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)

Hairy cell leukemia is a chronic but steadily progressive malignancy usually of older males. Clinically, patients present with splenomegaly and/or cytopenia. The diagnosis is made by demonstration of the hairy cell in Wright's-stained peripheral blood and in bone marrow and by the characteristic histologic findings in the bone marrow biopsy and spleen sections. Infection is the most significant problem complicating the course of patients with hairy cell leukemia and the role of granulocytopenia and/or monocytopenia is undoubtedly substantial. Splenectomy has produced an objective response in improving hematologic parameters in a large number of patients and may prolong survival in those patients who respond. The precise nature of hairy cells remains unknown. The cells exhibit features of both monocytes and B-lymphocytes in morphologic, cytochemical, immunologic and functional studies. A more complete understanding of the monocytic and lymphocytic stem cells and their maturation may provide insight into the origin of the hairy cell.
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PMID:Hairy cell leukemia: a review. 8 94

Clinical data on 24 civilian patients hospitalized for malaria in The New York Hospital were analyzed. Of 16 patients infected with Plasmodium falciparum, 14 acquired the disease in West Africa. Only three of the 24 had taken recommended courses of prophylaxis. Diagnosis was invariably, and often dangerously, delayed because physicians often made diagnoses of viral syndromes or used antibiotics; only one patient had a blood smear taken by a personal physician. Although all patients had fever and chills, classic malarial fever was seen in only seven patients; nausea, vomiting and diarrhea were common. Hepatomegaly and splenomegaly occurred in about half the patients. Blood smears stained in routine fashion by Wright's stain were positive in 23 of 24 patients. A normal leukocyte count was present in 19 of the 24 patients and thrombocytopenia in 16 of 23. The most frequent complications were those of central nervous system involvement. Therapy consisted mainly of chloroquine phosphate but other drugs, including quinine, pyrimethamine, sulfonamides and primaquine, were used in special situations. Suggestions for prophylaxis, diagnosis and therapy were made.
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PMID:Malaria - the mime. Recent lessons from a group of civilian travellers. 78 38

Felty's syndrome (FS) refers to the occurrence of rheumatoid arthritis, splenomegaly, and neutropenia. A subset of these patients has recently been described with a chronic T cell leukemia of large granular lymphocytes (LGCL). To examine the spectrum of lymphocyte abnormalities in FS and LGCL, we examined phenotypic and genotypic properties of lymphocytes from eight FS patients. In two of these FS patients, we observed an elevated proportion of T cells with an unusual phenotype (CD3+/Leu-7+/Leu-8-/CR3+) (46 +/- 5% of mononuclear cells). The FS lymphocytes had large granular morphology on Wright-Giemsa stain and were active in antibody-dependent cellular cytotoxic activity. This phenotype, morphology, and activity was similar to LGCL patients except that the latter T cells additionally expressed the Fc-IgG receptor recognized by monoclonal antibody Leu-11 (CD 15). In the remaining six FS patients, the proportion of CD3+/Leu-7+/CR 3+ T cells was only 10 +/- 8%, which was not significantly different from age-matched normal subjects (6.6 +/- 2.2%). To determine the clonality of T lymphocytes in FS and LGCL, we examined DNA for rearrangements of the T cell antigen receptor beta-chain (Ti beta) and gamma-chain (Ti gamma) genes by using Southern blotting techniques. We found a clonal rearrangement of the Ti beta 1 and Ti gamma genes in both LGCL patients. In contrast, no clonal rearrangements of Ti beta or Ti gamma genes were detected in lymphocytes from the FS patients. These results indicate that FS patients are heterogeneous in their phenotype and that one subset exhibits polyclonal expansion of an unusual lymphocyte subset.
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PMID:Comparison of T cell receptor gene rearrangements in patients with large granular T cell leukemia and Felty's syndrome. 310 95

Two subjects, not previously reported in detail, had severe inherited deficiencies of erythrocyte pyrimidine nucleotidase. This was manifested hematologically by moderate hemolytic anemia with splenomegaly, morphologically by punctate basophilic stippling of Wright's stained erythrocytes, and biochemically by intraerythrocytic accumulation of pyrimidine nucleotides, elevated concentrations of reduced glutathione, and partial deficiencies of ribosephosphate pyrophosphokinase. All 5 of their children were asymptomatic and phenotypically normal except for intermediate reductions in activities of pyrimidine nucleotidase consistent with heterozygosity for an autosomal recessive defect.
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PMID:Additional data from two kindreds with genetically induced deficiencies of erythrocyte pyrimidine nucleotidase. 625 90

A propositus, the offspring of a first-cousin marriage, was presented with severe hemolytic anemia, splenomegaly, jaundice, and growth retardation. Marked basophilic stippling of erythrocytes was shown by Wright's stain. Erythrocyte 5'-nucleotidase activity was found markedly decreased, whereas red blood cell glucose-6-phosphate dehydrogenase activity was elevated as the reduced glutathione level. His growth and anemia improved following splenectomy. His sister was also similarly affected.
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PMID:A case of hemolytic anemia due to erythrocyte pyrimidine 5'-nucleotidase deficiency. 627 Sep 45

We evaluated a 13-month-old boy with cytoplasmic inclusions in hematopoietic cells, transfusion-dependent anemia, splenomegaly, and striking grey skin discoloration. Bright blue inclusions, 1 to 5 microns in diameter, were observed, primarily in the cytoplasm, of 30% to 40% of myeloid cells and in occasional monocytes, megakaryocytes, and lymphocytes on Wright Giemsa-stained bone marrow and blood smears. They occasionally involved the nucleus. The inclusions lacked lysosomes, polysaccharides, or lipids. Ultrastructurally, they lacked limiting membranes and consisted of tightly packed microfilaments averaging 7 nm in diameter, consistent with the size of actin monofilaments. On light microscopy, the inclusions stained with a monoclonal antibody to muscle-specific actin. Inclusion-positive cells contained increased F-actin content and were defective in chemotactic factor-activated actin polymerization; inclusion-negative cells polymerized actin normally. Neutrophil and platelet numbers and functional studies were mildly abnormal. Anemia and skin discoloration resolved spontaneously after 18 months, but the giant inclusions have persisted to the present. We conclude that this child has a previously unreported constellation of clinical and laboratory findings comprising severe anemia, intermittent neutropenia and thrombocytopenia, abnormal neutrophil migration and platelet aggregation, giant inclusions of actin in hematopoietic cells, and grey skin discoloration.
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PMID:Giant actin inclusions in hematopoietic cells associated with transfusion-dependent anemia and grey skin discoloration. 820 94

Splenomegaly is uncommon in myelodysplasia (MDS) and, although cytopenias may be severe, therapeutic splenectomy is rarely performed. We report the histologic, histochemical, and immunophenotypic findings of nine cases of surgical splenectomy and four postmortem spleens from MDS patients. Four histologic patterns were identified: one dominated by erythrophagocytosis, one characterized by red pulp plasmacytosis, one with extramedullary hematopoiesis as the only salient finding, and one with marked red pulp expansion caused by a monocytic proliferation. Wright-Giemsa and histochemical stains were performed on touch preparations in three cases and played a critical role in the precise subclassification of one MDS patient's hematologic disorder, which ultimately proved to be chronic myelomonocytic leukemia. Splenectomy led to sustained improvement of cytopenias in three cases, but did not eliminate transfusion dependence for the remaining patients. Three splenectomy cases exhibited clustered Leder-negative mononuclear elements: two of these patients experienced disease progression to refractory anemia with excess blasts in transformation or acute myelogenous leukemia during post-splenectomy follow-up, whereas none of the three splenectomy patients without clustered mononuclear elements did. We conclude that splenomegaly in MDS usually reflects the sequelae of dyspoiesis rather than evidence of a proliferative phase, that clustering of Leder-negative large cells may correlate with either a substantial monocytic component or, possibly, increased risk of disease progression, and that the spleen can provide diagnostic as well as prognostic information in MDS patients with splenomegaly.
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PMID:Splenic pathology in myelodysplasia: a report of 13 cases with clinical correlation. 977 88

The aim of this study was to evaluate the clinical, laboratory findings and therapeutic features of patients with brucellosis. The diagnosis was made by clinical findings, automated blood culture, serology (Rose Bengal plate agglutination test, standard tube agglutination (Wright) and immunofluorerescence). The susceptibility of 13 strains was tested in vitro. The base sequence was determined for four strains. Forty-five cases were collected (31 acute and 14 sub-acute). Contamination was digestive in 62%. Symptoms of patients were fever (93%), sweating (82%), arthralgia (78%) and splenomegaly (51%). Elevated erythrocyte sedimentation rate was determined in 80%, leukopenia in 49% and anaemia in 37% of cases. Blood cultures were positives in 39% of cases. The four sequenced strains were identified as Brucella melitensis biovar abortus. Six strains were resistant to sufomethoxazol-trimetoprim (54%). In 93% of cases, the treatment was associated rifampicin and doxycyclin. One patient died. No relapse was reported.
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PMID:[Clinical manifestations, complications and treatment of brucellosis: 45-patient study]. 1838 52

A 31-year-old man with no significant medical history presented with a 5-day history of progressive left upper quadrant abdominal pain. Physical examination revealed a tender guarded abdomen, no icterus, and bilateral corneal "arcus senilis"-like changes. Laboratory workup showed a mild normocytic, normochromic anemia; and target cells were seen in the peripheral blood smear. Serum was turbid; and the lipid profile showed elevated total cholesterol, low high-density lipoprotein cholesterol, and elevated triglycerides. Urinalysis revealed nephrotic range proteinuria with microhematuria. An abdominal computed tomographic scan demonstrated a homogeneously enlarged spleen. The patient was discharged after symptomatic treatment to be followed as an ambulatory patient. Several days later, he returned with severe left upper quadrant pain and was admitted to the surgical service for further evaluation. A splenectomy was performed for a suspected splenic lymphoma. Upon gross examination, spleen was moderately enlarged, weighing 780 g. Sectioning revealed a beefy red cut surface without gross lesions. Wright-Giemsa-stained touch imprints showed many sea-blue histiocytes. A renal biopsy was also performed, demonstrating focal segmental glomerular sclerosis and mesangial expansion with extramembranous and intramembranous deposition of lipids. In the absence of hematologic malignancy and in light of the abnormal lipid profile, a disorder of lipid metabolism was suspected. Histologic and ultrastructural findings in the kidney and spleen raised the likelihood of lecithin-cholesterol acyltransferase (LCAT) deficiency, which was confirmed by the markedly decreased serum LCAT activity and serum LCAT mass. We describe a case with the triad of splenomegaly with sea-blue histiocytes, nephropathy, and dyslipidemia in a patient with LCAT deficiency.
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PMID:Splenomegaly with sea-blue histiocytosis, dyslipidemia, and nephropathy in a patient with lecithin-cholesterol acyltransferase deficiency: a clinicopathologic correlation. 1959 52

Brucellosis is considered a known widespread zoonotic disease and is endemic in Mediterranean region, like Iran. This study reviewed the clinical manifestations, laboratory findings, and therapeutic regimen in childhood brucellosis in Iran. In this retrospective study, we reviewed hospital-records of 34 consecutive children with a confirmed diagnosis of brucellosis among a total number of 10,864 patients admitted to Children's Medical Center, Tehran, Iran, between 2002 and 2010. Among the patients diagnosed with brucellosis, 22 (65%) were admitted during spring and summer. Clinical findings of these patients at admission were arthritis, splenomegaly, hepatomegaly, lymphadenopathy, maculopapular skin rashes, and fever. Anaemia (53%) and leukopenia (33%) were the most common findings in the children. Only one patient had presented with leukocytosis. Four children (12%) were thrombocytopenic, and none of patients had pancytopenia. Blood cultures were positive in 5 patients (23%). Only one patient underwent bone-marrow aspiration and had positive culture for Brucella spp. Positive titres were found in 33 cases (97%) in Wright test, 23 cases (96%) in Coombs test, and 16 patients (72.7%) in 2ME (2-Mercaptoethanol) test. In one case, Wright and Coombs test titres were below 1:80 while Brucella spp. were isolated from blood at the same time. It is concluded, prolonged fever with joint involvement and organomegaly may increase possibility of infection with Brucella spp. Appropriate treatment regimen by more tolerable oral drugs, with a duration of at least 8 weeks, is recommended.
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PMID:Clinical manifestations, laboratory findings, and therapeutic regimen in hospitalized children with brucellosis in an Iranian Referral Children Medical Centre. 2393 Mar 40


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