Progressive multifocal leucoencephalopathy, sclerosing cholangitis, bronchiectasis and disseminated warts in a patient with primary combined immune deficiency.
A 24 year old man presented with an unusual primary combined immune deficiency syndrome characterised by a profound lymphopenia of CD4 cells, selective serum IgG2 subclass deficiency, poor polysaccharide antibody responses, disseminated warts, recurrent sinopulmonary infection and bronchiectasis. The developed progressive multifocal leucoencephalopathy (PML) in association with sclerosing cholangitis. Progressive multifocal leucoencephalopathy (PML) usually occurs as an opportunistic infection in patients with secondary defects in cellular immunity.
Misbah
S A
SA
Department of Immunology, John Radcliffe Hospital, Oxford.
Spickett
G P
GP
Zeman
A
A
Esiri
M M
MM
Wallington
T B
TB
Kurtz
J B
JB
Chapel
H M
HM
D000328:Adult; D001987:Bronchiectasis / Q000209:etiology*; D002531:Cerebellum / Q000473:pathology; D015209:Cholangitis, Sclerosing / Q000209:etiology*; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000209:etiology* / Q000473:pathology; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications*; D012871:Skin Diseases / Q000209:etiology; D014860:Warts / Q000209:etiology*