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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytic medullary reticulosis (HMR) is a rare, progressive, fatal reticuleondothelial proliferative disorder. It was diagnosed in a 10-year-old boy who had pityriasis lichenoides et varioliformis acuta of Mucha-Haberman which was controlled by dapsone for 2 years. One month after cessation of dapsone therapy, cutaneous tumors associated with fever, lymphadenopathy, and hepatosplenomegaly developed. Tissue biopsy specimens of skin, liver, spleen, lymph nodes, and a bone marrow aspirate demonstrated histiocytic erythrophagocytosis and atypical histiocytosis compatible with HMR. A rapidly progressing, fatal course followed despite intensive chemotherapy.
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PMID:Histiocytic medullary reticulosis presenting as Mucha-Habermann disease. 7 36

Malignant histiocytosis, an affection in which there is proliferation of morphologically atypical histiocytes, traditionally associates high fever, deterioration of the general condition, adenopathy, hepatosplenomegaly, and less frequently, cutaneous lesions. Clinical, radiological, and histological signs of bone involvement are rarely observed, which demonstrates the interest of the case reported of a pure medullary form with massive necrosis and successive bone localisations of the osteolytic type. The clinical picture was completed by the progressive development of adenopathy and hepatosplenomegaly. Multiple chemotherapy, according to the A.V.E.C. procedure, controlled the affection for three months before it became totally ineffective. Survival for twelve months after clinical onset demonstrates the extreme malignancy of the affection.
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PMID:[Malignant histiocytosis with bone involvement (author's transl)]. 54 9

A young man with acute lymphoblastic leukemia was treated with vincristine sulfate, prednisone, and intrathecally injected methotrexate sodium for central nervous system involvement. A good remission was induced, but three months later he had hepatosplenomegaly, an enlarging mediastinal mass, and progressive anemia. Histiocytic medullary reticulosis was confirmed by a bone marrow biopsy specimen. The patient died of respiratory failure because of infiltration of the lungs by malignant histiocytes.
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PMID:Acute lymphoblastic leukemia terminating as histiocytic medullary reticulosis. 105 4

Two young females were admitted one by one because of peculiar skin eruptions suggesting mosquito-allergy and severe pancytopenia with normo-hypercellular bone marrow. The outcome of the first case was fatal on account of severe pancytopenia, immune deficiency and marked jaundice 14 months after the initial pancytopenic episode. In the post-mortem examination, HMR (histiocytic medullary reticulosis) was highly suggestive since hepatosplenomegaly with increase of Kupffer cells and large histiocytes including some atypical cells were detected. The second patient was treated with splenectomy because the past treatment were unsatisfactory in the first case and another cases in literatures. After the operation, the patient sustained almost normal blood cell level for 11 months without any treatment. As for the histopathological findings of resected spleen and lymphnodes, some erythrophagocytic mature histiocytes were found, but no atypical cells was detected. The relationship with pancytopenia, mosquito-allergy and HMR was discussed on the two cases and literatures.
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PMID:[Severe pancytopenic episodes in two cases with mosquito-allergy]. 197 33

Malignant histiocytosis (MH: sinusoidal hematolymphoid malignancy) is a rare lymphoreticular disorder characterized by an aggressive clinical course in younger patients with weight loss, hepatosplenomegaly, generalized lymphadenopathy and pancytopenia. Five cases of MH were identified over a five-year period (1982 to 1987) at Indiana University Medical Center. The patients' ages were 12, 16, 20, 30 and 57 years; all presented with classic clinical symptoms. Four cases were diagnosed by fine needle aspiration biopsy; one case was diagnosed by the examination of ascitic fluid. All patients had confirmatory surgical biopsies. The salient cytologic features of MH included (1) a lack of background lymphoglandular bodies, (2) a population of variably sized dyscohesive cells, (3) a component of large bizarre cells with abundant eccentric, deep-blue cytoplasm on Wright-Giemsa-stained preparations, (4) prominent cytoplasmic vacuolization and (5) inconspicuous erythrophagocytosis occurring in the most benign-appearing histiocytic cells. Ancillary studies on cytologic and histologic material (immunostaining for alpha-1-antichymotrypsin and alpha-1-antitrypsin and staining for nonspecific esterases) confirmed the histiocytic nature of the malignant cells. Recognition of the distinctive morphology of MH and the performance of ancillary studies on cytologic preparations should facilitate the rapid diagnosis and early treatment of this aggressive disease.
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PMID:The cytology of malignant histiocytosis (sinusoidal hematolymphoid malignancy). 278 72

A 32-year-old male was hospitalized with high fever, pancytopenia and hepatosplenomegaly. No atypical cells were found in the peripheral blood. Bone marrow aspiration resulted in dry taps. Superficial lymph node swelling was not observed. He had been treated twice for high fever, hepatosplenomegaly and leukopenia that were very similar to the present illness, 13 and 3 years before the onset and hepatosplenomegaly had been noted by the patient for 13 years. The patient died after a rapid course of 20 days. Histiocytic medullary reticulosis (HMR) was diagnosed at the autopsy, which revealed atypical histiocytic infiltration showing erythrophagocytosis in the liver, spleen, left adrenal, and mesenterial and pulmonary hilar lymph nodes. This patient had shown the same clinical signs and hepatosplenomegaly 13 years before the onset of HMR, which suggest a possible latent stage and acute exacerbation of HMR.
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PMID:An autopsy case of histiocytic medullary reticulosis presenting with marked hepatosplenomegaly for 13 years before the onset. 341 86

Malignant histiocytosis, also known as histiocytic medullary reticulosis, is a rare neoplasm of malignant histiocytes. Clinical findings include fever, weight loss, hepatosplenomegaly, lymphadenopathy, anemia, thrombocytopenia, and a rapidly worsening course. Approximately 7 percent of patients have skin manifestations early in the course of their disease. In these patients, diagnosis based on examination of a skin biopsy specimen may be crucial. A case of malignant histiocytosis is presented in which the diagnosis was first suggested by findings on the skin biopsy specimen.
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PMID:Malignant histiocytosis. 401 55

The clinical and pathologic features of 4 dogs with malignant histiocytosis were evaluated. The most common clinical signs were weight loss, lethargy, lymphadenopathy, hepatosplenomegaly, and anemia. Neoplastic histiocytic infiltrates most commonly were found in the spleen, bone marrow, liver, or lymph nodes. Malignant histiocytosis was considered as a differential diagnosis for anemic dogs with lymphadenopathy and/or hepatosplenomegaly.
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PMID:Malignant histiocytosis in four dogs. 405 15

Malignant histiocytosis is characterized by systemic, progressive, and invasive proliferation of malignant histiocytes. The disorder is typically accompanied by fever, general fatigue, lymphadenopathy, and hepatosplenomegaly. A case of a 21-year-old woman with primary malignant histiocytosis of the oropharynx is reported. Histologic diagnosis from the biopsy specimen was confused by infiltration of normal-appearing histiocytes and inflammatory cells. The titers of Epstein-Barr virus and the Paul-Bunnell test were elevated without atypical lymphocytes. The patient died 3.5 months after the onset of symptoms. Autopsy revealed systemic neoplastic proliferation of malignant histiocytes. A review of literature on this subject revealed no cases of malignant histiocytosis primarily involving the oropharynx.
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PMID:Malignant histiocytosis in the oropharynx. 647 63

Histiocytic medullary reticulosis (HMR) was originally defined as a neoplastic disorder. Some cases reported as HMR have been characterized by a systemic proliferation of mature histiocytes showing hemophagocytosis, bone marrow necrosis, pancytopenia, hepatitis, and coagulopathy. Clinically, these patients have fever and constitutional symptoms and often have hepatosplenomegaly and lymphadenopathy. Although there is a high mortality rate, this process appears to be reactive and has been associated with active viral infection. Similar cases have been briefly described that were associated with other agents or disease processes, but concomitant viral infections were not excluded. Three characteristic examples of this hemophagocytic syndrome that were associated with bacterial sepsis are described. Active infection by those viruses that have previously been associated with the syndrome was excluded. It appears that the hemophagocytic syndrome may be associated with various types of active disseminated infections.
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PMID:Bacteria-associated hemophagocytic syndrome. 649 70


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