[A case of hepatosplenic gammadelta T-cell lymphoma associated hemophagocytic syndrome].

Abstract

A 56-year-old woman was admitted to our hospital for examination of high fever, liver dysfunction, pancytopenia, elevated lactate dehydrogenase (LDH) and ferritin, which were not improved by methylprednisolone pulse therapy. Although bone marrow aspiration revealed hypocellularity with no apparent activated macrophages, all other data strongly suggested hemophagocytic syndrome (HPS). She was then treated with chemotherapy consisting of etoposide, prednisolone and cyclosporine, which resulted in transient improvement and allowed her to undergo whole-body fluorine-18fluorodeoxyglucose positron emission tomography (FDG-PET) analysis. FDG uptake was elevated especially in the spleen and liver. A liver biopsy was performed and the examination of the specimen with immunohistochemical staining and PCR analysis revealed monoclonal infiltration of gammadelta T-cell. Despite the repeated chemotherapy, she deteriorated rapidly and succumbed to multi-organ failure. A postmortem examination revealed massive infiltration of activated macrophages with hemophagocytosis in the spleen, liver, bone marrow and perisplenic lymph nodes.

• Authors: Satoshi Iyama, Takuya Matsunaga, Tsutomu Sato, Yasushi Sato, Tamotsu Sagawa, Kazuyuki Murase, Masaya Kida, Masayoshi Kobune, Risyu Takimoto, Junji Kato, Yoshiro Niitsu
• Journal: Gan to kagaku ryoho. Cancer & chemotherapy (Gan To Kagaku Ryoho) Vol. 35 Issue 9 Pg. 1623-7 (Sep 2008) ISSN: 0385-0684 [Print] Japan
• PMID: 18799926 (Publication Type: Case Reports, Journal Article)

Chemical References

• Receptors, Antigen, T-Cell, gamma-delta

Topics

• Biopsy
• Female
• Humans
• Liver Neoplasms (complications, diagnostic imaging, metabolism, pathology)
• Lymphohistiocytosis, Hemophagocytic (complications, diagnostic imaging, metabolism, pathology)
• Lymphoma, T-Cell (complications, diagnostic imaging, metabolism, pathology)
• Middle Aged
• Positron-Emission Tomography
• Receptors, Antigen, T-Cell, gamma-delta (genetics, metabolism)
• Splenic Neoplasms (complications, diagnostic imaging, metabolism, pathology)
• Tomography, X-Ray Computed