DOCK 8 Deficiency, EBV+ Lymphomatoid Granulomatosis, and Intrafamilial Variation in Presentation

DOCK 8 Deficiency, EBV+ Lymphomatoid Granulomatosis, and Intrafamilial Variation in Presentation

Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections....

[Lymphomatoid granulomatosis].

A case of lymphomatoid granulomatosis is presented demonstrating the most common chest radiograph findings as well as some infrequent but recognized associations. The clinical presentation and pathogenesis are discussed. The difficulty in radiological diagnosis is highlighted.

A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT) of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was con...

Choroidal involvement in lymphomatoid granulomatosis.

A 13-year-old boy with evidence of pulmonary lymphomatoid granulomatosis developed monocular diplopia. Fluorescein angiography revealed bilateral choroidal involvement. Following treatment with vincristine, cyclophosphamide, and prednisone his diplopia resolved and the angiographic appearances returned to normal.

Lymphomatoid granulomatosis with postoperative bronchospasm.