Neutrophil rich CD30 Positive Primary Cutaneous Anaplastic Large Cell Lymphoma: A Case Report and Review of Literature

Abstract

We describe a case of neutrophil rich variant of primary cutaneous anaplastic large cell lymphoma (PCALCL) and the clinicopathological features of cases reported in the literature. In all cases the male to female ratio is 1.4 : 1. The age at presentation ranges between 12 and 82 years with a mean age of 50.73 years. Various clinical lesions including nodules (47.8 %), tumormasses (34.8 %), plaques (21.7 %) and unusual variants (13.4 %) are noted. 47.8 % of lesions are associated with ulceration. Multifocal lesions are seen in about 18.4 % of cases. Lesions are located in extremities (44.7 %), trunk (36.8 %), head and neck (34.2 %). Around 10.5 % of patients are immunocompromised. About 5.3 % of cases show peripheral neutrophilia. Histopathological examinations show diffuse dermal infiltrate of large anaplastic (53.5 %), pleomorphic (27.9 %) or small to medium sized atypical lymphocytes (18.6 %) in inflammatory background. Subcutaneous tissues are involved in about 43.5 % of biopsies. Some biopsies show epidermal changes as epidermotropism (32.6 %), hyperplasia (20.9 %) or ulceration (7 %). 18.6 % of specimens show necrosis and angiodestruction. We try to increase awareness of this rare variant.

Zusammenfassung

Wir berichten, unter Berücksichtigung der bislang publizierten klinischen und histologischen Besonderheiten, von einer 66-jährigen Patientin mit einer seltenen neutrophilenreichen Variante eines primär kutanen großzellig-anaplastischen T-Zell-Lymphoms. Bisher wurden Ersterkrankungen an dieser seltenen Entität im Alter von 12 bis 82 Jahren beschrieben mit einem mittleren Erkrankungsalter von 50,73 Jahren. Klinisch-morphologisch sind verschiedene Varitanten beschrieben, am häufigsten Knoten (47,8 %), Tumore (34,8 %), Plaques (21,7 %) und selten andere Varianten (13,4 %). In 47,8 % der Fälle zeigen die Hautveränderungen zusätzlich Ulzerationen. Eine Disseminierung wurde bei 18,4 % der Fälle beobachtet. Hierbei traten die Hauterscheinungen zu 44 % an den Extremitäten, zu 36 % am Stamm und zu 34,2 % im Kopf-Hals-Bereich auf. Etwa ein Fünftel aller betroffenen Personen wies eine Immunkompromittierung sowie 5,3 % eine Blutneutrophilie auf.

Die histologischen Untersuchungen zeigten überwiegend ein diffuses dermales Infiltrat großer anaplastischer (53,5 %), pleomorpher (27,9 %) oder klein- bis mittelgroßer atypischer (18,6) Lymphozyten, eingebettet in einem dichten entzündlichen, neutrophilenreichen Begleitinfiltrat. Das subkutane Fettgewebe war in 43,5 % der Fälle mit einbezogen. Auch epidermale Veränderungen zeigten sich mit Epidermotropismus (32,6 %), Hyperplasie (20,9 %) oder Ulzeration (7,0 %) variabel. In 18,6 % der Präparate waren Nekrosen und Angiodestruktion nachzuweisen. Aufgrund der ausgeprägten Neutrophilie in der Histologie können die histologischen Veränderungen leicht als primär entzündlich respektive phlegmonös-abszedierende Erkrankung verkannt werden. Mit der vorliegenden Arbeit möchten wir das Bewusstsein für diese seltene Lymphomentität schärfen.

• References

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