Evidence map›Paper›PMID 38985311›Full record

SynthesisBlood advances2024

The clinical picture of Castleman disease: a systematic review and meta-analysis.

Christian Hoffmann, Eric Oksenhendler, Sarah Littler, Lisa Grant, Karan Kanhai, David C Fajgenbaum

Abstract readSystematic ReviewMeta-Analysis
In one paragraph

Synthesis in Blood advances, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

25 citing papers in PubMed.

  1. The bleeding puzzle: unmasking a hidden culprit.Internal and emergency medicine · 2026
    Article
  2. Article
  3. Article
  4. Article
  5. Article
  6. Article
  7. Castleman disease arising from the pleura: a case report and literature review.Journal of cancer research and clinical oncology · 2026
    Review
  8. Article
  9. Review
  10. Snapshot Look at Castleman Disease.Journal of cellular and molecular medicine · 2026
    Review
  11. Article
  12. Article
  13. Review
  14. Article
  15. Article
  16. Article
  17. Article
  18. Article
  19. [This is how I diagnose Castleman's disease].Zeitschrift fur Rheumatologie · 2025
    Article
  20. The sonographic characteristics of unicentric castleman disease - a single-center retrospective study.Cancer imaging : the official publication of the International Cancer Imaging Society · 2025
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors.

Christian HoffmannInfektionsmedizinisches Centrum Hamburg Study Center, Hamburg, Germany.ORCID 0000-0002-1082-8093
Eric OksenhendlerDepartment of Clinical Immunology, Hôpital Saint-Louis, Paris, France.ORCID 0000-0001-8588-7138
Sarah LittlerSelect Statistical Services, Exeter, United Kingdom.
Lisa GrantTVF Communications, London, United Kingdom.
Karan KanhaiMedical Affairs, Recordati Pharma Ltd, Hemel Hempstead, United Kingdom.
David C FajgenbaumDepartment of Medicine, Center for Cytokine Storm Treatment & Laboratory, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.ORCID 0000-0002-7367-8184

Funding

mTOR as a Central Regulator of iMCD Pathogenesis and Novel Therapeutic TargetR01HL141408 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI Taku Kambayashi · 2018 to 2026
$6.1M
ACCELERATE: An Efficient and Innovative Natural History Study Addressing Unmet Needs in Castleman DiseaseR01FD007632 · FDA · UNIVERSITY OF PENNSYLVANIA · PI Joshua D. Brandstadter, David C Fajgenbaum · 2022 to 2026
$2.2M
FDA HHS R01 FD007632NHLBI NIH HHS R01 HL141408
6 · The paper itself

Abstract

abstractCastleman disease (CD) encompasses a spectrum of rare disorders, including unicentric CD (UCD), idiopathic multicentric CD (iMCD), and human herpesvirus 8-associated MCD (HHV8+ MCD). We performed a systematic review of publications reporting ≥5 cases of CD between 1995 and 2021, following preferred reporting items for systematic reviews and meta-analyses guidelines, to describe and compare subtypes. We extracted data on clinical symptoms and laboratory parameters as stated in international consensus diagnostic criteria for iMCD and estimated the frequency of each criterion using meta-analyses. We analyzed 32 studies describing 559 UCD, 1023 iMCD, and 416 HHV8+ MCD cases. Although many symptoms and laboratory abnormalities occurred at similar rates in patients with iMCD and HHV8+ MCD, patients with HHV8+ MCD had significantly higher rates of constitutional symptoms (46.6% vs 98.6%; P = .038) and splenomegaly (48.2% vs 89.2%; P = .031). Renal dysfunction was significantly more common in patients with iMCD than in patients with HHV8+ MCD before adjustment (36.9% vs 17.4%; P = .04; adjusted P = .1). Patients with UCD had lower rates of symptoms and laboratory abnormalities, although these were present in 20% of patients and were particularly pronounced in pediatric UCD. There are many similarities in the symptomatology of iMCD and HHV8+ MCD; many patients experience constitutional symptoms and organ dysfunction. Differences between these subtypes likely reflect differences in pathophysiology and/or comorbidity burdens.

Indexed as

Castleman DiseaseHerpesviridae InfectionsHerpesvirus 8, HumanHumans

Identifiers

PMID38985311
PMCPMC11421328

What OpenQuestion holds

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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.