TY - JOUR
T1 - Recurrent Crow-Fukase syndrome associated with increased serum levels of vascular endothelial growth factor
T2 - A case report and review of the literature
AU - Mineta, Mitsuyoshi
AU - Hatori, Masahito
AU - Sano, Hirotaka
AU - Hosaka, Masami
AU - Kokubun, Shoichi
AU - Hiroki, Eri
AU - Hatakeyama, Akira
AU - Ogasawara, Tetsuro
PY - 2006/11/1
Y1 - 2006/11/1
N2 - Crow-Fukase syndrome (CFS) is a rare multi-system disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteins, skin changes and anasarca, with or without myeloma. The pathophysiology, diagnosis, and treatment of CFS are controversial. CFS may be associated with the overproduction of vascular endothelial growth factor (VEGF). However, there have been no reports of monitoring the serum VEGF level after recurrence, to the best of our knowledge. We report a 54-year-old man with CFS presenting with a 3-year history of ascites, anasarca, weakness of the lower extremities, and plasmacytoma in the scapula. At the initial examination, the VEGF level was 1,590 pg/ ml (the VEGF level of a healthy control, 78.4 ± 75.2 pg/ml). After initial treatment with chemotherapy and irradiation of the affected shoulder, the VEGF level decreased to 154 pg/ml and the symptoms disappeared. Twenty one months later, gate disturbance and anasarca recurred, and the VEGF level was over 2,000 pg/dl. After total scaplectomy, the VEGF level decreased to 730 pg/dl and the symptoms disappeared. The serum level of VEGF well correlated to the clinical course of the patient. In conclusion, measurement of the VEGF level is useful for diagnosing CFS and for monitoring its clinical course.
AB - Crow-Fukase syndrome (CFS) is a rare multi-system disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteins, skin changes and anasarca, with or without myeloma. The pathophysiology, diagnosis, and treatment of CFS are controversial. CFS may be associated with the overproduction of vascular endothelial growth factor (VEGF). However, there have been no reports of monitoring the serum VEGF level after recurrence, to the best of our knowledge. We report a 54-year-old man with CFS presenting with a 3-year history of ascites, anasarca, weakness of the lower extremities, and plasmacytoma in the scapula. At the initial examination, the VEGF level was 1,590 pg/ ml (the VEGF level of a healthy control, 78.4 ± 75.2 pg/ml). After initial treatment with chemotherapy and irradiation of the affected shoulder, the VEGF level decreased to 154 pg/ml and the symptoms disappeared. Twenty one months later, gate disturbance and anasarca recurred, and the VEGF level was over 2,000 pg/dl. After total scaplectomy, the VEGF level decreased to 730 pg/dl and the symptoms disappeared. The serum level of VEGF well correlated to the clinical course of the patient. In conclusion, measurement of the VEGF level is useful for diagnosing CFS and for monitoring its clinical course.
KW - Crow-Fukase syndrome
KW - Polyneuropathy
KW - Vascular endothelial growth factor (VEGF)
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U2 - 10.1620/tjem.210.269
DO - 10.1620/tjem.210.269
M3 - Article
C2 - 17077605
AN - SCOPUS:33750710096
VL - 210
SP - 269
EP - 277
JO - Tohoku Journal of Experimental Medicine
JF - Tohoku Journal of Experimental Medicine
SN - 0040-8727
IS - 3
ER -