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)

58-year-old man was admitted with cervical tumor and leukocytosis. Physical examination indicated splenomegaly and cervical abscess. Laboratory data showed WBC 55,000/microliters, Hb 7.9g/dl, and PLT 4.5 x 10(4)/microliters. After cure of the abscess, WBC counts were still high with 1,500-2,000/microliters monocytes, and anemia and thrombocytopenia persisted. Bone marrow aspiration showed myeloid hyperplasia and trilineage myelodysplasia. The Ph1 chromosome could not be detected. The case was diagnosed as chronic myelomonocytic leukemia and treated with oral etoposide (25mg/day). After 2 weeks, the dose was increased to 50mg, and then modified according to the blood counts. WBC counts are presently being maintained between 7,000 and 12,000/microliters, and RBC and PLT counts have gradually become normal. Splenomegaly almost disappeared and dysplastic change in bone marrow improved somewhat. At nine months following the start of chemotherapy with etoposide, remission is maintained by treatment with 25mg of etoposide on alternate days. This case suggests that low-dose etoposide is useful for treating CMMoL.
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PMID:[Chronic myelomonocytic leukemia with good response to low-dose etoposide]. 147 4

The French-American-British classification scheme of myelodysplastic syndromes includes a category of refractory cytopenia that includes refractory thrombocytopenia (RTC). Because dysmegakaryopoiesis manifesting as an isolated cytopenia can be difficult to identify morphologically and because it may be accompanied by megakaryocytic hyperplasia, RTC may be confused with idiopathic thrombocytopenic purpura. A review of 1,220 cases of myelodysplastic syndromes at Mayo Clinic Jacksonville and Mayo Clinic Rochester from 1979 to 1990 yielded 9 cases (0.7%) of isolated thrombocytopenia (RTC) associated with clonal chromosome abnormalities. Review of 319 marrow chromosome analyses performed at the cytogenetics laboratory at Mayo Clinic Rochester from 1979 to 1990 for patients with low platelet count yielded two additional cases of RTC (0.6%). Of the 11 RTC cases, 3 previously had been misdiagnosed as idiopathic thrombocytopenic purpura. All patients had oval macrocytes in peripheral blood smears and abnormal megakaryocyte morphology in bone marrow aspirates, lacked antiplatelet antibodies, and did not have splenomegaly on clinical examination. The most common clonal chromosome abnormalities involved chromosomes 3, 5, 8, or 20. Steroid therapy was ineffective. Clinical and laboratory findings can establish the diagnosis of RTC and allow the physician to avoid recommending inappropriate therapy (steroids or splenectomy) for these patients.
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PMID:Refractory thrombocytopenia. A myelodysplastic syndrome that may mimic immune thrombocytopenic purpura. 148

Ten patients with progressive follicular lymphomas (seven with follicular mixed lymphomas, three with follicular, small cleaved cell lymphomas) with clinical indications for systemic therapy received parenteral suramin. Each had failed from one to six prior chemotherapeutic regimens and three had in addition received prior radiation therapy. All had measurable disease and nine of the ten had documented bone marrow involvement at the start of therapy. Suramin was administered at an initial infusion rate of 350 mg/m2/day, which was then modified on the basis of subsequent weekly plasma suramin concentrations in order to reach a final plasma concentrations of 250-350 micrograms/ml. Treatment cycles were repeated at eight week intervals. Nine of ten patients are evaluable for response. Five of nine evaluable patients achieved a partial remission as defined by a greater than 50% decrease in the sum of the product of all measurable lesions. Sites of response include: Peripheral (five patients) and central (four patients) adenopathy, disappearance of biopsy-proven skin involvement (one patient), malignant pleural effusions (one patient) and shrinkage of an enlarged spleen (two patients). Disappearance of B symptoms occurred in the one responder with these symptoms. Response duration varied from 3 to 9 months (mean 5.6 months) with time to subsequent systemic therapy varying from 5 to 12 months (mean 8 months). Drug related toxicity included the development of polyradiculopathy (one case), liver function abnormalities (three cases), thrombocytopenia (five cases), vortex keratopathy (two cases) and bacterial infection (two cases). We conclude that suramin has significant activity against follicular lymphomas refractory to standard chemotherapy and that its precise role in the treatment of lymphoproliferative neoplasms in general warrants further investigation.
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PMID:A pilot study of suramin in the treatment of progressive refractory follicular lymphomas. 149 80

The most common haematological abnormalities in alcoholism are raised mean corpuscular volume of the erythrocytes and thrombocytopenia. The etiology is multifactorial including malnutrition with folate deficiency, a direct toxic influence of alcohol and sequestration in an enlarged spleen. Sideroblastic anaemia caused by interference of alcohol with the metabolism of pyridoxine is common and so is haemolytic anaemia caused by hypersplenism and megaloblastic anaemia. Leucopenia can be seen and is probably caused by a direct toxic effect of alcohol on the bone marrow. Other potentially toxic changes are impaired chemotaxis, motility and adherence of the granulocytes and impaired blast-transformation of the lymphocytes. In the bone marrow, vacuolized precursors of myelo- and erythropoiesis are seen. The bone marrow may be hypocellular. Other changes in the bone marrow are increased but ineffective erythropoiesis with defective iron metabolism, vacuolized pro-erythroblasts, multinucleated erythroblasts, megaloblasts and iron-containing plasma cells.
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PMID:[Hematological abnormalities in alcoholism]. 150 2

One-hundred-and-fifty-seven children admitted with brucellosis at Abha, Saudi Arabia, were studied prospectively. Ninety-two per cent gave a history of animal contact, usually with sheep or goats, or ingesting raw milk, milk products, or raw liver. Three-quarters of the patients had an acute or subacute presentation with diverse symptomatology: fever (100 per cent), malaise (91 per cent), anorexia (68 per cent), cough (20 per cent), abdominal symptoms (20 per cent), arthralgia (25 per cent). Hepatomegaly (31 per cent), splenomegaly (55 per cent), and lymphadenopathy (18 per cent) were common findings. Organ complications were rare except for arthritis (36 per cent) which usually presented as a peripheral oligoarthritis involving the hips and knees. All patients had significant agglutination titres; B. melitensis was grown from the blood in 7 of 16 (44 per cent) patients. Haematological variations were common, but non-specific: anaemia (64 per cent), thrombocytopenia (28 per cent), leucopenia (38 per cent), leucocytosis (12 per cent), and elevated erythrocyte sedimentation rate (81 per cent). Varying combinations of rifampicin, co-trimoxazole, tetracycline, and streptomycin resulted in a prompt pyrexial response (mean: 3.8 days), and a slower response in the arthropathy and hepatosplenomegaly. Relapses were related to poor compliance, use of a single drug or a shorter duration of chemotherapy. Brucellosis is a common childhood problem in southwestern Saudi Arabia as in other parts of the country and the Middle East. It should be considered in every child from an endemic area presenting with a febrile illness and a history of animal contact.
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PMID:Childhood brucellosis in southwestern Saudi Arabia: a 5-year experience. 152 11

The value of routine bone marrow examination (RBME) in children during and after treatment for standard risk acute lymphoblastic leukemia (SR-ALL) was investigated. The clinical symptoms and peripheral blood findings at the time of bone marrow relapse of 28 children were reviewed and compared with those of 28 matched controls in continuous complete remission. Five (45%) children with bone marrow relapse during maintenance therapy and six (35%) after cessation of cytostatic treatment were asymptomatic at the time of relapse. Signs indicative of relapse during treatment were lymphoblast cells in the peripheral blood, thrombocytopenia, hepatomegaly, anemia, or leukopenia in decreasing order of frequency. After cessation of treatment these signs were lymphoblasts in the peripheral blood, hepatomegaly, splenomegaly, thrombocytopenia, or leukocytosis. Except for one case with thrombocytopenia, no signs suspicious for relapse were found in the control groups. When each sign was evaluated separately only the presence of lymphoblasts in peripheral blood and hepatomegaly were significant symptoms for relapse after cessation of treatment. The mean percentage of lymphoblasts in the bone marrow at the time of relapse was significantly lower for patients with an unpredicted relapse (46.8%) than patients with clinical and/or laboratory evidence of relapse (79.5%). When lymphoblasts were present in the peripheral blood the percentage of lymphoblasts in the bone marrow was always more than 40%, both during and after cessation of treatment. These data suggest a relation between clinical and laboratory symptoms and progression of the disease. It is concluded that 46% of relapses are detected by RBME in the absence of clinical or laboratory symptoms. This early detection may have a positive prognostic influence with more effective treatment for relapsed ALL.
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PMID:Value of routine bone marrow examination for detection of bone marrow relapse in children with standard risk acute lymphoblastic leukemia. 155 75

An epidemic of dengue hemorrhagic fever occurred in Delhi during 1988. A total of 21 pediatric patients with that illness or dengue shock syndrome were evaluated from September-November 1988. All patients had fever, restlessness, ecchymotic spots, and ascites. Pleural effusion occurred in 19 patients (90%) and 18 (86%) exhibited each of the following: vomiting, thrombocytopenia, and hemoconcentration. Hepatomegaly was observed in 15 patients (71%) and splenomegaly in 3 (14%). Titers of hemagglutination inhibition (HI) antibodies against dengue virus type 2 were raised in all 15 cases from whom sera were collected during the acute stage. Convalescent sera from 5 patients had increased titers of HI antibodies to dengue virus type 2. The remaining 10 cases exhibited raised IgM antibody levels against dengue virus type 2. The fatality rate for serologically proven cases was 13% (2 of 15 patients) while for all patients (including those diagnosed clinically [n=6] and serologically [n=15]), it was 33.3% (7 of 21). Patients who survived had no sequelae, except 1 who had transient hypertension which continued for 2 weeks.
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PMID:Dengue haemorrhagic fever in children in Delhi. 156 74

We present a six-year follow-up of a boy with a novel type of hypolipoproteinemia, with clinical and biochemical features distinct from classical hypoalphalipoproteinemias. There were abnormally low concentrations of total and high-density lipoprotein (HDL) cholesterol, apolipoprotein (apo) B, apo A-I, and apo A-II, and the phospholipids were decreased. The most striking abnormality was an extra fraction containing mainly phospholipids and apo A-I in the HDL3 subfraction. This fraction is reminiscent of concentric 20- to 50-nm-diameter lamellar phospholipid liposomes. Plasma lecithin:cholesterol acyltransferase activity was strongly decreased. We noted a persisting polyclonal hypergammaglobulinemia, hematological abnormalities (hemolytic anemia and thrombocytopenia), and a progressive splenomegaly. After the five-year follow-up, the patient had recurrent severe infections; moderate hematuria and proteinuria developed gradually. Treatment with corticosteroids and immunoglobulins improved thrombocytopenia and hypolipoproteinemia. These clinical and biochemical findings differ from those in the known primary and secondary hypo-alpha-lipoproteinemia syndromes. Although investigation of the relatives suggests a familial predisposition for hypo-alpha-lipoproteinemia, the subject's condition can be regarded as acquired.
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PMID:Acquired hypolipoproteinemia. 158 35

Felty's syndrome is a variant of rheumatoid arthritis with a triad of leukopenia, splenomegaly and typical rheumatoid arthritis, and often accompanies the other extraarticular symptoms, such as subcutaneous nodules, lymphadenopathy, anemia, thrombocytopenia, leg ulcers, fever and weight loss. In addition to the decreased number of leukocyte, impaired functions of leukocytes were also revealed. These changes were considered as the cause of repeated infections, which was the major cause of death in Felty's syndrome. Splenectomy is not fully effective for leukopenia and for the repeated infection. Glucocorticoid therapy can increase leukocyte count, at least tentatively. It has been reported that gold was effective for leukopenia, repeated infection and skin ulcer. However, the definitive way of treatment of this syndrome based on the pathogenesis of this syndrome, remains to be established.
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PMID:[Felty's syndrome]. 158 59

Autoimmune neutropenia is thought to be an uncommon disorder in adults. Over a 2-year period, however, autoimmune neutropenia was diagnosed in seven adults in a county with a population of approximately 105,000 people. The median age of the patients was 52 years old (range of 22 to 81 years), and five of the seven patients were women. All seven patients had at least one other symptom of autoimmune disease. Three patients had splenomegaly, three patients had positive direct antiglobulin tests, and two patients had immune thrombocytopenia. Antibodies reacting to neutrophils were detected by either granulocyte agglutination (GA) or granulocyte immunofluorescence (GIF) testing in five of the seven patients. Antibodies in four patients reacted with an 80 kd neutrophil membrane glycoprotein, and antibodies from two people reacted with a 60 kd membrane glycoprotein. Three patients were given treatment with splenectomy, which resulted in only transient improvement in the neutrophil counts. Serious infections occurred in only three patients over the 2 years of observation. In summary, autoimmune neutropenia in adults may occur more often than appreciated. Most cases of autoimmune neutropenia in adults appear to be associated with other autoimmune phenomena.
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PMID:Autoimmune neutropenia in Sheboygan County, Wisconsin. 159 17


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