Suheyla Ocak, Turkey
Istanbul University Istanbul Medical Faculty Pediatric Hematology and OncologyPresenter of 1 Presentation
PULMONARY LYMPHOMATOID GRANULOMATOSIS IN A 5-YEAR-OLD GIRL: A SIGNIFICANT PRESENTATION FOR DIAGNOSIS OF A PRIMARY IMMUNE DEFICIENCY IN CHILDHOOD
Abstract
Background and Aims
Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder in childhood with frequent extranodal presentation and involvement of the respiratory system. It is one of the EBV related malignancies seen in immuncompromised patient. LYG is usually associated with primary immune deficiency. Herein we report a 5-year-old girl admitted with a pulmonary LYG with common variable immune deficiency (CVID).
Methods
A 5-year-old girl was first admitted to pediatric hematology and oncology department for evaluation of pulmonary mass. She was diagnosed as pneumonia 3 months ago, but no clinical and radiological improvement achieved despite oral and parenteral antibiotherapies. Medical history revealed repeated penumonia, autoimmune hemolytic anemia and perianal/vulvar granulomatous lesion with no signifaicant daignossis. On admission she had no systemic pathologic lymphadenopathy or organomegaly but had lymphopenia and thrombocytopenia with normal peripheral smear and also blood chemistry was normal. A 4x5 cm infiltrative mass was seen on right lower lung.
Results
The pathological examination was consistent with LYG Grade III. She had no isohemaglutinins, no vaccine response, low immunglobulin levels, and lymphocyte subgroup examination revealed significantly low CD3 and CD4 counts. She had been suspected to have CVID (common variable immune deficiency) and genetic testing was scheduled. R-CHOP protocol was started with regular IVIG replecement and she has still on treatment with complete response.
Conclusions
Atypical pulmonary malignant (EBV related or not) lesions should have rise the suspicion of primary immune deficiency in childhood. Through medical histroy and pre-treatment evaluation of immune status is very important for treatment plan and prediciton of prognosis.