Successful Treatment of Life-Threatening Cerebral Bleeding Associated with Disseminated Intravascular Coagulation Using Recombinant Human Soluble Thrombomodulin in a Patient with Mixed Phenotype Acute Leukemia with t (9; 22) (q34; q11.2); <i>Bcr-Abl1</i>
- 1 Department of Hematology, National Hospital Organization Disaster Medical Center, Tachikawa, Tokyo, Japan
- 2 Department of Hematology, National Hospital Organization Disaster Medical Center, Tachikawa, Tokyo, Japan
- 3 Department of Hematology, National Hospital Organization Disaster Medical Center, Tachikawa, Tokyo, Japan
- 4 Department of Hematology, National Hospital Organization Disaster Medical Center, Tachikawa, Tokyo, Japan
- 5 Department of Hematology, National Hospital Organization Disaster Medical Center, Tachikawa, Tokyo, Japan
- 6 Department of Hematol- ogy, National Center for Global Health and Medicine, Tokyo, Japan
Abstract
Recently, mixed phenotype acute leukemia (MPAL) with t (9; 22) (q34; q11.2); bcr - abl1 was described as one kind of acute leukemia of ambiguous lineage in the 2008 World Health Organization Classification of Tumors of Hematopoi etic and Lymphoid Tissues. However, treatment strategy remains difficult for this uncommon MPAL. In addition, this type of MPAL is at high risk of tumor lysis syndrome (TLS) because of high chemo-sensitivity. Here, we report a MPAL with t (9; 22) (q34; q11.2); bcr - abl1 case that suffered from life-threatening cerebral bleeding associated with disseminated intravascular coagulation (DIC) with TLS after bcr-abl positive acute lymphoblastic leukemia (ALL) type induction therapy who was successfully treated with recombinant human thrombomodulin (rhTM). This case reached complete remission without additive cerebral bleeding. In conclusion, bcr-abl positive ALL type induction therapy was effective for MPAL with t (9; 22) (q34; q11.2); bcr - abl1 and rhTM was effective against DIC with TLS.
- S. H. Swerdlow, E. Campo, N. L. Harris, et al., “Acute Leukemias of Ambiguous Lineage,” In: S. H. Swerdlow, E. Campo, N. L. Harris, et al., Eds., World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon, 2008, pp. 150-155.
- Y. Wang, M. Gu, Y. Mi, L. Qiu, S. Bian and J. Wang, “Clinical Characteristics and Outcomes of Mixed Phenotype Acute Leukemia with Philadelphia Chromosome Positive and/or Bcr-Abl Positive in Adult,” International Journal of Hematology, Vol. 94, No. 5, 2011, pp. 552-555. doi:10.1007/s12185-011-0953-1
- P. Tosi, G. Barosi, C. Lazzaro, V. Liso, M. Marchetti, E. Morra, A. Pession, G. Rosti, A. Santoro, P. L. Zinzani and S. Tura, “Consensus Conference on the Management of Tumor Lysis Syndrome,” Hae-matologica, Vol. 93, No. 12, 2008, pp. 1877-1885. doi:10.3324/haematol.13290
- N. Zojer and H. Ludwig, “Hematological Emergencies,” Annals of Oncology, Vol. 18, No. Supplement 1, 2007, pp. i45-i48. doi:10.1093/annonc/mdl450
- H. Wada, T. Nagano, M. Tomeoku, M. Kuto, Y. Karitani, K. Deguchi and S. Shirakawa, “Coagulant and Fibrinolytic Activities in the Leukemic Cell Lysates,” Thrombosis Research, Vol. 30, No. 4, 1983, pp. 315-322. doi:10.1016/0049-3848(83)90223-2
- M. Levi and H. T. Cate, “Disseminated Intravascular Coagulation,” The New England Journal of Medicine, Vol. 341, No. 8, 1999, pp. 586-592. doi:10.1056/NEJM199908193410807
- T. Iba, E. Nakarai, T. Takayama, K. Nakajima, T. Sasaoka and Y. Ohno, “Combination Effect of Antithrombin and Recombinant Human Soluble Thrombomodulin in a Lipopolysaccharide Induced Rat Sepsis Model,” Critical Care, Vol. 13, No. 6, 2009, p. R203. doi:10.1186/cc8210
- K. Yamakawa, S. Fujimi, T. Mohri, H. Matsuda, Y. Nakamori, T. Hirose, O. Tasaki, H. Ogura, Y. Kuwagata, T. Hamasaki and T. Shimazu, “Treatment Effects of Recombinant Human Soluble Thrombomodulin in Patients with Severe Sepsis: A Historical Control Study,” Critical Care, Vol. 15, No. 3, 2011, p. R123. doi:10.1186/cc10228
- S. Moll, C. Lindley, S. Pescatore, D. Morrison, K. Tsuruta, M. Mohri, M. Serada, M. Sata, H. Shimizu, K. Yamada and G. C. White, “Phase I Study of a Novel Recombinant Human Soluble Thrombomodulin, ART-123,” Journal of Thrombosis and Haemostasis, Vol. 2, No. 10, 2004, pp. 1745-1751. doi:10.1111/j.1538-7836.2004.00927.x
- H. Saito, S. Maruyama, S. Shimazaki, Y. Yamamoto, N. Akikawa, R. Ohno, A. Hirayama, T. Matsuda, H. Asakura, M. Nakashima and N. Aoki, “Efficacy and Safety of Recombinant Human Soluble Thrombomodulin (ART-123) in Disseminated Intravascular Coagulation: Results of a Phase III, Randomized, Double-Blind Clinical Trial,” Journal of Thrombosis and Haemostasis, Vol. 5, No. 1, 2007, pp. 31-41. doi:10.1111/j.1538-7836.2006.02267.x