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)

A 24 year old male with a history of eczema, recurrent mild infections, and thrombocytopenia consistent with the Wiskott-Aldrich syndrome (WAS) presented with a mediastinal mass, generalized lymphadenopathy, splenomegaly, and severe thrombocytopenia. Studies of immune function including immunoglobulin levels and T-cell subsets were normal. Furthermore, his T lymphocytes proliferated normally in response to phytohemagglutinin, concanavalin A, and the combination of neuraminidase/galactose oxidase. However, their proliferative responses to anti-CD43 antibody and periodate were diminished, consistent with the clinical diagnosis of WAS. An initial inguinal lymph node biopsy surprisingly revealed Kaposi sarcoma. However, following splenectomy to increase the platelet count, biopsy of the mediastinal mass revealed T-cell large cell lymphoma. Studies of biopsied tissue for the presence of Epstein-Barr virus and cytomegalovirus were negative, as were studies of blood, including the polymerase chain reaction, for the presence of the human immunodeficiency virus (HIV). This is the first report of Kaposi sarcoma arising in a patient with a congenital immunodeficiency syndrome. Although Kaposi sarcoma can arise in the face of the severe immunosuppression that follows allograft transplantation and in patients infected with HIV, we postulate that longevity in the face of mild immunosuppression was the major factor in the development of Kaposi sarcoma in this patient.
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PMID:Coincident Kaposi sarcoma and T-cell lymphoma in a patient with the Wiskott-Aldrich syndrome. 131 18

Since the onset of the AIDS epidemic, disseminated Histoplasma capsulatum infection has been reported with much greater frequency in both endemic and nonendemic areas. Abdominal CT scans of 16 patients with disseminated histoplasmosis were reviewed retrospectively to identify radiologic features of this disease. The diagnosis was confirmed by autopsy (three patients), bone marrow biopsy (10 patients), lymph node biopsy (three patients), bronchoscopic biopsy (three patients), liver biopsy (two patients), and/or colonoscopic biopsy (one patient). Fourteen patients had serologic evidence of human immunodeficiency virus infection. Disseminated histoplasmosis was either the only initial manifestation of AIDS (seven patients) or was accompanied simultaneously by cytomegalovirus infection (four patients), or Kaposi sarcoma, Toxoplasma encephalitis, or cryptosporidiosis (one patient each). Abdominal CT findings included hepatomegaly (63%); splenomegaly (38%); diffuse splenic hypottenuation (19%); bilateral adrenal enlargement or hypoattenuating masses (13%); and enlarged lymph nodes with homogeneous soft-tissue density (44%), diffuse or central low density (13%), or both (19%). Histoplasmosis should be included in the differential diagnosis when abdominal CT scans show such nonspecific findings as hepatomegaly, splenomegaly, enlarged soft-tissue-density or hypoattenuating lymph nodes, or adrenal enlargement or masses in an immunodeficient patient. An uncommon but possibly specific CT finding in histoplasmosis is diffuse splenic hypoattenuation.
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PMID:Disseminated histoplasmosis: abdominal CT findings in 16 patients. 192 16

To establish the indications for splenectomy in patients with human immunodeficiency virus (HIV) infection we retrospectively analyzed 12 patients who underwent splenectomy. Patients with HIV infection who had immune thrombocytopenic purpura (ITP) were excluded as they had no splenomegaly and a definite indication for splenectomy exists in some of these patients. All 12 patients were anemic; 6 were thrombocytopenic and 6 leukopenic. All patients had splenomegaly and all were febrile. At surgery 3 patients were found to have Mycobacterium avium intracellulare (MAI) infection; 2 had splenic abscess due to Salmonella group D; 1 each had cytomegalovirus (CMV) splenitis and localized Kaposi's sarcoma (KS) of the spleen. No definite histopathologic diagnosis could be made in five patients, all of whom had evidence of extramedullary hematopoiesis. The degree of splenic enlargement did not correlate with the outcome. Both clinical and hematologic improvements were achieved in patients with splenic abscess and in patients who had splenomegaly, anemia, and thrombocytopenia. The presence of either of these findings constitutes an indication for splenectomy. Anemia and/or leukopenia without thrombocytopenia failed to improve; the presence of MAI and active CMV infection also resulted in failure. The presence of either of these conditions may be considered a contraindication to splenectomy.
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PMID:Splenectomy in patients with AIDS. 281 11

Co-infection with acquired immunodeficiency syndrome (AIDS) and Kaposi's sarcoma is not uncommon in Europe, but is rare in Africa and not previously reported in infants. This article documents the case of an 11-month-old African boy with lymphocutaneous Kaposi's sarcoma. The infant was brought to a hospital in the Central African Republic with chronic diarrhea and disseminated lymphadenopathy. Also present were fever, cough, weight loss, a gingivostomatitis with herpes-like vesicles, hepatomegaly, splenomegaly, and cervico-axillo-inguinal lymphadenopathy. The adenopathies 1st occurred when the infant was 7 months of age and were followed 1 month later by the emergence of 12 dark brown or black velvet raised cutaneous nodules. The diagnosis of Kaposi's sarcoma was confirmed by lymph node and skin nodule biopsies. Also indicative of Kaposi's sarcoma was the presence of abortive vascular foci at a distance from the skin's surface and the cell proliferation. Both the infant and his asymptomatic mother were seropositive for antibodies to human immunodeficiency virus (HIV)-1. The skin lesions in this case presented the special infiltrative characteristic of AIDS-related Kaposi's sarcoma. The infant died 2 months after presentation at the hospital. By the last weeks of his life, the cutaneous nodules had covered the entire body. Death was from pleuropneumopathy. Given the high prevalence of HIV-1 infection in the Central African Republic, more such cases can be expected.
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PMID:Lympho-cutaneous Kaposi's sarcoma in an African pediatric AIDS case. 292 57

The Celebes black macaque (Macaca nigra) colony at the Oregon Regional Primate Research Center has a high incidence of an immunodeficiency syndrome characterized by recurrent diarrhea and the development of retroperitoneal fibromatosis (RF). We have examined the relationship of type D viral infection to the immunodeficiency syndrome by surveying the colony for viral infection and for mitogen reactivity. Type D virus-positive monkeys (28% of the colony) have a higher prevalence of diarrhea, splenomegaly, lymphadenopathy and weight loss than do virus-negative monkeys, and RF has been found to occur only in virus-positive animals. Comparison of the concanavalin A (con-A) and phytohemagglutinin reactivities of the virus-positive and -negative populations has revealed no significant difference. However, within the virus-positive population, those with RF have reduced con-A reactivity and there are both high and low mitogen responders in the groups lacking RF. Thirty-two percent of the virus-positive monkeys are free of clinical symptoms, 40% have clinical symptoms but no RF, and 27% have clinical symptoms and RF. Five of the six monkeys with RF are older than the RF-free monkeys but monkeys are susceptible to type D retrovirus infection regardless of age or sex. The progressive nature of this immunodeficiency syndrome, its broad age range, and the probability that the etiological agent is also a type D retrovirus and the similarity of RF to Kaposi's sarcoma make this a potentially useful model for human AIDS.
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PMID:Relationship of mitogen reactivity to type D retrovirus infection in Celebes black macaques (Macaca nigra). 301 9

Acquired immunodeficiency syndrome (AIDS) is a lethal infectious disease that has reached epidemic proportions in urban centers of the United States. Intraabdominal opportunistic infections and malignancies are common features of this syndrome. A prodromal phase or possibly milder form of infection is known as the AIDS-related complex. Abdominal computed tomography (CT) in patients with AIDS-related complex often demonstrates a triad of mild retroperitoneal and mesenteric adenopathy, splenomegaly, and perirectal inflammation. Lymph node enlargement greater than 1.5 cm is unusual in the AIDS-related complex and should prompt CT-guided biopsy. Abdominal adenopathy (greater than 1.5 cm) in AIDS, in our experience, is most commonly related to non-Hodgkin lymphoma, Kaposi sarcoma, or infection with Mycobacterium avium-intracellulare. In most instances, CT-guided biopsy with appropriate staining technique can readily distinguish these entities. However, the subtyping of non-Hodgkin lymphoma by fine-needle aspiration biopsy alone remains controversial. Unusual features of abdominal malignancies are common in AIDS. These include a purely lymphadenopathic form of AIDS-related Kaposi sarcoma and a predilection for extranodal sites of lymphoma in AIDS. In general, patients with AIDS-related lymphoma present with advanced stages of disease with highly malignant histologic subtypes. Abdominal CT may be useful clinically for diagnosing intraabdominal complications of AIDS.
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PMID:Abdominal CT in acquired immunodeficiency syndrome. 351 46

Two homosexual men with the acquired immunodeficiency syndrome (AIDS) who developed a multicentric variant of angiofollicular lymph node hyperplasia (AFLNH) (Castleman's disease) and Kaposi's sarcoma are reported. Both had diffuse adenopathy, splenomegaly, and a systemic inflammatory state. Both had an absolute increase in Leu 1+ lymphocytes, which was associated with markedly decreased Leu 3+ lymphocytes, markedly increased Leu-2+ lymphocytes, and a very low Leu 3/2 ratio. The lymphocytes of both patients had a normal blastogenic response to PHA. The lymphocytes of patient 1 had a poor response to autologous or allogenic cells in the mixed lymphocyte culture reaction. AFLNH represents another lymphoreticular complication of AIDS. Given the interrelationships between AFLNH, the development of Kaposi's sarcoma, and the aggressive clinical course seen in our two patients and those in the literature, the aggressive use of lymph node biopsy may be an important prognostic tool for the patient with the acquired immunodeficiency syndrome.
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PMID:Multicentric angiofollicular lymph node hyperplasia (Castleman's disease) followed by Kaposi's sarcoma in two homosexual males with the acquired immunodeficiency syndrome (AIDS). 387 3

A spontaneous multifocal subcutaneous fibromatosis is described in 6 pig-tailed macaques (Macaca nemestrina) with simian acquired immune deficiency syndrome (simian AIDS). The lesions consisted of a proliferation of vascular fibrous tissue that was infiltrated by lymphocytes and plasma cells. One animal also had retroperitoneal fibromatosis, which has also been found in this colony of pig-tailed macaques. Progressive weight loss, diarrhea, lymphadenopathy, and neutropenia were seen. Peripheral lymph nodes were hyperplastic, and there was splenomegaly. Aggregates of lymphocytes were present in the bone marrow, kidneys, liver, and lungs. Type D retrovirus particles were found in three nodules by electron microscopy; intracytoplasmic type A and budding particles were identified in fibroblasts. In a setting of acquired immunodeficiency, these subcutaneous tumors in pig-tailed macaques present a striking analogy to Kaposi's sarcoma in human AIDS.
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PMID:Subcutaneous fibromatosis associated with an acquired immune deficiency syndrome in pig-tailed macaques. 401 42

The radiographic findings in 39 patients with acquired immunodeficiency syndrome (AIDS) were reviewed. Twenty five of the 39 patients had infections and 14 had Kaposi sarcoma and opportunistic infections. A prodromal phase, which is seen in many patients and is associated with lymphoid hyperplasia, lymphadenopathy, and splenomegaly, is described. Recognition of the problem is important in alerting the patient to the early detection of infections and/or Kaposi sarcoma.
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PMID:The prodrome, Kaposi sarcoma, and infections associated with acquired immunodeficiency syndrome: radiologic findings in 39 patients. 660 29

Abdominal computed tomography (CT) was performed in 31 patients with Kaposi sarcoma (KS) related to acquired immunodeficiency syndrome (AIDS), three patients with classic KS, and 12 patients with the newly described lymphadenopathy syndrome (LNS). The frequency, distribution, and appearance of lymphadenopathy and splenomegaly were similar in the AIDS-related KS and LNS groups. Rectal and perirectal disease was identified in 86% of the homosexual men studied; rectal KS could not be distinguished from proctitis on CT criteria alone. No CT abnormalities were seen in patients with classic KS. The CT demonstration of retroperitoneal, mesenteric, or pelvic adenopathy or of rectal or perirectal disease in patients with AIDS-related KS is not necessarily indicative of widespread involvement with the disease.
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PMID:Kaposi sarcoma and lymphadenopathy syndrome: limitations of abdominal CT in acquired immunodeficiency syndrome. 669 Nov 5


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