Primär kutanes anaplastisches grosszelliges 30-positives Lymphom (multifokale Läsionen rezidivierend)

Zuletzt aktualisiert: 2022-11-16

Autor(en): -

ICD11: 2B03.0

Stein, 1985.

  • Häufigkeitsgipfel: Kinder, Jugendliche und auch im höheren Alter vorkommend
  • Männer > Frauen

  • Solitäre oder gruppiert auftretende, teils ulzerierte Knoten
  • Lymphknoten-, Lungen- und ZNS-Beteiligung möglich

  • Klinik
  • Labor
    • BSG/CRP, Diff.-BB (Leukozytose, rel. Lymphozytose), Leber- und Nierenwerte, LDH, Elektrolyte
    • FACS-Analyse, CD4/CD8 Ratio, Bestimmung der CD4+CD7- Zellen
    • Ggf. Immunelektrophorese
    • Ggf. HTLV-Serologie
    • Ggf. Borrelienserologie
  • Biopsie
    • Dermatopathologie
    • Immunhistologie
      • CD30+ von mindestens 75% der Tumorzellen, CD3+ und CD4+
      • ALK-1 negativ
    • Molekularbiologisch: Klonales Rearrangment von T-Zell-Rezeptor-Genen
  • Ganzkörper-CT, Lymphknotensonographie, ggf. PET-CT

Anaplastische und pleomorphe Zellen bilden noduläre dermale Infiltrate.

5-Jahres-Überleben: 90%

Ausdehnung

Therapie der 1. Wahl

Therapie der 2. Wahl
  • Solitäre oder lokalisierte Läsionen
  • Multifokale Läsionen rezidivierend
  • evtl. mit spontaner Remission
  • Exzision und/oder Radiotherapie
  • (PCALCL)
  • Beobachtung (LyP)
  • Beobachtung (LyP)
  • Low-dose Methotrexat
  • PUVA
  • IFN-α
  • Bexaroten
  • Gemcitabin

Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.

 

  • Biologicals
    • Anti-CD30-Antikörper
    • Brentuximab i.v. 1,8 mg/kg KG (max. 180 mg) alle 3 Wochen
    • Infusion über 30  Minuten
    • min. 8, max. 16 Zyklen

  1. CD4+/CD56+ hematodermic neoplasm (blastic NK-cell lymphoma). Journal of Cutaneous Pathology, 2008. 35(10): p. 975-977.
  2. Adachi, M., et al., High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am. J. Hematol., 1994. 47(4): p. 278-282.
  3. Alobeid, B., et al., Eosinophil-Rich CD30+ Lymphoproliferative Disorder of the Oral Mucosa. American Journal of Clinical Pathology, 2004. 121(1): p. 43-50.
  4. Ascani, S., et al., CD4-negative variant of CD4+/CD56+ hematodermic neoplasm: description of three cases. Journal of Cutaneous Pathology, 2008. 35(10): p. 911-915.
  5. Bagdades, E.K., et al., Relationship between herpes simplex virus ulceration and CD4+ cell counts in patients with HIV infection. AIDS, 1992. 6(11): p. 1317-1320.
  6. Beljaards, R.C., et al., Primary cutaneous T-cell lymphoma: Clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and cd30-positive large cell lymphoma. J. Pathol., 1994. 172(1): p. 53-60.
  7. Belongia, E.A., et al., An Investigation of the Cause of the Eosinophilia–Myalgia Syndrome Associated with Tryptophan Use. New England Journal of Medicine, 1990. 323(6): p. 357-365.
  8. Benner, M.F., et al., Diagnostic and prognostic evaluation of phenotypic markers TRAF1, MUM1, BCL2 and CD15 in cutaneous CD30-positive lymphoproliferative disorders. British Journal of Dermatology, 2009. 161(1): p. 121-127.
  9. Blaya-Nováková, V., et al., Dealing with Ebola virus disease in Spain: epidemiological inquiries received by the Department of Public Health Alerts, April to December 2014. Euro Surveill., 2015. 20(44).
  10. Cargill, M., et al., A Large-Scale Genetic Association Study Confirms IL12B and Leads to the Identification of IL23R as Psoriasis-Risk Genes. The American Journal of Human Genetics, 2007. 80(2): p. 273-290.
  11. Chang, S.E., et al., Congenital CD34-positive granular cell dendrocytosis. J Cutan Pathol, 1999. 26(5): p. 253-258.
  12. Chapman, A.S., et al., Cluster of Sylvatic Epidemic Typhus Cases Associated with Flying Squirrels, 2004–2006. Emerg. Infect. Dis., 2009. 15(7): p. 1005-1011.
  13. Charrel, R.N., et al., Toscana Virus RNA in Sergentomyia minuta Flies. Emerg. Infect. Dis., 2006. 12(8): p. 1299-1300.
  14. Cooper, H.L., et al., Treatment of resistant pemphigus vulgaris with an anti-CD20 monoclonal antibody (Rituximab). Clin Exp Dermatol, 2003. 28(4): p. 366-368.
  15. Dworkin, R.H., et al., A randomized, placebo-controlled trial of oxycodone and of gabapentin for acute pain in herpes zoster. Pain, 2009. 142(3): p. 209-217.
  16. Ekback, M., K. Wijma, and E. Benzein, "It is always on my mind": women's experiences of their bodies when living with hirsutism. Health Care Women Int, 2009. 30(5): p. 358-72.
  17. Farquhar, C., et al., Spironolactone versus placebo or in combination with steroids for hirsutism and/or Akne. Cochrane Database Syst Rev, 2003(4): p. CD000194.
  18. Fujimoto, E., et al., AGE-Modified Collagens I and III Induce Keratinocyte Terminal Differentiation through AGE Receptor CD36: Epidermal–Dermal Interaction in Acquired Perforating Dermatosis. Journal of Investigative Dermatology, 2010. 130(2): p. 405-414.
  19. Garcia-Herrera, A., et al., Primary Cutaneous Small/Medium CD4+ T-Cell Lymphomas: A Heterogeneous Group of Tumors With Different Clinicopathologic Features and Outcome. Journal of Clinical Oncology, 2008. 26(20): p. 3364-3371.
  20. Grogg, K.L., et al., Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma: a clonal T-cell lymphoproliferative disorder with indolent behavior. Mod Pathol, 2008. 21(6): p. 708-715.
  21. Jonuleit, H., et al., Induction of Interleukin 10–Producing, Nonproliferating Cd4 + T Cells with Regulatory Properties by Repetitive Stimulation with Allogeneic Immature Human Dendritic Cells. The Journal of Experimental Medicine, 2000. 192(9): p. 1213-1222.
  22. Kadin, M.E., Primary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorders, in Hematopathology. 2011, Elsevier BV. p. 604-616.
  23. Kadin, M.E., et al., Hodgkin's lymphoma of T-cell type: Clonal association with a CD30+ cutaneous lymphoma. Human Pathology, 2001. 32(11): p. 1269-1272.
  24. Kakabadze, T., et al., Oral lesions in HIV-positive patients in Georgia. Georgian Med News, 2008(165): p. 60-5.
  25. Kalman, L., et al., Mutations in genes required for T-cell development:IL7R, CD45, IL2RG, JAK3, RAG1, RAG2, ARTEMIS, and ADA and severe combined immunodeficiency: HuGE review. Genetics in Medicine, 2004. 6(1): p. 16-26.
  26. Kaudewitz, P., et al., Morphological, Immunological, and Clinical Features of Cutaneous CD30-Positive Large-Cell Tumors. The American Journal of Dermatopathology, 1989. 11(3): p. 285.
  27. Kerkhof, P.C.M. and C.M.R. Weemaes, Skin manifestations in congenital deficiency of leucocyte-adherence glycoproteins (CDLG). Br J Dermatol, 1990. 123(3): p. 395-401.
  28. Krieger, J.N., New Sexually Transmitted Diseases Treatment Guidelines* *A free copy of the Sexually Transmitted Diseases Treatment Guidelines (HHS Publication No. CDC 93-8017) may be obtained from the Superintendent of Documents, U. S. Government Printing Office, Washington, D. C. 20402; telephone (202) 783-3238 or Centers for Disease Control and Prevention, Atlanta, Georgia 30333; telephone (404) 332-4555. The Journal of Urology, 1995. 154(1): p. 209-213.
  29. Leib, S.R., T.C. McGuire, and D.J. Prieur, Comparison of the Tyrosine Aminotransferase cDNA and Genomic DNA Sequences of Normal Mink and Mink Affected with Tyrosinemia Type II. Journal of Heredity, 2005. 96(4): p. 302-309.
  30. Liu, H.L., et al., Cd30+ cutaneous lymphoproliferative disorders: The stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. Journal of the American Academy of Dermatology, 2003. 49(6): p. 1049-1058.
  31. MacDonald, A. and A. Forsyth, Nutritional deficiencies and the skin. Clin Exp Dermatol, 2005. 30(4): p. 388-390.
  32. McDougall, A.C., A GUIDE TO LEPROSY CONTROL, WHO/LEP/79.9. Leprosy Review, 1980. 51(1).
  33. Mohamed, E.E., et al., Trichoscopic changes in hair during treatment of hirsutism with 1064-nm neodymium:yttrium-aluminum-garnet laser. J Cosmet Dermatol, 2016. 15(1): p. 31-5.
  34. Na, The US Centers for Disease Control and Prevention (CDC) Advisory Committee on Immunization Practices (ACIP) has issued an update of its 2004 recommendations regarding influenza vaccination. PharmacoEconomics & Outcomes News, 2005. &NA;(483): p. 2.
  35. Nachbar, F., et al., The ABCD rule of dermatoscopy. Journal of the American Academy of Dermatology, 1994. 30(4): p. 551-559.
  36. Natkunam, Y., et al., Co-expression of CD56 and CD30 in lymphomas with primary presentation in the skin: clinicopathologic, immunohistochemical and molecular analyses of seven cases. Journal of Cutaneous Pathology, 2000. 27(8): p. 392-399.
  37. Oji, V., et al., Loss of Corneodesmosin Leads to Severe Skin Barrier Defect, Pruritus, and Atopy: Unraveling the Peeling Skin Disease. The American Journal of Human Genetics, 2010. 87(2): p. 274-281.
  38. Pham-Ledard, A., et al., IRF4 Gene Rearrangements Define a Subgroup of CD30-Positive Cutaneous T-Cell Lymphoma: A Study of 54 Cases. Journal of Investigative Dermatology, 2010. 130(3): p. 816-825.
  39. Pimpinelli, N., Primary cutaneous CD30+ T-cell lymphomas: hypothesis for a highly distinctive clinicobiological behaviour. Journal of the European Academy of Dermatology and Venereology, 2001. 15(2): p. 108-109.
  40. Poenitz, N., et al., Jessner’s Lymphocytic Infiltration of the Skin: A CD8+ Polyclonal Reactive Skin Condition. Dermatology, 2003. 207(3): p. 276-284.
  41. Price, M.L., et al., Lichen aureus: A localized persistent form of pigmented purpuric dermatitis. Br J Dermatol, 1985. 112(3): p. 307-314.
  42. Rotz, L.D., Public Health Assessment of Potential Biological Terrorism Agents. Emerg. Infect. Dis., 2002. 8(2): p. 225-230.
  43. Salisbury, C.L., S.D. Budnick, and S. Li, T-Cell Receptor Gene Rearrangement and CD30 Immunoreactivity in Traumatic Ulcerative Granuloma With Stromal Eosinophilia of the Oral Cavity. American Journal of Clinical Pathology, 2009. 132(5): p. 722-727.
  44. Svensson, L., et al., Leukocyte adhesion deficiency-III is caused by mutations in KINDLIN3 affecting integrin activation. Nature Medicine, 2009. 15(3): p. 306-312.
  45. Sweeney, M.H. and P. Mocarelli, Human health effects after exposure to 2,3,7,8-TCDD. Food Additives and Contaminants, 2000. 17(4): p. 303-316.
  46. Tardío, J.C. and R. Granados, The cellular component of the mucinous nevus consists of CD34-positive fibroblasts. Journal of Cutaneous Pathology, 2009. 37(9): p. 1019-1020.
  47. Van der Spuy, Z.M. and P.A. le Roux, Cyproterone acetate for hirsutism. Cochrane Database Syst Rev, 2003(4): p. CD001125.
  48. van Zuuren, E.J., et al., Interventions for hirsutism (excluding laser and photoepilation therapy alone). Cochrane Database Syst Rev, 2015. 4: p. CD010334.
  49. Vonderheid, E.C., A. Sajjadian, and M.E. Kadin, Methotrexate is effective therapy for lymphomatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders. Journal of the American Academy of Dermatology, 1996. 34(3): p. 470-481.
  50. Werner, B., et al., Large CD30-positive cells in benign, atypical lymphoid infiltrates of the skin. Journal of Cutaneous Pathology, 2008. 35(12): p. 1100-1107.
  51. Yang, X.R., et al., Identification of modifier genes for cutaneous malignant melanoma in melanoma-prone families with and without CDKN2A mutations. International Journal of Cancer, 2009. 125(12): p. 2912-2917.
  52. Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.