Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A trial is presented of evaluation of the frequency of clinical symptoms and signs, activity of disease process, and course of the disease in a group of children with juvenile chronic arthritis with generalized manifestations at onset. The analysed group comprised 36 children aged from 2 to 15 years. The duration of the disease was from 6 months to 13 years. The evaluation of the most frequent symptoms and signs in the so called early period of the disease covered the first year after the beginning of the first clinical signs. During the disease involvement of many internal organs develops and establishing of correct diagnosis at the onset is difficult, particularly, since in this initial period the signs of articular involvement may be absent or slight. Arthritis in this early period was observed in only 7 of 36 children. The most frequent symptoms and signs in decreasing frequency were: fever, rash, arthralgias, lymphadenopathy, hepatosplenomegaly. Heart involvement was noted in 16 of 36 cases, and pneumonia in 7 of 36. Anaemia and leucocytosis were present in all cases. Amyloidosis was recognized in 6 of 36 cases. In view of the severe course of the disease all children were treated with glycocorticoids, and cytostatic agents were given to those with evidence of amyloidosis.
Mater Med Pol
PMID:Considerations on juvenile chronic arthritis with generalized (polysystemic) onset. 248 68

Preliminary clinical course and the results of treatment of the acute T-cell lymphoblastic leukemia in 11 children have been analysed. All children has been treated with aggressive, multidrug chemotherapy with modified BFM scheme 79/81. In the majority (approximately 80%) of cases baseline high leucocytosis, hepatosplenomegaly and/or lymphadenopathy were seen. Mediastinal tumor was present in nearly 50% of cases. The first complete remission was achieved in the majority of children but complete recovery was noted only in 4 cases (36.4%). In the remaining patients therapy has failed due to early recurrence involving bone marrow or CNS. All recurrence developed within the first 18 month of the treatment. Further studies on new therapy regimens in T-cell acute lymphoblastic leukemia in childhood are needed.
Pol Tyg Lek
PMID:[Treatment results of T-cell acute lymphoblastic leukemia therapy in children from 1983-1985]. 780 50

A case of a healthy 23-year-old woman is reported with cytomegalovirus mononucleosis as a result of infection of cytomegalovirus probably primary. The patient presented with symptoms of generalized adenopathy, migratory arthralgias and arthritis, hepatosplenomegaly, long lasting rash as well as complications of pneumonia and myocarditis. Because on histopathological examination of lymph node the Hodgkin-Reed-Sternberg-like cells were found a misdiagnosis of Hodgkin's disease was initially made. After about 8 weeks period there was a complete recovery. The current problems related to cytomegalovirus infection are presented.
Pol Arch Med Wewn 1995 Jul
PMID:[Difficulties in diagnosis of cytomegalovirus mononucleosis syndrome]. 852 1

We present a case of a 17-year old patient with extreme hepatosplenomegaly, hyperthrombocytosis, hyperleucocytosis and the presence of myelo- and megakaryoblasts in the peripheral blood film. Numerous complications that occurred in the course of the disease made cytostatic treatment difficult. Since Ph chromosome and hybrid gene bcr/abl were absent, the diagnosis of unclassified chronic myeloproliferative syndrome in the phase of blast crisis was established. Immunophenotyping confirmed a mixed myelo- megakaryoblastic character of the crisis. In the differential diagnosis other myeloproliferative syndromes were taken into account including i(17q) syndrome. The patient died after a 13-month observation due to neoplasm progression and sepsis.
Acta Haematol Pol 1996
PMID:[Unclassified chronic myeloproliferative Ph(-); i(17q); +8 syndrome with mixed myelo-megakaryoblastic crisis--case report]. 862 49

A newborn with symptoms of congenital cytomegalovirus infection (interuterine dystrophy, thrombocytopenia, leukopenia, hepatosplenomegaly, chronic pneumonia, pleocytosis in CSF) is described in whom tests for specific anti- CMV-IgM antibodies were negative. Changes typical for cytomegaloviral infection were found on autopsy. The authors discuss the difficulties in interpreting various serologic tests (CFT, anti-CMV IgM) in congenital infections, suggesting the necessity of introducing new diagnostic methods for better diagnosis and treatment.
Pediatr Pol 1996 Apr
PMID:[Suppression of immunological response in a newborn with congenital cytomegalovirus infection: diagnostic difficulties]. 897 25

A women, 50 years old, has been observed for 4 years because of recurrent infiltrations in both lungs. Biopsy of those lesions revealed sarcoid-like granulomas. Tubercle baccilli were not present in those lesions nor in the sputum or bronchial washings. Anti-tuberculosis therapy was without effect. On admission to our hospital she was in good performance state. There was an infiltration in the base of the left lung. Hepatosplenomegaly was observed on USG examination. Aspartate aminotransferase was 49 UI/l, alanine aminotransferase 70 UI/l. Alkaline phosphatase was 167 UI/l and the titer of antimitochondrial antibodies was 1:2000. Primary biliary cirrhosis was suspected, but the patient refused liver biopsy. Prednisone in the dose of 60 mg per day was given to suppress the granuloma formation in the lungs. During this treatment there was a decrease in size of liver and spleen, lung lesion disappeared and the titer of antimitochondrial antibodies decreased to 1:40. After 3 months of treatment the dose of prednisone was reduced gradually. When she was receiving 15 mg of prednisone every other day the titer of antimitochondrial antibodies rose to 1:8000 and the activity of alkaline phosphatase to 448 UI/l. At this time she accepted liver biopsy. Primary biliary cirrhosis was diagnosed. The possible connection between sarcoid-like granulomas in the lungs and the primary biliary cirrhosis is discussed.
Pneumonol Alergol Pol 1998
PMID:[Primary biliary cirrhosis with sarcoid-like infiltrations in the lung]. 985 54

We describe 4 cases of non-Hodkin's lymphomas that were interesting because of their curiosal clinical courses and spontaneous complete remissions during the course of high malignancy lymphoma. We present three of them for the first time in Poland. Case 1: a 61-year old woman was admitted to the hospital because of the headache, lasting for 4 months before hospitalization and right hemiparesis. CT scans revealed the presence of tumor in the temporo-occipital region. The diagnosis of B-cell lymphoma was established at histopathological examination of the postoperative material. Co60--therapy of these region was applied after the operation with good response. Case 2: a 38-year woman was admitted to the hospital because of L5-S1 spondylolisthesis to operate it. During the hospitalization haemolytic anaemia of unknown origin, thrombocytopoenia, splenomegaly, fever and rising acute insufficiency of kidneys, heart, liver and CNS were occurred. The patient died, despite applying corticosteroidotherapy. The diagnosis of intravascular lymphoma was established at postmortem examination. Case 3: a 51-year old woman was admitted to the hospital with diagnosis: anaplastic non-Hodgkin lymphoma B-cell type high malignancy established after the double histopathological examination of lymph nodes and biopsy of the lung. At the admission to the Department of Haematology we stated absolute regression of these changes. The patient had been only observed in the Outpatient Department over 1 year. She died after 6 months since the beginning of the relapse despite intensive chemotherapy. Case 4: a 43-year old man was admitted to the hospital because of great hyperleukocytosis, hepatosplenomegaly and neurological symptoms. The diagnosis: chronic prolymphocytic leukaemia was established. The cerebrospinal fluid examination showed presence of mononuclears which infiltrated CNS. CT scans of the brain revealed leucaemic infiltrations of the hemispheres and cerebellum. The patient died despite intensive therapy due to rising progressive multiorgan failure.
Pol Arch Med Wewn
PMID:[Unusual cases of non-Hodgkin's lymphomas--case reports]. 1123 49

Post-transplant lymphoproliferative disorder (PTLD) constitutes a serious complication of allogeneic bone marrow transplantation. We describe a case of PTLD in a twenty-six year-old male treated with bone marrow transplantation for aplastic anemia of unknown cause. The patient received unmanipulated marrow graft from his HLA-matched brother. Fifty-one days post transplant he developed progressive enlargement of cervical lymph nodes, followed by hepatosplenomegaly and generalized lymphadenopathy. Polymorphic PTLD was diagnosed basing on the lymph node histopathology, positive EBV detection, flow cytometry and IgH rearrangement studies proving monoclonality (capillary electrophoresis with ABI PRISM 310 Genetic Analyzer). There was no response to anti-CD20 antibody, cessation of immunosuppression, donor lymphocyte infusion and cytostatic therapy. The patient died on the 65th day of multiple organ failure. We discuss the diagnostics and management of PTLD in the setting of bone marrow transplantation.
Pol J Pathol 2002
PMID:Fatal post-transplant lymphoproliferative disorder following allogeneic bone marrow transplantation for aplastic anemia. 1201 24

Authors present the case of 15-year-old girl with diagnosed pre T-acute lymphoblastic leukaemia with hyperleukocytosis--at admission 213 x 10(9)/l. At clinical evaluation a major peripheral and abdominal lymphadenopathy, mediastinal mass, hepatosplenomegaly were revealed. After administration of prednisone for two times--11.4 mg/m2/24 h--the features of tumour lysis syndrome rapidly increased. Hyperphosphatemia, hyperkalemia and hyperuricemia resulted in acute renal failure. Renal replacement therapy was introduced with simultaneous application of cytoreduction phase therapy and induction of remission according to New York protocol. Total number of performed hemodialysis sessions was 12. Obtained haematological remission in 8th week of treatment is still present. Renal function remains normal. Remission supportive treatment has been continued according to above-mentioned protocol.
Pol Merkur Lekarski 2003 Aug
PMID:[Tumor lysis syndrome in the course of acute lymphoblastic leukemia as the consequence of prednisone monotherapy]. 1464 89

The aim of this article is to present a case report of Gaucher disease which was diagnosed in patient with hepatosplenomegaly. A 43 years old man has complained of weakness, stomach pain and yellowish skin color for several years. The severity of symptoms has increased during the last 2 years. Laboratory data revealed thrombocytopenia (platelet count 108 G/l) and slightly elevated bilirubin level (1.68 mg/dl). In ultrasound examination hepatosplenomegaly was observed. Computer tomography evaluated the size of the spleen to 20 x 12 cm. Gaucher cells were found in bone marrow. The some type of cells was recognized in liver biopsy. To confirm diagnosis of Gaucher disease enzyme test was performed. Chitotriosidase level in serum was pronouncedly increased (11,540 nmol/mg protein/hr) while normal level is under 150 nmol/mg protein/hr. Glucocerebrosidase activity in leucocytes was within the limit. Thus the enzyme activity was 21.8 nmol/mg protein/hr and was below the normal range which is between 111-455 nmol/mg protein/hr. The last assay, measuring glucolukocerebrosidase activity in cultured fibroblast confirmed the diagnosis of Gaucher disease.
Pol Arch Med Wewn 2004 Sep
PMID:[Gaucher disease--one of the possible causes of splenomegaly--case report]. 1572 93


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