Castleman-like lymfadenopatie u pacienta se smíšenou chorobou pojiva: kazuistika a přehled literatury

Stáhnout PDF English version

Autoři: Evelina Rogges ¹; Sabrina Pelliccia ²; Gianluca Lopez ³; Roberta Soscia ⁴; Arianna Di Napoli ³
Působiště autorů: PhD School in Translational Medicine and Oncology, Department of Medical and Surgical Sciences and Translational Medicine, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy ¹; Haematology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea University Hospital, Sapienza University, Rome, Italy ²; Pathology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea University Hospital, Sapienza University, Rome, Italy ³; Department of Neurosciences, Mental Health and Sensory Organs, Sapienza University of Rome, Rome, Italy ⁴
Vyšlo v časopise: Čes.-slov. Patol., 62, 2026, No. 1, p. 50-57
Kategorie: Original article

Souhrn

Odlišení reaktivních lymfadenopatií v kontextu autoimunitních onemocnění od idiopatické multicentrické Castlemanovy choroby (iMCD) představuje významnou diagnostickou výzvu. Histologické znaky podobné Castlemanově chorobě byly popsány u různých autoimunitních onemocnění, což vyžaduje striktní a komplexní integraci klinických a laboratorních nálezů k dosažení správné diagnózy. Ačkoli konsenzuální doporučení Castleman Disease Collaborative Network (CDCN) uvádějí několik autoimunitních onemocnění jako vylučovací kritéria pro diagnózu iMCD, smíšené onemocnění pojiva (MCTD) mezi nimi v současnosti uvedeno není. Popisujeme případ 77leté ženy s únavou, Raynaudovým fenoménem, sklerodaktylií a mírnou generalizovanou lymfadenopatií, u níž biopsie lymfatické uzliny prokázala histologii podobnou Castlemanově chorobě. Absence systémových zánětlivých příznaků a přítomnost vysokých titrů protilátek anti-U1-RNP však nebyly v souladu s iMCD a spíše podporovaly diagnózu reaktivní Castleman-like lymfadenitidy sekundární k MCTD. Tato kazuistika zdůrazňuje, že Castleman-like lymfadenopatie se může vyskytovat i u MCTD a může velmi těsně napodobovat iMCD. U pacientů s autoimunitními onemocněními, která nejsou výslovně uvedena mezi vylučovacími kritérii CDCN, je proto nezbytná důkladná klinicko-patologická integrace, aby se předešlo chybné diagnóze a potenciálně nevhodné terapii založené na blokádě IL-6.

Klíčová slova:

smíšené onemocnění pojiva – Castleman-like lymfadenopatie – autoimmunitní onemocnění – sklerodaktylie – Raynaudův fenomén - anti-U1-RNP

--------

Zdroje

1. Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 2017; 129(12): 1646-1657.

2. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood 2020; 135(16): 1353–1364.

3. Dispenzieri A. POEMS syndrome: 2017 Update on diagnosis, risk stratification, and management. Am J Hematol 2017; 92(8): 814-829.

4. Kawabata H, Takai K, Kojima M, et al. Castleman-Kojima disease (TAFRO syndrome) : a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (Anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly : a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012). J Clin Exp Hematop 2013; 53(1): 57–61.

5. van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018; 132(20): 2115-2124.

6. van Rhee F, Oksenhendler E, Srkalovic G, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv 2020; 4(23): 6039-6050.

7. Gérard L, Bérezné A, Galicier L, et al. Prospective study of rituximab in chemotherapy-dependent human immunodeficiency virus associated multicentric Castleman’s disease: ANRS 117 CastlemaB Trial. J Clin Oncol 2007; 25(22): 3350-3356.

8. Nijim S, Fajgenbaum DC. Identifying Castleman disease from non-clonal inflammatory causes of generalized lymphadenopathy. Hematology Am Soc Hematol Educ Program 2024; 2024(1): 582-593.

9. Nishikori A, Nishimura MF, Nishimura Y, et al. Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease. Int J Mol Sci 2022; 23(18): 10301.

10. van Rhee F, Fayad L, Voorhees P, et al. Siltuximab, a novel anti-interleukin-6 monoclonal antibody, for Castleman’s disease. J Clin Oncol 2010; 28(23): 3701-3708.

11. Frizzera G, Peterson BA, Bayrd ED, Goldman A. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985; 3(9): 1202-1216.

12. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012; 25(9): 1181-1192.

13. Pelliccia S, Rogges E, Cardoni A, et al. 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. Br J Haematol 2024; 204(2): 534-547.

14. Krishnan J, Danon AD, Frizzera G. Reactive lymphadenopathies and atypical lymphoproliferative disorders. Am J Clin Pathol 1993; 99(4): 385-396.

15. John KJ, Sadiq M, George T, et al. Clinical and Immunological Profile of Mixed Connective Tissue Disease and a Comparison of Four Diagnostic Criteria. Int J Rheumatol 2020; 2020 : 9692030.

16. Bennett RM, O’Connell DJ. Mixed connective tisssue disease: a clinicopathologic study of 20 cases. Semin Arthritis Rheum 1980; 10(1): 25-51.

17. Okawa-Takatsuji M, Aotsuka S, Uwatoko S, et al. Endothelial cell-binding activity of anti-U1-ribonucleoprotein antibodies in patients with connective tissue diseases. Clin Exp Immunol 2001; 126(2): 345-54.

18. Biggiogera M, Bottone MG, Martin TE, Uchiumi T, Pellicciari C. Still immunodetectable nuclear RNPs are extruded from the cytoplasm of spontaneously apoptotic thymocytes. Exp Cell Res 1997; 234(2): 512-520.

19. Okawa-Takatsuji M, Aotsuka S, Uwatoko S, Kinoshita M, Sumiya M. Increase of cytokine production by pulmonary artery endothelial cells induced by supernatants from monocytes stimulated with autoantibodies against U1-ribonucleoprotein. Clin Exp Rheumatol 1999; 17(6): 705-712.

20. Stypińska B, Lewandowska A, Felis-Giemza A, Olesińska M, Paradowska-Gorycka A. Association study between immune-related miRNAs and mixed connective tissue disease. Arthritis Res Ther 2021; 23(1): 19.

21. Nanki T, Tomiyama J, Arai S. Mixed connective tissue disease associated with multicentric Castleman’s disease. Scand J Rheumatol 1994; 23(4): 215-217. 22. Chrispal A, Vasuki Z, Thomas EM, Boorugu HK. Mixed connective tissue disorder and Castleman’s disease. J Assoc Physicians India 2010; 58 : 515-517.

23. Karuppan AV, Ganesh D, Jayaseelya CPS. Case Report on Presentation of Castleman’s Disease in a Patient with Mixed Connective Tissue Disorder. Indian Journal of Pharmacy Practice 2024; 17(1): 75-77.

24. Popović Dragonjić L, Jovanović M, Vrbić M, et al. Castleman’s disease associated with mixed connective tissue disorder and cerebral ischaemia and vasculitis: A rare case and a diagnostic challenge for an infectologist. Vojnosanit Pregl 2020; 77(8): 872–877.

25. Wojtyś M, Piekarska A, Kunc M, et al. Clinicopathological comparison and therapeutic approach to Castleman disease-a case-based review. J Thorac Dis 2019; 11(11): 4859-4874.

26. Saparov D, Gold-Olufadi S, Wasifusddin M, et al. Atypical Combination of Mixed Connective Tissue Disease and Multicentric Castleman Disease. Cureus 2024; 16(9): e70325.

27. Szodoray P, Szollosi Z, Gyimesi E, et al. Sarcoidosis in patients with mixed connective tissue disease: clinical, genetic, serological and histological observations. Rheumatol Int 2008; 28(8): 743-747.

28. Fernandes BM, Bernardes M, Barroca H, Costa L. Kikuchi-Fujimoto Disease Associated With Mixed Connective Tissue Disease: A Late Recurrence Case. J Clin Rheumatol 2021; 27(8S): S779-S780.

29. Gourley I, Bell AL, Biggart D. Kikuchi’s disease as a presenting feature of mixed connective tissue disease. Clin Rheumatol 1995; 14(1): 104-107.

30. Pallet N, Aaron L, Larousserie F, Therby A, Dupont B, Viard JP. Kikuchi-Fujimoto disease associated with mixed connective tissue disease. Scand J Rheumatol 2004; 33(6): 434-436.

31. Aqel NM, Amr SS, Najjar MM, Henry K. Kikuchi’s lymphadenitis developing in a patient with mixed connective tissue disease and Hashimoto’s thyroiditis. Br J Rheumatol 1997; 36(11): 1236-1238.

32. Shiokawa S, Yasuda M, Kikuchi M, Yoshikawa Y, Nobunaga M. Mixed connective tissue disease associated with lupus lymphadenitis. J Rheumatol 1993; 20(1): 147-150.

33. Sharma V, Rankin R. Fatal Kikuchi-like lymphadenitis associated with connective tissue disease: a report of two cases and review of the literature. Springerplus 2015; 4 : 167.

34. Karsulovic C, Hojman LP, Seelmann DL, Wurmann PA. Diffuse Lymphadenopathy Syndrome as a Flare-Up Manifestation in Lupus and Mixed Connective Tissue Disease Following Mild COVID-19. Am J Case Rep 2021; 22: e932751.

35. Erlij D, Cuellar MC, Badilla N, et al. Poliadenopatías de origen reumatológico y las claves del diagnóstico diferencial: Análisis de 19 casos [Lymphadenopathies in patients with rheumatic diseases. Review of 19 cases]. Rev Med Chil 2020; 148(3): 320-326.

36. Guit GL, Shaw PC, Ehrlich J, Kroon HM, Oudkerk M. Mediastinal lymphadenopathy and pulmonary arterial hypertension in mixed connective tissue disease. Radiology 1985; 154(2): 305-306.

37. Frayha RA, Nasr FW, Mufarrij AA. Mixed connective tissue disease, Sjögren’s syndrome, and abdominal pseudolymphoma. Br J Rheumatol 1985; 24(1): 70-73.

38. Yiannopoulos G, Daoussis D, Kourea H, Kalogeropoulou C, Andonopoulos AP. Massive Abdominal Lymphadenopathy as a Manifestation of Connective Tissue Diseases. Report of Two Cases and Review of the Literature. J Orthop Rheumatol 2013; 1 : 1.

39. Bagio MT, Queiroz Guedes L, Takeshi Ishizava R, et al. Lymphadenopathy with high PET-CT uptake: a case of mixed connective tissue disease mimicking lymphoma. Rev Eletr Acervo Saude 2025.