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)

Myelofibrosis is observed in 1/5 of the cases of C.M.L. It consists of reticulin fibers with few collagen and no osteosclerosis. It involves signs which usually indicate the extension of the myelosis to other organs and other types of cells: hepatosplenomegaly, erythroblastosis, thrombocytemia. Its prognosis is always bad. In one third of the cases, myelofibrosis develops early, and in two third it is late. Chimiotherapy is not responsible for it. These forms of C.M.L. with myelofibrosis appear as a special type of myeloproliferative disorder apart from the true C.M.L. and the true osteomyelofibrosis.
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PMID:[Myeloid leukemia with myelofibrosis (author's transl)]. 12 27

The data on 31 patients who fit into the clinical spectrum of subacute myeloid leukemia have been reviewed. The majority of patients were male with a median age of 61 years. The interval from onset of symptoms to actual diagnosis was extremely variable, with a mean of 16 months and a median of six months. Most patients presented with anemia and thrombocytopenia, although the white blood cell count varied from striking leukopenia to marked leukocytosis. Examination of the bone marrow invariably revealed abnormalities of all cell lines with megaloblastoid erythrogenesis and dysplastic megakaryocytopoiesis. Although the white cell line showed prominence of immature forms, there was more maturation than is seen in acute myeloid leukemia. Survival from diagnosis was variable, from less than one month to greater than 68 months, with a median of only six months. Anemia and hepatosplenomegaly were prognosticators of a poor outlook; patients with hepatosplenomegaly in association with either leukocytosis or thrombocytopenia had a particularly poor outlook, with a median survival of only one and a half months. Approximately half the patients received chemotherapy with no demonstrated effect on survival.
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PMID:Subacute myeloid leukemia: a clinical review. 28 73

Observations in six adult patients with leukaemic differential white counts, predominantly mature-celled, and with hepatosplenomegaly show that the mature-celled but fulminant (para-)neutrophil leukaemia must be differentiated from Ph1-positive chronic myeloid leukaemia. This (para-)neutrophil leukaemia is probably identical with the previously described atypical chronic myelosis of the adult, chronic myeloid leukaemia of childhood and the Pelger-like chronic myeloid leukaemia. Cardinal signs are a mature-celled differential count, short life expectancy (1 year), initial platelet deficiency, increased activity of granulocyte alkaline phosphatase, absence of Ph1-chromosome, and poor therapeutic response to busulfan. This curious and yet apparently not uncommon disease has been observed in the adult age group predominantly in men. The frequently high HbF level observed in juvenile chronic myeloid leukaemia could not be demonstrated in adults. Some of these neutrophil leukaemias are characterized by medullary fibrosis and terminal increase of immature blast cells (blast crises?) of which the diagnostic reliability is still disputed.
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PMID:[Differential diagnosis of atypical chronic myeloid leukaemia]. 106 23

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 considerable reduction of hepatosplenomegaly and leucocytosis in leukemic rats of the Brown Norway Myeloid Leukemia (BNML) can be achieved by exposure to 50% nitrous oxide/50% oxygen. In this study the differential antiproliferative effect of nitrous oxide, inactivating vitamin B12, on normal and leukemic hemopoiesis was investigated in this rat model. Rats injected with leukemic cells and exposed to nitrous oxide for 10 days showed 30% reduction of hepatosplenomegaly and 50% reduction of leukocytosis. Similarly treated healthy rats showed no signs of impaired hemopoiesis as measured by peripheral blood parameters. Clonogenic assays of erythroid and myeloid progenitors from both healthy and leukemic rats revealed that exposure to nitrous oxide did not suppress normal bone marrow functioning. On the contrary, the reduction of leukemic proliferation by nitrous oxide retarded the leukemic infiltration of the bone marrow compartment.
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PMID:Nitrous oxide selectively reduces the proliferation of the malignant cells in experimental rat leukemia. 273 Nov 56

Infection of BALB/c mice with the RLV-A virus typically results in an erythropoietic dysplasia characterized by hepatosplenomegaly, erythroblastosis, erythroblastemia and severe anemia without reticulocytosis. Mice hypertransfused weekly with 75%-packed red cells for 42 days prior to RLV-A infection and viral potency controls manifested this typical RLV-A response. Mice that were hypertransfused prior to and following RLV-A infection never developed the "typical" RLV-A pathogenesis. Instead, a transplantable myeloid leukemia was established. Although the reason for altered pathogenesis remains uncertain, it seems plausible that continued hypertransfusion, presumably after establishment of an altered granulopoietic microenvironment, resulted in a completely different viral expression and development of the transplantable myeloid leukemia.
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PMID:Sustained hypertransfusion and induction of a transplantable myeloid leukemia in RLV-A-infected BALB/c mice. 273 54

Infection of BALB/c mice with the RLV-A virus normally induces an erythropoietic dysplasia characterized by hepatosplenomegaly, erythroblastosis, erythroblastemia and severe anemia without reticulocytosis. Time to death varies between 20-30 weeks. Mice were inoculated with RLV-A after being hypertransfused with 75% packed red cells for 42 days which has been shown to eliminate erythropoiesis and modify the microenvironment to favor granulopoiesis. Following RLV-A inoculation, one group did not receive further transfusion (short-term) and another group continued with hypertransfusion weekly (long-term). The pathogenesis of RLV-A in the short-term group paralleled the characteristic RLV-A response. In the long-term group however, the characteristic RLV-A response was never observed. Instead, a granulocytic leukemia was developed. Continued hypertransfusion presumably after establishment of an altered microenvironment resulted in a completely different viral pathogenesis and the development of a transplantable myeloid leukemia.
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PMID:Effect of sustained hypertransfusion on Rauscher leukemia virus-variant A (RLV-A) infection in BALB/c mice. 322 6

Acute megakaryocytic myelosis represents a distinct disease entity. As shown by a survey of the literature, it is a rapidly fatal disease, not preceded by a chronic myeloproliferative disorder, and mostly refractory to cytotoxic treatment. The first manifestation is pancytopenia with initial absence but quick development of hepatosplenomegaly. In contrast to "acute myelofibrosis", which is characterized by hyperplasia of all three haematopoietic lineages, a purely megakaryocytic proliferation develops together with a slight reticulinic fibrosis. Immature megakaryocytic cells may appear in the circulation, allowing classification of the disease as a variant of acute non-lymphatic leukaemia. This view is stressed by the observation of a preceding myelodysplastic phase in the case reported here.
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PMID:Acute megakaryocytic myelosis preceded by myelodysplasia. Report of a case and review of the literature. 375 40

There have been few reports of acute leukemia presenting with a hypocellular bone marrow. All patients diagnosed as having acute leukemia were identified during a recent six-year interval who had blast cells plus promyelocytes of greater than 30% and marrow cellularity of needle biopsy less than or equal to 50%. Of 195 patients analyzed, 15 (7.7%) fulfilled the criteria. Ten patients were men and five women; the median age was 68 years with a range of 40-82. Seven complained of fatigue of 6-12 months duration, five were seen with occult infection, and three were asymptomatic. Hepatosplenomegaly was absent in 93% and none had lymphadenopathy. Fourteen patients were pancytopenic with median leukocyte count at presentation of 1.5 X 10(9)/liter, hemoglobin of 9.0 g/dl, and platelet count of 55 X 10(9)/liter. Circulating blast cells were not observed in ten patients; in the other five they were less than 0.7 X 10(9)/liter. The morphology of all cases appeared myeloid and Auer rods were seen in three patients; however, in one the peroxidase was negative. Classification according to FAB criteria revealed ten to be M1, three to be M2, one M4, and one L2. Median survival of the entire group was seven months. Of seven patients receiving no chemotherapy, two survived longer than 1 year (14, 24.5 months), one is alive at 7+ months, and the median survival was seven months. Eight patients with life-threatening complications received various combination regimens including an anthracycline, cytosine arabinoside, 6-thioguanine, vincristine, and prednisone. Five died of treatment complications; two achieved durable complete remission and are free of disease at 17 and 27 months. It can be concluded that hypoplastic acute leukemia is a distinct nosologic entity affecting primarily older patients with myeloid leukemia. Remission induction therapy in patients who are seriously ill has a low success rate, and in some patients prolonged survival is possible with supportive care alone.
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PMID:Hypoplastic acute leukemia. 727 65

In a 77-year-old Caucasian female B-cell chronic lymphocytic leukaemia was diagnosed and classified as stage 1 according to the Rai classification. The disease remained stable and therefore no antileukaemic therapy had to be initiated. Four years after the initial diagnosis the patient developed hepatosplenomegaly, anaemia and leukopenia. Bone marrow biopsy revealed megakaryocytic myelosis supervening upon the pre-existing chronic lymphocytic leukaemia.
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PMID:Suppression of chronic lymphocytic leukaemia by megakaryocytic myelosis. 795 70


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