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)

The clinical and hematological profile of 53 patients in whom primary myelofibrosis (PMF) had been diagnosed during the last 15 years was evaluated. Median age was 64 years (range 17-86). Thirty-five patients were males and 18 females. The most frequent symptoms were associated with the hypermetabolic state, anemia and splenomegaly. The latter was found in 96% of patients, while 83% had hepatomegaly and 9% had lymphadenopathy. Thirty-three patients had anemia at the time of diagnosis. The leukocyte and platelet counts were normal or moderately high in most cases. Myelemia was found in 83% of patients, with circulating erythroblasts in 72%. The most common biochemical abnormalities were the increased serum LDH (84%) and hypocholesterolemia (62%). Bone marrow aspirate was not analyzable in most cases. Bone marrow biopsy showed myelofibrosis in hypercellular phase in 22 patients, myelofibrosis without osteosclerosis in 17, and myelofibrosis with osteosclerosis in 14. The median survival of the series was 3.8 years; 34 patients had died at the time of the analysis. The major causes of death were infection, cardiovascular complications and hemorrhage. In 4 patients the evolution from PMF to acute leukemia was observed.
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PMID:[Primary myelofibrosis: description of a series of 53 patients]. 266 65

The lactate dehydrogenase mouse mutant Ldh-1c/Ldh-1c is afflicted with a severe hemolytic anemia associated with extreme reticulocytosis (95%) and splenomegaly. Ninety-one percent of the total body colony-forming units--erythroid (CFU-E) have been quantified in the seven- to ten-times enlarged spleens of the mutant mice. Moreover, the splenic fraction of morphologically recognizable erythroid precursors was 134 times normal. From these data it was apparent that the spleen crucially contributes to the maintenance of steady state erythropoiesis in the mutants. On the other hand, an enhanced sequestration of red blood cells in the enlarged spleen may augment the anemia. Splenectomy experiments were performed with LDH mutant and wild type mice in order to investigate the role of the spleen in this particular hemolytic disease. Following splenectomy, the peripheral blood values and the frequency of femoral stem and progenitor cells were determined, and histological investigations were carried out. The life span of the splenectomized mutants was not shortened, in spite of a very low red blood cell count (25% of the untreated mutant value). Compared to the splenic loss only a moderate increase in bone marrow erythropoiesis was observed, such as a 250% increase of CFU-E. It is concluded that the reduction in red blood cell survival due to splenic sequestration in the mutants is of such a magnitude that it counterbalances a significant portion of splenic erythropoiesis.
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PMID:The role of the spleen in a lactate dehydrogenase mutant mouse (Ldh-1c/Ldh-1c) with hemolytic anemia. 336 65

Neurological involvement in acute myeloid leukaemia has become more common in recent years. The increase seems to be related to the longer survival rates made possible by more intensive treatment protocols. The predictive elements appear to be the tumour mass, cytomorphological variety M5, splenomegaly and serum LDH. Prophylaxis with craniospinal radiotherapy or spinal chemotherapy does not modify the course of the leukaemia or diminish the frequency of neuromeningeal complications. Six cases of AML involving the central nervous system were examined, two at onset and 4 at first relapse. Neuromeningeal complications are to be feared since they are extremely difficult to eradicate completely and the prognosis is extremely unfavourable especially if a bone marrow relapse occurs simultaneously.
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PMID:[Neurological complications of acute myeloid leukemia in adults]. 345 91

Interferon-beta (GKT-beta) was administered to a patient with adult T-cell leukemia (ATL), presumably of acute type, with a marked organ infiltration and increases in serum calcium, LDH and bilirubin. The therapy produced a fall in the number of peripheral blood abnormal lymphocytes, disappearance of jaundice, reduction in the degree of hepatomegaly and splenomegaly and a remarkable improvement of general condition with a performance status of grade 3 improving to grade 1. The response has been maintained for more than seven months. Dose, schedule and criteria for drug discontinuation are matters for further investigation.
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PMID:[A case of adult T-cell leukemia responding to recombinant interferon-beta (GKT-beta)]. 348 33

A 66-year-old man was admitted for shortness of breath and showed fever, abdominal fullness and paraplegia. Monocytosis amounting to 25% and an elevation of serum LDH (4,281 mIu), were remarkable in the laboratory findings. He died of pulmonary insufficiency about a month after admission. On autopsy hepatomegaly (1950 g), splenomegaly (780 g), but no lymphadenopathy and small infarction in the thoracic spinal cord causing paraplegia was noted. Histopathologically, the invasion of the tumor cells into the liver, spleen, lymph nodes, bone marrow and other organs was observed. Malignant histiocytosis was diagnosed by histologic and immunohistochemical studies (lysozyme positive, S-100 protein negative).
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PMID:[A case of malignant histiocytosis with paraplegia]. 362 41

Inspite of its non-inclusion in the prescribed list of food colors, orange II is extensively employed to color a variety of foodstuffs. Oral LD50 value of orange II in both male and female rats was calculated to be more than 10.56 g/kg body weight. In short-term studies, animals were exposed to diets containing 0.0 (control), 0.1, 0.5 or 3.0% (w/w) of orange II, daily for 90 days. Hematological examination revealed a slight decrease in erythrocyte count and hemoglobin content, whereas leucocyte count, PCV, ESR, MCV, MCH and MCHC showed normal values. There was no change in the activities of LDH, GOT, GPT, alkaline/acid phosphatases and bioconstituents, lactic acid, cholesterol and protein in serum as well as in liver, indicating normal functioning of the liver. Histopathological examination of various body organs such as liver, heart, lung, kidney, testes, adrenal, stomach, large and small intestine presented normal appearance. Animals receiving 3.0% orange II showed marked splenomegaly and deposition of Perl's positive iron pigments. Testicular LDH, hyaluronidase and lactic acid did not reveal any deviation from controls, suggesting normal spermatogenic process. No changes in testicular cholesterol, fructose content of coagulating glands and dorso-lateral prostate, and activities of alkaline phosphatase in seminal vesicle and acid phosphatase in ventral prostate support normal androgenic status.
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PMID:Acute and short-term toxicity studies on orange II. 362 8

There have been many reports of cases in which chronic increases in the numbers of natural killer (NK) cells have been reported. Whether this is reactive or neoplastic in nature has been debated. We report the first case of an aggressive NK cell leukemia in an adult with establishment of an NK cell line. A 70-year-old man had two spontaneous episodes of jejunal perforation and one month later developed a severe febrile illness with moderate splenomegaly. Hemoglobin was 13.1 g/L, and WBC count was 1.8 X 10(9)/L with 2% large granular lymphocytes (LGLs). Platelet count was 143 X 10(9)/L; prothrombin time (PT) and partial thromboplastin time (PTT) were normal. Bone marrow was infiltrated with 25% to 30% LGLs; serum lysozyme was normal. Serum LDH was initially 1,191 U/L and rose to 6,408 (normal 240 to 525 U/L). Ten days later, the WBC count increased to 99.9 X 10(9)/L with 70% LGL cells; the PT and PTT increased, and the platelet count dropped. No bacterial or viral cause of fever was identified. The cells from peripheral blood were LGLs that stained positively for acid phosphatase. All of the LGLs reacted with a monoclonal antibody reactive with NK cells (LEU-11b). Functionally, the patient's peripheral blood mononuclear cells (PBMs) demonstrated 100 times more lytic activity against K562 tumor cell lines than did normal PBMs. The patient's PBMs were propagated in vitro. The cultured cells showed the morphological, cytochemical, immunological, and functional characteristics of NK cells. In addition, partial trisomy involving chromosome 1 q with duplication in regions of q21 through q31 was observed in all metaphases analyzed. The extra chromosome 1q with duplication in regions q21 through q31 was translocated to the p-terminal of chromosome 5. One percent to 5% of normal PBMs comprise NK cells; in most cases, leukemias arise from normal phenotypic counterparts. This case demonstrated that aggressive NK cell leukemia may occur in adults. In addition, the chromosomal abnormalities suggest that this is not a reactive process but a malignancy.
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PMID:Aggressive natural killer cell leukemia in an adult with establishment of an NK cell line. 395 37

Clinical and laboratory findings at the time of diagnosis were correlated with the survival of 242 patients with chronic myelocytic leukemia. Twelve patients with the blastic stage of the disease (blasts greater than or equal to 29%) had a median survival of eight months. Of the nonblastic patients, 28 without the Philadelphia chromosome had a relatively constant mortality averaging 43% per year and a median survival of 13 months, markedly worse than the Ph1-positive group (mortality, 6% in the first year, 17% in the second year, and the 25% per year, with a median survival of 43 months; P less than 0.001). In the latter group of 202 patients, features reflecting the "quantity" of leukemia (leukocyte count, marrow cellularity, and M:E serum B12, different degrees of splenomegaly, presence or absence of symptoms) had weaker or short-term correlations with mortality, while "qualitative" abnormalities (e.g., increased percentage of circulating blast, extramedullary leukemic tumors, major abnormalities of erythropoiesis or platelet production, marked basophilia or eosinophilia) had strong and persistent correlations with mortality. Chromosome abnormalities in addition to the Ph appeared to have a delayed though significant effect on survival. Serum alkaline phosphatase and SGOT levels did not correlate significantly with survival, but major elevations of serum LDH were associated with increased mortality throughout the course of the disease.
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PMID:Prognostic features at diagnosis of chronic myelocytic leukemia. 694 41

We report a 20 year-old woman with hemophagocytic syndrome. In February 1993, she developed high fever, arthralgia, salmon-like pink eruption, leukocytosis and splenomegaly. She was diagnosed as adult Still's disease and successfully treated with intravenous immunoglobulin and oral prednisolone. In September 1993, she was re-admitted to our hospital complaining of general fatigue and low grade fever and treated with oral prednisolone at a daily dose of 15 mg. On October 2, 1993, she suddenly developed high fever and salmon-like pink eruption on her leg followed by the marked increase of serum transaminase and LDH levels (GOT 3,270 IU/l, GPT 1,880 IU/l, LDH 5,480 IU/l) on October 7. Since hepatic failure progressed, we started methylprednisolone pulse therapy and plasmapheresis. However, because of the progression of pancytopenia caused by hemophagocytosis, the treatment with VP-16 was initiated. However, she died of DIC on November 2, 1993. Autopsy revealed submassive necrosis of the hepatocytes with moderate infiltration of histiocytes. She was retrospectively diagnosed as hemophagocytic syndrome whose manifestations are very similar to those in adult Still's disease and acute viral hepatitis.
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PMID:[A case of hemophagocytic syndrome manifesting adult Still's disease and acute hepatitis]. 755 62

A 66-year-old male experienced the sudden onset of amnesia and generalized convulsions, and during the subsequent month developed consciousness disturbance, disorientation, memory disturbance, abnormal speech and behavior, pseudobulbar palsy, unsteadiness and urinary/fecal incontinence. The initial brain CT scan was unremarkable, and the MRI findings were also unremarkable, except for evidence of several scattered lacunar strokes in the cerebral white matter. 99mTc-HM-PAO-SPECT, however, revealed multifocal large hypoperfused lesions in the cerebrum. The erythrocyte sedimentation rate and serum CRP, LDH and gamma-globulin levels were elevated. CSF studies showed mild pleocytosis and increased protein levels. The patient's subsequent course was marked by fever, SIADH, adrenal enlargement, splenomegaly, pulmonary infiltration and pancytopenia. The neurological signs progressively worsened, until the patient lapsed into an apallic state and died 3 months after the onset of symptoms. Autopsy disclosed diffuse intravascular B lymphomatous proliferation in the brain, lungs, kidneys, adrenals, spleen and pancreas. Until now, the brain SPECT findings of IL were not very well known, but in our patient, SPECT was capable of demonstrating the pathophysiologic changes very precisely. SPECT was able to provide valuable information on the pathophysiology of IL and may therefore become another powerful tool in the diagnosis of IL.
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PMID:[A case of intravascular lymphomatosis (IL) with diffuse cerebral hypoperfusion detected by SPECT]. 766 6


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