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)

Results from transabdominal fine-needle aspiration of the spleen in 28 dogs and 5 cats are reported. Splenomegaly was present in 79% of these patients, and splenic masses were present in 15%. Extramedullary hematopoiesis, the most common cytologic diagnosis, was found in 24% of the patients and was associated with a variety of diseases including immune hemolytic anemia, hemangiosarcoma, and bone marrow hypoplasia. Hematopoietic neoplasms including lymphosarcoma, plasmacytoma, myelogenous leukemia, and systemic mastocytosis were diagnosed in 24% of the patients. Other diagnoses included malignant neoplasia of undetermined cell type and lymphoreticular hyperplasia. Splenic aspirates were considered normal in 18% of the animals. Two (6%) of the aspirates contained liver tissue rather than spleen. Histologic evaluation of splenic tissue was performed in 42.5% of the patients. All cytologic diagnoses correlated well with their final histologic diagnoses. Complications from the aspiration procedure were not observed, even in thrombocytopenic patients.
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PMID:Fine-needle aspiration of the spleen as an aid in the diagnosis of splenomegaly. 350 95

On clinical examination, a six-year-old Hassian gray gelding with a history of impaired performance, slight cough, colic, and edema of the ventral abdomen, prepuce and the legs had reduced skin turgor, pale mucous membranes, forced costoabdominal breathing, reduced venous return, enlarged lymph nodes, and splenomegaly. Hematologic findings revealed anemia, leukocytosis and a high percentage of monocytoid leukemic cells. Generalized lymphadenopathy, splenomegaly, ascites, hydrothorax, and a diffusely thickened gut wall were found at necropsy. Massive infiltration with monocytoid leukemic cells was detected in lymph nodes, spleen, bone marrow, liver, gut wall, kidneys, and choroid plexus. Incubation of living cells obtained from a leukocyte concentrate with latex particles revealed phagocytosis in the leukemic cells on light and electron microscopy. The leukemic cells also had a marked alpha-naphthyl-acetate and naphthol-AS-acetate esterase activity, but were only weakly positive to naphthol-AS-D-chloroacetate esterase. A very weak alkaline phosphatase activity only was demonstrated in a few leukemic cells. On scanning electron microscopy, the leukemic cells had prominent ruffles and ridge-like profiles. These features of the leukemic cells excluded lymphocytic and granulocytic leukemia, and monocytic leukemia was diagnosed.
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PMID:Monocytic leukemia in a horse. 658 70

Four cases of acute myelogenous leukemia and six cases of chronic myelogenous leukemia after treatment with azathioprine and prednisone for renal allotransplantation have been described in the literature. We report another two cases of acute leukemia 10 and 5 years after successful renal allotransplantation. Patient 1, a 29-year-old farmer, exhibited the signs of acute lymphatic leukemia resistent to treatment with cytostatic agens. Death was due to pneumonia. Patient 2, a 47-year-old salesman, developed pancytopenia together with splenomegaly. After splenectomy an atypical subacute myeloid leukemia became apparent which was not treated due to withdrawal of the patient. He died 2 months after diagnosis. Both patients received long-term immunosuppressive therapy with azathiopine and prednisone until the leukemia was diagnosed. A relationship between long-term immunosuppression and the occurrence of leukemia is postulated.
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PMID:[Acute leukemia after kidney allotransplantation (author's transl)]. 699 44

In an autopsied female primary polycythemia was definitely diagnosed only after histological examination. A classical form of Vaquez-Osler disease was characterized by pronounced normo-, granulo- and megakaryocyte hyperplasia of the bone marrow, spleen and liver myelosis with an admixture of atypical megakaryocytes and splenomegaly. There was a complication in the form of two coronary arteries thrombosis. Lethal outcome resulted from myocardial infarction.
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PMID:[Polycythemia vera, complicated by myocardial infarction]. 767 90

Diagnosis of chronic myeloproliferative disorders (CMPD) can encounter difficulties due to overlaps and possible transitions between the different entities and their similarity to reactive myeloproliferations. In this study DNA analysis has been applied to improve differentiation of CMPD. All subtypes of CMPD analyzed, including chronic myeloid leukemia (CML), agnogenic myeloid metaplasia (AMM), polycythemia vera (PV), and essential thrombocythemia (ET), had in common that granulocytes and bone marrow cells were clonal in origin as shown by X-chromosome-linked DNA polymorphism in conjunction with methylation patterns. Reactive myeloproliferations, by contrast, revealed a polyclonal inactivation pattern. Clonality could not distinguish CMPD from cases of myelodysplastic syndrome (MDS) since the latter also exhibited clonal hematopoiesis. AMM belongs to the group of myeloproliferative syndromes. Up to now the cellular phase at onset of the disease (megakaryocytic myelosis) has not been analyzed for clonality of the hematopoietic cells. Granulocytes as well as bone marrow cells from the cellular phase and advanced stages of the disease revealed a monoclonal inactivation pattern of X-chromosomal genes. These results show that the cellular phase already represents a monoclonal, and hence probably a neoplastic, proliferation of a pluripotent stem cell. The monoclonality of granulocytes could also be demonstrated in patients with splenomegaly and strongly argues against a compensatory proliferation of regular hematopoiesis in this organ. Because of their clonal origin, peripheral granulocytes were used in all cases (n = 244) for the detection of bcr-gene rearrangement. Despite possible morphological overlaps between different types of CMPD, bcr-gene rearrangement proved to be specific for CML and could be applied to differentiate CML from other CMPD in cases of uncertain morphological diagnosis. It is concluded that CMPD represent clonal hemopoietic disorders that probably have specific underlying genetic defects. Thus, DNA analysis can substantially aid in the differential diagnosis of CMPD.
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PMID:[Histopathology and molecular pathology of chronic myeloproliferative disorders]. 837 86

Here we report a 65-year-old male patient with idiopathic myelofibrosis (IMF) accompanied with acromegaly. He was admitted because of anemia and splenomegaly. No favorable effects were observed when hydroyurea was administered. However, his symptoms were successfully treated by administration of vincristine and prednisolone (VP therapy), which is usually applied for choronic myelogenous leukemia in the accelerated phase. Therefore, VP therapy might be one possible for IMF. Since there have been several reports suggesting the possible association of hematological malignancies with acromegaly, we speculate that acromegaly was implicated in the initiation or progression of IMF in this case.
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PMID:[Successful treatment of splenomegaly and anemia by VP therapy in idiopathic myelofibrosis complicated with acromegaly: a case report]. 853 37

Expression of the natural killer (NK) cell antigen CD56 is uncommon among lymphomas, and those that do are almost exclusively of non-B-cell lineage and show a predilection for the nasal and nasopharyngeal region. This study analyzes 49 cases of nonnasal CD56+ lymphomas, the largest series to date, to characterize the clinicopathologic spectrum of these rare neoplasms. All patients were Chinese. Four categories could be delineated. (1) Nasal-type NK/T cell lymphoma (n = 34) patients were adults 21 to 76 years of age (median, 50 years), including 25 men and 9 women. They presented with extranodal disease, usually in multiple sites. The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen. Only 7 cases (21%) apparently had stage I disease. The neoplastic cells were often pleomorphic, with irregular nuclei and granular chromatin, and angiocentric growth was common. The characteristic immunophenotype was CD2+ CD3/Leu4- CD3epsilon+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV). Follow-up information was available in 29 cases: 24 died at a median of 3.5 months; 3 were alive with relapse at 5 months to 2.5 years; and 2 were alive and well at 3 and 5 years, respectively. (2) Aggressive NK cell leukemia/lymphoma (n = 5) patients presented with hepatomegaly and blood/marrow involvement, sometimes accompanied by splenomegaly or lymphadenopathy. The neoplastic cells often had round nuclei and azurophilic granules in the pale cytoplasm. All cases exhibited an immunophenotype of CD2+ CD3/Leu4- CD56+ CD16- CD57- and all were EBV+. All of these patients died within 6 weeks. (3) In blastoid NK cell lymphoma (n = 2), the lymphoma cells resembled those of lymphoblastic or myeloid leukemia. One case studied for CD2 was negative and both cases were EBV-. One patient was alive with disease at 10 months and one was a recent case. (4) Other specific lymphoma types with CD56 expression (n = 8) included one case each of hepatosplenic gammadelta T-cell lymphoma and S100 protein+ T-cell lymphoproliferative disease and two cases each of T-chronic lymphocytic/prolymphocytic leukemia, lymphoblastic lymphoma, and true histiocytic lymphoma. All of these cases were EBV-. Six patients died at a median of 6.5 months. Nonnasal CD56+ lymphomas are heterogeneous, but all pursue a highly aggressive clinical course. The nasal-type NK/T-cell lymphoma and aggressive NK cell leukemia/lymphoma show distinctive clinicopathologic features and a very strong association with EBV. Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.
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PMID:Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. 919 74

The chronic myeloproliferative syndromes are bone marrow stem cell disorders. An increase of cell counts of one or rather all three blood cell types is characteristic for these disorders. The most important diseases in this group are: chronic myelogenous/granulocytic leukemia, polycythemia rubra vera, osteomyelosclerosis or agnogenic myeloid metaplasia and essential thrombocythemia. The cells are normally differentiating in these diseases, while the control of cell dividing is abnormal and therefore the cells are produced and accumulated in excess. Splenomegaly is a common and characteristic clinical finding. The fibrotic or sclerotic transformation of the bone marrow can take place in all forms of the syndrome. Extramedullary haematopoiesis can occur in all of the above diseases, but it is most common in myelofibrosis/agnogenic myeloid metaplasia. In the last phase of the disease a terminal blastic crisis may terminate the course of chronic myeloproliferative diseases. The myeloproliferative disorders can be transformed in each other--the most common transformation is that of the polycythemia rubra vera into myelofibrosis. The greatest progress in the therapy of chronic myeloproliferative diseases is achieved in chronic myelogenous leukemia: bone marrow transplantation and interferon treatment (the latter also in essential thrombocythemia and polycythemia rubra vera) are routine modalities worldwide. A new drug, anagrelide is effective in the treatment of myeloproliferative thrombocytosis and thrombocythemia.
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PMID:[Chronic myeloproliferative diseases]. 971 44

There has not been a reported series of children with therapy-induced myelodysplastic syndrome/acute myeloid leukemia (tMDS/tAML) who were treated systematically. This paper describes 24 children with tMDS/tAML who were assigned randomly to standard- or intensive-timing induction on protocol CCG 2891. Presenting features and outcomes of those children were compared with those of 960 patients with de novo MDS (62 patients) or AML (898 patients). Children with tMDS/tAML were older at presentation (P =.015), had lower white blood cell counts (P =.01), and were more likely to have MDS (21% vs 7%) (P =.02) and trisomy 8 (P =.06). Fewer had hepatomegaly (P =.02), splenomegaly (P =.03), hepatosplenomegaly (P =.02), or classic AML translocations [t(8;21), t(15;17), 16q22; P =.02]. They had a poorer induction rate (50% vs 72%, P =.016), overall survival (26% vs 47% at 3 years, P =.007), and event-free survival (21% vs 39% at 3 years, P =.023). Disease-free survival after achieving remission was similar (45% vs 53%, P =.868). Children with tMDS/tAML who received intensive-timing induction had better outcomes than those who received standard-timing induction (overall survival 32% vs 0%, P =.54). In this study, the latency period to development of tMDS/tAML was the same for presumed alkylator-induced as for topoisomerase-induced myeloid leukemia. The findings of this study confirm that most children with tMDS/tAML have disease resistant to current therapies. Standard-timing induction appears less effective for this population.
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PMID:Acute myeloid leukemia and myelodysplastic syndrome in children treated for cancer: comparison with primary presentation. 1209 32

While studying the unique Nramp1 (Slc11a1)-independent susceptibility to Mycobacterium bovis (BCG) infection of BXH-2 mice, we noted that these mice develop important splenomegaly and enlargement of lymph nodes. Segregation analyses in several F2 crosses showed that splenomegaly segregates as a single recessive trait caused by a novel mutation in BXH-2, independent of the infection. Histologic and fluorescence-activated cell sorter (FACS) analyses indicated that splenomegaly is associated with a large increase in Mac1+/GR1+ (macrophage antigen-1+/granulocyte differentiation antigen 1+) granulocyte precursors in spleen, lymph nodes, and bone marrow, resembling a myeloproliferative syndrome. This is concomitant to extramedullary erythropoiesis in the spleen, as measured by proportion of Ter119+ erythroid cells. The locus controlling this myeloproliferative syndrome and splenomegaly was designated Myls and maps to an 18 centimorgan (cM) region of chromosome 8, which also contains an integrated copy of an N-ecotropic murine leukemia virus (MuLV) provirus (Emv2). The relationship between Myls, expansion of Mac1+/GR1+ cells, and Emv2 was investigated. Homozygosity at Myls is necessary but not sufficient for B-ecotropic virus replication in splenocytes, the extent of which appears to be under separate genetic control. Our results suggest a model in which Myls-dependent myeloproliferation in BXH-2 acts as a predisposing factor for the subsequent development of virally induced myeloid leukemia characteristic of this strain.
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PMID:Genetic control of myeloproliferation in BXH-2 mice. 1463 Aug 19


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