Nasal T-cell lymphoma associated with hemophagocytic syndrome. Immunohistochemical and genotypic studies.
A 53-year-old man with nasal T-cell lymphoma exhibited hemophagocytic syndrome as a terminal event. Immunohistochemical studies revealed that neoplastic cells were derived from T cells. Genotypic analysis of DNA samples that were obtained from the frozen tissue specimens demonstrated clonal rearrangements of the T-cell receptor beta-chain genes. No rearrangement was observed in the immunoglobulin heavy-chain gene. To our knowledge, only three cases of nasal T-cell lymphoma with hemophagocytic syndrome have been reported before the present case. All of these cases occurred in Oriental patients. The present report suggests the beneficial effect of high-dose glucocorticoid therapy on the prolongation of survival compared with that of the other three cases.
Chubachi
A
A
Third Department of Internal Medicine, Akita University School of Medicine, Japan.
Imai
H
H
Nishimura
S
S
Saitoh
M
M
Miura
A B
AB
D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D005838:Genotype; D006644:Histiocytes / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000235:genetics / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity; D009669:Nose Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology; D013154:Spleen / Q000473:pathology