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Hematology AND DIC [keywords]
- 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.
- Disseminated Intravascular Coagulation: Testing and Diagnosis. [REVIEW]
- Clin Chim Acta 2014 Apr 29.
Abnormalities of the hemostatic system in patients with DIC result from the sum of vectors for hypercoagulation and hyperfibrinolysis. DIC is classified into hyperfibrinolysis, hypercoagulation, massive bleeding or nonsymptomatic types according to the balance of the two vectors. Both the antithrombin (AT) and protein C (PC) levels are significantly low in patients with septic DIC, and reduced amounts of AT and PC result in the lack of inhibition of thrombin and activated FVIII, respectively. Thrombin activates FVIII, while activated FVIII accelerates the coagulation pathway to generate thrombin; thus activation of the coagulation system persists. Three sets of diagnostic criteria have been established by the Japanese Ministry of Health, Labour and Welfare, International Society of Thrombosis and Haemostasis and Japanese Association for Acute Medicine, respectively. Although these three diagnostic criteria score hemostatic abnormalities using similar global coagulation tests, the sensitivity and/or specificity for death differ. Treatment with AT or activated PC may not improve the outcomes of patients with sepsis at the early stage, although they may improve the outcomes in those with DIC. Therefore, new diagnostic criteria for determining the appropriate time to initiate anticoagulant treatment required.
- [Analysis of empirical treatment for newly diagnosed acute promyelocytic leukemia combined with disseminated intravascular coagulation]. [English Abstract, Journal Article]
- Zhongguo Shi Yan Xue Ye Xue Za Zhi 2014 Mar; 22(2):315-22.
This study was aimed to explore the clinical chracteristics and optimal therapentic methods for newly diagnosed acute promyelocytic leukemia (APL) combined with disseminated intravascular coagulation (DIC) so as to guide the clinical therapy. The clinical date and therapeatic ontcome of 25 cases of APL combined with DIC treated from January 2008 to March 2013 in our departement were analysed retrospectively. The 25 patients were given ATRA 20 mg orally twice a day and arsenic trioxide (ATO) 10 mg intravenously once a day to induce differentiation therapy, the chemotheray was added after degranulation of promyelocytes. At the same time the platelets, fresh frozen plasma, fibrinogen, eroprecipitate,prothrombin complex and amino methylbenzoic acid, low molecular weight heparin were given to treat DIC. According to the laboratorial examination of coagulation and fibrinolysis, the medication was adjusted.The white blood cell count, platelet level, prothrombin time (PT), partial thromboplastin time of plasma (APTT), fibrinogen level were detected, and the relation of those factors and age with bleeding severity was analyzed by multivariate manner. The results showed that among 25 patients with APL (low-risk 5 cases, intermediate risk 13 cases and high risk 7 cases), 22 cases combimed with DIC, incidence of DIC was 88%. Out of 22 patients with DIC 21 patients (95.5%) were corrected, except 1 case death. After the first cource of treatment, 23 cases (92%) gained complate remission (CR) with average CR time 31.8 ± 7.2 days. During the induction of CR, the average platelet transfusion level was 75.68 ± 55.88 U, the RBC level was 8.90 ± 5.69 U, the average level of fresh frozen plasma transfusion of APL patients with DIC was 21.92 ± 19.32 U. The recovery time of platelet level to normal was 29.3 ± 9.3 days, the recovery time of PT, APTT, FDP and fivrinogen to normal were 12.7 ± 9.5 days, 11.6 ± 8.6 days, 16.0 ± 9.3 days and 125.3 ± 85.3 days respectively. The multivariate analysis showed that WBC count at onset was >10 × 10(9)/L and APTT was prolonged. These two factors were main reasons resulting in severe bleeding. It is concluded that the newly diagnosed APL always combined with DIC, therefore in the early phase of disease active transfusion of blood products, application of anti-coagulation and anti-fibrinolytic drugs as well as heparin should be performed; the coagulation function should be as soon as recovered to normal so as to early correct DIC. These measures can significantly decrease the mortality of APL patients resulting from DIC. The hyperleukocytosis and prolonged APTT are the main factors for severe bleeding.
- Quantitative optical microscopy: measurement of cellular biophysical features with a standard optical microscope. [Journal Article]
- J Vis Exp 2014; (86)
We describe the use of a standard optical microscope to perform quantitative measurements of mass, volume, and density on cellular specimens through a combination of bright field and differential interference contrast imagery. Two primary approaches are presented: noninterferometric quantitative phase microscopy (NIQPM), to perform measurements of total cell mass and subcellular density distribution, and Hilbert transform differential interference contrast microscopy (HTDIC) to determine volume. NIQPM is based on a simplified model of wave propagation, termed the paraxial approximation, with three underlying assumptions: low numerical aperture (NA) illumination, weak scattering, and weak absorption of light by the specimen. Fortunately, unstained cellular specimens satisfy these assumptions and low NA illumination is easily achieved on commercial microscopes. HTDIC is used to obtain volumetric information from through-focus DIC imagery under high NA illumination conditions. High NA illumination enables enhanced sectioning of the specimen along the optical axis. Hilbert transform processing on the DIC image stacks greatly enhances edge detection algorithms for localization of the specimen borders in three dimensions by separating the gray values of the specimen intensity from those of the background. The primary advantages of NIQPM and HTDIC lay in their technological accessibility using "off-the-shelf" microscopes. There are two basic limitations of these methods: slow z-stack acquisition time on commercial scopes currently abrogates the investigation of phenomena faster than 1 frame/minute, and secondly, diffraction effects restrict the utility of NIQPM and HTDIC to objects from 0.2 up to 10 (NIQPM) and 20 (HTDIC) μm in diameter, respectively. Hence, the specimen and its associated time dynamics of interest must meet certain size and temporal constraints to enable the use of these methods. Excitingly, most fixed cellular specimens are readily investigated with these methods.
- A case of thrombotic microangiopathy/microangiopathies. [JOURNAL ARTICLE]
- Blood Coagul Fibrinolysis 2014 Mar 7.
We report a case of Streptococcus pneumonia sepsis-associated disseminated intravascular coagulation (DIC) with features of acute renal failure and microvascular thrombosis characterized by skin purpura and bilateral foot necrosis. The persistence of laboratory features of microangiopathic hemolytic anemia despite aggressive correction of DIC-associated coagulopathy suggests the possibility of an additional concomitant microangiopathic process. Here, we discuss the management and diagnostic approach, particularly highlighting the difficulties in making a definitive diagnosis. Although unconfirmed, our differentials include the concomitant process of sepsis-induced DIC occurring together with an indeterminate form of plasmapheresis and plasma exchange-responsive thrombotic microangiopathy, processes which are previously believed to be mutually exclusive.
- Elevated fibrin-related markers in patients with malignant diseases frequently associated with disseminated intravascular coagulation and venous thromboembolism. [Journal Article, Research Support, Non-U.S. Gov't]
- Intern Med 2014; 53(5):413-9.
Many patients with malignant diseases are frequently complicated with some type of thrombosis, such as venous thromboembolism (VTE) or disseminated intravascular coagulation (DIC).This retrospective study was designed to examine the frequency of thrombosis in 478 patients with malignant diseases in comparison to that observed in 121 patients without malignant diseases and to evaluate the efficacy of fibrin-related markers (FRMs), such as soluble fibrin, fibrinogen and fibrin degradation products and D-dimer, in diagnosing thrombosis.The frequency of thrombosis, including 62 cases of VTE, 63 cases of DIC and nine cases of cerebrovascular thrombosis, was significantly higher in the patients with malignant diseases (28.0%) than in the patients without malignant diseases (12.5%). DIC was frequently detected in the patients with hepatic cell cancer and hematopoietic malignancy, while VTE was frequently observed in the patients with colon cancer, breast cancer and urinary tract cancer. The FRMs levels were significantly higher in the patients with thrombosis than in the patients without thrombosis. A receiver operating characteristic analysis showed these markers to be useful for diagnosing thrombosis.Patients with malignant diseases have a high risk of thrombosis, and elevated FRMs levels are useful for diagnosing thrombosis in patients with malignant diseases.
- Primary intrarenal neuroblastoma with hypertension and disseminated intravascular coagulation. [Journal Article]
- Case Rep Oncol Med 2013.:684939.
The primary intrarenal neuroblastoma (IRNB) is a rare condition. Intrarenal neuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma, but true intrarenal neuroblastoma originates either sequestered adrenal rests during the fetal life or intrarenal sympathetic ganglia. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms' tumor is an important challenge since both tumors have major differences in prognostic and therapeutic response. We present a 3-year-old boy of primary intrarenal neuroblastoma with extensive abdominal and mediastinal mass, persistent hypertension, and disseminated intravascular coagulation (DIC).
- Effective control of relapsing disseminated intravascular coagulation in a patient with decompensated liver cirrhosis by recombinant soluble thrombomodulin. [Journal Article]
- Intern Med 2014; 53(1):29-33.
A 70-year-old Japanese man was hospitalized for expanding purpura and chronic disseminated intravascular coagulation (DIC) caused by decompensated liver cirrhosis. As there are no effective treatments for chronic DIC caused by liver cirrhosis, we decided to administer recombinant human soluble thrombomodulin (rhsTM) after he provided informed consent. The DIC was rapidly improved; however, the purpura and coagulopathy recurred after two months, and repeated rhsTM treatments were required. The rhsTM treatment sufficiently controlled the coagulopathy for two years, without any complications, including bleeding. This is the first report demonstrating that rhsTM can be administered safely and repeatedly to a patient with decompensated liver cirrhosis, and that it appears to be associated with a favorable outcome.
- Elevated plasma levels of soluble platelet glycoprotein VI (GPVI) in patients with thrombotic microangiopathy. [Journal Article, Research Support, Non-U.S. Gov't]
- Thromb Res 2014 Mar; 133(3):440-4.
Thrombotic microangiopathy (TMA) is caused by various conditions, such as decreased a ADAMTS13 level, activated or injured vascular endothelial cells or activated platelets. This study examined the soluble platelet glycoprotein VI (sGPVI) levels in patients with TMA to evaluate the activation of platelets in thrombotic states.The plasma levels of sGPVI, ADAMTS13 activity, von Willebrand factor (VWF) and VWF propeptide (VWFpp) were measured in patients with TMA.The plasma levels of sGPVI were significantly higher in postoperative patients, patients with TMA and those with disseminated intravascular coagulation (DIC) than in those without thrombosis. The plasma levels of sGPVI were the highest in patients with TMA without markedly reduced ADAMTS13 and those were significantly reduced after plasma exchange.The measurement of sGPVI level is therefore considered to be important for the diagnosis and evaluation of TMA.