[A case of hepatosplenic gammadelta T-cell lymphoma associated hemophagocytic syndrome].
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.
Iyama
Satoshi
S
Fourth Dept. of Internal Medicine, Sapporo Medical University, School of Medicine.
Matsunaga
Takuya
T
Sato
Tsutomu
T
Sato
Yasushi
Y
Sagawa
Tamotsu
T
Murase
Kazuyuki
K
Kida
Masaya
M
Kobune
Masayoshi
M
Takimoto
Risyu
R
Kato
Junji
J
Niitsu
Yoshiro
Y
D001706:Biopsy; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D008875:Middle Aged; D049268:Positron-Emission Tomography; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics / Q000378:metabolism*; D013160:Splenic Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology*; D014057:Tomography, X-Ray Computed
D016692:Receptors, Antigen, T-Cell, gamma-delta