Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.
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PMID:Mixed connective tissue disease associated with skin defects of livedoid vasculitis. 1105 28

A 24-year-old woman was admitted to our hospital because of a high fever that had persisted for two weeks. She complained of a sore throat and arthralgia, and had evanescent rash, lymphadenopathy, liver dysfunction, and hyperferritinemia. Tests for RF and ANA were negative. Adult-onset Still's disease was diagnosed. On the fifth day of hospitalization, acute respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC) developed. Treatment consisted of mechanical ventilation and administration of steroid pulse-therapy and gabexate mesilate. Analysis of fluid obtained by bronchoalveolar lavage showed increases in the total cell count, predominantly of neutrophils and lymphocytes. Bilateral pulmonary infiltration seen on chest radiographs was alleviated, and the arterial blood gas data gradually improved. After cyclosporine was given, all the above symptoms associated with adult-onset Still's disease disappeared. Plasma levels of inflammatory cytokines decreased with the improvement of the patient's clinical condition.
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PMID:[A case of adult-onset Still's disease complicated with acute respiratory distress syndrome]. 1264 11

A 67-year-old man presented with a 3-day history of abdominal pain, fever, and significant weight loss over 2 months. Physical examination revealed left upper quadrant tenderness, hepatomegaly, splenomegaly, and bilateral pitting edema but peripheral lymphadenopathy was absent. Laboratory tests showed anemia, thrombocytopenia, elevated prothrombin time (PT), partial thromboplastin time (PTT), and increased lactate dehydrogenase (LDH). PTT was corrected completely in mixing study. Further workup for the cause of coagulopathy revealed decreased levels of all clotting factors except factor VIII and increase fibrinogen levels, which ruled out disseminated intravascular coagulation (DIC). Flow cytometry of peripheral blood was normal. Contrast-enhanced computed tomography (CECT) revealed splenomegaly with multiple splenic infarcts without any mediastinal or intraabdominal lymphadenopathy. Further investigations for infective endocarditis (blood cultures and transthoracic echocardiography) and autoimmune disorders (ANA, dsDNA, RA factors) were negative. The patient received treatment for sepsis empirically without any significant clinical improvement. The diagnosis remained unclear despite extensive workup and liver biopsy was conducted due to high suspicion of granulomatous diseases. However, the liver biopsy revealed high-grade diffuse large B-cell lymphoma (DLBCL). Unfortunately, patient died shortly after the diagnosis. Here we report a case of high-grade DLBCL with hepatosplenomegaly and splenic infarcts in the absence of any lymphadenopathy or focal lesions. This case highlights the fact that unusually lymphoma can present in the absence of lymphadenopathy or mass lesion mimicking autoimmune and granulomatous disorders. The diagnosis in these cases can only be made on histology, and hence the threshold for biopsy should be low in patients with unclear presentations and multiorgan involvement.
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PMID:Unusual Presentation of Diffuse Large B-Cell Lymphoma With Splenic Infarcts. 2820 80