Concomitant IgA nephropathy and cyclical neutropaenia.

Area of Science:

• Nephrology
• Hematology
• Immunology

Background:

• Cyclical neutropaenia is a rare hematologic disorder characterized by periodic drops in neutrophil counts.
• Immunoglobulin A (IgA) nephropathy is a common glomerular disease often presenting with hematuria.

Observation:

• A patient presented with recurrent aphthous ulceration and hematuria coinciding with neutropaenic episodes.
• Peripheral blood studies confirmed cyclical neutropaenia.
• Renal biopsy revealed IgA nephropathy, confirmed by light, electron, and immunofluorescence microscopy.

Findings:

• This case represents the first reported instance of concomitant cyclical neutropaenia and IgA nephropathy.
• Elevated serum immunoglobulin levels were observed, with a notable disproportionate increase in IgA.
• The clinical presentation and biopsy findings support IgA nephropathy diagnosis.

Implications:

• The case highlights the potential role of infection as an initiating factor in IgA nephropathy pathogenesis.
• It provides further evidence for the involvement of poorly soluble immune complex formation in the development of IgA nephropathy.
• This unique combination underscores the complex interplay between hematologic and renal conditions.