Sonographic diagnosis of venocclusive disease of the liver and danazol therapy for autoimmune thrombocytopenia in an autologous marrow transplant patient.
A 16-Year-old boy with lymphoblastic lymphoma underwent an autologous bone marrow transplantation (ABMT) after conditioning with high-dose busulfan and cyclophosphamide. On day 39 post-transplant, right upper quadrant pain occurred with an increase in the size of the liver. Liver function tests showed a subsequent deterioration. Ultrasonographic studies of the abdomen disclosed hepatosplenomegaly, ascites, thickening of the gall bladder wall and a failure to visualize the major hepatic veins. The venocclusive disease of the liver (VOD) diagnosis was confirmed from these findings. Ultrasonographic monitoring reflected the disease status well and demonstrated a complete recovery from the VOD. We emphasize, thus, that abdominal ultrasonography can be applied easily, being a non-invasive procedure, and is useful in diagnosing VOD. Furthermore, the procedure can be repeated serially for evaluating the severity of VOD. Although engraftment was confirmed with granulocytes exceeding 500/microliters, platelet recovery was delayed; the megakaryocytes had not decreased in bone marrow aspirates and platelet-associated IgG was significantly elevated. Since autoimmune thrombocytopenia was highly suspected, to prevent immunosuppression danazol was given as an immune modulator instead of prednisolone, and a complete recovery was obtained. Accordingly, danazol can be used as an alternative to prednisolone for the treatment of autoimmune thrombocytopenia after bone marrow transplantation.
First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka.
E
E
Matsuishi
K
K
Anzai
K
K
Dohmen
S
S
Taniguchi
H
H
Gondo
J
J
Kudo
T
T
Shibuya
H
H
Ishibashi
M
M
Harada
Y
Y
Niho
D000293:Adolescent; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy*; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D003613:Danazol / Q000627:therapeutic use*; D006504:Hepatic Veno-Occlusive Disease / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000097:blood / Q000601:surgery*; D008297:Male; D011245:Pregnadienes / Q000627:therapeutic use*; D013921:Thrombocytopenia / Q000150:complications / Q000188:drug therapy*; D014463:Ultrasonography
D011245:Pregnadienes; D003613:Danazol