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 patient with rhabdomyosarcoma is described in whom the presenting clinical and laboratory features were those of disseminated intravascular coagulation. The patient's rapid downhill course was primarily expressed by haemorrhagic tendency. An alveolar rhabdomyosarcoma, affecting many organs, including vascular and cardiac lumina, was found at necropsy and was considered to be the cause of the consumption coagulopathy.
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PMID:Alveolar rhabdomyosarcoma presenting as subacute intravascular coagulation. 88 20

Thirty-two children with solid tumors (lymphangioma, fibrosarcoma, hepatocarcinoma, osteogenic sarcoma, rhabdomyosarcoma, lymphosarcoma, mesenchymoma, hepatoma, Ewing's sarcoma, reticulum cell sarcoma, neuroblastoma, Hodgkin's disease, and brain tumors) were studied for alterations in coagulation by means of platelet counts, platelet aggregation, thrombelastogram, procoagulant and antigenic factor VIII, fibrin split products, and antithrombin III level. Results indicated hypercoagulability as shown by abnormally short thrombelastograms and elevated factor VIII levels and platelet counts in approximately one-half of the group. With the exception of increased fibrin split products in a third of the patients, little laboratory or clinical evidence for disseminated intravascular coagulation was seen. Hypercoagulability, as noted in adult carcinoma patients, can also occur in childhood sarcoma patients.
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PMID:Hypercoagulability in childhood cancer. 120 73

Disseminated intravascular coagulation (DIC) has been described in association with many tumors. We describe a patient with alveolar rhabdomyosarcoma in whom DIC developed with the initiation of chemotherapy. The patient achieved complete remission of his tumor for 14 months. An unusual factor VIII antigen was identified on crossed immunoelectrophoresis that was present at initial diagnosis, disappeared with remission, and returned with relapse of the tumor.
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PMID:Alveolar rhabdomyosarcoma associated with disseminated intravascular coagulation and a unique factor VIII antigen. 680 36

The authors describe a case of undifferentiated rhabdomyosarcoma from unknown primary site, presenting as an acute hematologic malignancy with generalized lymphadenopathy, extensive bone marrow involvement and clinical and laboratory features of disseminated intravascular coagulation. Such a peculiar behaviour is known for rhabdomyosarcoma but is rare and can be a serious diagnostic problem for the clinician and the pathologist. The importance of a large spectrum immunohistochemistry as first diagnostic approach to any undifferentiated small-cell malignant tumor is stressed, together with the knowledge of the different immunoreactivity patterns. Desmin, MS-actin and myoglobin are the most reliable markers of this type of myogenic sarcoma.
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PMID:Rhabdomyosarcoma presenting as acute hematologic malignancy: case report and review of the literature. 889 Sep 82

Pancytopenia and fulminant disseminated intravascular coagulation in a 68-year-old woman suggested an acute hematologic malignancy. However, cytogenetic analysis on a bone marrow sample revealed a near-tetraploid karyotype with an isochromosome 1q and a translocation (2;13) (q35;q14), which was suggestive of an alveolar rhabdomyosarcoma (ARMS). This diagnosis was subsequently confirmed by indirect immunohistochemistry. ARMS has not yet been observed in a patient of this age. Thus, our case underlines the importance of cytogenetics, to establish an a priori unexpected tumor diagnosis.
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PMID:Alveolar rhabdomyosarcoma in a 68-year-old patient identified by cytogenetic analysis of bone marrow. 980 33

Unusual, intense splenic radioactivity was seen on bone scintigraphy with Tc-99m HMDP in a 14-year-old boy with alveolar rhabdomyosarcoma complicated by disseminated intravascular coagulation. Abnormal splenic radioactivity was resolved after recovery from the disseminated intravascular coagulation. During treatment of disseminated intravascular coagulation and tumors, the patient received repeated blood transfusions, resulting in iron overload, but this did not prevent the abnormal splenic uptake from resolving. This case indicates that disseminated intravascular coagulation may be a cause of splenic accumulation of bone-seeking agents, and that abnormal splenic uptake can be resolved.
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PMID:Resolved splenic accumulation of Tc-99m HMDP after recovery of disseminated intravascular coagulation in a patient with rhabdomyosarcoma. 1036 36

A 68-year-old woman presented with profuse hemorrhage and other signs suggesting an acute leukemia. Histologic and cytogenetic evaluation of her bone marrow revealed alveolar rhabdomyosarcoma as the underlying cause of massive disseminated intravascular coagulation and hyperfibrinolysis. A review of the literature reveals that coagulopathy appears to be a common feature of alveolar rhabdomyosarcoma.
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PMID:Massive disseminated intravascular coagulation and hyperfibrinolysis in alveolar rhabdomyosarcoma: case report and review of the literature. 1046 47

A 50-year-old unmarried rural man was referred for a giant painless scrotal lump which had developed over a period of about six years. At the admission physical examination disclosed a large swelling scrotum and palpable left supraclavicular lymph nodes. Blood counts, renal and hepatic function tests, lactate dehydrogenase, beta-HCG, and alpha-fetoprotein were normal. Computed tomographic scan of thorax and abdomen revealed supraclavicular, mediastinal, and retroperitoneal adenopathies. The patient underwent right-sided radical orchiectomy. The final pathological diagnosis was paratesticular spindle cell rhabdomyosarcoma. Unfortunately, few days after surgery, patient presented a clinical and laboratory picture of disseminated intravascular coagulation followed by exitus.
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PMID:Unique case of giant adult paratesticular spindle cell rhabdomyosarcoma. 1903 16

The paper presents diagnostic and therapeutic difficulties in two adolescents with widespread rhabdomyosarcoma (RMS) presenting with severe haemorrhages resulting from disseminated intravascular coagulation (DIC) and with laboratory features of acute tumour lysis syndrome (ATLS). Other published cases of childhood RMS with DIC at admission have been listed and reviewed. It has been concluded that the clinical picture of a widespread RMS in children may resemble acute hematologic malignancy and pose a big diagnostic problem. That is why the presence of small blue round cells morphologically similar to lymphoblasts and/or myeloblasts in bone marrow (BM), lacking hematopoietic makers, should prompt the pathologist to consider possible diagnosis of RMS. Inclusion of desmin, MyoD1 and myogenin Myf4 to the immunohistochemical panel is obligatory in such cases. When the representative histopathological tumour specimens are difficult to obtain, the flow cytometric immunophenotyping of BM metastases could help the standard morphological/immunohistological diagnostic procedures and advance the diagnosis. Recently, the flow cytometric CD45- CD56+ immunophenotype together with Myf4 transcript has been assigned to RMS cells infiltrating BM. In children with disseminated RMS complicated with DIC rapid polychemotherapy aimed at diminishing the malignancy-triggered procoagulant activity should be initiated. However, in cases with concomitant ATLS the initial doses of chemotherapy should be reduced and the metabolic disorders and renal function monitored. The prognosis in children with RMS metastatic to BM with signs of DIC or ATLS at admission depends on the response to chemotherapy, however generally it is highly disappointing.
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PMID:Childhood rhabdomyosarcoma metastatic to bone marrow presenting with disseminated intravascular coagulation and acute tumour lysis syndrome: review of the literature apropos of two cases. 2051 38

A 16-day-old female was referred with congenital swelling on her right shoulder. On examination, there was a hard, round, ecchymotic, nontender, slightly movable, warm and shiny 10x15 cm mass on the right axillary pits which was extended to the right side of neck and chest wall. The mass separated the shoulder from the chest wall causing paralysis of right hand. Chest X-ray, ultrasound and MRI with contrast demonstrated a soft tissue mass suspected to be a hemangioma. The mass rapidly increased in size despite aggressive steroid therapy with rupture and bleeding. On the 45th post natal day the baby was taken to operating room to control the bleeding and if possible total excision of the mass. The mass was separated easily from the surrounding tissue and was excised along with right upper extremity. At the end of surgery the baby had cardiac arrest, and apparently died of Disseminated Intravascular Coagulation (DIC). The final pathology report was Rhabdomyosarcoma (RMS).
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PMID:Congenital rhabdomyosarcoma of shoulder. 2562 36


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