Premium
Rare Case of Multicentric Castlemanˈs Disease and Progressive Multifocal Leukoencephalopathy in a Severely Immunocompromised Patient
Author(s) -
Solieman Noha,
Chapman Sherita,
Mehta Preema,
Kurukumbi Mohankumar
Publication year - 2012
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.26.1_supplement.lb507
Subject(s) - progressive multifocal leukoencephalopathy , medicine , pathology , castleman disease , malignancy , lymphoid hyperplasia , cervical lymphadenopathy , lymphoma , disease , virus , immunology
Background HIV‐associated Multicentric Castleman's Disease (MCD) is a rare malignant lymphoproliferative disorder in which human herpes virus 8 (HHV8) has been found to be a universal factor. Progressive multifocal leukoencephalopathy (PML) is a rare disease of the central nervous system white matter, which has an increased risk in severely immunocompromised patients. Here we present an interesting patient with newly diagnosed HIV infection with PML and Castleman's disease. Case Presentation Forty‐six year old female presented with progressive speech difficulty and left facial droop. Clinical examination was normal except for axillary lymphadenopathy, mild motor dysphagia, and decreased visual acuity on the left eye. Imaging revealed a left temporal lesion and significant cervical lymphadenopathy. HIV testing was positive with CD‐4 count of twenty‐six. Cerebrospinal fluid analysis was negative for malignancy; few scattered lymphoid cells present and JC virus (John Cunningham virus) was detected. Biopsy of axillary nodes showed atypical lymphoid hyperplasia with regressed germinal centers, paracortical expansion and increased plasma cells, suggestive of Castleman's disease. Immunohistochemical stain with HHV8 was negative. Conclusion This case is presented because of the rarity of occurrence of HIV infection, PML and MCD that has never been reported in the literature.