Immunotactoid hepatopathy: A novel entity with histologically proven recurrence post liver transplantation

by Selleckchem | Feb 12 2024

Immunotactoid deposition is a rare fibrillary deposition disease that is primarily seen in the kidney associated with paraproteinemia. Here, we report a case of hepatic immunotactoid deposition in a 67-year-old male with a history of smoldering myeloma and chronic kidney disease who underwent liver transplantation for metabolic dysfunction-related cirrhosis. Immunotactoid deposition was first identified in the explanted liver and recurred in the allograft within only seven weeks following transplantation, presenting as ascites with normal liver function tests. The patient's post-transplantation course was complicated by proteinuria and renal failure requiring dialysis. Histologic examination of both native and allograft liver demonstrated pink amorphous material occupying sinusoidal spaces that were Congo-red negative and IgM Kappa-restricted. Electron microscopy revealed characteristic deposits of electron-dense bundles of hollow microtubules with a 40 nm diameter within the sinusoids and space of Disse, consistent with immunotactoids. Therapy of the patient's underlying plasma-cell dyscrasia utilizing a Daratumumab-based regimen showed decreased serum paraproteins, resolution of ascites, and improved kidney function no longer requiring dialysis, without inducing rejection. The patient continues to respond to treatment 10 months post transplantation.

Comments:

That's quite an intricate case! Immunotactoid deposition diseases, while rare, can have significant implications, especially when associated with conditions like paraproteinemia. The presentation of hepatic immunotactoid deposition following liver transplantation is particularly interesting and challenging.

The recurrence of immunotactoid deposition in the allograft within such a short duration is noteworthy and reflects the systemic nature of the disease. It's fascinating how the histologic examination, Congo-red negativity, and restriction to IgM Kappa supported the diagnosis. Electron microscopy revealing characteristic deposits further confirmed the presence of immunotactoids.

The approach to managing this complex scenario by targeting the underlying plasma-cell dyscrasia with a Daratumumab-based regimen is promising. The decrease in serum paraproteins, resolution of ascites, and improved kidney function without inducing rejection post-transplantation are positive indicators. It's particularly encouraging that the patient continues to respond well to treatment even after 10 months post-transplantation.

This case underscores the importance of a multidisciplinary approach involving expertise in pathology, nephrology, hematology, and transplant medicine. It also highlights the potential efficacy of targeted therapies in managing complications arising from immunotactoid deposition diseases.

Do you work in a medical or research field related to this case, or are you studying it out of interest?