Chronic Disseminated Intravascular Coagulation Research Articles

ADENOSQUAMOUS LUNG CARCINOMA COMPLICATED WITH MARANTIC ENDOCARDITIS AND CHRONIC DISSEMINATED INTRAVASCULAR COAGULATION

Background: Adenosquamous lung carcinoma is a relatively rare subtype of non-small-cell lung cancer that contains both adenocarcinoma (ADC) and squamous cell carcinoma (SCC) components. It is difficult to reach a proper diagnosis before surgery, with the histopathological examination of the resected specimen being the method of choice. A supplementary immunohistochemistry examination of the sample is needed. In order to initiate targeted treatment, molecular testing is mandatory. Case presentation: A 47-year-old female, smoker (15 packs-year), without pathological personal history, presented for fatigue and moderate bilateral leg edema for about 3 months. The blood tests showed severe microcytic, hypochromic anemia and chronic disseminated intravascular coagulation. Computed tomography (CT) scan revealed a tumor in the right basal pleura with secondary pleural effusion, multiple lymphadenopathies, disseminated in the mediastinum, abdominal and pelvic cavity. Two other tumors were noticed, one in the right breast (7 mm) and the other in the vesicouterine pouch (7/10 mm). Prior to lung biopsy, a transthoracic and then transesophageal echocardiography were performed, highlighting the presence of a band on the aortic valve, suggesting marantic endocarditis. Tumor markers were also elevated. In evolution, the patient became bradypsychic, with head CT showing a brain metastasis in the left high-parietal region. Immunohistochemistry examination of the biopsy sample suggested a adenosquamous lung carcinoma. Conclusion: We reported the diagnostic path of a rare subtype of lung cancer in a young female without known comorbidities, with an atypical presentation - multiple extrapulmonary non-metastatic manifestations: metabolic etiology - weight loss, fatigue; vascular and hematological etiology – marantic endocarditis (a very rare complication), severe microcytic, hypochromic anemia and chronic disseminated intravascular coagulation; neurological etiology – peripheral sensorimotor neuropathy of the right arm. A tissue biopsy was performed from the most accessible region – 1/3 inferior right thorax, posterior axillary line. The particularity of the adenosquamous lung carcinoma in this case lies in multiple metastases in less common sites (breast, vesicouterine pouch and probably kidney).

Chronic disseminated intravascular coagulation in a patient with Takayasu arteritis.

Takayasu arteritis (TA) is a large-vessel vasculitis that predominantly affects the aorta and although it usually causes stenosis, aneurysms have been reported in ∼10-25% of the patients. Here, we present a male TA patient with chronic disseminated intravascular coagulation (DIC) as a rare complication of endovascular grafted aortic aneurysm (AA). Chronic DIC usually presents as mild or even hidden clinical symptoms, and early diagnosis of this condition can only be possible with laboratory tests. Due to the silent clinical scenario, this complication should be kept in mind among AA patients with unexplained thrombocytopenia or coagulation abnormalities.

Large intraosseous chronic expanding hematoma after total hip arthroplasty presenting with chronic disseminated intravascular coagulation: a case report and literature review

BackgroundA chronic expanding hematoma (CEH) is a rare complication caused by surgery or trauma; it mostly affects the soft tissues, such as those in the trunk or extremities. We present the first case of a large intraosseous CEH presenting with chronic disseminated intravascular coagulation (DIC), 22 years after total hip arthroplasty (THA); the CEH was treated with a single-stage excision and revision THA.Case presentationA 67-year-old man presented to our hospital with left thigh pain and an enlarging mass. He had no history of trauma, anticoagulant use, or a collagen vascular disorder. The patient initially declined surgery. Two years later, radiographs and computed tomography images revealed progressive osteolysis, marginal sclerosis, and calcification in the left femur, in addition to loosening of the femoral component. Laboratory data revealed anemia and chronic DIC of unknown causes. Magnetic resonance imaging revealed a “mosaic sign” on the mass, indicating a mix of low- and high-signal intensities on T2-weighted images. Needle biopsy prior to surgery revealed no infection or malignant findings. An intraosseous CEH was suspected due to extensive osteolysis and loosening of the femoral component. No other factors that could induce chronic DIC were identified, such as sepsis, leukemia, cancer, trauma, liver disease, aneurysms, or hemangiomas. Therefore, we speculated that the anemia and chronic DIC were caused by the large intraosseous CEH. A single-stage revision THA with surgical excision was performed to preserve the hip function and improve the chronic DIC. The postoperative histopathological findings were consistent with an intraosseous CEH. The anemia and chronic DIC improved after 7 days. There was no recurrence of intraosseous CEH or chronic DIC at the 6-month follow-up. The left thigh pain improved, and the patient could ambulate with the assistance of a walking frame.ConclusionsThe loosening of the femoral component caused persistent movement, which may have caused intraosseous CEH growth, anemia, and chronic DIC. It is important to differentiate CEHs from malignant tumors with hematomas. Furthermore, the “mosaic sign” noted in this case has also been observed on magnetic resonance images in other cases of CEH.

Anti-PD-L1 monoclonal antibody for the management of chronic disseminated intravascular coagulation secondary to a urothelial carcinoma: a case report

BackgroundThrombocytopenia is often considered a risk factor for bleeding, but conversely may be associated with an increased thrombotic risk in several clinical situations. Here we present a patient with arterial thrombosis and chronic disseminated intravascular coagulation caused by metastatic urothelial carcinoma. As the treatment for a disseminated intravascular coagulation caused by a neoplasia is the treatment of the underlying disease itself, our case highlights a new therapeutic approach—immunotherapy—in a patient prone to hematological complications due to conventional chemotherapy.Clinical caseA 74-year-old Caucasian male patient with a history of urothelial carcinoma of the bladder and moderate thrombocytopenia had multiple arterial thrombotic events despite antiplatelet therapy and anticoagulation. A diagnosis of chronic disseminated intravascular coagulation in the setting of a metastatic bladder urothelial carcinoma was made. The patient was treated with an anti-PD-L1 monoclonal antibody, and achieved a rapid response with subsequent reversal of the disseminated intravascular coagulation.ConclusionUnexplained arterial or venous thrombosis despite adequate thromboprophylaxis should be investigated, especially in the setting of thrombocytopenia. Chronic disseminated intravascular coagulation is a possible, life-threatening reason for this clinical picture, and should prompt rapid identification of the underlying disease. To the best of our knowledge, this is the second case of chronic disseminated intravascular coagulation due to neoplastic disease treated with immunotherapy.

Pharmacological Thromboprophylaxis in Patients With Cancer


 Six evidence-based guidelines were identified regarding the long-term (6 months or longer) use of pharmacological thromboprophylaxis for the management of cancer-associated thrombosis. The guidelines used rigorous methodology, systematically searched for evidence, and were clearly reported.
 Anticoagulation therapy for 6 months or longer is recommended by 5 guidelines for patients with active cancer and venous thromboembolism to prevent recurrences of venous thromboembolism. However, the recommendations are weak and made based on low-quality evidence or expert consensus.
 Two guidelines recommend a low-molecular-weight heparin or direct oral anticoagulant for long-term use (6 months or longer) in patients with cancer. This recommendation is based on low- to high-certainty evidence.
 Two guidelines strongly recommend direct oral anticoagulants in patients with cancers in locations other than gastrointestinal or genitourinary cancers. This recommendation is based on high-quality evidence.
 No guidelines were identified regarding arterial thrombosis or chronic disseminated intravascular coagulation associated with cancer.

Inflammation/coagulopathy/fibrinolysis: Dynamic indicators of COVID-19 progression in patients with moderate COVID-19 in Wenzhou, China

BackgroundThe majority of the coronavirus disease 2019 (COVID-19) non-survivors meet the criteria for disseminated intravascular coagulation (DIC). Although timely monitoring of clotting hemorrhagic development during the natural course of COVID-19 is critical for understanding pathogenesis, diagnosis, and treatment of the disease, however, limited data are available on the dynamic processes of inflammation/coagulopathy/fibrinolysis (ICF). MethodsWe monitored the dynamic progression of ICF in patients with moderate COVID-19. Out of 694 COVID-19 inpatients from 10 hospitals in Wenzhou, China, we selected 293 adult patients without comorbidities. These patients were divided into different daily cohorts according to the COVID-19 onset-time. Furthermore, data of 223 COVID-19 patients with comorbidities and 22 critical cases were analyzed. Retrospective data were extracted from electronic medical records. ResultsThe virus-induced damages to pre-hospitalization patients triggered two ICF fluctuations during the 14-day course of the disease. C-reactive protein (CRP), fibrinogen, and D-dimer levels increased and peaked at day 5 (D) 5 and D9 during the 1st and 2nd fluctuations, respectively. The ICF activities were higher during the 2nd fluctuation. Although 12-day medication returned high CRP concentrations to normal and blocked fibrinogen increase, the D-dimer levels remained high on days 17 ± 2 and 23 ± 2 days of the COVID-19 course. Notably, although the oxygenation index, prothrombin time and activated partial thromboplastin time were within the normal range in critical COVID-19 patients at administration, 86% of these patients had a D-dimer level > 500 μg/L. ConclusionCOVID-19 is linked with chronic DIC, which could be responsible for the progression of the disease. Understanding and monitoring ICF progression during COVID-19 can help clinicians in identifying the stage of the disease quickly and accurately and administering suitable treatment.

Severe Bleeding Tendency due to Excessive Fibrinolysis in Two Patients With Aortic Disease: Role of Tranexamic Acid in Non-Surgical Candidate

Disseminated intravascular coagulation (DIC) is characterized by systemic activation of coagulation pathway which can lead to thrombosis and bleeding. Chronic DIC associated with aortic aneurysm or dissection commonly presents with bleeding symptoms with variable severity due to enhanced fibrinolysis. Patients with unexplained bleeding diathesis with DIC should include aortic disease as one of the differential diagnoses. This paper reports two patients with severe hemorrhagic complications due to aortic disease. Long-term prophylactic treatment is recommended if the underlying aortic disease cannot be surgically managed. Tranexamic acid is a potentially safe option to relieve bleeding tendency. J Med Cases. 2020;11(10):303-305 doi: https://doi.org/10.14740/jmc3549

1672 Appearances Are Glimpse of the Unseen

INTRODUCTION: Breast cancer metastasis is seen in nearly 30% of cases. It most commonly spreads to the liver, brain, bones and lungs1,2. Gastrointestinal tract (GI) especially large bowel involvement is rare3,4. Usually diagnosed because of obstructive symptoms or bleeding. Endoscopic evaluation usually demonstrates obvious ulcerations, masses or stenosis. Finding a normal looking colon infiltrated by metastatic breast cancer cells is unique with incidence unknown5. CASE DESCRIPTION/METHODS: We report a case of a 69-year-old African American female with Stage 4 Invasive lobular left breast carcinoma with metastasis to bone and chronic disseminated intravascular coagulation who was admitted for GI bleeding. She underwent upper and lower endoscopies which were unrevealing for the cause of bleeding. Her upper scope revealed erythema in the stomach and duodenal villous blunting both of which were biopsied. Colonic mucosa looked congested and ulcerations were found in the terminal ileum which were also biopsied. These findings are not per literature review typical of metastatic breast cancer. To our surprise biopsies revealed metastatic lobular breast cancer infiltration in all samples collected from stomach, duodenum, terminal ileum and colon. DISCUSSION: Lobular breast carcinoma more commonly metastasizes to the gastrointestinal tract6 as compared to infiltrating ductal breast cancer. In instances that it does metastasize to the colon it shows obvious endoscopic findings which was not the case in our patient. Hence we purpose that vigilant sampling of tissue be performed in patients inflicted with this disease even with subtle findings on endoscopy.

Oral tranexamic acid combined with low molecular weight heparin only during dialysis sessions successfully controlled chronic disseminated intravascular coagulation associated with aortic aneurysm and aortic dissection in a dialysis patient: a case report with literature review

BackgroundDisseminated intravascular coagulation (DIC) is a relatively rare but important cause of bleeding diathesis in patients on maintenance dialysis. When the control of underlying disorders causing DIC is not achieved and anticoagulant therapy could not ameliorate the symptoms, other therapeutic options might be considered. While the use of antifibrinolytic agents, such as tranexamic acid, is generally not recommended in patients with DIC, the combined use of these agents with anticoagulants has produced good results in some cases with enhanced fibrinolytic-type DIC. Although the dose of tranexamic acid should be adjusted for patients with renal impairment to avoid neurotoxic complications, there are no widely accepted recommendations for dosage adjustment in dialysis patients. Therefore, the optimal indication and dosage of tranexamic acid in dialysis patients with hyper fibrinolytic type DIC remain unestablished.Case presentationWe herein report a 94-year-old male patient on maintenance hemodialysis with hyperfibrinolytic DIC induced by chronic aortic aneurysm and aortic dissection. He suffered from hemorrhagic diathesis and was successfully treated with oral administration of 750 mg tranexamic acid per day combined with intravenous infusion of low molecular weight heparin (LMWH) every dialysis session. There were no apparent adverse events. Unintended dose reduction of tranexamic acid resulted in exacerbation of DIC along with alarming recurrence of blood flow in the previously thrombosed aortic false lumen, which was ameliorated soon after surely performing medication.ConclusionsCombined use of oral tranexamic acid and minimum anticoagulant only during dialysis sessions successfully controlled aneurysm-induced DIC in a dialysis patient. Although the exact dosage and indication require further investigation, the treatment may be worth considering, even in dialysis patients, when other treatment options have failed to obtain good results.

A Paraneoplastic Syndrome to Remember: A Case of Disseminated Intravascular Coagulation in Lung Cancer.

Disseminated intravascular coagulation (DIC) is an acquired syndrome characterized by the widespread activation of coagulation. It can present as an acute life-threatening emergency or as a chronic process. Mortality is highly dependent on the reversibility of the aetiology and degree of coagulation impairment, so treatment of the underlying cause is vital. The authors present the case of a 57-year-old man whose inaugural presentation of lung cancer was chronic DIC, characterized by three thrombotic events, followed by acute DIC, culminating in death. Metastatic lung cancer was diagnosed only after death.LEARNING POINTSDisseminated intravascular coagulation (DIC) is an acquired syndrome characterized by systemic intravascular activation of coagulation, originating from and causing damage to the microvasculature, which can present as an acute or chronic condition, manifested by bleeding or thrombotic events.Unexplained thrombotic events should raise suspicion of chronic DIC.Treatment will fail, despite supportive measures, if the cause of the DIC is not identified.

Disseminated Mycobacterium avium-Intracellulare Complex Infection Presenting With Disseminated Intravascular Coagulation in an AIDS Patient.

Disseminated Mycobacterium avium-intracellulare complex (MAC) infection is one of the relatively common opportunistic infections seen in severely immunocompromised AIDS patients. A constellation of clinical, laboratory, and pathological features involving multiple organ systems are often present in disseminated MAC infection but disseminated intravascular coagulation (DIC) has not been previously described in association with this condition. To our knowledge, this is the first reported case of DIC complicating disseminated MAC infection in an AIDS patient. In this article, we present the case of a 33-year-old AIDS patient with high viral load, CD4 lymphocyte count of 1/mm3, who presented with nonspecific symptoms, anemia, thrombocytopenia, and increased lactate dehydrogenase, alkaline phosphatase, and ferritin. She also had abnormal coagulation parameters and features compatible with chronic DIC. Bone marrow biopsy assisted in making the correct diagnosis. She also later grew MAC from blood and sputum cultures. There were no other factors identified after a complete workup to explain DIC in this patient. After commencement of appropriate MAC therapy, she initially had a good response with some improvement of her coagulation parameters. Few months later, however, probably attributable to poor medication compliance, her condition deteriorated with development of thromboembolism, full-fledged DIC, sepsis, and an eventual fatal outcome. This case illustrates the importance of including disseminated MAC in the differential diagnosis of DIC in an AIDS patient.

Aortic aneurysm and chronic disseminated intravascular coagulation: a retrospective study of 235 patients

Chronic disseminated intravascular coagulation (DIC) is a rare but devastating complication of aortic aneurysm (AA). This study investigated the clinical manifestations, laboratory findings, and treatment of patients with AA-associated chronic DIC (AA-DIC) and explored the mechanisms, duration, and therapeutic response of AA-DIC. We retrospectively reviewed the medical records of 235 AA patients admitted at the Peking Union Medical College Hospital between September 2009 and January 2015. The patients were classified as those with DIC (AA-DIC) and those without DIC (non-DIC). The AA-DIC group showed a significantly higher proportion of female patients and a significantly longer AA disease course than the non-DIC group did. The AA-DIC patients presented mural thrombi, dissecting aneurysms, a family history of AA, and diabetes significantly more frequently than the non-DIC patients did. Furthermore, multiple regression analyses revealed that sex, mural thrombus, aneurysm type, diabetes, and stent surgery are possible independent risk factors for AA-DIC patients. Fifty-two (22.1%) patients presented AA-DIC. Among these patients, 43 had non-typical DIC and 9 had typical DIC; the mortality rate of the latter was 22.2%. The mean age of the patients with typical DIC was significantly higher than of that of patients with non-typical DIC. The non-typical DIC patients also presented abnormal coagulation disorders of varying degrees. Furthermore, heparin or low-molecular-weight heparin improved the clinical symptoms and laboratory parameters in patients with AA and typical DIC. Thus, chronic DIC should be considered in patients with AA.

Thoracic aortic dissection mimicking primary hematological disorder: A case report

Chronic disseminated intravascular coagulation (DIC) is uncommon and less clinically pronounced. Its diagnosis and therapymay be further delayed due to the subtle abnormal laboratory results. This may potentially contribute to an unfavorablepatient outcome. We describe a 60-year-old lady with chronic DIC who presented with progressive and recurrent unprovokedsubcutaneous hematoma for 6 months prior to presentation. On presentation, she was anemic and thrombocytopenic with amildly deranged coagulation profile. She was suspected to have hematological malignancies therefore a bone marrow aspirate andtrephine biopsy was done but revealed no abnormality. Serial DIC panel revealed persistent low fibrinogen and elevated d-dimerwhich were consistent with chronic DIC. When she was referred to our centre, baseline chest radiograph showed mediastinalwidening and subsequent computed tomography (CT) imaging confirmed the presence of Standford A dissection of thoracicaortic aneurysm. The gradual occult blood loss from the dissection lead to the consumptive coagulopathy and triggered the DICcascade. Essentially, chronic DIC never occur alone and unprovoked, therefore the main principle of management is to recognizeand address the underlying etiology in particularly aneurysms and malignancies.

Kasabach-Merritt症候群により抜歯後出血を来した1例

Kasabach-Merritt syndrome is a coagulation disorder caused by hemangioma, usually resulting in chronic disseminated intravascular coagulation (DIC). We report a case of post-extraction hemorrhage with Kasabach-Merritt syndrome. A 54-year-old man was referred to our department because of persistent hemorrhage after extraction of the left lower first molar. Although we performed local hemostatic treatment, intermittent hemorrhage persisted. Blood examination revealed abnormal coagulation, and the patient had a medical history of giant hemangioma of the liver. We therefore suspected post-extraction hemorrhage caused by Kasabach-Merritt syndrome and consulted with a hematologist. The patient was given a diagnosis of Kasabach-Merritt syndrome and received camostat mesilate. We continued local hemostatic treatment, and hemostasis was successfully achieved. About 10% of patients with hemangioma of the liver have Kasabach-Merritt syndrome. In patients with hemangioma who have abnormal bleeding such as post-extraction hemorrhage, the possibility of chronic DIC should be included in the differential diagnosis.

Perioperative management of diabetic foot.

Perioperative management of diabetic foot.

Successful Hemodialysis Initiation in a Patient with Chronic Disseminated Intravascular Coagulation

We report a 72-year-old man with end-stage renal disease due to chronic glomerulonephritis and chronic disseminated intravascular coagulation (DIC) caused by aortic aneurysm. He had had graft replacement for abdominal aortic aneurysm in 2012 at another hospital. He indicated development of thoracic aortic aneurysm, which developed chronic DIC with much subcutaneous hemorrhage. His renal function gradually decreased and we planned hemodialysis initiation. We administered camostat mesilate and platelet concentrate transfusion to treat hemorrhagic tendency before the arteriovenus fistula in May 2014. After the operation, we added tranexamic acid because of difficulty in hemostasis. About 1 month later, the patient reported with hemorrhage from the nose and his renal function was exacerbated. He was initiated into hemodialysis with transfusion of red blood cells and fresh frozen plasma. We raised the dose of camostat mesilate and adjusted the dose of anticoagulant with reference to activated clotting time. He was successfully initiated into dialysis without major trouble.

Effective Control of Relapsing Disseminated Intravascular Coagulation in a Patient with Decompensated Liver Cirrhosis by Recombinant Soluble Thrombomodulin

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.

Malignancies and Disseminated Intravascular Coagulation

The article entitled “Predictive Value of Baseline Plasma D-dimers for Chemotherapy- induced Thrombocytopenia in Patients with Stage III Colon Cancer: A Pilot Study” written by Tanriverdi (2013) and published in one of the recent issues of your journal was quite interesting. Here, we would like to emphasize some points. Disseminated intravascular coagulation (DIC) is a well known hemostatic complication of solid tumors. In a retrospective study of 1,117 patients with solid tumors, a clinical and laboratory diagnosis of DIC could be made in 76 (6.8%) (Sallah et al., 2001) Classiclaboratoryfindingsassociated with chronicDIC and solid tumors include thrombocytopenia and circulating fibrindegradation products. Hypofibrinogenemiaalso occurs. Fragmented red blood cells, although reported in patients with DIC, rarely constitute greater than 10% of the red cells. However, in some cases of chronic DIC with elevated D-Dimers but normal coagulation screening assay results, the presence of fragmented red cells may provide supportive evidence. Several scoring system combining clinical and laboratory date have been devised to assist in the diagnosis and determination of prognosis of patients with DIC (overt or non-overt). The International Society of Thrombosis and Haemostasis (ISTH) scoring system uses a combination of risk assessment and simple, widely available coagulation assays [including platelet count, fibrinrelated marker, prolongation of prothrombin time (PT), fibrinogen level] (Taylor et al., 2001). In conclusion, to me, low platelet count and high D-dimer value in the first group compared with the other group may be associated with non- overt DIC. The diagnosis can be supported with fibrinogen,fragmented red blood cells and PT levels.

Intermittent administration of recombinant human soluble thrombomodulin successfully controlled chronic disseminated intravascular coagulation in a patient with dissecting aortic aneurysm on an outpatient basis

Chronic disseminated intravascular coagulation (DIC) is a rare but life-threatening complication of dissecting aortic aneurysm. Although anticoagulant therapy may often proves effective for controlling DIC itself, patients would have to be hospitalized for a long period due to continuous infusion therapy. Subcutaneous injection of a highly concentrated preparation of heparin calcium may offer one alternative treatment for DIC; however, daily subcutaneous use of heparin for the treatment of DIC has impaired quality of life (QOL). The other alternative therapy is intravenous administration of recombinant human soluble thrombomodulin (rTM), which includes the active extracellular domain of thrombomodulin. Reportedly, rTM effectively resolves DIC by only 6 consecutive days of administration; however, how frequently rTM should be administered after the resolution of chronic DIC to have good control of it has been unclear. We report herein a case of chronic DIC complicated with dissecting aortic aneurysm, whose resolution of chronic DIC achieved by 6 consecutive days of rTM has been maintained by once a week administration of rTM on an outpatient basis.

Disseminated Intravascular Coagulation In a Patient with Angiosarcoma of the Face Treated with Fondaparinux

AbstractAbstract 4426 Introduction:We present a case with disseminated intravascular coagulation (DIC) successfully treated with low dose Fondaparinux. Case Presentation:A 50 year old male with past medical history of diabetes, hypertension was diagnosed with angiosarcoma localized to the face (Figure 1 & Figure 2). The patient had baseline mild coagulopathy before start of chemotherapy most likely from the angiosarcoma. He received the first cycle of paclitaxel and bevacizumab but required hospitalization four days later for chest pain and acute coronary syndrome was ruled out. He then developed febrile neutropenia with an ANC of 0.0 and progressive pancytopenia. On day 9 of chemotherapy, the patient had a platelet count of 12 K/μ L with hemoglobin of 6.1gm/dl, hematocrit of 18% and WBC count of 3 k/μ l. The peripheral smear revealed many schistocytes, in addition elevated LDH 1112U/L, with an indirect bilirubin of 2.62 mg/dl, total bilirubin of 3.80mg/dl, and a haptoglobin of less than 10. His coagulopathy progressed with a PT of 17.3, PTT 65.3, fibrinogen degradation products (FDP) which were less than 10 on day 6 post chemo, were more than 40 on day 8 and his D-Dimers were 13.5 mg/l(Figure 5). ADAMTS13 level was drawn and though his creatinine and mental status remained normal, plasmapharesis was initiated with the presumptive diagnosis of thrombotic thrombocytopenic purpura (TTP).The patient developed a mild to moderate pericardial effusion without evidence of tamponade and was monitored in the critical care unit. Echocardiogram also revealed an elevated pulmonary artery pressure of 60 mmHg. He was treated with broad spectrum antibiotics, daily plasmapharesis and blood products as needed. Initial platelet count responded to plasmapharesis going up to 50 by day 22. His ADAMTS 13 level on day 9 and 12 were 392ng/ml (ref > 590ng/ml) and 297ng/ml respectively.After 38 days of plasmapharesis platelet count stayed in the range of 10–20 and pericardial effusion was stable. We decided to consider the alternative diagnosis of acute on chronic DIC. Patient was started on Fondaparinux at a dose of 2.5mg initially for 3 days and then increased to 5mg SQ. On day 7 of Fondaparinux the platelet count was 137 from 20, PTT 35.5 (baseline 40), D-Dimers 0.74 and FDP were less than 10. (Figure 3,Figure 4 and Figure 5) Discussion:Microangiopathic hemolytic anemia (MAHA) can be is seen in tumors of vascular origin and could be caused due to TTP or DIC. These tumors can present as Kasabach Merritt phenomenon which is characterized by severe thrombocytopenia and or coagulopathy due to platelet trapping in the vascular tumor. This phenomenon usually responds to chemotherapy (1). In our case initially anticoagulation I was a difficult choice because of acute pericardial effusion. The rationale for using Fondaparinux were two; one a theoretical benefit of anticoagulation in DIC(2), second patient also was developing pulmonary hypertension observed on serial ECHO's done for pericardial effusion indicating an ongoing chronic thrombo-embolic process. We decided to avoid heparin because it can itself cause thrombocytopenia and may confound with our clinical condition. Patient responded well and has been stable on low dose Fondaparinux.1.Kasabach-Merritt syndrome associated with angiosarcoma of the scalp sucessfully treated with chemoradiotherapy. mafuku S, Hosokawa C, Moroi Y, Furue M. cta Derm enereol. 2008;88(2):193-4.2.Angiosarcoma variant of Kasabach-Merritt syndrome. Alliot C, Tribout B, Barrios M, Gontier MF. Eur J gastroentrol Hepatol. 2001 Jun;13(6):731-4 [Display omitted] [Display omitted] [Display omitted] Disclosures:Off Label Use: Fondaprinux is a direct thrombin inhibitor used for anticoagulation for venous thromboembolism. It is only technical off label because it is not mentioned in the label of the drug.