[Werner's syndrome associated with acute myelofibrosis].
A 47-year-old man was admitted to our hospital in June 1997 because of nasal bleeding. He presented with anemia in addition to physical characteristics of Werner's syndrome (WS). Peripheral blood examination disclosed pancytopenia with 4% blasts. Bone marrow aspiration was a dry tap; biopsy specimens revealed myelofibrosis. Chromosomal analysis of peripheral blood revealed hypodiploidy with complex abnormalities including -5 and del(7)(q21). Serum levels of PDGF, FGF, and TGF beta 1 were normal. A diagnosis of acute myelofibrosis was made. The patient's condition became quickly deteriorated and he died of pneumonia in October 1997. In the literature, we found 6 reported cases of myelofibrosis associated with WS. Considering that only approximately 1,100 cases of WS have been reported so far, the incidence of myelofibrosis in WS seems relatively high. This case suggested a link between WS and myelofibrosis, and the mechanism of myelofibrosis in WS was discussed.
Shimura
K
K
Second Department of Medicine, Kyoto Prefectural University of Medicine.
Shimazaki
C
C
Okamoto
A
A
Takahashi
R
R
Hirai
H
H
Sumikuma
T
T
Ashihara
E
E
Inaba
T
T
Fujita
N
N
Komatsu
S
S
Hirano
S
S
Nakagawa
M
M
D000208:Acute Disease; D017809:Fatal Outcome; D006801:Humans; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics; D014898:Werner Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics