Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Littoral cell angioma is a rare tumor of the spleen, usually being considered benign and typically discovered incidentally. There are three different modalities of presentation: tumoral splenomegaly, long-standing iron deficient anemia or thrombocytopenia due to hypersplenism. However, some of its manifestations could generate the suspicion of a lymphoma or other more serious condition. We present the case of a 46-year-old man with splenomegaly and iron deficiency anemia. The tumor affected the whole spleen, which was surgically removed. The histopathological examination, together with immunophenotyping, established the diagnosis. Six months after the procedure, the patient is in very good condition. Several differential diagnoses were discussed, as well as the prognostic factors. The case illustrates a rare cause of anemia and the importance of pathology in uncovering such unusual causes for this.
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PMID:Littoral cell angioma of the spleen--a surprising cause of anemia. 2432 45

Littoral cell angioma (LCA), a primary vascular neoplasm originating from splenic red pulp littoral cells, was initially thought to be an extremely rare pathology. There have been an increasing number of cases reported in the literature. However, the etiology and prevalence of LCA is still unclear, partly due to the rarity of cases. The association of LCA with internal organ cancers, specifically lymphoma, has also been reported. In the patients with a history of cancer/lymphoma, the accurate diagnosis of LCA as the cause of the splenomegaly is challenging. Here we present a case of LCA in a patient with non-Hodgkin B-cell lymphoma and alpha-thalassemia trait. To our knowledge, this is the first report of the coexistence of LCA and thalassemia and only the second report of LCA and marginal-zone non-Hodgkin B-cell lymphoma. We review the literature and discuss the radiologic and pathologic findings of this case compared with the previously reported cases.
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PMID:Littoral cell angioma: review of the literature and case report. 2507 59

Littoral cell angioma (LCA) is a rare endothelial cell neoplasm in the spleen. Although many cases of LCA are asymptomatic, some present with signs and symptoms related to splenomegaly, whereas others manifest with haematological abnormalities, including anaemia and/or thrombocytopaenia (ie, hypersplenism). We report a case of LCA presenting with chronic thrombocytopaenia, probably due to splenic sequestration of platelets or phagocytosis of platelets by neoplastic cells. Following therapeutic splenectomy, the patient suffered from a marked rebound thrombocytosis and subsequently developed pulmonary embolisms. He was treated with anticoagulant therapy combined with antiplatelet therapy, and his symptoms were quickly resolved. This case emphasises an exclusion of primary splenic disorders in patients with chronic thrombocytopaenia, especially in those with splenomegaly and the contemplation of thromboembolism prophylaxis postsplenectomy.
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PMID:Persistent thrombocytopaenia in a young man with splenomegaly, rebound thrombocytosis after splenectomy and subsequent pulmonary embolism: splenic littoral cell angioma and associated events. 2660 37

Littoral cell angioma (LCA) is a unique lesion of the spleen that arises from the cells lining the venous sinuses of the splenic red pulp and shows the features of combined endothelial and histiocytic differentiation. Several cases of LCA have been reported in the literature; however, the cytological findings have been described for only a few cases. We report the case of an 11-year-old boy with anemia, epigastric abdominal pain, and splenomegaly. The splenic lesions showed anastomosing vascular channels with cyst-like spaces filled by many sloughed endothelial cells, which were positive for CD68 and CD31 and negative for CD34. Scraping cytology revealed isolated and clusters of three-dimensional bland looking, epithelioid foamy tumoral cells with low nuclear cytoplasmic ratio, which mostly contained intracytoplasmic hemosiderin pigment. Although the fine needle aspiration cytology of splenic lesions is uncommon and LCA is a rare splenic lesion, it must be noted in the differential diagnosis of any splenic vascular neoplasm.
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PMID:Littoral cell angioma of the spleen: Cytological findings and review of the literature. 2846 25

Introduction: Being a rare benign vascular tumor of the spleen, Littoral cell angioma involves multiple focal nodules in the spleen and splenectomy is used for both definitive diagnosis and treatment.Patients and methods: A 30-year-old male patient presented to our clinic with the complaints of intermittent abdominal pain and fever. In physical examination, splenomegaly was found and he was further evaluated with ultrasonography, tomography and magnetic resonance imaging that have revealed multiple solid mass lesions of various sizes in his spleen. The patient was administered open splenectomy and recovered uneventful.Results: After histopathological and immunohistochemical analyses of the splenectomy material, he was diagnosed with Littoral cell angioma.Conclusions: For a timely diagnosis, Littoral cell angioma should be considered among the initial diagnoses in patients who were found to have splenomegaly in their physical examination and multiple masses in the spleen in radiological tests.
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PMID:A rare vascular tumor of the spleen: Littoral cell angioma. 3198 95


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