The application of a multidisciplinary approach in the diagnosis of Castleman disease and Castleman‐like lymphadenopathies: A 20‐year retrospective analysis of clinical and pathological features

Abstract

SummaryBackgroundCastleman disease (CD) comprises a group of rare and heterogeneous haematological disorders, including unicentric (UCD) and multicentric (MCD) forms, the latter further subdivided into HHV8‐MCD, POEMS‐MCD and idiopathic‐MCD (iMCD). However, according to the Castleman Disease Collaborative Network guidelines, the diagnosis of CD can only be achieved through collaboration between clinicians and pathologists.MethodsWe applied these clinical and pathological criteria and implement with clonality testing to a retrospective cohort of 48 adult and paediatric Italian patients diagnosed with reactive lymphadenitis with CD‐like histological features.ResultsWe confirmed the diagnosis of CD in 60% (29/48) of the cases, including 12 (41%) UCD and 17 (59%; five HHV8‐MCD, three POEMS‐MCD and nine iMCD) MCD. Of the remaining 19 cases (40%) with multiple lymphadenopathy, 5 (26%) were classified as autoimmune diseases, 1 (5%) as autoimmune lymphoproliferative disorder, 1 (5%) as IgG4‐related disease, 11 (83%) as reactive lymphadenitis and 1 (5%) as nodal marginal zone lymphoma.ConclusionsOur study emphasizes the importance of the multidisciplinary approach to reactive lymphadenitis with CD‐like features in order to achieve a definitive diagnosis and choose the appropriate treatment.

Correctly marking the 70th anniversary of Castleman disease

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To the Editor, The article by Pelliccia et al 1 published in the British Journal of Haematology commenced in the introduction with the statement 'Castleman disease (CD), first described in 1954 as an enlarged mediastinal lymph node, now…' and the given reference was case 40351 of the Case Records of the Massachusetts General Hospital, 2 but in fact the anatomical diagnosis in this case 40351 was rheumatic heart disease with consequences-not the real first case of CD that should be the case 40011 who had mediastinal mass that was shown to be hyperplasia of mediastinal lymph nodes. 3 This very first case of CD, case 40011, was published on 7 January 1954; thus, this marks the 70th anniversary of CD.

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