A Man with Paraneoplastic Retinopathy plus Small Fiber Polyneuropathy Associated with WaldenstrÃ¶m Macroglobulinemia (Lymphoplasmacytic Lymphoma): Insights into Mechanisms.

[waldenstrÃ¶m macroglobulinemia]

Abstract Purpose : To report a well-characterized WaldenstrÃ¶m 's macroglobulinemia (WM ) case that provides insight into the mechanisms of two paraneoplastic complications -- cancer-associated retinopathy (CAR ) and small fiber polyneuropathy (SFPN ). Methods : Retrospective medical chart review . Results : A 58 -year old man with WM developed vision loss and bilateral lower extremity pain . CAR was diagnosed by history , a depressed electroretinogram (ERG ) and positive anti-retinal antibodies . SFPN diagnosis was based on abnormal autonomic nerve function testing and a distal -leg skin biopsy that demonstrated absent epidermal small -fiber innervation , IgM and complement deposition and microvasculopathy . Plasma exchange (PLEX ) led to dramatic pain relief and subjective improvement in eye symptoms along with improvement of some ERG parameters . Repeat skin biopsy after treatment showed less microvascular abnormalities and decreased complement deposition . Conclusions : The concurrence of CAR and SFPN in this patient suggest that both were complications of WM and their common response to PLEX suggests co -mediation by humoral factors that accessed target antigens through IgM-triggered , complement-mediated vascular damage .

Diseases
Validation

Diseases presenting "their common response to plex suggests co-mediation by humoral factors that accessed target antigens through igm-triggered" symptom

waldenstrÃ¶m macroglobulinemia

You can validate or delete this automatically detected symptom