Glomerulopatia da depositi di catene leggere
Table of Contents
Definizione
La glomerulopatia da depositi di catene leggere fu descritta per la prima volta nel 1967 e considerata una complicanza del mieloma. Solo nel 1976 è stata definita come un’entità a sé stante, sindrome di Randall e si osserva sia nel mieloma sia nelle altre malattie maligne. Tuttavia recentemente si è visto che al momento dell’esordio, il 30% dei pazienti con malattia da depositi di catene leggere non presenta alcuna patologia maligna.
La malattia è caratterizzata dalla presenza didepositi di catene leggere sia a livello renale che di altri organi. Colpisce prevalentemente il sesso maschile nella 5° e 6° sesta decade. La malattia si può presentare con entrambi i tipi di catene ma il riscontro più frequente è quello della catena κ rispetto alla λ. La malattia coinvolge tutti gli organi con la deposizione di una sostanza amorfa, rifrangente, costituita da catene leggere delle immunoglobuline.
Eziopatogenesi
Il danno renale è prodotto dallaprecipitazione di catene leggere a livello glomerulare e tubolareche stimola la produzione e la deposizione di matrice extracellulare in notevoli quantità.
Sintomatologia
Il quadro clinico è rappresentato da unasindrome nefrosicao da un insufficienza renale rapidamente progressiva. La malattia può iniziare con una proteinuria modesta, senza ematuria néipertensione. La deposizione delle catene leggere avviene prevalentemente a livello renale, ma può interessare in modo più evidente alcuni organi come ilcuore o il fegatosenza la comparsa di alcun sintomo. In questi casi un occasionale esame bioptico o ecografico può diagnosticare la malattia. I depositi possono interessare anche le fibre nervose causando una neuropatia periferica. La malattiasi può associare a mieloma nel 50% dei pazienti,mentre il mieloma si può presentare con la malattia da deposito di catene leggere nel 90% dei casi.
Dati di laboratorio
La diagnosi si avvale del referto immunoistologico della biopsia renale, tuttavia è interessante effettuare l’elettroforesi del siero e delle urine seguita da immunoelettroforesi che potrebbe evidenziare lecatene leggere libere nelle urine e occasionalmente nel siero.
Diagnosi
La malattia deve essere sospettata nel paziente anziano che presenta unasindrome nefrosica, una nefrite tubulo-interstizialerapidamente progressiva e un’ immunoglobulina monoclonale nel siero e/o nelle urine. I criteri diagnostici sono rappresentati dalla positività renale dell’immunofluorescenza con antisieri specifici anti-κ o λ. I criteri clinici sono sono la presenza di un mieloma con insufficienza renale rapidamente progressiva, una sindrome nefrosica con insufficienza renale rapidamente progressiva, un’ insufficienza renale rapidamente progressiva da causa sconosciuta. Biopsia renale.
Decorso clinico e prognosi
Il decorso clinico della glomerulopatia da depositi di catene leggere è rapido e causa la morte del paziente in pochi mesi quando vi è un interessamento di molti organi. Se la deposizione è solo a livello renale il decorso verso l’uremia terminale può essere rapido per cui il paziente deve ricevere un trattamento emodialitico. La sopravvivenza è condizionata dal tipo di malattia ematologica presente e dal grado di coinvolgimento extrarenale.
Terapia
I presidi terapeutici per la glomerulopatia da depositi di catene leggere sono rappresentati dachemioterapia, plasmaferesie dialisi.
Potresti essere interessato a:
- Insufficienza renale acuta (IRA)
- Glomerulonefrite con depositi mesangiali di IgA o malattia di Berger
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