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)

The case report of a 52-year-old woman with an eight-year history of cyclic autoimmune hemolytic anemia and progressive splenic enlargement is presented. At laparotomy the enlarged spleen and splenic hilar lymph nodes showed lymphocyte-predominant Hodgkin's disease. No specific antitumor treatment was given and three years following splenectomy the patient is in excellent health and has not experienced any new hemolytic episodes. The patient illustrates the association between Hodgkin's disease and autoimmune hemolytic anemia as well as the extreme variation in the clinical picture of the disease.
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PMID:Cyclic autoimmune hemolytic anemia as a presenting manifestation of splenic Hodgkin's disease. 706 69

Two patients with splenomegaly and a mild degree of hypersplenism are presented. Both underwent diagnostic splenectomy and in both the histologic examination of the spleen disclosed follicular lymphoid hyperplasia. The clinical picture and histologic findings were compatible with the diagnosis of idiopathic splenomegaly. 33 months after splenectomy a non-Hodgkin lymphoma was diagnosed in one patient. The literature on idiopathic splenomegaly is reviewed and the high incidence of non-Hodgkin lymphoma in the follow-up of such patients is pointed out.
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PMID:[Idiopathic splenomegaly: the preliminary stage of a malignant lymphoma? Report on 2 cases]. 710 Aug 69

The term lymphogranulomatosis X (LgX) designates a clinicopathological entity of unknown etiology ("X"), which was first described by Forster and Moeschlin in 1954. LgX includes the "immunoblastic lymphadenopathy" of Lukes and to a large extent the "angioimmunoblastic lymphadenopathy" of Rappaport (except for the cases with active germinal centers), but in LgX there is another morphologic variant not mentioned either by Lukes or by Rappaport. To establish the morphologic diagnosis of LgX three typical changes of the affected lymph nodes are needed: effacement of the nodal architecture, absence of active germinal centers, and markedly increased proliferation of epithelioid venules. Cases with only partial effacement of nodal architecture, and especially with active germinal centers, were considered hyperimmune reactions (HR). This "hyperimmune reaction" may be an early stage of LgX: 4 of 39 cases showed later transformation into LgX. Based on cytologic aspects, five different variants of LgX are distinguished: immunoblastic predominance, plasma cell predominance, mixed cell type, epithelioid cell predominance and lymphocytic predominance. Case history and clinical picture of the five variants of LgX (172 patients) are reported and compared with HR (37 patients). In LgX the age peak is in the 7th decade; the youngest patient was 16 years old. HR are seen in children as well as in elderly people, the mean age being 49 years. In LgX males predominate and in HR females. In LgX the disorder is usually more generalized than in HR (80% versus 46% with generalized lymph node enlargement, 69% versus 24% with hepatomegaly and 62% versus 27% with splenomegaly). Skin involvement and high sedimentation rate are less frequent in HR. In LgX a Coombs-positive anemia is occasionally found; its origin is "aplastic" rather than hemolytic. Based on the data presented, some reflections relative to the etiology and pathogenesis of LgX are presented. Rubella virus should be considered a possible etiologic agent. In most cases with drug hypersensitivity, allergic reactions to drugs appear only in the course of the illness; in these cases drugs are ruled out as an etiologic factor. Occasionally, an augmented number of azurophil granulated lymphocytes (suppressor T-cells?) is observed in the blood, a fact that could be a pointer to the pathogenesis of LgX and possibly explain the high incidence of infections seen in this disorder.
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PMID:[History and clinical picture of lymphogranulomatosis X (including (angio)immunoblastic lymphadenopathy]. 726 65

No significant excess of deep vein thrombosis (DVT) as measured by the 125I-labelled fibrinogen method was observed in patients having staging laparotomy and splenectomy for Hodgkin's disease (HD) compared with patients having elective cholecystectomy under highly standardized surgical conditions. Patients who did have DVT all had splenic involvement with HD. There was no correlation between the post-splenectomy thrombocytosis and the occurrence of DVT. Patients with non-Hodgkin's lymphoma (NHL) and splenomegaly had a high incidence of DVT after splenectomy.
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PMID:Thrombotic risks of staging laparotomy with splenectomy in Hodgkin's disease. 731 64

In the case reported here the patient presented with Stage IVB mixed cellularity Hodgkin's disease was complicated by massive splenomegaly, bone marrow infiltration with Hodgkin's disease, and severe pancytopenia. The pancytopenia was reversed by glucocorticosteroid therapy but recurred when steroids were tapered. Reinstitution of steroids resulted in recovery of peripheral blood counts, allowing administration of full-dose combination chemotherapy leading to a complete clinical remission and obviating the need for emergency splenectomy.
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PMID:Hodgkin's disease presenting as steroid-responsive pancytopenia. 738 54

The authors reviewed retrospectively 139 splenopathies detected during 432 US examinations of the abdomen in AIDS patients to assess the role of US in correlation with anamnestic-clinical data and histologic findings in 45 cases. Splenomegaly was the main sign of abnormal splenic conditions, since it was present in all the examined patients. Twenty-one cases exhibited focal lesions. Non-Hodgkin's lymphomas were the only kind of neoplastic condition, which were detected in 15% of cases. In 57% of cases splenopathy was correlated with an infective agent, with a marked prevalence of Mycobacteria-i.e., tubercular in 26% and atypical in 22%. In the splenopathies with histologic confirmation which exhibited a focal US pattern (47%), US proved to be useful in assessing splenic involvement, however aspecific its signs, and in its monitoring, especially in the lesions with unexpected colliquative evolution, as in two cases of atypical mycobacteriosis.
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PMID:[Ultrasound aspects in AIDS-related splenic diseases]. 771 87

The traditional method of splenectomy involves a 7- to 10-cm abdominal incision, with its attendant morbidity that includes sepsis. Recently, the laparoscopic technique has been adopted for splenectomy. Many surgeons still believe that laparoscopic splenectomy should be carried out only for idiopathic thrombocytopenia purpura and Hodgkin's disease with a normal-sized spleen. A 16-year-old girl with marked splenomegaly (spleen size for times normal and weighing 600 g) due to congenital spherocytosis underwent laparoscopic splenectomy and retrieval of the spleen through the umbilical trocar site. Preoperative arterial embolization was not used, and the operative blood loss was estimated to be only 250 mL. Her postoperative course was uncomplicated, and she was discharged on the 4th postoperative day. The operative technique is described and the advantages of laparoscopic splenectomy are discussed.
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PMID:Laparoscopic splenectomy for congenital spherocytosis with splenomegaly: a case report. 788 14

Peripheral blood mononuclear cells (PBLs) from 14 patients with low grade non-Hodgkin's B-cell lymphomas with predominant splenomegaly were studied by means of scanning (SEM) and transmission electron microscopy (TEM). All patients had peripheral blood and bone marrow involvement, the absence of lymphoadenopathy, and, except in one case, immunophenotypic features of a malignant proliferation of mature spleen B-cells arising from outside the germinal center, but not consistent with CLL or HCL. Several distinctive cytological features were observed in PBLs of the different subgroups. The SEM surface features of PBLs in patients with intermediate differentiation lymphocytic lymphoma (IDL) (five cases), lymphoplasmacytoid immunocytoma (LP-IC) (two cases), and mixed small and large cells malignant lymphoma (one case) were characterized by the presence of numerous well-developed microvilli. Some distinctive TEM ultrastructural features were also seen in the different cases. In the two cases of splenic lymphoma with villous lymphocytes (SLVL), SEM revealed large and elongated surface microvilli generally arising from two or three poles of the cells. This surface morphology, confirmed by TEM analysis, may be pathognomonic of this disease. Four additional cases, tentatively classified as small lymphocytic lymphoma on the basis of immunophenotypic data, were extremely heterogeneous at both SEM and TEM analysis. The ultrastructural features revealed by SEM and TEM may be useful for the more precise characterization of this heterogeneous group of diseases, which is generally difficult to define even when immunophenotypic and molecular approaches are used.
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PMID:Scanning and transmission electron microscopy of clonal peripheral blood lymphocytes in low grade non-Hodgkin's B-cell lymphomas with predominant splenomegaly. 791 21

Laparoscopic splenectomy was attempted in 16 patients and was performed successfully in 13 (81%) patients with the diagnosis of idiopathic thrombocytopenic purpura (ITP), AIDS-related thrombocytopenia, Hodgkin's disease, or lymphoma. The operative time averaged 157 min, and autologous transfusion was required in four patients. The postsurgical stay averaged 3 days in patients with completely laparoscopic splenectomies and 4 days in patients whose spleens were removed through small counterincisions. No major complications secondary to the procedure itself occurred postoperatively. Conversion to open operation was necessary in three (19%) patients because of bleeding or splenomegaly. With careful selection of patients and mastery of the technique, laparoscopic splenectomy can be safely performed on normal or slightly enlarged spleens. The advantages are less pain, shorter hospitalization, and reduced disability as compared to "open" splenectomy.
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PMID:Laparoscopic splenectomy. 799 69

Of splenectomies performed in the Cork Regional Hospital over an 11 year period, ten were undertaken primarily for diagnostic purposes. A definitive histological diagnosis was established in nine patients, seven of whom had lymphoma, two with Hodgkin's disease and five with non-Hodgkin's lymphoma. The weight of the excised spleen in all patients with lymphoma exceeded 1 kg; in all those with a diagnosis other than lymphoma, the spleen weighed less than 1 kg. A majority of patients also had symptomatic improvement from reversal of hypersplenism and from relief of the mechanical pressure effects of an enlarged spleen. Operative mortality was zero. Diagnostic splenectomy is a worthwhile procedure. Most patients will have lymphoma.
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PMID:Splenectomy in patients with undiagnosed splenomegaly. 818 75


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