Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 64-year-old man who had a two year history of hairy cell leukemia became feverish with night sweats. Splenomegaly and enlarged nodes in the neck and in the anterior mediastinum were detected. Splenectomy was performed and multiple white nodules on a dark-red background were seen in the spleen. The white nodules represented a large cell malignant lymphoma; other areas of the spleen contained mononuclear cells typical in hairy cell leukemia. One of the hilar lymph nodes of the spleen was involved by malignant lymphoma, and the other eight lymph nodes were involved by hairy cell leukemia. The coincidence of the two diseases tends to favor the view that hairy cells are of lymphocytic origin.
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PMID:Coincidental hairy cell leukemia and large cell malignant lymphoma. 31 72

Spontaneous (pathologic) rupture of the spleen in lymphoma is a rare event. A 38-year-old man with a 6-week history of fever, night sweats, and weight loss, and with known splenomegaly presented in shock as a result of a ruptured spleen. There was no known antecedent trauma. Hodgkin's disease was demonstrated in the spleen and liver. Review of the literature failed to reveal any previous report of spontaneous rupture of the spleen due to undiagnosed Hodgkin's disease. Only one survivor of spontaneous rupture of the lymphomatous spleen has been reported previously.
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PMID:Spontaneous (pathologic) rupture of the spleen in previously undiagnosed Hodgkin's disease: report of a case with survival. 83 49

A 36 year old white man was diagnosed as having Whipple's disease after a prolonged illness of lethargy, night sweats, and weight loss associated with lymphadenopathy and splenomegaly. Biopsy specimen of an inguinal lymph node confirmed the presence of periodic acid Schiff positive macrophages and culture gave a pure growth of Corynebacterium jeikeium. Twelve hours after the introduction of oral co-trimoxazole and streptomycin the patient's condition deteriorated. He became confused, feverish, and developed florid retinal vasculitis with associated visual impairment. Both the systemic symptoms and the retinal vasculitis responded to treatment with corticosteroids and his vision returned to normal. We think this was a Jarisch-Herxheimer reaction not previously described in Whipple's disease and advise inspection of the fundi of such patients before starting treatment.
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PMID:Whipple's disease complicated by a retinal Jarisch-Herxheimer reaction: a case report. 137 Dec 61

A 17-year-old, previously healthy woman developed an acute "mononucleosis-like" illness with an associated "atypical" pneumonitis, followed by years of debilitating chronic fatigue, fevers, a 10-kg weight loss, night sweats, and neurocognitive symptoms. Thereafter, her sister developed a similar but less severe illness. The patient developed marked, chronic lymphadenopathy and splenomegaly, with associated persistent relative lymphocytosis and atypical lymphocytosis and with thrombocytopenia. After 3 years of illness, a splenectomy was performed, which resulted in some symptomatic improvement, prompt weight gain, and resolution of all hematologic abnormalities. Serial immunologic studies revealed a strikingly elevated number of activated B lymphocytes and a T lymphopenia, which improved but did not return to normal postsplenectomy. No causal association was found with any of several infectious agents that could produce such a lymphoproliferative illness. However, both the patient and her sister had evidence of active infection with the recently discovered human herpesvirus-6. Seven years after the onset of the illness, the patient and her sister remain chronically ill.
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PMID:A chronic "postinfectious" fatigue syndrome associated with benign lymphoproliferation, B-cell proliferation, and active replication of human herpesvirus-6. 196 94

The authors present 4 cases of Hodgkin's disease developing in homosexual men with persistent, generalized lymphadenopathy. Laboratory abnormalities associated with the acquired immunodeficiency syndrome (AIDS) and the lymphadenopathy syndrome were present in these patients. In 2 patients, diagnosis of lymphadenopathy syndrome preceded the diagnosis of Hodgkin's disease by 2-3 years; in the other 2, the 2 conditions were noted simultaneously. 2 patients had nodular sclerosing Hodgkin's disease, while the other 2 had mixed cellularity disease. All patients presented with clinical stage III or IV Hodgkin's disease. 2 patients in this group have died: 1 of progressive Hodgkin's disease with evidence of atypical myobacterial infection, and 1 with no evidence of Hodgkin's disease but with AIDS-related infections. The lymphadenopathy syndrome has a presentation similar to that of Hodgkin's disease: lymphadenopathy often accompanied by malaise, fever, night sweats, weight loss, and splenomegaly. When a homosexual man presents with painless adenopathy, with or without constitutional symptoms, the potential diagnosis of Hodgkin's disease or other malignancy should be considered in addition to lymphadenopathy syndrome. Evaluation by lymph node biopsy is advisable. The natural history of Hodgkin's disease in patients at risk for AIDS may be altered to a more aggressive form. Unusual features of Hodgkin's disease observed in this group of patients included presentation with stage IV disease, cutaneous Hodgkin's disease, and bone marrow but no splenic involvement. The effects of the treatment modes used for Hodgkin's disease in homosexual patients should be evaluated for their effect on the risk of AIDS.
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PMID:Hodgkin's disease in homosexual men with generalized lymphadenopathy. 396 48

42 homosexual or bisexual men with persistent generalised lymphadenopathy not attributable to an identifiable cause have been followed longitudinally since February, 1981. Lymphadenopathy was accompanied by fatigue, low-grade fever and/or night sweats (57%), splenomegaly (29%), leucopenia (40%), hypergamma - globulinaemia (76%), and diminished proportion and absolute numbers of helper T cells (95%). Of the 26 patients who had lymph node biopsy, all showed benign reactive hyperplasia. After 15-30 (median 22) months, 8 patients have met criteria for the diagnosis of acquired immunodeficiency syndrome (AIDS). This outcome was associated with previous heavy nitrite inhalant use, with the presence of night sweats, with leucopenia, and with the triad of constitutional symptoms, splenomegaly, and leucopenia. In addition, a lower mean absolute helper T cell count and an increased frequency of anergy to mumps intradermal antigen and of herpes simplex virus isolation distinguished these patients from those remaining in the cohort, who seem to be stable and in some cases to have improved.
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PMID:Longitudinal study of persistent generalised lymphadenopathy in homosexual men: relation to acquired immunodeficiency syndrome. 614 74

12 patients with symptomatic chronic myelofibrosis were treated with either busulphan or 6-thioguanine. Therapy was without significant side effects and resulted in a reduction in the size of liver and spleen and an improvement in well-being in all cases. In 7 patients the Hb value rose and the extent of marrow fibrosis was reduced in the 5 patients in whom bone marrow examination was repeated after treatment. Chemotherapy is an effective and safer alternative to splenectomy in patients with myelofibrosis with symptomatic anaemia, symptomatic splenomegaly or constitutional symptoms such as fever, weight loss and night sweats.
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PMID:Chemotherapy resolves symptoms and reverses marrow fibrosis in myelofibrosis. 651 28

A 25-year-old patient presented with fever over a period of more than three months, night sweats, fatigue and a weight loss of more than 10 kg. A splenomegaly and an enlargement of cervical, thoracic and abdominal lymph nodes were found. The suspected malignant hematologic disorder could not be confirmed. Instead, epithelioid noncaseating granulomas in the bone marrow and a cervical lymph node as well as an elevated serum ACE and a lymphocytic alveolitis were found. These findings led us to the conclusion that the patient was suffering from sarcoidosis. Treatment with corticosteroids resulted in complete regression of all symptoms, including the splenomegaly and the enlargement of the lymph nodes. Sarcoidosis is an important consideration in differential diagnosis of fever of unknown origin, even in the absence of pathological changes on X-ray films of the chest.
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PMID:[Fever of unknown origin]. 763 Nov

In an attempt to ascertain whether the presenting features of idiopathic myelofibrosis (IM) have changed in recent years, 2 groups of patients diagnosed with IM in a single institution in different time periods were compared. The first group included 53 patients diagnosed from 1975 to 1986, and the second included 56 patients diagnosed from 1987 to 1997. No significant differences were observed between the two groups with regard to age, gender, delay from first symptoms to disease diagnosis, peripheral blood hematological values and serum biochemical parameters. Patients diagnosed prior to 1987 presented more often with constitutional symptoms (fever, night sweats, weight loss), but the difference did not reach statistical significance. These latter patients had, however, a higher frequency of splenomegaly (91% vs. 73%, p=0.01) and hepatomegaly (79% vs. 48%, p=0.002), and were more often in the osteosclerotic phase at diagnosis (p=0.05) than patients more recently diagnosed. Finally, no significant differences were found between both groups in either the distribution by prognostic scores or survival. The above results seem to indicate a trend towards a less florid clinical picture of IM at presentation in recent years. This, however, does not result in a longer patient survival.
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PMID:The changing profile of idiopathic myelofibrosis: a comparison of the presenting features of patients diagnosed in two different decades. 950 50

An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, approximately 140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy.
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PMID:Symptomatic splenic hamartoma: case report and literature review. 956 43


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