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)

Splenic erythropoiesis was demonstrated by surface counting of 59Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agnogenic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodysplastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombocythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II-III myelofibrosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis 1) is encountered in a variety of clinical conditions; 2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myeloproliferative disorders; 3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and 4) can be evaluated in a semiquantitative manner by surface counting.
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PMID:Ferrokinetic study of splenic erythropoiesis: relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis. 275 9

Several groups of authors have recently reported that removal of the massively enlarged spleen (1,500 grams or greater) is associated with a greater morbidity than that encountered in patients with spleens of smaller sizes. In our experience, 46 of 51 patients with massively enlarged spleens had a myeloproliferative disorder (leukemia, lymphoma or agnogenic myeloid metaplasia) and usually had a limited life expectancy. When morbidity in these patients was compared with that in patients with the same diagnosis but spleens of smaller size, no increase in complications or death was found other than a greater operative blood loss in patients with non-Hodgkin's lymphoma. Limitation of operative morbidity in patients with very large spleens depends on careful selection and appropriate operative and perioperative management.
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PMID:Splenectomy for massive splenomegaly. 277 94

The case of a chronic myelogenous leukemia (CML) starting in an unusual form in a young woman is reported. Rapidly progressing icterus was the first and leading symptom of the disease. Simultaneously with the exclusion of the possibility of hepatitis and extrahepatic obstruction of the bile duct the qualitative blood picture roused the suspicion of a myeloproliferative disease. Detailed hematological examinations confirmed Philadelphia chromosome (Ph1) negative CML. Besides the histologically diffuse leukemic infiltration intrahepatic cholostasis could be demonstrated in the background of the icterus. In the chronic and accelerated phase clinical symptoms developing as a consequence of hepatic organic manifestation were dominating. In the authors's case the moderate leukocytosis, initial thrombocytopenia, absence of splenomegaly, early blast-phase and short survival were atypical, characteristic of Ph1 negative CML. The diagnosis and the absence of other associated hepatopathies was supported also by the post-mortem examination. CML beginning with icteric symptoms due to intrahepatic cholostasis is considered as rarity in the literature.
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PMID:[Onset of Philadelphia chromosome negative chronic myeloid leukemia with symptoms of intrahepatic cholestasis]. 281 60

A new kind of myeloproliferative disorder (L-8313) has been discovered. It was transplantable into syngeneic mice with spleen cells. The mice showed hepato-splenomegaly with a marked leukocytosis and anemia 3 weeks after transplantation of L-8313 cells. The number of GM-CFU and CFU-S per spleen increased to more than 40 times normal. The results of chromosomal and PGK analysis demonstrated that these increased stem cells were of host origin. Both the culture medium of the spleen cells and the serum from L-8313 bearing mice showed high levels of IL-3, BPA and CSF. Consequently, hematopoietic cells of the host mice underwent remarkable proliferation in response to these stimulating factors when L-8313 cells were transplanted. We also have been successful in establishing an in-vitro cell line and have maintained it for over one year. The phenotype of L-8313 cells was Thy 1.2 positive. Some L-8313 cells showed a positive acid phosphatase reaction but the cytochemical character of myeloid lineage was not observed. Therefore, L-8313 is considered to be a T-cell derived hematopoietic regulatory cell neoplasm with the ability to produce several hematopoietic stimulating factors.
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PMID:Myeloproliferative disorder due to abnormal production of hematopoietic stimulators. 288 2

A 16 year review of 391 splenectomies performed at New England Medical Center was done to evaluate the morbidity and mortality of patients with drained splenic weights greater than 1,000 grams. Thirty-six met the criteria for study. Twenty men and 16 women with an average age of 55.4 years were identified. Myeloproliferative disorders were the most predominant cause of massive splenomegaly. Pancytopenia and hemolytic complications of the disease processes were the most acute indications for operations. The average time between diagnosis and operative intervention was 42 months. An average of 10 units of blood products were required to correct preoperative coagulopathy. Eleven of 36 patients had postoperative complications. Eight of 21 with drains and an equal number of patients with preliminary splenic arterial ligation had complications. Eight-one per cent of all complications were infection related. Complication increased the length of stay 11 days. The 30 day mortality rate was 11.1 per cent. Sepsis was the major cause of mortality. Closed drainage system provided no demonstrable benefit nor appeared to be the cause of sepsis. No episodes of pulmonary embolic phenomenon or peripheral venous thrombosis were demonstrated. Elective splenectomy in patients with smaller spleens was performed without operative mortality and with 3 per cent morbidity rate. For patients with massive splenomegaly, the average survival time was 28.5 months. The majority of these patients died from complications of the disease. Preoperative coagulopathy, failure to demonstrate a hematologic response to splenectomy and reoperation were clear predictors for decreased long term survival periods.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Massive splenomegaly. 291 90

By means of a scintigraphic method for estimation of spleen size, the relationship was studied between spleen size and anaemia, total blood volume and red cell volume in patients with myeloproliferative disorders. There was significant correlation between spleen size and Hb, RBC and PCV in myelofibrosis. The size of the enlarged spleen did not, however, correlate with the degree of anaemia in the leukaemias. There was positive correlation between spleen size and red cell volume in these conditions. It was not possible to evaluate the severity of polycythaemia from spleen size and the degree of enlargement of the spleen does not appear to be a reliable parameter for staging and deciding on treatment in patients with PPP.
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PMID:The splenomegaly of myeloproliferative disorders: effects on blood volume and red blood count. 292 87

This report deals with myeloproliferative disorders associated with chronic, persistent Epstein-Barr virus (EBV) infection and with monosomy 7 and aberrations concerning chromosomes 3 and 5. Altogether five children were affected, their age ranging from 1 to 4 years at time of clinical diagnosis. Principal symptoms were: hepatomegaly, splenomegaly, recurring upper respiratory tract infection and anaemia. The serum IgG level remained persistently increased. Anti EBV antibody concentrations were measured over a period of 9 months to 6 years, demonstrating persistently increased concentrations of IgG antibodies to viral capsid antigen (VCA) and against early antigen (EA). In three patients IgA antibodies were also studied and were found to be elevated. Within 2-5 years two children developed chronic myelomonocytic leukaemia from the chronic myeloproliferative syndrome. A third patient who initially was diagnosed as chronic myelomonocytic leukaemia developed acute leukaemia within a period of 12 months. A fourth patient with myeloproliferative syndrome went into spontaneous remission after an observation period of 2 years. A fifth patient, the only one with translocation t(3;5)(q27;q33), displayed symptoms and a clinical course that can best be characterized as juvenile chronic myelocytic leukaemia. The clinical, haematological, serological and cytogenetic findings may be related.
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PMID:Persistent Epstein-Barr virus infection associated with monosomy 7 or chromosome 3 abnormality in childhood myeloproliferative disorders. 298 97

A novel murine retrovirus complex was derived from the in vivo passage of a molecularly cloned Friend ecotropic helper virus. The virus isolate, myeloproliferative leukemia virus (MPLV), causes an acute (2-3 weeks) and generalized myeloproliferative disorder in adult mice. All strains of mice examined, including the C57BL/6J strain, developed the acute syndrome. This syndrome is characterized by a rapid hepatosplenomegaly, no thymus or lymph node involvement, granulocytosis, thrombocytosis, and erythroblastosis leading to polycythemia. The most prominent feature at the terminal phase of the disease is a granulocytic hyperplasia. The MPLV isolate replicates in vitro on NIH 3T3 fibroblasts but does not induce foci of transformed cells. Thus, MPLV exhibits unique biological properties that distinguish it either from the Friend virus complexes or from acutely transforming sarcomatogenic murine retrovirus which also induced a rapid splenomegaly.
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PMID:MPLV: a retrovirus complex inducing an acute myeloproliferative leukemic disorder in adult mice. 300 28

The distribution of ABO and Rhesus (D) blood groups was studied retrospectively in 40 patients with primary myelofibrosis (PMF). Only patients with a leukoerythroblastic peripheral blood, splenomegaly and marrow fibrosis in whom chronic myeloid leukemia and secondary myelofibrosis was absent were included in the study. In 14 patients (35%), PMF was preceded by another myeloproliferative disorder (polycythemia rubra vera, essential thrombocythemia or unclassified myeloproliferative disorder), while 26 patients (65%) represented agnogenic myeloid metaplasia (AMM). Comparison with Hospital and Irish blood group distribution showed a significant increase in blood group B (p less than 0.01) in PMF. This increase remained statistically significant for both the AMM and the non-AMM subgroup of PMF when each subgroup was considered separately. This finding supports previous suggestions that the various myeloproliferative disorders which proceed to myelofibrosis are a closely related group rather than a heterogeneous collection of diseases.
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PMID:Excess of blood group B in primary myelofibrosis. 311 Oct 93

The investigation of elderly patients presenting with raised PCV values has been described. Suitable clinical and laboratory investigation enables the separation of those with a raised red cell mass (RCM) into three groups: primary proliferative polycythaemia (PPP), secondary polycythaemia and idiopathic erythrocytosis. Those patients with a raised PCV but normal RCM either have apparent polycythaemia (normal plasma volume) or relative polycythaemia (low plasma volume). PPP is a clonal disorder with a peak incidence in the elderly. It commonly presents with vascular occlusive symptoms/signs involving larger vessels, both arterial and venous. The microvasculature may also be involved, particularly when there is associated thrombocythaemia. Effective treatment is required to minimize the future vascular occlusive incidence and diminish the complication rate of surgery if it is ever required. Both the PCV and the platelet count, if elevated, should be adequately controlled. 32P is probably the simplest treatment and is very effective, but venesection and intermittent low-dose busulphan is equally satisfactory in the co-operative patient with good peripheral veins. Secondary polycythaemia may arise from a variety of causes, particularly from arterial hypoxaemia and renal lesions. Occasionally, more than one pathology is identified in the elderly patient. Lung disease is the most common cause of hypoxaemia. Venesection may be indicated in those patients with excessively raised PCV values. The term idiopathic erythrocytosis should only be used for patients who have been adequately investigated. These patients most commonly present with ischaemic or vascular occlusive symptoms/signs. Relative polycythaemia may be caused by fluid loss, but generally the origin of the low plasma volume is not established. Apparent polycythaemia may represent a physiological variant or a stage before the development of a definitely raised RCM. The management of idiopathic erythrocytosis, and relative and apparent polycythaemia, should initially involved removal of known risk factors if present (e.g. hypertension) with the addition of venesection in selected patients. Reactive thrombocytosis in the elderly is most commonly due to malignant disease of chronic infection. The high platelet count is usually asymptomatic, and antiplatelet therapy is rarely required. Primary thrombocythaemia (PT) is a clonal myeloproliferative disorder similar to PPP. The finding of splenomegaly, abnormal platelet morphology or function helps to separate PT from reactive thrombosis. PT most commonly presents with digital or transient cerebral ischaemia or haemorrhage.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Polycythaemia and thrombocythaemia in the elderly. 332 42


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