History of primary immunodeficiency diseases.

Purpose Of Review: This issue of Current Opinion in Allergy and Clinical Immunology is focused on primary immunodeficiency diseases (PIDs). Like every disorder, PID has its own specific history, starting with the discovery of agammaglobulinemia in 1952 and still expanding at a rapid pace, covering, at the time of this publication, more than 180 genetically determined disorders. However, as this report attempts to demonstrate, the history of PID is intertwined with the development of modern medicine and is the direct result of the innovative thinking of physician scientists who introduced new concepts in pathology, microbiology, biochemistry, and immunology, based on carefully designed experiments.

The discovery of agammaglobulinaemia in 1952.

Unlabelled: Fifty years ago a new disease, agammaglobulinaemia, was described. This was made possible by a great number of preceding technical innovations and theories on different fields of research, in particular haematology, microbiology/immunology and basic sciences. The widely used name "Bruton disease" credits one single man with a new observation which, however, was simultaneously made by several physicians.

A rare entity among childhood malignant lymphomas (large anaplastic T-cell Ki-1 +).

A case of peripheral type T-cell lymphoma is presented to underline the difficulty in distinguishing the initial clinical findings of an inflammatory neoplastic disorder since the diagnosis could only be arrived at after several repeated lymph node biopsies. An 11 10/12-year-old boy admitted to the hospital with inguinal lymph node enlargement was diagnosed as having adenitis and periadenitis. The disease had progressed and the patient had remittent fever rising to 39 degrees C, and another biopsy was taken.