An unusual case of a splenic gamma/delta T-cell lymphoma with angiocentric tendency and haemophagocytic syndrome.
We report here an unusual post-thymic T-cell neoplasia of the spleen associated with a rapidly progressive haemophagocytic syndrome. The lymphoma was classified as a medium- to large sized pleomorphic T-cell lymphoma with angiocentric tendency (CD3+, CD43+, CD45RO+ CD45+). Clonality was confirmed by PCR and revealed rearrangement of the T-cell receptor gamma chain. Serological tests excluded a recent EBV infection and in situ hybridization with the EBER probe was negative. Haemophagocytic syndrome was the initial finding in an otherwise symptomless patient and this deteriorated with progression of the T-cell malignancy. Both, the T-cell lymphoma and the haemophagocytic syndrome remained unaffected by chemotherapy. Splenic gamma/delta T-cell lymphoma associated with haemophagocytosis is an uncommon entity which has until now not been widely recognized.
Zabernigg
A
A
Department of Internal Medicine, Hospital Kufstein, Austria.
Fend
F
F
Thaler
J
J
Gattringer
C
C
D000368:Aged; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000473:pathology; D006801:Humans; D016399:Lymphoma, T-Cell / Q000139:chemically induced* / Q000473:pathology / Q000648:ultrastructure; D008297:Male; D016692:Receptors, Antigen, T-Cell, gamma-delta; D013160:Splenic Neoplasms / Q000139:chemically induced* / Q000473:pathology / Q000648:ultrastructure
D016692:Receptors, Antigen, T-Cell, gamma-delta