Advances in the diagnosis and treatment of disseminated intravascular coagulation in haematological malignancies
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PROGRESS IN HEMATOLOGY Advances in diagnosis and treatment of disseminated intravascular coagulation
Advances in the diagnosis and treatment of disseminated intravascular coagulation in haematological malignancies Takayuki Ikezoe1 Received: 24 June 2020 / Revised: 22 August 2020 / Accepted: 28 August 2020 © Japanese Society of Hematology 2020
Abstract Haematological malignancies, including acute leukaemia and non-Hodgkin lymphoma, are one of the underlying diseases that frequently cause disseminated intravascular coagulation (DIC), an acquired thrombotic disorder. Concomitant DIC is associated with the severity of the underlying disease and poor prognosis. The Japanese Society on Thrombosis and Hemostasis released the new DIC diagnostic criteria in 2017. This criteria include coagulation markers such as soluble fibrin and the thrombin-antithrombin complex to more accurately evaluate the hypercoagulable state in patients. Among several groups of anticoagulants available, recombinant human soluble thrombomodulin is most frequently used to treat DIC caused by haematological malignancies in Japan. DIC is remitted in parallel with the improvement of the underlying haematological diseases; thus, there is room for debate regarding whether the treatment of DIC would improve the prognosis of patients. Haematopoietic stem cell transplantation as well as the recently introduced chimeric antigen receptor (CAR)-T-cell therapy are innovative therapies to produce a cure in a subset of patients with haematological malignancies. However, coagulopathy frequently occurs after these therapies, which limits the success of the treatment. For example, DIC is noted in approximately 50% of patients after CAT-T-cell therapy in conjunction with cytokine release syndrome. Hematopoietic stem cell transplantation (HSCT) causes endotheliitis, which triggers coagulopathy and the development of potentially lethal complications, such as sinusoidal obstruction syndrome/veno-occlusive disease and transplant-associated thrombotic microangiopathy. This review article describes the pathogenesis, clinical manifestation, diagnosis, and treatment of DIC caused by haematological malignancies, CAR-T-cell therapy, and HSCT. Keywords DIC · Haematological malignancies · APL · VOD/SOS · TA-TMA · Cytokine release syndrome
Introduction Disseminated intravascular coagulation (DIC) is an acquired thrombotic disorder that is caused by various underlying diseases, with sepsis being the leading cause, followed by shock, solid cancer, and haematological malignancies, including non-Hodgkin lymphoma and acute leukaemia [1, 2]. The disease that is most frequently accompanied by DIC is acute promyelocytic leukaemia (APL); nearly 70% of newly diagnosed APL patients develop DIC [3]. Approximately 17% and 11% of non-APL acute myeloid leukaemia * Takayuki Ikezoe [email protected] 1
Department of Haematology, Fukushima Medical University, Fukushima 960‑1295, Japan
(AML) and non-Hodgkin lymphoma patients, respectively, develop DIC at the time of diagnosis [4, 5]. The pr
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