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Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
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Leg-type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic

Thomas Ballul1, Nabil Belfeki2, Adèle de Masson3

  • 1Clinical Immunology Department Hôpital Saint Louis, Université de Paris Paris France.

Ejhaem
|July 18, 2022
PubMed

Insights

Idiopathic multicentric Castleman disease (iMCD) presenting in the lower limbs is a rare condition. This study highlights a novel iMCD entity with severe leg ulcers, unresponsive to standard treatments like rituximab.

Area of Science:

  • Medicine
  • Pathology
  • Immunology

Background:

  • Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder of unknown cause.
  • Understanding iMCD pathogenesis is crucial for improving patient management.
  • A specific subtype of iMCD affecting lower limb lymphatics was identified.

Purpose of the Study:

  • To characterize a novel entity of sub-diaphragmatic iMCD.
  • To investigate the clinical presentation and treatment response in patients with iMCD affecting lower limb-draining areas.
  • To explore potential etiologies and optimal management strategies for this iMCD variant.

Main Methods:

  • Case series identification of patients with stereotyped iMCD.
  • Clinical data review, including dermatological manifestations.
  • Pathological examination of lymph nodes (mixed or plasma-cell type).
  • Shotgun metagenomics analysis in a subset of patients.
  • Assessment of treatment responses to antibiotics, rituximab, and tocilizumab.

Main Results:

  • Six cases of sub-diaphragmatic iMCD involving lower limb lymphatics were identified.
  • Patients presented with severe, often ulcerative, chronic lower extremity dermatological conditions.
  • Pathology revealed mixed or plasma-cell type MCD; no pathogens were detected by metagenomics.
  • Antibiotics provided temporary relief, but rituximab and tocilizumab were ineffective.
  • This suggests a distinct iMCD entity not responsive to common iMCD therapies.

Conclusions:

  • A novel, stereotyped iMCD entity associated with severe lower extremity dermatological disease is described.
  • This condition appears distinct from typical iMCD and may not respond to rituximab or tocilizumab.
  • A specific diagnostic and therapeutic approach is warranted, avoiding potentially harmful immunomodulation.

Keywords:
Castleman diseasechronic venous diseaselymphedema

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