Successful treatment with recombinant thrombomodulin for disseminated intravascular coagulation complicated with hemophagocytic syndrome

• Published in: Rinshō ketsueki Vol. 56; no. 3; p. 312
• Main Authors: Yamazaki, Hiroyuki, Kondo, Tadakazu, Tatsumi, Goichi, Kotani, Shin-Ichi, Arai, Yasuyuki, Shirakawa, Kotaro, Kitano, Toshiyuki, Hishizawa, Masakatsu, Kadowaki, Norimitsu, Takaori-Kondo, Akifumi
• Format: Journal Article
• Language: Japanese
• Published: Japan 01.03.2015
• Subjects: Aged; Disseminated Intravascular Coagulation - complications; Disseminated Intravascular Coagulation - diagnosis; Disseminated Intravascular Coagulation - drug therapy; Female; Humans; Lymphohistiocytosis, Hemophagocytic - complications; Lymphohistiocytosis, Hemophagocytic - therapy; Male; Middle Aged; Recombinant Proteins - administration & dosage; Recombinant Proteins - therapeutic use; Thrombomodulin - administration & dosage; Thrombomodulin - therapeutic use; Treatment Outcome; Young Adult
• ISSN: 0485-1439
• DOI: 10.11406/rinketsu.56.312

Recombinant human thrombomodulin (rTM) improves the blood coagulation disorder characteristic of disseminated intravascular coagulation (DIC) as well as, or even better than, other anti-DIC drugs. On post-marketing surveillance, its effectiveness has been recognized for hematologic disorders, sepsis and solid tumor subgroups. However, the effect on hemophagocytic syndrome (HPS) complicated by DIC remains unclear. We treated three HPS patients with rTM in addition to chemotherapy for the underlying diseases including nasal NK/T cell lymphoma, angioimmunoblastic T-cell lymphoma and refractory acute myeloid leukemia post cord blood transplantation. Although being refractory to medical management was suspected in our cases, clinical status rapidly came under control including not only amelioration of the blood coagulation disorder but also inflammatory reactions, such as serum ferritin and lactic acid dehydrogenase abnormalities, which represent HPS activity. These observations suggest that rTM might exert marked synergistic effects on HPS with DIC. Given the results obtained in these three cases, administration of rTM appears to offer a promising method of treating HPS complicated by DIC.