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)

Splenic lymphoma with circulating villous lymphocytes is a rare B-lymphoproliferative disorder of the elderly which has been only recently defined. Clinical features are spleen enlargement of various degree without lymphadenopathy and an indolent course, with a long survival, in most cases. Absolute lymphocytosis is present; atypical circulating lymphocytes show a medium or large size, a small prominent nucleolus and a few short and thin cytoplasmic protrusions and projections (villi), which are distributed at one or both poles of cell surface. Reaction for tartrate-resistant acid phosphatase is almost always negative. Immunological markers are as follows: CD 19+, CD 20+, CD 22+, CD 11c+/-, CD 5-, CD 23-, CD 25-, HLA DR+, SmIg+. Differential diagnosis with other chronic lymphoproliferative disorders, particularly chronic lymphocytic leukemia, hairy cell leukemia, prolymphocytic leukemia, follicular and mantle-cell lymphoma in leukemic phase, is based on clinical and immunocytomorphologic criteria. Bone marrow biopsy shows involvement of different degree and pattern; splenic involvement mostly occurs in the white pulp; hepatic nodules in portal areas may be present. Cytogenetic alterations are often present but not specific, such as increased serum LDH and monoclonal gammopathy. No therapy should be made in asymptomatic patients. In case of systemic symptoms, symptomatic splenomegaly or cytopenias, treatment may consist on splenectomy, splenic irradiation or alkylating agents. A case of splenic lymphoma with circulating villous lymphocytes is reported; differential diagnosis, particularly with other B lymphoproliferative disorders, is discussed.
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PMID:[Splenic lymphoma with villous lymphocytes. A clinical case]. 930 75

Splenic lymphoma with circulating villous lymphocytes (SLVL) is a special kind of lymphoproliferative disease characterized by specific clinical, haematological, histomorphological and immunophenotypic features that make it different from hairy cell leukaemia, prolymphocytic leukaemia and non-Hodgkin lymphoma. Four patients suffering from SLVL, 3 men and 1 woman, 62 years in average (range 55-67 years), are presented. All patients had anaemia and splenomegaly. One patient had huge pseudocyst of the spleen, two had mild lymph-adenopathy, while 3 patients had thrombocytopenia. The number of WBC ranged from 13-29 x 10(9)/l with lymphocytes ranging from 62-80%. Monoclonal IgM paraprotein was found in 3 patients. Immunophenotyping showed cells with features of mature B lymphocytes. Splenectomy was carried out in three patients. They all recovered and stayed symptomfree. One patient refused surgery. In our opinion SLVL is not a rare disease as it was thought; it should especially be taken into diagnostic consideration in elder patients, particularly in males with splenomegaly and lymphocytosis.
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PMID:[Splenic lymphoma with circulating villous lymphocytes]. 1797 22

Splenic lymphoma, or primary malignant lymphoma of the spleen (PMLS), is an uncommon condition whose true nature is difficult to define due to the variable ways it has been classified. Out of all non-Hodgkin's lymphomas it comprises less than 2% of cases. Some experts suggest that PMLS only involves the spleen and splenic hilum, while others consider PMLS to be an entity that develops within the spleen and later has the potential for invading adjacent organs and metastasizing. Clinical features of splenic lymphoma are characterized by nonspecific systemic symptoms such as low grade fevers, night sweats and symptoms related to considerable splenomegaly. Most of these lymphomas are of B-cell origin showing low or intermediate-grade lymphoma on histological analysis. The case we present here is of a patient presenting with left sided flank pain, and given a previous history of nephrolithiasis, a presumably simple diagnosis of kidney stones was made. However, further investigation led to the discovery of splenic lymphoma, which was asymptomatic earlier but may have manifested symptoms that mimicked renal colic.
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PMID:Splenic non-Hodgkin's lymphoma presenting as recurrent kidney stones -- an "incidentaloma"? 1954 77

Splenic lymphoma without splenomegaly is uncommon. We report the case of a 68-year-old female who presented with fulminant B symptoms and thrombocytopenia, only to be found to have micronodular large B-cell lymphoma of the spleen post-mortem. Diagnosis of this rare entity remains difficult and overall prognosis is poor.
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PMID:Splenic non-Hodgkin's lymphoma without organomegaly: occult presentation with fatal course and post-mortem diagnosis. 2113 46