Primär kutanes Marginalzonen B-Zell-Lymphom (multiple Läsionen)

Letztmals aufdatiert: 2020-08-06

Autor(en): -

Immunozytome, Kutane Plasmozytome.

Niedrig malignes, selten vorkommendes, primär kutanes B-Zell Lymphom.

Chornische Stimuli (Borrelia burgdorferi, Hepatitis C, Helicoobacter pylori).

Solitäre oder multiple erytheamötse prall-elastisch palpable, nicht schuppende Plaques und Knoten.

  • Klinik
  • Labor
  • BSG/CRP, Diff.-BB (Leukozytose, rel. Lymphozytose), Leber- und Nierenwerte, LDH, Elektrolyte.
  • Borrelienserologie
  • Ggf. Immunelektrophorese
  • Ggf. HTLV-Serologie
  • Biopsie
  • Dermatopathologie
  • Immunhistologie:   CD 19, CD20, CD79a, bcl-6 und CD10 pos. und meist bcl-2 neg.
  • Molekularbiologisch: PCR für die Immunglobulinkette
  • Lymphknotensonographie, Abdomensonographie, Röntgen-Thorax.
  • Knochenmarksbiopsie optional.

5-Jahres-Überlebensrate: 98%.

Ausdehnung Therapie der ersten Wahl Therapie der zweiten Wahl

Solitäre Läsionen

 

 

 

 

 

 

 

Multiple Läsionen

·         Antibiotika (falls borrelienassoziiert)

·         Exzision und/oder Radiotherapie

·         Intraläsional Rituximab

·         Intraläsional IFN-α

·         Intraläsional Steroid

 

·         Antibiotika (falls borrelienassoziiert)

·         Radiotherapie

·         Intraläsional IFN-α

·         Intraläsional Rituximab

·         i.v. Rituximab

 

 

 

 

 

 

 

·         Bendamustin in Kombination mit i.v.

·         Rituximab

·         Doxorubicin oder Gemcitabin

·         ggf. in Kombination mit Rituximab

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

 

1.         Arai, E., M. Shimizu, and T. Hirose, A review of 55 cases of cutaneous lymphoid hyperplasia: reassessment of the histopathologic findings leading to reclassification of 4 lesions as cutaneous marginal zone lymphoma and 19 as pseudolymphomatous follikulitis. Human Pathology, 2005. 36(5): p. 505-511.

2.         Bailey, E.M., et al., Marginal Zone Lymphoma (Low-grade B-cell Lymphoma of Mucosa-associated Lymphoid Tissue Type) of Skin and Subcutaneous Tissue. The American Journal of Surgical Pathology, 1996. 20(8): p. 1011-1023.

3.         Baldassano, M.F., et al., Cutaneous Lymphoid Hyperplasia and Cutaneous Marginal Zone Lymphoma. The American Journal of Surgical Pathology, 1999. 23(1): p. 88-96.

4.         Cerroni, L., et al., Primary Cutaneous Marginal Zone B-Cell Lymphoma: A Recently Described Entity of Low-Grade Malignant Cutaneous B-Cell Lymphoma. The American Journal of Surgical Pathology, 1997. 21(11): p. 1307-1315.

5.         Deutsch, A.J.A., et al., Primary Cutaneous Marginal Zone B-Cell Lymphomas Are Targeted by Aberrant Somatic Hypermutation. Journal of Investigative Dermatology, 2009. 129(2): p. 476-479.

6.         Goldberg, L.J., Cicatricial marginal alopecia: is it all traction? British Journal of Dermatology, 2009. 160(1): p. 62-68.

7.         Lan, T.T., N.A. Brown, and A.C. Hristov, Controversies and considerations in the diagnosis of primary cutaneous CD4(+) small/medium T-cell lymphoma. Arch Pathol Lab Med, 2014. 138(10): p. 1307-18.

8.         van Maldegem, F., et al., The majority of cutaneous marginal zone B-cell lymphomas expresses class-switched immunoglobulins and develops in a T-helper type 2 inflammatory environment. Blood, 2008. 112(8): p. 3355-3361.

9.         Willemze, R., et al., Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol, 2013. 24 Suppl 6: p. vi149-54.

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