Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinicopathological findings of six cases of Hairy cell leukaemia are presented. All the patients were males, the age ranged between 32-57 years. Complications of anaemia and neutropenia were common modes of presentation. Hepatomegaly and splenomegaly were present in all the cases whereas only 2 patients had lymphadenopathy. Severe pancytopenia was detected in 3 cases and circulating hairy cells were present in all the cases. Trephine biopsy done in all six patients was found to be diagnostic. Tartrate resistant acid phosphatase was detected in the hairy cells of 2 cases.
 ...
PMID:A clinico-pathological study of six cases of hairy cell leukaemia. 179 14

Fever in systemic lupus erythematosus (SLE) may be caused by exacerbation of the disease itself or by infection. We report on a patient with a long standing history of SLE that was complicated by fever and pancytopenia with no splenomegaly. SLE disease activity was suspected because of an elevated DNA-antibody titer. The early positive response to corticoid therapy may have masked the underlying infection. Visceral leishmaniasis was diagnosed by a repeated bone marrow biopsy and serological testing.
 ...
PMID:Visceral leishmaniasis mimicking a flare of systemic lupus erythematosus. 180 3

A 26-year-old male patient with pachydermoperiostosis is reported. He had severe anemia with myelofibrosis. Treatment with iron, prednisolone, oxymethorone and 1 alpha (OH)D3 were not satisfactory. But steroid pulse therapy with parenteral iron improved his anemia and pancytopenia, but was not sufficient to relieve the bone marrow fibrosis or splenomegaly. The mechanism of anemia which was considered to be multifactorial including gastro-intestinal bleeding associated with peptic ulcer or erosion and bone marrow failure due to myelofibrosis, is discussed.
 ...
PMID:Pachydermoperiostosis with myelofibrosis and anemia: report of a case of anemia of multifactorial causes and its improvement with steroid pulse and iron therapy. 186 80

This report describes three cases of acute malignant myelofibrosis characterized by pancytopenia, absence of splenomegaly, bone marrow fibrosis with an immature cell proliferation and rapidly fatal outcome. The authors investigated the origin of blast cells with the use of immunohistochemical markers on paraffin embedded material with anti-factor VIII, BNH9 and anti-lysozyme. They studied the expression of megakaryoblastic, erythroblastic and myeloblastic differentiation in these cells. They demonstrated the heterogeneity of blast cells which are capable of differentiating along the three hematologic cell lines. The morphometric study showed the mutilating or systematized character of myelofibrosis. The increase in reticulin fiber density compared with normal bone marrow was not significantly different from two other types of myelofibrosis. It would be interesting to correlate a quantitative parameter with the course of this disease in order to evaluate the prognosis and the treatment.
 ...
PMID:[Acute malignant myelofibrosis. Report of 3 cases]. 192 69

We retrospectively studied the incidence of familial hemophagocytic lymphohistiocytosis (FHL) in children during the 16-year period 1971-86. First, all departments of pediatrics, pathology, and infectious diseases were enquired for children with FHL or disorders resembling FHL. Secondly, the causes of death of all children who died during the study period in Sweden (n = 19,542) were also investigated. Files and histological specimens were further studied in selected children. By using a set of inclusion/exclusion criteria, we found 32 children with FHL. The incidence was 1.2/1,000,000 children per year. One child per 50,000 live borns developed FHL during this period. The sex ratio was close to 1:1. Prominent early clinical signs were fever (91%), splenomegaly (84%), hepatomegaly (90%), rash (43%), and lymph node enlargement (42%). Neurological symptoms, which developed in 47%, could totally dominate the clinical picture and develop prior to other symptoms and signs. Common laboratory findings were pancytopenia, hypertriglyceridemia, hypofibrinogenemia, elevated serum transaminases, hyperbilirubinemia, hyponatremia, hypoalbuminemia, and a moderate spinal fluid pleocytosis. Chest X-ray often revealed mostly discrete pulmonary infiltrates. FHL is an underdiagnosed disease and in only 11/32 children was diagnosis made during their lifetime. It is important to be aware of the disorder as potential therapy now exists.
 ...
PMID:Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. 205 92

Seven cases of myelodysplastic syndrome with myelofibrosis, which is defined using the following criteria: (1) pancytopenia with less than 5% blasts in the peripheral blood; (2) minimal or no splenomegaly; (3) myelofibrosis with cellular marrow; (4) absence of diffuse proliferation of blasts in the bone marrow; and (5) presence of myelodysplastic features of bone marrow or peripheral blood cells, are presented. They were in the range of 52-82 years old and consisted of 3 males and 4 females. Six out of 7 cases developed into acute leukaemia after 5 to 8 months from the onset and died from between 2 weeks to 8 months from the evolution to leukaemia. The type of leukaemia was acute myeloblastic in 3 patients, and acute myelo-megakaryoblastic in 3 patients. Another patient died of severe hepatic injury after 5 months from the onset of the disease. These findings revealed that the complication of myelofibrosis in the patients with myelodysplastic syndrome was an indicative sign of rapid progression to overt leukaemia or otherwise poor prognosis for survival. In addition myelodysplastic syndrome is thought to be major primary disorder for acute myelofibrosis. Myelodysplastic syndrome with myelofibrosis is closely associated with the neoplastic proliferation of megakaryoblasts in a considerable number of patients.
 ...
PMID:Myelodysplastic syndrome with myelofibrosis: myelodysplastic syndrome as a major primary disorder for acute myelofibrosis. 206 Feb 60

We report a case of a 61-year-old woman with large granular lymphocytosis associated with pulmonary tuberculosis. She was admitted to our hospital because of high fever, anemia and splenomegaly. On admission, the leukocyte count was 6,890/microliters with 52% of large granular lymphocytes. Immunophenotypical analysis of the increased cells showed following results; CD2+, CD3-, CD16+, CD57+. These cells had natural killer (NK) activity. Molecular genetical analysis showed these cells had germline configuration of the T cell receptor beta chain genes. About four months after admission, chest X-P revealed multiple mass shadow and the diagnosis of pulmonary tuberculosis was made by the examination of gastric juice. Anti-tuberculosis therapy was started, and soon after clinical symptom and pancytopenia were improved. For about one year, anti-tuberculosis therapy was continued, and now hematological abnormality is not found. We considered that this case was reactive large granular lymphocytosis of NK cells to lung tuberculosis.
 ...
PMID:[Transient large granular lymphocytosis associated with pulmonary tuberculosis: a case report]. 207 32

Reactive hemophagocytic syndrome (RHS) or hemophagocytic histiocytosis is a disease with anatomo-pathological features of systemic proliferation of non-neoplastic histiocytes, with prominent hemophagocytosis, associated to infection of other diseases. The cases of three patients afflicted with RHS are presented. 2 of them secondary to a brucellosis and the other of unknown origin. The clinical features were similar: high fever, wasting, and splenomegaly. Pancytopenia existed together with liver disfunction, CID and hyperferremia. Marrow infiltration of reactive histiocytes with important hemophagocytic phenomenon, demonstrated by aspirated and bone marrow biopsies, were observed in all cases. Studies of the immunology system were performed, showing changes in two of them. All of them fully recovered after antibiotic treatment.
 ...
PMID:[The reactive hemophagocytic syndrome associated with infection: a study of 3 cases]. 210 68

An 18 years old female was admitted to hospital due to pancytopenia on May 25, 1987 and found to have petechiae, mild hepatomegaly and severe splenomegaly. The bone marrow was found to contain 12% of blast cells. Splenectomy was performed followed by CHOP therapy. In September, 1987 the peripheral blood was found to contain tumor cells, which turned out to be resistant to various combination chemotherapies. The patient died on August 21, 1988. The phenotype of tumor cells in this case was CD2+ CD7+ CD3+ CD4- CD8- WT31-. Genetic analysis detected rearrangement of the beta and gamma chain of TcR but not transcription or translation of the beta chain of TcR, while the antibodies of delta TCS 1 and TcR delta 1 to the delta chain of TcR were positive. From this fact, the present case was considered to be the malignant counterpart of normal CD3+ WT31- double negative T cells. The reactivity of this tumor cells to IL-2 and IL-1 beta suggested the association of the IL-2R beta chain.
 ...
PMID:[T gamma lymphoma with CD3+ CD4- CD8- WT31- and TcR gamma delta]. 213 75

We describe two patients suffering from a prolonged fever, pancytopenia, and splenomegaly. The spleen of both patients was grossly enlarged and the liver was moderately enlarged. The blood pictures were characterized by anemia and leukopenia to a severe degree, with moderate thrombocytopenia and moderate reticulocytosis. The serum transaminases were in the normal range, but the alkaline phosphatase was greatly increased. Splenectomy and wedge liver biopsies were performed on both patients. Histological findings of the spleen were variable. The histopathologic findings of the liver showed infiltration of lymphocytes, plasma cells, monocytes, and neutrophils into the dilated hepatic sinusoids. The sinusoidal infiltrate was more prominent in zones 1 and 2 and less prominent in zone 3 of the acinus. Erythrocyte inclusions that were confirmed by immunohistochemical methods were seen in the cytoplasm of hepatocytes. Electron microscopy revealed a penetration of erythrocytes into the cytoplasm of hepatocytes and caused leakage of cytoplasmic contents into Disse's spaces and sinusoids.
 ...
PMID:Intrahepatocellular erythrocyte inclusions with hepatic sinusoidal infiltrates and splenomegaly. 215 67


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>