Hodkin's disease, mixed cellularity ICD-10: 81.2; ICD-O: 9652/3
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Frau, 27 J. Lymphknotenexzision wegen schmerzloser Vergrösserung
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- Zerstörung der Struktur des Lymphknotens durch buntes Zellinfiltrat
- Diagnose durch Nachweis von zwei- und mehrkernigen Sternberg-Reed'schen
Zellen und von einkernigen Hodgkin-Zellen
in einem Mischzellinfiltrat:
- Hodgkin-Zellen: Grosse, blastoide,
einkernige Zellen mit nierenförmigem Kern und grossen, einschlussartigen,
eosinophilen Nukleolen
- Sternberg-Reed'sche Zellen:
Entstehen aus Hodgkin-Zellen und besitzen dementsprechende Kerne (meist
zwei), die typischerweise spiegelbildlich angeordnet sind
- Mischzelleninfiltrat aus reaktiven, nicht malignen Zellen:
- Lymphozyten
- +/- epitheloidzellige Histiozyten
- Eosinophile Leukozyten
- Plasmazellen
- In einigen Präparaten finden sich zusätzlich sarkoidoseartige
Granulome mit Epitheloidzellen und Riesenzellen vom Langhans-Typ (Langhans-RZ)
Klinik:
Häufigkeitsgipfel bimodal, 2. - 3. und 6. - 7. Lebensdekade,
Lymphknotenschwellung, undulierendes Fieber vom Pel-Epstein-Typ, Juckreiz
"Staging" zur klinischen Stadieneinteilung, wichtig für
Prognose und Therapie
27-year-old female experienced painless swelling of supraclavicular lymph nodes on her left side. A lymph node was excised.
- The architecture of the lymph node is effaced by a mixed cellular infiltrate.
- Within the background of the mixed infiltrate numerous mononuclear Hodgkin and multinucleated Reed-Sternberg cells (Sternberg-Reed'schen
Zellen) are distinguished. The latter are diagnostic of classical
Hodgkin’s lymphoma.
- Hodgkin cells (Hodgkin-Zellen) are large slightly acidophilic to amphophilic cells containing one nucleus outlined by a thick nuclear membrane.
A large usually centrally located, highly acidophilic nucleolus surrounded by a clear halo is characteristic. Reed-Sternberg cells (Sternberg-Reed'sche Zellen)
are the multinucleated variants of Hodgkin cells and thus display according nuclear
features. To be diagnostic, Reed-Sternberg cells must contain minimally two nucleoli in two separate nuclear lobes. If the two nuclei are arranged in a mirror image like fashion,
the typical ‘owl eye’ appearance is observed.
- Hodgkin and Reed-Sternberg cells, the actual tumor cells, account for only a minority of the cells of the infiltrate. The background infiltrate is composed of lymphocytes (Lymphozyten),
eosinophilic granulocytes, plasma cells (Plasmazellen) and histiocytes.
- On occasion, non-caseating granulomas may be observed (not seen on the section shown) with Langhans giant cells.
Epidemiology: Classical Hodgkin lymphoma accounting for 95% of all Hodgkin lymphomas shows a bimodal age distribution with a first peak incidence at 15-35 years and a second
between the 6th and 7th decade. Mixed cellularity classical Hodgkin lymphoma accounts for 20-25% of classical Hodgkin’s lymphoma and does not display a bi-modal age
distribution (median age 37 years).
Clinical features: Most frequent presenting symptom of classical Hodgkin lymphoma is peripheral lymphadenopathy affecting 1 to 2 node-bearing areas. Around 40% of patients
suffer from systemic symptoms (B symptoms), namely relapsing fever (so-called Pel-Epstein fever), night sweats and significant body weight loss. Patients with mixed cellularity
Hodgkin lymphoma often present at an advanced stage (III or IV).
Staging: Clinical and occasionally pathological staging determine the mode of treatment of Hodgkin’s lymphomas. Stage and the presence of B symptoms are important predictive
factors regarding prognosis.