficiency (CIVD)

Dr. Arnaldo C. Porto Neto, MD, PhD, FAAAAI, Dr. Julio Mella

Pierezan, Student, Dr. Joao Paulo Bordin, Student, Dr. Julia Noschang,

Dr. Juliana Gotardo, Student, Dr. Juliana Moro, Student, Dr. Jorge Luigi

Orso, Student, Dr. Jamile Pedroni, Student and Dr. Jessica Zandona,

Student; School of Medicine UPF, PASSO FUNDO, Brazil.

RATIONALE: Jacobsen syndrome is a rare 11q terminal deletion disorder

associated with multiple dysmorphic features, short stature, psychomotor

retardation, congenital heart disease, thrombocytopenia (Paris- Trosseau

syndrome), genitourinary anomalies, ophthalmologic disorder and occa-

sional digital anomalies . While recurrent upper respiratory infections in

these patients are common, life-threatening or opportunistic infections are

few reported.

METHODS: We report 15 year-old male diagnosed as having 11q23.3

terminal deletion at 4 years-old. He has experienced a multitude

of problems, include Paris-Trosseau syndrome, developmental delay,

psychomotor retardation, gastroesophagal reflux, atrioventricular septal

defect, agenesis of right kidney, food allergy, asthma and recurrent otitis

media and persistent sinopulmonary infections, requiring frequent and

prolonged courses of antibiotic. In 2009 he was hospitalized by sepsis and

endocarditis.

RESULTS: Immunologic evaluation: IgG5375mg/dl, IgM519mg/dl,

IgA57mg/dl, CD195 77.2/mm3, CD25 1482/mm3 CD35 1436/

mm3, CD45 684/mm3, CD85693/mm3and pneumococal antibody ti-

ters were nonresponsive (pre and pos vaccination), isohemagglutinins

were low:, anti-A 1:4, platelets (18,000 to 83,000 in blood tests) .

Treatment with IVIG has led to normalization of IgG levels and

clinical improvement.

CONCLUSIONS: A significant number of Jacobsen syndrome have

recurrent infections, there have been no reports to our knowlegde of CVID

or life-threatening in Brazil with this syndrome. It is therefore prudent that

Jacobsen syndrome patients have immunologic assessment in face of

recurrent infections.