Cavernous lymphangioma of the spleen in a patient with Klippel-Trenaunay-Weber syndrome.
A 31-year-old woman presented with hypertrophy of the left upper extremity and thrombocytopenia. Physical examination revealed splenomegaly, and laboratory investigation revealed thrombocytopenia, elevation of cross-linked fibrin degradation products (XDP), and thrombin-antithrombin III complex (TAT). A diagnosis of Klippel-Trenaunay-Weber (K-T-W) syndrome was established by the dermatologic findings and angiography of the extremities. A splenic cavernous lymphangioma was diagnosed by ultrasonography and angiography, and was confirmed by pathology following splenectomy. Post-operatively, the platelet count increased, and hemostatic parameters normalized. Cavernous lymphangioma is a rare complication of Klippel-Trenaunay-Weber syndrome. Splenectomy proved to be an effective therapy for both cavernous lymphangioma and consumptive coagulopathy in Klippel-Trenaunay-Weber syndrome.
Yamazaki
M
M
Department of Internal Medicine (III), Kanazawa University School of Medicine.
Kawamura
Y
Y
Ohka
T
T
Katada
S
S
Morita
K
K
Nakagawa
M
M
Kubo
E
E
Kawashima
A
A
Shimizu
H
H
Nobata
K
K
D000328:Adult; D004211:Disseminated Intravascular Coagulation / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications*; D008202:Lymphangioma / Q000209:etiology* / Q000601:surgery; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000209:etiology* / Q000601:surgery