[Thrombotic complication in the course of aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome; possible involvement of dysplasminogenemia (plasminogen Tochigi) in the pathogenesis of thrombosis].
A 44-year-old Japanese man having aplastic anemia (AA)-paroxyamal nocturnal hemoglobinuria (PNH) syndrome was referred to our hospital because of purpuras due to thrombocytopenia in July 1994. He suffered from pneumonia after admission, complicated with cerebral, splenic, and left renal infarction. Pulmonary infaction was also confirmed by perfusion lung scan. He had a plasma plasminogen (PLG) functional activity of 54.4% with a normal level of PLG antigen. The gel isoelectrofocusing pattern of the plasminogen derived from the patient showed 10 normal bands and 10 additional doublet bands with slightly higher isoelectric points than the normal components. Abnormal PLG is converted by urokinase to an inactive two-chain plasmin molecule. These findings were similar to those of a case with dysplasminogenemia (PLG Tochigi) reported by Aoki et al. He was given warfarin for the prevention of thrombosis in December 1994. As of October 1995, these was no recurrence of thrombosis. The cause of thrombosis in the present case have been the association with PNH, predisposition to PLG Tochigi, or the complication of pneumonia. This is the first report of AA/PNH syndrome associated with dysplasminogenemia.
Takahashi
H
H
Department of Medicine, Jichi Medical School, Tochigi, Japan.
Izumi
T
T
Toshima
M
M
Kawano
C
C
Komatsu
N
N
Imagaua
S
S
Hatake
K
K
Madoiua
S
S
Baba
M
M
Sakata
Y
Y
Miura
Y
Y
D000328:Adult; D000741:Anemia, Aplastic / Q000150:complications*; D000925:Anticoagulants / Q000627:therapeutic use; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D008297:Male; D010958:Plasminogen / Q000172:deficiency*; D011014:Pneumonia / Q000150:complications; D013577:Syndrome; D013927:Thrombosis / Q000188:drug therapy / Q000209:etiology*; D014859:Warfarin / Q000627:therapeutic use
D000925:Anticoagulants; D014859:Warfarin; D010958:Plasminogen