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Hematology AND DIC [keywords]
- Thrombotic events in acute promyelocytic leukemia. [JOURNAL ARTICLE]
- Thromb Res 2014 Dec 4.
Thrombotic events (TE) appear to be more common in acute promyelocytic leukemia (APL) than in other acute leukemias, with reported prevalence ranging from 2 to10-15%.We retrospectively analyzed the data on TE appearance in 63 APL patients.TE occured in 13 (20.6%) cases, four arterial (6.3%) and nine venous (14.3%). TE were more frequently diagnosed after initiation of weekly D-dimer monitoring (7 TE during 20months vs 6 during 76months, P=0.032). Patients with and without venous thrombosis were significantly different regarding female/male ratio (P=0.046), PT (P=0.022), aPTT (P=0.044), ISTH DIC score (P=0.001), bcr3 (P=0.02) and FLT3-ITD (P=0.028) mutation. The most significant risk factor for venous TE occurrence in multivariate analysis was FLT3-ITD mutation (P=0.034). PAI-1 4G/4G polymorphism was five times more frequent in patients with venous TE than without it (P=0.05). Regarding risk factors for arterial TE we failed to identify any.We have demonstrated that APL-related TE rate is higher than previously reported and that weekly D-dimer monitoring might help to identify patients with silent thrombosis. Moreover, our study suggests a possible relationship between venous TE occurrence and several laboratory findings (PT, aPTT, ISTH DIC score, bcr3 isoform, FLT3-ITD mutation and PAI 4G/4G). Prophylactic use of heparin might be considered in patients with ISTH DIC score<5, bcr3 isoform, FLT3-ITD mutation and PAI 4G/4G.
- Diagnosis and treatment of disseminated intravascular coagulation (DIC) according to four DIC guidelines. [Journal Article, Review]
- J Intensive Care 2014; 2(1):15.
Disseminated intravascular coagulation (DIC) is categorized into bleeding, organ failure, massive bleeding, and non-symptomatic types according to the sum of vectors for hypercoagulation and hyperfibrinolysis. The British Committee for Standards in Haematology, Japanese Society of Thrombosis and Hemostasis, and the Italian Society for Thrombosis and Haemostasis published separate guidelines for DIC; however, there are several differences between these three sets of guidelines. Therefore, the International Society of Thrombosis and Haemostasis (ISTH) recently harmonized these differences and published the guidance of diagnosis and treatment for DIC. There are three different diagnostic criteria according to the Japanese Ministry Health, Labour and Welfare, ISTH, and Japanese Association of Acute Medicine. The first and second criteria can be used to diagnose the bleeding or massive bleeding types of DIC, while the third criteria cover organ failure and the massive bleeding type of DIC. Treatment of underlying conditions is recommended in three types of DIC, with the exception of massive bleeding. Blood transfusions are recommended in patients with the bleeding and massive bleeding types of DIC. Meanwhile, treatment with heparin is recommended in those with the non-symptomatic type of DIC. The administration of synthetic protease inhibitors and antifibrinolytic therapy is recommended in patients with the bleeding and massive bleeding types of DIC. Furthermore, the administration of natural protease inhibitors is recommended in patients with the organ failure type of DIC, while antifibrinolytic treatment is not. The diagnosis and treatment of DIC should be carried out in accordance with the type of DIC.
- Primary Evan Syndrome With Disseminated Intravascular Coagulation Suggests Progressive Immune Dysregulation and Early Immunosuppressive Intervention is Key to Improving Outcomes. [JOURNAL ARTICLE]
- Am J Ther 2014 Nov 20.
Evan syndrome (ES) is a rare hematological disorder that involves 2 or more immune cytopenias. It usually includes autoimmune hemolytic anemia and autoimmune thrombocytopenia. Although occasionally associated with immune neutropenia, its association with disseminated intravascular coagulation (DIC) is rare. And, early diagnosis with appropriate intervention is important because mortality from ES is known to be greater than that of isolated immune hemolytic anemia and probably worse in the presence of DIC. Considering that the presence of DIC can make the diagnosis of ES challenging, a strong clinical suspicion is important as early initiation of therapy is critical to reducing the morbidity and mortality associated with this syndrome. We report a case of ES complicated by DIC.
- Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome. [Journal Article]
- Int J Clin Exp Pathol 2014; 7(10):7110-3.
Epstein-Barr virus (EBV) associated lymphoproliferative disease (LPD) are commonly derived from B-cells, however, it is becoming more and more apparently that EBV can also infect T-lymphocytes. Systemic EBV positive T-cell LPD of childhood is rare and characterized by an extremely aggressive course and poor prognosis. Here, we report a 22-year-old female of systemic EBV positive TLPD with acute EBV infection and review the clinical features of this disorder. A 22-year-old previously healthy female without immunocompromised status presented with persisting coach and fever resistant to conventional therapies. Physical examination showed hemorrhage and hepatosplenomegaly. Laboratory examinations revealed severe pancytopenia, disseminated intra-vascular coagulopathy (DIC), and anti-EBV-IgM positivity. Peripheral blood smears and bone marrow investigation identified a number of atypical lymphocytes. Flow cytometry (FCM) did not show any significant evidence of leukemia or lymphoma. The lymph node biopsy showed apparent infiltration of lymphocytes, which expressed CD2+, CD3+, CD7+ and TIA1+. There was no CD20+ or CD56+ cells. EBV early RNA (EBER) was positive. Cytogenetic analysis showed a normal karyotype. T-cell receptor (TCR) gene rearrangement revealed a polyclonal pattern. The patient received prednisolone and IVIG therapy with a transient good condition, and then died of multiorgan failure one week after diagnosis.
- Recombinant human soluble thrombomodulin (thrombomodulin alfa) to treat disseminated intravascular coagulation in solid tumors: results of a one-arm prospective trial. [JOURNAL ARTICLE]
- Int J Clin Oncol 2014 Nov 12.
Disseminated intravascular coagulation (DIC) associated with solid tumors (DIC-ST) is often encountered in clinical practice. Patients with DIC-ST are usually in poor condition and have bleeding diathesis due to advanced or metastatic diseases. Although some affected patients are treated with heparin, this strategy has not been prospectively studied. Recombinant human soluble thrombomodulin (thrombomodulin alfa, TM-α) is a new anticoagulant developed in Japan. We conducted a prospective study that evaluated the efficacy and safety of TM-α in patients with DIC-ST.A prospective one-arm study with TM-α was conducted for DIC-ST. TM-α (380 U/kg) was given for 30 min intravenously once daily for 6-14 days. The primary efficacy endpoint was the DIC resolution rate. Change in DIC scores and improvement in bleeding symptoms and outcomes were also evaluated. Safety endpoints included the incidence of bleeding-related adverse events.A total of 101 patients were treated with TM-α. The three main underlying malignant diseases were lung, stomach, and breast cancer, which accounted for 60 % of all patients. The DIC resolution rate was 34.0 % at the end of TM-α treatment. Improvement in DIC scores was seen in 55.2 % of patients, while only 22.9 % of patients had worsening of DIC scores. The overall survival rate was 55.4 % on day 28. The incidence of hemorrhage related to TM-α was 12.9 % until day 28. Cases of severe hemorrhage related to TM-α did not occur.TM-α is effective and safe for DIC-ST. This agent is the treatment of choice for the management of DIC-ST.
- [Chronic disseminated intravascular coagulopathy associated with aortic dissecting aneurysms: two cases report and literature review]. [English Abstract, Journal Article]
- Zhonghua Xue Ye Xue Za Zhi 2014 Sep; 35(9):831-4.
To report two cases of chronic disseminated intravascular coagulation associated (DIC) with aortic dissecting aneurysm, and discuss the treatment strategy.The clinical data of two patients with chronic DIC associated with aortic dissecting aneurysm were analyzed and the related literature was reviewed.Case 1: female, 53 years old, she had gingival bleeding, skin ecchymosis and haematuria for 2 months, the laboratory test revealed PLT 48 × 10⁹/L, APTT 38.0 s and fibrinogen 0.53 g/L; Case 2: male 86 years old, he had skin petechia and ecchymosis,gingival bleeding for 2 weeks, the laboratory test revealed PLT 17×10⁹/L, APTT 37.5 s and fibrinogen 0.51 g/L. CT scan for both cases revealed aortic aneurysm. They were diagnosed as aortic aneurysm associated chronic DIC. Both of them received blood component transfusion. After the treatment, they showed improvement in bleeding symptoms and laboratory data. They gave up operation, and were discharged from the hospital at last.Blood replacement can alleviate bleeding tendency in those patients with chronic DIC associated with aortic dissecting aneurysm.
- Detection of dicentric chromosome (9;20) in paediatric B-cell acute lymphoblastic leukaemia: prognostic significance. [JOURNAL ARTICLE]
- Ann Hematol 2014 Sep 6.
The dicentric chromosome (9;20) (dic(9;20)) is described in 2 % of childhood B-acute lymphoblastic leukaemia. Fluorescence in situ hybridization (FISH) is the most reliable method to identify dic(9;20) when compared with conventional cytogenetics. To define the prognostic importance of dic(9;20), we evaluated treatment response and patient survival. This was a retrospective study in three French university centres. Patients' clinical and laboratory characteristics and treatment response are described. Nine children with dic(9;20) have been identified since 1995. All patients had at least one poor prognostic feature either among the clinical features, the initial laboratory results or in the initial treatment response: central nervous system involvement (2/9), high median leucocyte count (≥50 G/L) (8/9) and poor response to prednisone (2/9). All patients were in complete cytological remission after induction therapy but only three had a good molecular response with minimal residual disease (MRD) <10(-3). Five out of nine patients relapsed and two died, 4 and 12 months after diagnosis, respectively. The event-free survival rate in this population was 44 % (95 % confidence interval (CI) = 0.09-0.79) and overall survival 78 % (95 % CI = 0.51-1.05). In this population, dic(9;20) is associated with a relatively poor prognosis. Patients showing dic(9;20), whether this cytogenetic abnormality is associated with other poor prognostic factors or not, should be identified at the outset in order to be offered a more intensive treatment protocol.
- A Report of Disseminated Carcinomatosis of the Bone Marrow Originating from Transverse Colon Cancer Successfully Treated with Chemotherapy Using XELOX plus Bevacizumab. [Journal Article]
- Case Rep Oncol 2014 May; 7(2):426-34.
A 61-year-old male, who had been admitted to another hospital due to disseminated intravascular coagulation (DIC), was referred to our hospital. Total colonoscopy, abdominal dynamic CT and positron-emission tomography revealed bone metastasis and multiple lymphocytic metastases from transverse colon cancer in addition to disseminated carcinomatosis of the bone marrow (DCBM). We immediately performed chemotherapy with XELOX + bevacizumab and denosumab against DCBM from transverse colon cancer in order to avoid radical surgery. In addition, we initiated the administration of recombinant human soluble thrombomodulin for 1 week to treat DIC. The patient was able to tolerate and receive 4 cycles of chemotherapy without any severe side effects. After receiving the 4 cycles of treatment, he recovered from DIC, and the bone and multiple lymphocytic metastases disappeared.
- Primary angiosarcoma of the breast complicated by the syndrome of disseminated intravascular coagulation (DIC): Case report and literature review. [Journal Article]
- Rep Pract Oncol Radiother 2014 May; 19(3):221-5.
Primary angiosarcoma of the breast (PAB) accounts for 0.04% of all breast malignant tumors. It affects young women usually at third or fourth decades of life. PAB clinically manifests as a painless, movable mass with sharp limits. A bluish red discoloration of the overlying skin is often observed. Enlargement of axillary lymph nodes generally does not occur. Angiosarcoma of the breast has a very poor prognosis due to the tendency to metastasize haematogenously and high frequency of local recurrence. Mastectomy and chemotherapy are preferable treatment choices. This paper presents a case of primary angiosarcoma of the breast with a syndrome of disseminated intravascular coagulation (DIC).
- Supportive transfusion therapy in cancer patients with acquired defects of hemostasis. [Journal Article]
- Thromb Res 2014 May.:S56-62.
Bleeding occurs in approximately 10% of patients with cancer: supportive transfusion therapy with Platelets Concentrates (PC), Fresh Frozen Plasma (FFP) and plasma-derived or recombinant concentrates is often required for the cessation and prevention of the bleeding episodes. The most frequent causes of bleeding in cancer is thrombocytopenia followed by liver insufficiency with or without vitamin K deficiency, disseminated intravascular coagulation (DIC) and the inappropriate or excessive use of anticoagulants. Other acquired hemostatic defects such as acquired hemophilia (AHA) and acquired von Willebrand syndrome (AVWS) are rare but they can be life-threatening. Thrombocytopenia in cancer patients may be the consequence of marrow invasion, chemotherapy or platelet auto-antibodies; patients with severe hypoproliferative thrombocytopenia, must be treated with PC and carefully followed to assess refractoriness to PC. The management of the other acquired defects of hemostasis usually requires the use of FFP and specific plasma-derived or recombinant concentrates. PC, FFP and plasma-derived concentrates can induce complications and/or adverse events in cancer patients: these include mainly allergic (ALR) or anaphylactic reactions (ANR), Transfusion-Associated Graft-Versus-Host Disease (TA-GVHD), Trasfusion-transmitted bacteriemia (TTB), Transfusion-Related Acute Lung Injury (TRALI), Acute Hemolytic Transfusion Reactions (AHTR), Febrile Non Hemolytic Transfusion Reactions (FNHTR). Therefore, modifications such as leukocyte-reduction and irradiation of the blood components to be transfused in cancer patients are recommended to reduce the risk of these complications.