Zusammenfassung
Die aktuelle WHO/EORTC-Klassifikation differenziert in der Gruppe der seltenen primär
kutanen B-Zell-Lymphome (I) das Marginalzonen B-Zell-Lymphom (PCMZL), (II) das follikuläre
Lymphom (PCFCL), und (III) das kutane großzellige B-Zell-Lymphom vom Bein-Typ (PCLBCL-LT)
und (IV) andere primär kutane großzellige B-Zell-Lymphome (PCLBCLO) [1]. Letztere sind diffuse großzellige B-Zell-Lymphome, die weder in die Gruppe der
PCLBCL-LT noch der PCFCL passen. Während PCFCL und PCLBCL-LT klare Unterschiede in
der Klinik, Histopathologie und Prognose zeigen, ist das PCLBCLO durch überlappende
klinisch-pathologische Merkmale gekennzeichnet.
Ein 47-jähriger Mann bemerkte seit einigen Monaten eine rötliche Makula an der rechten
Schulter, die er als Akneeffloreszenz ansah, da er in seiner Jugend unter starker
Akne litt. Eine systemische Doxycyclintherapie bei Hinweis auf eine floride Borreliose
erbrachte keine Besserung. Eine Hautbiopsie ergab die Diagnose eines follikulären
Lymphoms mit Übergang in ein diffuses großzelliges B-Lymphom. Die Therapie mit einer
modifizierten R-CHOP (Rituximab, Cyclophosphamid, pegyliertes liposomales Doxorubicin,
Vincristin und Prednisolon) zeigte ein gutes Ansprechen.
Follicular Lymphoma with Transition to a Diffuse Large B-Cell-Lymphoma
In the new World Health Organization/European Organization for Research and Treatment
of Cancer (WHO/EORTC) classification primary cutaneous B-cell-lymphomas are categorized
in marginal zone B-cell lymphoma (PCMZL), follicle centre lymphomas (PCFCL), large
B-cell-lymphoma leg-type (PCLBCL-LT) and other large B-cell-lymphoma (PCLBCLO) [1]. While PCFCL and PCLBCL-LT show distinct characteristic presentations, clinical
and pathological, PCLBCLO share characteristics of both categories.
A 47-year old man observed a red macule on his right shoulder for some month. He assumed
it as an acne skin lesion because he suffered from severe acne in his youth. Under
suspicion of an infection with borrelia he underwent a systemic therapy with doxycyclin
without any clinical improvement. A subsequent biopsy revealed the diagnosis of a
follicle center cell lymphoma with a transition into a diffuse large B-cell-lymphoma.
The patient was started on a modified therapy with R-CHOP (rituximab, cyclophosphamide,
pegylated liposomal doxorubicine, vincristine and prednisolone) and experienced a
good clinical response.
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Dr. Claudia-S. Vetter-Kauczok
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie
Universitätsklinikum Würzburg
Josef-Schneider-Straße 2
97080 Würzburg
eMail: kauczok_c@klinik.uni-wuerzburg.de
