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)

Malignant histiocytosis is an uncommon, progressive disease with a poor prognosis which is characterized by malaise, fever, lymphadenopathy, and splenomegaly. Lymphadenopathy is commonly present at presentation as well as during the course of the disease. The lymphographic findings in this case report were very similar to those encountered in malignant lymphoma, a not unexpected situation since the clinical and pathological resemblances to histiocytic lymphoma are striking.
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PMID:Lymphography in malignant histiocytosis: case report. 78 10

A 66-year-old man was admitted for shortness of breath and showed fever, abdominal fullness and paraplegia. Monocytosis amounting to 25% and an elevation of serum LDH (4,281 mIu), were remarkable in the laboratory findings. He died of pulmonary insufficiency about a month after admission. On autopsy hepatomegaly (1950 g), splenomegaly (780 g), but no lymphadenopathy and small infarction in the thoracic spinal cord causing paraplegia was noted. Histopathologically, the invasion of the tumor cells into the liver, spleen, lymph nodes, bone marrow and other organs was observed. Malignant histiocytosis was diagnosed by histologic and immunohistochemical studies (lysozyme positive, S-100 protein negative).
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PMID:[A case of malignant histiocytosis with paraplegia]. 362 41

A 64-year-old man was admitted with complaints of lethargy, malaise, weight loss and transient left-hemiplegia and aphasia. Initial physical and laboratory findings showed splenomegaly and pericardial effusion. During his hospital stay, his mental status deteriorated progressively. The characteristic pathology of malignant reticulosis was noted at autopsy. Microscopic examination of the brain demonstrated accumulations of malignant histiocytic cells confined within small vessels and subsequent multiple hemorrhages and necroses in the gray matter. Malignant reticulosis with antemortem manifestations of pericardial effusion and central nervous system involvement is rare.
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PMID:[A case of malignant reticulosis with pericardial effusion and central nervous system involvement]. 685 69

Malignant histiocytosis is a rare invasive proliferation of neoplastic histiocytes. Cases previously reported as malignant histiocytosis were shown to be lymphomas of T or B lineage, especially anaplastic large-cell lymphomas. A case of malignant histiocytosis is described, in which a patient presenting with symptoms suggestive of pneumonia suddenly deteriorated and died. At autopsy, a large quantity of fresh blood, originating from several ruptured nodules on the enlarged spleen, was seen in the peritoneal cavity. Extensive infiltration by pleomorphic tumour cells and erythrophagocytosis by tumour cells were seen on histological examination of the spleen. Immunohistochemical analysis and staining were carried out. This is the second reported case of malignant histiocytosis presenting with spontaneous splenic rupture.
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PMID:Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture. 1680 51

Malignant histiocytosis (MH) is a progressive systemic neoplastic proliferation of morphologically atypical histiocytes, well characterised in humans and dogs but only recently identified in the cat. In all species, liver, lung, lymph nodes, spleen and bone marrow are infiltrated by atypical histiocytes, and the disease is rapidly fatal. The purpose of this study was to describe the clinical, histological, immunohistochemical and ultrastructural findings of MH in a cat, together with the diagnostic work-up and a list of differential diagnoses. Clinical evaluation included a complete blood-cell count, serum biochemistry, urinalysis, serology and ultrasound examination. The cat had clinical signs of depression, thinness, dehydration, pale mucous membranes and tachycardia. Abdominal ultrasonography revealed generalised splenomegaly and hepatomegaly. Necroscopy showed whitish nodules, randomly scattered throughout the parenchyma in the spleen and liver. The periportal lymph nodes were greatly enlarged and the cut surface was uniformly greyish-white and translucent. Histological examination revealed pleomorphic proliferation of large round tumour cells, with numerous phagocytic vacuoles containing erytrocytes, leukocytes and haemosiderin. By immunohistochemistry, positivity for lysozyme and alpha1-antitrypsin and a scattered positivity for Mac 387 were observed. Ultrastructural features of tumour cells included cytoplasmic lipid droplets, lysosomes and phagolysosomes. MH in the cat needs to be differentiated from diffuse granulomatous disease, non-Hodgkin's lymphoma and Hodgkin's-like disease. The morphological features of the tumour cells, combined with immunohistochemical and ultrastructural observation, are consistent with a diagnosis of MH in the cat.
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PMID:Morphological characterisation of malignant histiocytosis in a cat. 1908 73

We report an interesting case of Malignant Histiocytosis (MH) with polyploid clones characterized by i(17q) and t(9; 22) translocation. A 47-year-old man had lymphadenopthy, splenomegaly and leukopenia at presentation. Bone marrow (BM) cytology showed 10.5% abnormal histiocytes. Karyotypic analyses with R- and G-banding techniques on BM cells revealed complex abnormalities: 88, XXYY, add(2) (p25), -4, -8, -11, i(17q), -21[4]/89, idem, t(9; 22) (q34; q11), +22[26]/46, XY [47], of which, t(9; 22) was confirmed by fluorescence in situ hybridization using a chromosome 22 paint wcp 22+. This patient was treated with interferon-alpha and COP regimen. 10 months later he achieved a complete hematologic and cytogenetic remission (CR). However, relapse occurred one year after achieving CR. At that time, cytogenetic examination showed a new polyploid clone characterized by add(1) (p36), add(2) (p25), -4, -8, t(9; 22), -11, i(17q), -21, +22 in addition to the other two polyploid clones observed previously. RT-PCR indicated that the BCR/ABL transcript (165bp) observed in classic chronic myeloid leukemia, was present, MH with t(9; 22) has not previously been described in the literature. This case may be the first one of MH with t(9; 22), and is likely a secondary event.
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PMID:A Case of Malignant Histiocytosis having Polyploid Clones Characterized by an Isochromosome of the Long Arm of Chromosome 17 [i(17q)] and t(9;22) Translocation. 2740 5