Poster Display Malignancy and PID

COMMON VARIABLE IMMUNODEFICIENCY: AUTOIMMUNITY, GASTROINTESTINAL MANIFESTATION AND NEOPLASIA IN A TEENAGE BOY.

Lecture Time
11:13 - 11:14
Presenter
  • Ekaterini Goudouris, Brazil
Room
Poster Area
Date
20.09.2019, Friday
Session Time
10:00 - 17:00
Board Number
39
Presentation Topic
Malignancy and PID

Abstract

Background and Aims

Common variable immunodeficiency (CVID) is a primary immunodeficiency with heterogeneous clinical manifestations which include recurrent infections, chronic lung diseases, heightened susceptibility to lymphoma and autoimmunity.

Methods

Case report

Results

Here we describe a 16 year-old boy that presented with recurrent pneumonia since 4 months of age, several upper respiratory tract infections, recurrent herpes zoster and chronic diarrhea. Immunoglobulin levels were below the 3rd percentile for the age and the number of B, T and NK cells was normal. The diagnosis of CVID was performed and he was kept on antibiotic prophylaxis and intravenous human immune globulin (IVIG) from the age of 16 years. Two years later he presented with urticaria that in the beginning seemed to be related with the IVIG, but evoluted as a chronic urticaria with no relation with IVIG or antibiotic prophylaxis. At the same year he presented with immune thrombocytopenia that disappeared after a high dose of IVIG. Diagnostic imaging revealed lymphonodular hyperplasia and a solid oval mass in right iliac fossa compressing and shifting the bladder. The biopsy was performed and Hodgkin lymphoma grade IA was confirmed. The patient was kept on IVIG, ABVD chemotherapy was initiated with partial response and autologous hematopoietic cell transplantation was performed. Since then he presented with no recurrence of the lymphoma.

Conclusions

This patient illustrates a case of CVID and concomitant severe clinical manifestations that can be presented in this disease. It also reinforces the importance of thinking in cancer, especially in lymphoma, in patients with CVID even in childhood or adolescence.

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