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Kidney Week

Abstract: FR-PO0730

Problems with Podocytes and Platelets: Minimal Change Disease (MCD) and Acquired Amegakaryocytic Thrombocytopenia (AAMT)

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Evans, Neil Asn, UC Davis Health, Sacramento, California, United States
  • Kaur, Harpreet, UC Davis Health, Sacramento, California, United States
  • Gupta, Rajib K., UC Davis Health, Sacramento, California, United States
  • Wiegley, Nasim, UC Davis Health, Sacramento, California, United States
  • Ananthakrishnan, Shubha, UC Davis Health, Sacramento, California, United States
  • Madan, Niti, UC Davis Health, Sacramento, California, United States
Introduction

First reported concurrent presentation of Minimal change disease (MCD) and acquired amegakaryocytic thrombocytopenia (AAMT). Both are immune-mediated. MCD is T-cell dysregulation with anti-nephrin, while AAMT is CD8+ T-cell inhibiting megakaryocytes and autoantibodies against c-Mpl/TPO.

Case Description

A middle-aged male with latent tuberculosis (TB) and chronic NSAID use presented with progressive nephrotic syndrome (Cr 0.8 mg/dL, UPCR 3.4 g/g, and Alb 2.7 g/dL) and macrocytic thrombocytopenia (Hgb 13.2 g/dL, MCV 109 fL, and Platelet 42 K/mm^3).
Renal biopsy confirmed MCD. Cytopenias worsened and bone marrow biopsy showed an absence of megakaryocytes CD61+ <5%. NGS, FISH, PNH, PET scans ruled out MDS / malignancy; confirming AAMT.
Eltrombopag and cyclosporine started with symptomatic improvement; except persistent bleeding required stem cell transplant.

Discussion

Double-hit T-cell dyscrasia likely involves imbalance of (Th17) / T (Treg) cells, generating podocyte permeability factors (IL-13/Angptl4) alongside autoantibody oppression of megakaryocytes. The divergent response between the conditions may stem from the anti-TPO antibodies shielding the TPO receptor agonist.
Take-Away:
Expand the Differential: In nephrotic syndrome with cytopenias consider bone marrow biopsy
The Hypoproliferative Signal: Macrocytosis and absent megakaryocytes distinguish AAMT from ITP/TMA
Anticipate Resistance: Immune dysregulation may require combined T- and B-cell targeting or hematopoietic stem cell transplant