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 xenogeneic graft-versus-host reaction model is described, evoked in neonatal mice by injection of rat spleen cells, and registered as splenomegaly at day 7. The following arguments are given as support for the idea that the reaction is indeed a graft-versus-host reaction: only living rat cells can give the reaction; rat cells treated with antilymphocyte serum cannot do so. The reaction is of almost the same strength in mice which have thymus dysplasia and are probably incapable of mounting a host-versus-graft reaction. Pretreatment of the grafted cells with mitomycin C, which abolishes the cells' capacity for DNA synthesis and proliferation, also eliminates their capacity to cause splenomegaly. This is probably because the grafted cell clone, reactive to mouse antigens, is small and has to be expended in order to be effective. Dividing rat cells have been demonstrated by chromosome studies in the enlarged mouse spleens 2--6 days after grafting.
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PMID:Studies on a systemic xenogeneic graft-versus-host reaction model in newborn mice. 1 Jun 44

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

Myelodysplasia is an increasingly recognized complication of polycythemia vera (PCV) which often precedes leukemic transformation. This paper describes two patients with aggressive chronic myelomonocytic leukemia, previously undescribed as a complication of PCV. Both patients presented with rapidly increasing splenomegaly which was resistant to treatment with hydroxyurea and external beam irradiation. Splenectomy precipitated fatal hepatic failure in one patient. The other died shortly after transformation to acute myelomonocytic leukemia (FAB M4 classification). Pathology of the bone marrow, spleen, and liver was remarkable for extensive infiltration by dysplastic myeloid elements. Survival was short, only 4-6 months from diagnosis. The unique characteristics in these patients were: (i) prior history of PCV; (ii) rapidly increasing splenomegaly resistant to standard therapy; (iii) absence of overt marrow fibrosis; (iv) hypercellularity (greater than or equal to 90% cellular) of the bone marrow with dysplasia in the myeloid, erythroid, and megakaryocytic cell lines; (v) peripheral monocytosis greater than 1 x 10(9); and (vi) extensive infiltration of the spleen and liver by dysplastic myeloid cells. In addition, the patient who subsequently developed acute leukemia had been treated with hydroxyurea under the PVSG-08 protocol, providing further evidence of the potential leukemogenic effects of this agent.
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PMID:Chronic myelomonocytic leukemia transformation in polycythemia vera. 207 46

We have treated three cases of splenic artery aneurysms recently, so we reported them with a review of 181 cases in Japan. All three cases are women and have characteristic clinical courses and pathogenesis in each to which we performed a reasonable operation so that they could be saved. First one is a ruptured case. So we performed ligation of the splenic artery from inside the aneurysm under 9 min.'s clamp of the aorta. Second one was pointed out the splenic artery aneurysm during admission for cholecystectomy, so we performed splenectomy and aneurysmectomy, and after that we reconstructed the splenic artery with end-to-end anastomosis. Third one suffered from splenomegaly and portal hypertension. We performed splenectomy with the aneurysm. From the clinical and pathological findings, we concluded that an aneurysm in our first case was associated with arterial dysplasia, second with hemodynamic changes in parous women, and third with portal hypertension. Aneurysms of the splenic artery have been rarely reported until recently, when developments in diagnostic procedures made their discovery easier. We have diagnosed splenic artery aneurysm in three patients preoperatively. The clinical symptoms and operative procedure was different in each case, and are reported here. We demonstrated the summary of our three cases of that at Table 1.
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PMID:Clinical courses and treatment of splenic artery aneurysms--report of 3 cases and review of literatures in Japan. 207 73

We report on 12 patients who fulfilled the FAB diagnosis criteria of chronic myelomonocytic leukemia (CMML). Some clinical findings (splenomegaly) as well as biological parameters (WBC count, cellularity of bone marrow sections, presence of dyserythropoiesis in bone marrow smears) were subjected to particular analysis. This allowed the assignment of each patient onto one of three separate hematological syndromes: (1) RA or RAEB with monocytosis, (2) myelomonocytic dysplasia (a novel syndrome) and (3) true CMML. Such separation may have distinct prognostic significance and may, therefore, predict life expectancy.
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PMID:FAB classification of chronic myelomonocytic leukemia: heterogeneity revealed on the basis of bone marrow findings. 321 30

We reviewed a series of 109 reported Japanese cases of "plasma cell dyscrasia with polyneuropathy and endocrine disorder." This syndrome shows 1) polyneuropathy with increased protein level in the cerebrospinal fluid and sometimes papilledema, 2) endocrinological symptoms, including skin pigmentation, sclerosis, hypertrichosis, gynecomastia, impotence, amenorrhea, decreased glucose tolerance, edema, pleural effusion and ascites, 3) hepatomegaly, splenomegaly and lymphadenopathy, 4) polycythemia, leukocytosis and thrombocytosis, 5) osteosclerotic changes and 6) plasma cell dyscrasia. Plasma cell dyscrasia is considered to be the cardinal change in this syndrome. Most of the patients have low levels of IgG lambda or IgA lambda M-protein in the serum and a slightly increased number of plasma cells in the bone marrow. The clinical course is usually chronic. Surgical excision or irradiation of the local lesion and administration of corticosteroids and/or anti-cancer drugs are effective in improving polyneuropathy and other systemic symptoms. This syndrome is apparently more common in Japan than in the United States and European countries. The pathogenesis of the association of a variety of symptoms in this syndrome is still unclear.
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PMID:Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. 631 93

A syndrome which is known as plasma cell dyscrasia with polyneuropathy and various endocrine manifestations or plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, abnormal M protein and skin changes is very interesting because this syndrome has miscellaneous manifestations such as skin hyperpigmentation, hypertrichosis, polyneuropathy, M protein abnormality, plasma cell dyscrasia and endocrine disturbances. Miscellaneous endocrine abnormalities which have not been described so far are reported here. A 47 year old female was admitted with the chief complaints of edema and gait disturbance. Past and family histories were noncontributory. In April 1981, edema appeared in her face and legs. In June she noticed paresthesia in her legs. Edema increased gradually and she had difficulty walking. Her skin became pigmented and hairy. In October she was admitted because of polyneuropathy with increased cerebrospinal fluid protein without pleocytosis. Prednisolone was started. Walking improved slightly, but edema and paresthesia remained unchanged. Prednisolone was stopped at the end of the following March. In May 1982, she was admitted for further evaluation of edema and polyneuropathy. The patient was alert and cooperative. On standing the skin of her legs became cyanotic. There was hypertrichosis on the arms and legs. Her fingers were clubbed. A moderate swelling of the cervical lymph nodes was noted. There was mild hepatomegaly without splenomegaly. All tendon reflexes were lost. Plantar response was flexor. Muscular strength diminished mildly. She complained of paresthesia on the soles. Superficial sensation was normal. Vibratory sense decreased mildly. Cerebellar function and cranial nerves were normal. There was no sphincter disturbance. The examination of urine, stool and peripheral blood was normal.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Marked endocrine abnormalities in polyneuritis with skin hyperpigmentation, hypertrichosis, edema and increased lambda type immunoglobulin A: a case report]. 641 99

A 14-yr-old white male with a severe form of progressive diaphyseal dysplasia (Engelmann-Camurati disease) was referred to our institution for evaluation of splenomegaly, which is not usually associated with the disease. Our studies included bone-marrow, and liver-spleen scans. These scintigraphic findings, along with the probable cause for splenomegaly, are discussed.
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PMID:Scintigraphic findings in progressive diaphyseal dysplasia. 646 31

Haemopathologic changes were studied in 19 patients (13 male, six female, age 33-85 years, mean 56 years) with relapsing polychondritis (RP). Anaemia was found in eight, thrombocytopenia in two and splenomegaly in three patients. A total of 17 bone marrow biopsies were obtained from seven individuals. Bone marrow evaluation revealed myelodysplastic syndromes (MDS) with marked trilineage hyperplasia and dysplasia in three cases. Since an excess of myeloblasts or an increase of CD34 positive progenitor cells was not seen, the disorders were designated as 'refractory anaemia' or with regard to the dysplastic megakaryopoiesis 'MDS, unclassifiable'. Two of the three patients died after 10 and 55 months of follow-up due to infectious complications. In a further patient, bone marrow analysis repeatedly showed an unexplained granulopoietic hyperplasia, which, however, was not dysplastic enough to allow a diagnosis of MDS. The remaining patients had clearly reactive changes. Our findings support the notion that RP is a heterogenous disorder and suggest that RP may at times represent a paraneoplastic phenomenon of an underlying MDS. Since HLA typing revealed a significantly increased frequency of the antigen DR4 (10/17 patients positive = 59%), we hypothesize that immunological imbalances due to the MDS in conjunction with a specific immunogenetic background may play key roles in the pathogenesis of RP in these patients.
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PMID:Bone marrow pathology in relapsing polychondritis: high frequency of myelodysplastic syndromes. 777 18

We report here two cases of a previously undescribed myeloproliferative disorder. Both were young adult males who presented with generalized lymphadenopathy, splenomegaly, leukocytosis, polycythemia, and persistent thrombocytopenia. The leukocyte alkaline phosphatase (LAP) score was low in both cases, and the bone marrow was hypercellular without dysplasia or fibrosis, but lacked the Philadelphia chromosome, BCR gene rearrangement, or other karyotypic abnormalities. The clinical course was indolent in each case. One patient died from an unusual "blast crisis" after 12 years, while the second patient remains in a complete hematologic remission on hydroxyurea and alpha interferon 4 years from diagnosis. Interestingly, changes in therapy in this patient have consistently resulted in precise and concerted fluctuations in his blood counts, with the red and white cells cycling together and the platelets and mean corpuscular volume (MCV) changing concomitantly but in the opposite direction. This unique myeloproliferative disorder is distinguishable from all previously described forms of chronic myeloid leukemia and other myeloproliferative syndromes.
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PMID:A new myeloproliferative syndrome. 786 27


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