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[Idiopathic multicentric Castleman's disease].

V I Vasilyev1, S G Palshina1, A I Pavlovskaya2

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Idiopathic multicentric Castleman disease, a rare lymphoproliferative disorder, presents challenges in diagnosis due to overlapping symptoms with other conditions. This study highlights key diagnostic features and presents a rare case of extranodal hip muscle involvement.

Keywords:
IgG4-related diseaseextranodal lesions in Castleman’s disease.idiopathic multicentric Castleman’s diseaselymphoproliferative diseases

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder.
  • iMCD shares clinical, radiological, and pathological similarities with IgG4-related disease and other lymphadenopathies.
  • Cytokine dysregulation, particularly elevated interleukin-6, drives systemic inflammation and lymphadenopathy in iMCD.

Purpose of the Study:

  • To review the diagnostic criteria for idiopathic multicentric Castleman disease.
  • To present two cases of iMCD, including a novel instance of extranodal hip muscle involvement.
  • To enhance understanding of the clinical, laboratory, and morphological features aiding iMCD diagnosis.

Main Methods:

  • Literature review of idiopathic multicentric Castleman disease.
  • Case report analysis of two patients diagnosed with iMCD.
  • Histopathological examination of lymph node and extranodal tissue.

Main Results:

  • Idiopathic multicentric Castleman disease diagnosis requires exclusion of HHV-8 and HIV infections.
  • Histological findings in iMCD can mimic reactive, tumoral, or IgG4-related lymphadenopathy.
  • The study reports the first known case of extranodal hip muscle involvement in iMCD.

Conclusions:

  • Accurate diagnosis of iMCD relies on a comprehensive evaluation of clinical, laboratory, and morphological data.
  • Distinguishing iMCD from similar conditions necessitates careful exclusion of infectious etiologies.
  • Recognition of extranodal manifestations, such as hip muscle involvement, expands the understanding of iMCD's presentation.