Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute myelofibrosis is a rare but distinct accelerated variant of agnogenic myeloid metaplasia that is characterized by marked anemia, peripheral blood myeloblastosis and normoblastosis, a lack of teardrop poikilocytosis, and prominent myelofibrosis. There is usually no palpable hepatosplenomegaly or lymph node enlargement. The clinical course is remarkable short. We describe a 63-year-old man who presented with idiopathic acquired sideroblastic anemia and subsequently developed acute myelofibrosis. Intensive polychemotherapy with vincristine, cytosine arabinoside, and prednisone and a later trial of oxymetholone therapy were ineffective. He died 134 days after the diagnosis of acute myelofibrosis was established. The 11 previously reported cases of acute myelofibrosis are reviewed, and the relationships of acute myelofibrosis to other myeloproliferative disorders and to idiopathic acquired sideroblastic anemia are discussed.
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PMID:Idiopathic acquired sideroblastic anemia terminating in acute myelofibrosis: case report and review of leterature. 31 17

A 60 year old man had well documented agnogenic myeloid metaplasia with pancytopenia. Frequent red cell transfusions were required for two and a half years. Androgen therapy was not beneficial and was discontinued 26 months before a spontaneous hematologic remission occurred. The remission was documented by bone marrow biopsy and reticuloendothelial scanning of the marrow. Hepatosplenomegaly, poikilocytosis and expansion of the marrow space have persisted despite hematologic remission.
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PMID:Spontaneous hematologic remission in agnogenic myeloid metaplasia. 93 47

Plasmacytoid T-cell (PTC) lymphoma is a rare clinicopathologic entity characterized by generalized lymphadenopathy in association with a myeloproliferative disorder. Hepatosplenomegaly and weight loss frequently are present. Nodal T-zone expansion by mononuclear cells with ultrastructural and immunohistochemical features typical of PTC is diagnostic. All of the five previously reported cases of PTC lymphoma coincided with or heralded the onset of a clinically aggressive myeloid leukemia. This strong association and recent immunohistochemical findings in reactive or neoplastic PTC favored a monocyte/macrophage derivation of these cells, and it has been suggested that they be renamed plasmacytoid monocytes (PM). Two additional cases of PTC lymphoma were studied at the institutions of the authors, and the findings supported the concept that PTC belong to the monocytic lineage. The disease presentation was generalized lymphadenopathy with constitutional symptoms. One patient also had hepatosplenomegaly and bilateral renal enlargement concomitantly with myelofibrosis with myeloid metaplasia that progressed within months to acute myelogenous leukemia. Similar rapid evolution of acute monoblastic leukemia occurred in the other patient. Tumor cells within subtotally effaced lymph nodes had positive findings for CD45, CD4, CD7, and LN2 and negative findings for CD3, CD8, and beta F1. Occasional cells had positive findings for CD2. One case demonstrated CD5, HLA-DR, CD71, and CD43 (Leu-22)-positive cells. The myeloid/monocyte-associated antigens CD14 and CD68 were identified in both. The tumor cells lacked the B-cell markers LN1, CD20 (L26), CD19, and CD22 and did not rearrange immunoglobulin heavy chain genes and T-cell receptor beta, gamma, and delta chain genes. The term plasmacytoid T-zone lymphoma or PM proliferation is more appropriate for this rare disease. The close association of the PM proliferation with a myeloproliferative disorder indicates that the two entities are related.
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PMID:Plasmacytoid monocyte proliferation associated with myeloproliferative disorders. 154 Aug 83

A female patient with agnogenic myeloid metaplasia presented with hepatosplenomegaly and a palpable mass in the right breast. Mammography showed an asymmetric pattern of confluent densities and sonography revealed several smooth, homogeneous and echo-poor masses of variable sizes in both breasts. Excision biopsy of the mass in the right breast revealed extramedullary haematopoiesis as did histopathologic examination of the liver and the spleen. The data are discussed with emphasis on the complementary character of both diagnostic modalities and the resemblance to lymphoma of the breast, although the findings are too non-specific to rule out breast carcinoma. Knowledge of the clinical history and histopathology are necessary to make the proper diagnosis.
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PMID:Case report: mammographic and sonographic demonstration of extramedullary haematopoiesis of the breast. 187 57

The authors make a review of recent data in the literature and compare them to their own cases between 1983 and 1988. They analyzed eight patients with idiopathic myelofibrosis confirmed with bone marrow biopsy of the posterior iliac wrist with Jamshidi's needle. Most patients were between 55 and 60 years old (5 male and 3 female). Major symptoms were weakness and bleeding (6/8 cases), weight loss and bone distress (4/8 cases). In general, physical signs were splenomegaly and anemia (7/8 cases), hepatomegaly (5/8 cases), and jaundice (2/8 cases). Laboratory features were variable. Most cases were diagnosed in an advanced stage of the disease. Therapy with busulfan, prednisone, oxymetholone and radiotherapy of the spleen was used alone or in combination to relieve compressive abdominal symptoms. This review shows that idiopathic myelofibrosis should be included in the differential diagnosis of patients with hepatosplenomegaly and anemia.
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PMID:[Idiopathic myelofibrosis]. 188 77

From 1982 to 1985, four cases of primary myelofibrosis were diagnosed in our department. Three were boys and one was a girl. Their ages ranged from 7 months to 15 years. The diagnosis was made based on anemia, leukoerythroblastic change and presence of giant platelets in the peripheral blood, and a bone marrow biopsy showing myelofibrosis. Most of them had anemia, fever, and hepatosplenomegaly on admission. The anemia was severe and refractory to repeated transfusions and steroid therapy in 3 out of the 4 cases. Splenectomy was performed in 1 case, but without satisfactory results. The clinical course and blood pictures in one case resembled leukemia of megakaryocyte lineage (M7), but results of marker studies of the blast cells ruled out the possibility of M7. Three of them underwent leukemic transformation within 2 years and died soon after. The other one died of sepsis 2 weeks after diagnosis. Myelofibrosis in childhood occurs rarely, however, when it does, it always runs a rapid and fatal course.
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PMID:Primary myelofibrosis in children: report of 4 cases. 198 Dec 37

A 29-year-old male was diagnosed as having essential thrombocythemia (ET) in 1975. From that time, his platelet count gradually increased to more than 2 X 10(6)/microliter until 1979. However, his platelet count gradually decreased to less than 6 X 10(5)/microliter in 1985. Also, in 1982, erythroblasts and immature myeloid cells began to appear in the peripheral blood, and the liver and spleen became palpable in 1985. Bone marrow then revealed osteomyelosclerosis. These findings suggested that ET had transformed to myelofibrosis with myeloid metaplasia. Increased hepatosplenomegaly was accompanied by the appearance of ascites in June, 1988, and an esophageal varix ruptured in December of the same year. The varix was resected and the spleen was removed. After the operation, ascites did not recur and his condition became stable. Portal hypertension in this patient was considered to be due mainly to increased blood flow from the enlarged spleen.
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PMID:[Essential thrombocythemia transformed to myelofibrosis with myeloid metaplasia after seven years]. 204 Nov 65

Idiopathic myelofibrosis is a disease of unknown cause characterized by systemic marrow fibrosis and extramedullary hematopoiesis. We report here a patient of myelofibrosis treated successfully by busulfan pulse therapy which was reported first by chang et al in 1988. The patient was a 62-year-old woman who was referred to us for anemia and hepatosplenomegaly in August 1984. Further examination established a diagnosis of idiopathic myelofibrosis. During the subsequent 4-year follow-up period without specific treatment in our outpatient clinic, there occurred gradual progression of anemia and hepatosplenomegaly with the spleen extending beyond the level of the umbilicus. In September 1988, she was initiated on 4-day pulse therapy of busulfan with a daily dose of 12 mg, which was repeated 10 times until July 1989. This was followed by marked improvement of anemia and hepatosplenomegaly. Post-treatment iliac marrow biopsy showed some reduction of reticulin fibers with increased hematopoietic elements as compared to pretreatment iliac marrow biopsy. The busulfan pulse therapy, therefore, appears to be a treatment of choice in idiopathic myelofibrosis.
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PMID:[Successful treatment of primary myelofibrosis by busulfan pulse therapy: report of a case]. 224 32

A case of bronchial carcinoid tumor with foci of extramedullary hematopoiesis is presented. The patient had a 6-year history of agnogenic myeloid metaplasia and hepatosplenomegaly. All three hematopoietic cell lines were represented in different areas of the bronchial tumor. To our knowledge, this is the first case of extramedullary hematopoiesis described in a bronchial carcinoid and only the fourth report of hematopoietic cells in a neoplasm of otherwise unrelated origin. The pathogenesis of this uncommon finding is discussed and the literature is reviewed.
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PMID:Extramedullary hematopoiesis in a bronchial carcinoid tumor. An unusual complication of agnogenic myeloid metaplasia. 225 28

A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Women's Medical School, was admitted to our hospital because of left hip-joint pain in May 1988. Physical examinations revealed marked hepatosplenomegaly and multiple reddish papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis. Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis. He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later. At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen. As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.
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PMID:[Primary myelofibrosis with extramedullary haematopoiesis of the skin transformed into acute megakaryoblastic leukemia]. 232 83


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