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)

A quantitative scanning method employing cyclotron-produced 52Fe has been developed to assess splenic erythropoiesis in patients with myeloproliferative disorders. In 12 patients with myelofibrosis splenic uptake of 52Fe was from 5.0% to 48% of the injected dose. Although a single patient with classical polycythaemia vera had a minor uptake of 2.8% of six other patients with this diagnosis showed no concentration of isotope in the splenic area. The fraction of 52Fe in the spleen of four patients with 'transitional' myeloproliferative disorders characterized by a high red cell mass, hypercellular bone marrow and a leucoerythroblastic blood film varied from 5% to 41%. No clear relationship was noted between the degree of splenic erythropoiesis as defined by this technique and the level of haemoglobin, the degree of splenomegaly, the effectiveness of erythropoiesis of traditional 59Fe surface counting. If splenectomy is considered in patients with myelofibrosis splenic 52Fe quantitation will provide more precise data on the contribution of splenic erythropoiesis than 59Fe surface counting alone.
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PMID:Quantitative studies of splenic erythropoiesis in polycythaemia vera and myelofibrosis. 99 Jan 84

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.
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PMID:Myeloproliferative disorders. 105 48

Lethally irradiated mice transplanted with bone marrow cells infected with a novel recombinant retrovirus (murine stem cell virus-interleukin 6 [MSCV-IL-6]) bearing a mouse IL-6 gene developed a fatal myeloproliferative disease within 4 wk of engraftment. The hematologic manifestations of the syndrome included elevated peripheral leukocyte counts (up to 430 x 10(3) cells/mm3) with a predominance of neutrophilic granulocytes, microcytic anemia, and thrombocytosis or thrombocytopenia. The mice showed extensive neutrophil infiltration of the lungs, liver, and occasionally lymph nodes, plus splenomegaly resulting from enhanced splenic myelopoiesis (30-60-fold increase in progenitor numbers). Despite the chronic stimulation of neutrophil excess by IL-6, bone marrow from affected mice was capable of repopulating the hematopoietic tissues (bone marrow and spleen) of lethally irradiated hosts during repeated serial transplantation. In the longest documented case, the progeny of a single MSCV-IL-6-marked cell transferred the myeloproliferative disease to two secondary, four tertiary, and two quaternary recipients (the clone endured for a total of 72 wk). These results, demonstrating considerable proliferative longevity of the IL-6-producing cells, support an in vivo role of IL-6 in the maintenance of hematopoietic precursors. Dysregulated IL-6 production also had significant systemic effects. The mice displayed increased mesangial cell proliferation in the kidney, frequent liver abnormalities, and marked alterations in plasma protein levels. Unlike previous studies where constitutive expression of exogenous IL-6 genes resulted in lymphoproliferative disorders characterized by massive plasmacytosis, minimal plasma cell expansion occurred in the MSCV-IL-6 mice during the observation period. Potential explanations for the differences in disease phenotypes observed in the present and previous studies are different cell types expressing the exogenous IL-6 genes, higher sustained circulating levels of IL-6 achieved using the MSCV-IL-6 retroviral delivery system, and/or the premature death (3-15 wk after transplantation) of the MSCV-IL-6 mice before the onset of plasmacytosis. This animal model should prove useful for further investigation of the function of IL-6 in normal and abnormal hematopoiesis and in inflammatory responses.
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PMID:Transplantable myeloproliferative disease induced in mice by an interleukin 6 retrovirus. 140 59

Idiopathic myelofibrosis is a rare myeloproliferative disorder characterized by excessive accumulation of connective tissue in the bone marrow in association with anemia, splenomegaly, and extramedullary hematopoiesis. The cause of this disease is unknown, and the prognosis is generally poor. To our knowledge, this is the first case report of a patient with idiopathic myelofibrosis who carried a term pregnancy. In spite of the increased perinatal risks, a favorable outcome was possible with close antepartum surveillance.
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PMID:Idiopathic myelofibrosis in pregnancy: a case report and review of the literature. 144 51

In myeloproliferative disorders, aggravation of splenomegaly was reported as an adverse effect of erythropoietin (EPO). Recently, we experienced the adverse effect of EPO in myelodysplastic syndrome (MDS). A 65-year-old male was admitted to our hospital for scrutiny of pancytopenia in July 8, 1991. He was diagnosed as having MDS (refractory anemia: RA). After discharge, daily subcutaneous administration of EPO (3,000U) was started on August 1 because his Hb concentration had decreased to 9.2 g/dl. After the daily dose of EPO was increased up to 6,000U in August 15, left hypochondralgia gradually developed. EPO administration was haltedon August 22. His splenomegaly was aggravated from 2 finger breadths below the left costal margin before EPO administration to 4.5 finger breadths. Bone marrow examination revealed a change to extremely hypercellular marrow from slightly hypocellular marrow before EPO administration. The peripheral blood cell count was not altered. We concluded that he was a rare case of MDS in which aggravation of splenomegaly was observed, probably as a result of extramedullary hematopoiesis induced by administration of EPO.
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PMID:[Rapid aggravation of splenomegaly by administration of erythropoietin in a case of myelodysplastic syndrome]. 147 99

Agnogenic myeloid metaplasia is a chronic myeloproliferative disorder characterized by splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, teardrop-shaped red blood cells, and fibrosis of the bone marrow. It is a disease of adults, with only one case report in the pediatric literature. The symptoms of the patient in this case clearly fit the diagnostic criteria of this disease. Myelofibrosis in children is usually of the acute type, presenting in infancy and running a fulminant, fatal course with minimal or mild splenomegaly. Red blood cells are usually normal on morphologic examination. Three infants, including two siblings, presented at ages 9, 10, and 16 months with clinical and laboratory findings consistent with agnogenic myeloid metaplasia. The occurrence of the disease in these siblings is suggestive of an autosomal recessive mode of inheritance.
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PMID:Agnogenic myeloid metaplasia in children. 163 66

We have used recombinant human erythropoietin (rHuEPO) in a phase I/II clinical trial to evaluate its ability to reverse refractory anemia in hematologic disorders. rHuEPO was administered subcutaneously 5 days per week at escalating doses (50 to 150 U/kg per day). The aim of treatment was a hemoglobin (Hb) level greater than or equal to 10 g/dL without blood transfusion. Of 25 patients treated, 17 were evaluable, most of them with a regular need for transfusion. Eight of these had lymphoproliferative disorders (three cases of malignant lymphoma and five of monoclonal gammopathy) and were exposed to cytotoxic therapy. The other nine patients had hematopoietic stem cell disorders (four cases of myelodysplastic syndrome, three of idiopathic myelofibrosis, and two of chronic myelogenous leukemia). All patients with lymphoproliferative disorder had serum EPO levels inappropriately low for the degree of anemia, while patients with stem cell disorder showed variable values. Erythroid marrow activity was inadequate in all cases. Seven of eight patients with lymphoproliferative disorder responded to treatment maintaining Hb above 10 g/dL without transfusion. The median dose of rHuEPO required for correction of anemia was 75 U/kg. In four cases response was maintained with 50 U/kg, three times per week. There was no complete response among patients with hematopoietic stem cell disorder, although transfusion requirement was eliminated or reduced in four cases. Four patients developed functional iron deficiency during rHuEPO treatment and required iron supplementation to obtain response. Aggravation of splenomegaly was observed in two cases of myeloproliferative disorder. We conclude that: (1) subcutaneous administration of rHuEPO can be effective and safe in patients with lymphoproliferative disorder exposed to chemotherapy and showing inappropriate EPO response to anemia; (2) this is less likely in hematopoietic stem cell disorders, although favorable responses may be observed in occasional patients; and (3) functional iron deficiency as a cause of nonresponse to rHuEPO is frequent also in nonrenal anemia.
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PMID:Subcutaneous erythropoietin for treatment of refractory anemia in hematologic disorders. Results of a phase I/II clinical trial. 163 33

A 20-year old patient is presented with generalized lymphadenopathy, splenomegaly, hyperleukocytosis and a bone marrow biopsy showing panmyelosis with predominance of immature granulocytes. Lymph node biopsy showed a histopathological feature that was diagnosed as a chronic granulocytic leukemia in blast crisis. The cell surface phenotype of these blast cells showed predominance of immature CD1+, CD7+ T lymphocytes. The T cell lineage was confirmed by DNA rearrangement studies. In addition, the patient showed erythrocytosis, arterial O2 saturation of 92% and thrombocytosis, characteristics of polycythemia vera. After chemotherapy, the patient relapsed with similar symptoms and lymph node cells of similar immature T phenotype. With a revised diagnosis of immature T cell lymphoma associated to a myeloproliferative disorder and polyglobulia, the patient received a combined treatment of Cyclophosphamide-Adriamycin-Vincristine-VM26-Prednisone. Two months later, the patient relapsed again. He received the first phase of induction of the BFM protocol, with partial clinical remission. Five months later, the patient returned with fever, polyadenopathy and splenomegaly. Lymph node cells showed again immature T cell phenotype. The patient was next treated with the m-BACOD scheme, with no response and progression of the disease and he died few days later due to massive bleeding and cardiorespiratory failure.
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PMID:[T lymphoma of immature phenotype associated with polycythemia vera]. 182 May 2

Idiopathic myelofibrosis is a myeloproliferative disorder of unknown origin. The bone marrow becomes fibrotic with an associated decrease in hematopoiesis resulting in anemia, bleeding problems, splenomegaly, and other secondary abnormalities. Although idiopathic myelofibrosis is usually diagnosed in middle age, there have been a few reports of the disorder in the pediatric population. This case report documents dental treatment considerations in a 6-year-old female with idiopathic myelofibrosis, severe anemia, and abnormal blood coagulation studies. The patient was successfully treated in a hospital after medical consultation, transfusion of packed red blood cells, and administration of prophylactic antibiotics. Local hemostatic measures following multiple extractions of carious teeth controlled bleeding. No postoperative sequelae occurred.
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PMID:Idiopathic myelofibrosis: dental treatment considerations. 183 Dec 96

We report on 22 patients with myelodysplastic syndrome (MDS), all of whom showed striking marrow fibrosis. Variable blood counts, often with teardrop poikilocytosis and a leukoerythroblastic picture, were present at diagnosis. Visceral enlargement was detected in 17 patients with a distinct splenomegaly in seven cases. All cases demonstrated dysplasia in at least two cell lineages. No specific cytogenetic abnormality seems to characterize this group of patients. Southern blot analysis showed no breakpoint cluster region rearrangement as observed in classical chronic myeloid leukemia. Ferrokinetic studies revealed quantitatively deficient erythropoiesis in all except two cases and an abnormally high fraction of ineffective erythropoiesis in all. Splenic erythropoiesis was present in eight patients. The median survival was 18 months. At the time of this report, 12 patients had died. The causes of death were disease progression (7 patients) and infection (5 patients). One might speculate that the present series of cases represents a transition between MDS and myeloproliferative disease, thereby displaying characteristics of both groups of diseases.
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PMID:Myelodysplastic syndromes with bone marrow fibrosis: a myelodysplastic disorder with proliferative features. 195 47


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