Guidance on the assessment of iMCD for pathologists

Guidance on writing pathology reports for iMCD or ‘Castleman Disease-like changes’

Our expert panel has collaborated to provide guidance on the writing of pathology reports where you have identifed histopathological features consistent with the Castleman Disease spectrum. To access the guidance most appropriate to you, please select the scenario that applies.

Please note, the scenarios below assume you were provided with appropriate tissue. It is important to be aware that an excisional lymph node biopsy is recommended, and other biopsies - most commonly needle/core lymph node biopsies - can return false negatives.

I have confirmed the presence of histopathological features consistent with the Castleman Disease spectrum, BUT DO NOT have enough clinical information to confirm a diagnosis of iMCD.

OR

I have confirmed the presence of histopathological features consistent with the Castleman Disease spectrum AND I have enough clinical information to confirm a diagnosis of iMCD.

Abbreviations

iMCD, idiopathic Multicentric Castleman Disease.

This tool has been funded and produced by Recordati. All images have been provided by an international panel of expert pathologists.
The concept, functionality and expert guidance found within this tool has also been developed with the support of expert pathologists.

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I have confirmed the presence of histopathological features consistent with the Castleman Disease spectrum AND I have enough clinical information to confirm a diagnosis of iMCD.

Confirmed diagnosis of iMCD

Observed morphological features are consistent with a HHV-8/HIV negative (idiopathic) Castleman Disease. Suspected variant type:

  • Hypervascular
  • Mixed
  • Plasmacytic

The provided clinical data CONFIRM the diagnosis of idiopathic Multicentric Castleman Disease (iMCD).

RECOMMENDED ACTION:

  • Review international treatment guidelines
  • Seek input of expert specialist (if required)

Abbreviations

HHV-8, human herpesvirus-8; HIV, human immunodeficiency virus; iMCD, idiopathic Multicentric Castleman Disease.

This tool has been funded and produced by Recordati. All images have been provided by an international panel of expert pathologists. The concept, functionality and expert guidance found within this tool has also been developed with the support of expert pathologists.

I have confirmed the presence of histopathological features consistent with the Castleman Disease spectrum, BUT DO NOT have enough clinical information to confirm a diagnosis of iMCD.

Morphological OVERLAP with Castleman Disease

Features observed indicate morphological OVERLAP with a HHV-8/HIV negative (idiopathic) Castleman Disease. Suspected variant type:

  • Hypervascular
  • Mixed
  • Plasmacytic

The provided clinical data support the diagnosis of idiopathic Multicentric Castleman Disease (iMCD).

RECOMMENDED ACTION:

  • Clinical evaluation for diagnostic features and exclusions required. Targeted treatment available if diagnosis confirmed. See diagnostic criteria according to Fajgenbaum DC, et al. Blood 2017.

Morphological CONSISTENCY with Castleman Disease

Features observed indicate morphological CONSISTENCY with a HHV-8/HIV negative (idiopathic) Castleman Disease. Suspected variant type:

  • Hypervascular
  • Mixed
  • Plasmacytic

The provided clinical data support the diagnosis of idiopathic Multicentric Castleman Disease (iMCD).

RECOMMENDED ACTION:

  • Clinical evaluation for diagnostic features and exclusions required. Targeted treatment available if diagnosis confirmed. See diagnostic criteria according to Fajgenbaum DC, et al. Blood 2017.

Reactive lymphadenopathy with Castleman Disease-like changes

Features observed indicate reactive lymphadenopathy with Castleman Disease-like changes.

Suspicion of Castleman Disease, but histopathological features not fully met.

RECOMMENDED ACTION:

  • Clinical evaluation for diagnostic features and exclusions required. Targeted treatment available if diagnosis confirmed. See diagnostic criteria according to Fajgenbaum DC, et al. Blood 2017.
  • In case of well-founded suspicion an additional excisional lymph node biopsy is highly recommended.

Abbreviations

HHV-8, human herpesvirus-8; HIV, human immunodeficiency virus; iMCD, idiopathic Multicentric Castleman Disease.

This tool has been funded and produced by Recordati. All images have been provided by an international panel of expert pathologists. The concept, functionality and expert guidance found within this tool has also been developed with the support of expert pathologists.

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