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)

Primary (idiopathic) thrombocythaemia is a rare disease in childhood. Haemorrhagic diathesis and splenomegaly are principal findings besides thrombocytosis. The cases of a nine year old girl with thrombocyte levels between 2.8 and 4.5 X 10(6) microliter is described. The examination in light and electron microscopy revealed various morphological abnormalities in thrombocytes and megakaryocytes. Platelet function was disturbed in various degrees. The impact of functional abnormalities on the haemorrhagic diathesis is discussed. The findings in this child were compared to those of the better characterized picture of primary and secondary thrombozytosis in adulthood. Possible pathogenic relations to myeloproliferative diseases are also discussed.
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PMID:[Morphological and functional studies in a case of primary (idiopathic) thrombocythaemia in childhood (author's transl)]. 92 13

Systemic mastocytosis is a rare disease of mast cell proliferation with cutaneous and multi-visceral involvement. Portal hypertension and ascites are rare manifestations of systemic mastocytosis. We report a case of systemic mastocytosis presenting with extensive nodular cutaneous lesions and hepatic dysfunction, manifested by portal hypertension (ascites, splenomegaly) and derangement of metabolic function (hyperammonemia, hypoalbuminemia, hypocholesterolemia), a picture resembling that of a common cirrhotic form. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. On the basis of our and other observations we suggest that systemic mastocytosis be added to the list of infiltrative diseases of the liver with potential evolution to portal hypertension and compromise of biochemical functions.
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PMID:[Systemic mastocytosis with portal hypertension and hepatocellular failure]. 143 8

A male patient, aged 78, was found with chronic neutrophilic leukemia (CNL). The patient showed sustained mature neutrophilic leukocytosis, splenomegaly, a high leukocyte alkaline phosphatase score, elevated serum vitamin B12 and uric acid, myeloid hyperplasia and absence of ph' chromosome in the bone marrow, with no evidence suggesting this condition to be a leukemoid reaction to an underlying disease. In addition to the above mentioned features, some functional characteristics of CNL cells were compared with normal cells. CNL is a very rare disease; some thought it as a variant of chronic myelogenous leukemia (CML). In this report a review of the literature is also included.
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PMID:Chronic neutrophilic leukemia--report of a case and review of the literature. 217 62

Clinical, hematologic, and histologic material from five patients with chronic monocytic leukemia is analyzed, and the literature is reviewed on 28 patients either reported as having this disease or referenced as such in subsequent publications. Patients with chronic monocytic leukemia have a characteristic clinical course. All have splenomegaly of uncertain etiology, and initial hematologic evaluation usually shows anemia with normal leukocyte and differential counts. One of the authors' patients had slight monocytosis at presentation. In the four patients for whom presplenectomy bone marrow smears were available for review, there was no detectable increase in the number of monocytes. The patients developed monocytosis, usually with accompanying leukocytosis, at intervals ranging from 3.5 to 24 months after splenectomy. In all patients, there was a corresponding increase in the numbers of mature monocytes in the bone marrow. Survival times from the detection of monocytosis ranged from five to eight months. Of the 28 cases reported in the literature, only two were considered by the authors of this study to have the characteristics of chronic monocytic leukemia, and of these, only one had features identical to those reported in the present series. The remainder represent a variety of hematologic disorders including chronic myelomonocytic leukemia, acute leukemia, histiomonocytic proliferations that cannot be sub-classified, and cases in which the data are insufficient for analysis. The differential diagnosis includes chronic myelomonocytic leukemia, chronic malignant histiocytosis, and hairy cell leukemia. In chronic monocytic leukemia, in contrast to hairy cell leukemia, splenectomy does not appear to be beneficial. Although chronic monocytic leukemia is an extremely rare disease, it is a distinct hematologic entity that appears to have a characteristic clinical course.
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PMID:Chronic monocytic leukemia in adults. 694 97

Solitary splenic abscess is a rare disease and usually treated with splenectomy. A 78-year-old female with diabetes mellitus and liver cirrhosis was admitted to our hospital in a state of fever and left hypochondralgia. The abdominal ultrasonogram and CT scan showed the splenomegaly and one abnormal mass in the spleen. Drainage under sonographic guidance was attempted to the splenic mass but only a little amount of aspiration fluid was obtained, from which Salmonella Enteritidis was detected. No other abscess in the other organs was detected, resulting in the diagnosis as solitary splenic abscess caused by S. Enteritidis. Antibiotic therapy was carried out and the abscess disappeared judging from the ultrasonogram, CT scan, and laboratory data. The present case suggests that the fully application of ultrasonogram, CT scan, and drainage for the diagnosis leads to the healing of solitary splenic abscess without operation. This is the first case of S. Enteritidis infection in the spleen reported in Japan.
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PMID:[A case of solitary splenic abscess caused by Salmonella enteritidis and cured completely by antibiotic therapy alone]. 831 5

A 59-year-old man was admitted to our hospital on May 17, 1991 because of dizziness and a sense of abdominal fullness. Physical examination on admission showed splenomegaly without hepatomegaly or lymphadenopathy, and blood examination revealed normocytic anemia, thrombocytopenia and marked leukocytosis of 16,800/microliters with 87% lymphoid cells. Prolymphocytoid cells formed 28% of the lymphoid cells. Bone marrow aspiration revealed massive infiltration of lymphoid cells. Surface marker analysis showed that the lymphoid cells were positive for anti-HLA-DR, CD 5, CD19, CD20, CD21, SmIgM and SmIgD. The patient was diagnosed as having B-CLL/PL, according to the classification advocated by Melo in 1986, and initially treated with vindesine + prednisolone + pirarubicin (VP-THP). However, the prolymphocyte count increased, so we changed to VP-THP + cyclophosphamide (VEP-THP), and remission was obtained. CLL/PL is a rare disease in Japan but we obtained a good response to chemotherapy.
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PMID:[A case of B-chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL)]. 842 81

One hundred and two cases of chronic lymphocytic leukaemia (CLL) were seen at the Tikur Anbessa (Black Lion) Hospital, in Addis Ababa, Ethiopia, from January 1982 to December 1994. The age range was 35-91 (mean 55.6 +/- 11.08) years. The male to female ratio was 3.6:1. The commonest symptoms were weakness, weight loss, fever and sweating. The commonest signs were lymphadenopathy, splenomegaly and hepatomegaly. Fifty six per cent had Rai stage III and IV, only three patients were in stage 0. Of those treated with chemotherapy, 22.0% and 48.8% achieved complete and partial remissions respectively. Twelve patients are still alive and on follow up for 2-138 (median 18) months, 69 are lost to follow up after 0-132 (median 3) months and 21 are dead 0.5-84 (median 2.8) months after diagnosis. Of those that died, 13 were in stage IV and five in stage III. The main causes of death were septicaemia of undetermined origin in eight and pneumonia in seven. Thus CLL is not a rare disease in this centre. Its presentations are similar to cases reported in the literature. Optimal treatment is not possible due to lack of chemotherapeutic agents and supportive care. Therefore, we suggest that referral centres be equipped for better management of CLL patients.
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PMID:Chronic lymphocytic leukaemia in Ethiopians. 899 43

Noncirrhotic portal vein thrombosis (PVT) is a rare disease that usually presents with small nonfatal "herald bleeding" with low mortality. Classic findings of noncirrhotic PVT include esophageal varices, splenomegaly, a normal liver, and an organized thrombus of the portal vein. We present a case of previously unreported sudden unexpected death from noncirrhotic PVT in an asymptomatic elderly woman, review the literature of this entity, and examine the pathophysiology of the formation of fatally ruptured varices. The portal vein must be carefully examined in all cases where there is no coexisting cirrhosis.
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PMID:Noncirrhotic portal vein thrombosis causing sudden unexpected death. A rare cause of fatally ruptured esophageal varices. 918 42

Virus-associated hemophagocytic syndrome (VAHS) is associated with a systemic viral infection and is mainly observed in immunosuppressed adult patients. This rare disease is characterized by symptoms which include a high fever, pancytopenia, and splenomegaly and sometimes results in a fatal outcome. However, thus far, little has been reported on VAHS in general surgical patients. We herein report this rare complication which occurred in a patient with hepatocellular carcinoma, as well as chronic hepatitis C, after a hepatic resection. A 66-year-old man with chronic hepatitis C and recurrent hepatocellular carcinoma underwent a repeat hepatic resection without any blood transfusions. In the early postoperative period, he recovered uneventfully. However, he suddenly began to suffer from a high fever (38.4 degrees C) and severe pancytopenia 8 days after surgery. Activated macrophages, which phagocytosed erythrocytes, were identified by a cytological study of the bone marrow. The patient was therefore treated with methylprednisolone pulse therapy 13 days after surgery. On the day following the initial administration of methylprednisolone, his clinical condition drastically improved. Fortunately, with methylprednisolone therapy, our patient recovered from acute, severe pancytopenia. In general surgery, it is often difficult for surgeons to use steroids due to their negative side effects. However, when symptoms such as fever, general fatigue and pancytopenia are observed, even in posthepatectomy patients with hepatocellular carcinoma and hepatitis, a bone marrow aspiration should be performed as soon as possible, and when VAHS is suspected, steroid pulse therapy should be the first treatment of choice. This rare but sometimes fatal complication should thus be taken into consideration in the postoperative management of hepatectomized patients with chronic hepatitis C.
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PMID:Virus-associated hemophagocytic syndrome after hepatic resection: a case report. 949 16

Adult onset Stills disease (ASD), an adult variant of systemic onset juvenile rheumatoid arthritis, is a rare disease entity. The diagnosis is solely a clinical one and often difficult. Clinical and laboratory features are not pathognomonic. The diagnosis of ASD has to be considered in patients with high spiking fever, transient rash, arthralgias, oligo- or polyarticular arthritis, leukocytosis, sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction and high serum ferritin levels. We give a brief review of the clinical features, differential diagnosis, treatment and prognosis.
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PMID:[Still disease in adults]. 962 78


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