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)

Hairy cell leukemia is a rare lymphoproliferative disorder which is morphologically characterized by circulating lymphatic cells with prominent 'hairy' cytoplasmatic projections. Patients typically present with pancytopenia and splenomegaly. The bone marrow cannot be aspirated because of reticulin fibrosis. Chemotherapy was unable to influence the course of the disease satisfactorily. Therefore, splenectomy was the treatment of choice. The introduction of alpha-interferons into the therapy of hairy cell leukemia was a decisive breakthrough and is exemplary for the use of interferons in the treatment of malignant diseases. However, there are still many questions to be answered. The discussion of clinical implications is achieving growing importance in consequence of the availability of diverse commercial alpha-interferons for treatment of hairy cell leukemia.
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PMID:[Therapy of hairy cell leukemia with interferon]. 305 67

We have reported the case of a 47-year-old man who had massive splenomegaly and marked pancytopenia for which no cause was determined. Splenectomy produced dramatic improvement. The clinical, hematologic, and pathologic findings are characteristic of nontropical idiopathic splenomegaly, or Dacie's syndrome. In 20% of the 46 cases described in the literature, malignant lymphoma has subsequently developed.
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PMID:Nontropical idiopathic splenomegaly. 305 22

Hairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and transfusion-dependent anemia after splenectomy. Each subsequently acquired transfusion hepatitis and in two patients marked hematologic improvement was noted within 2 months. Complete peripheral blood remission occurred within 17 months in all patients although bone marrow infiltration with hairy cells persisted. One patient remains in remission for 12 years; the other two succumbed to infectious illnesses but with normal blood counts. The mechanism by which hepatitis virus induces hematologic recovery in patients with hairy cell leukemia is unknown but may involve augmentation of the interferon system.
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PMID:Peripheral blood remission of hairy cell leukemia after transfusion hepatitis. 311 Dec 50

Kikuchi's disease is characterized by lymphadenopathy in young patients and may be mistaken for malignant disease both clinically and histologically. We report the case of a 26-year-old man with persistent fever for 3 weeks and splenomegaly, in whom pancytopenia developed afterwards. A bone marrow aspiration was normal. Blood, urine, throat, stool and bone marrow cultures were negative as were serological tests for lues, Toxoplasma, Epstein-Barr virus and Widal's test. An abdominal CT scan showed enlarged retroperitoneal lymph nodes and an exploratory laparotomy was performed. Two lymph nodes were excised and a wedge biopsy of the liver was performed. The histological findings in the lymph nodes were compatible with the diagnosis of Kikuchi's disease. The patient became afebrile on the 2nd postoperative day without any treatment. He has been well for 4 months after discharge.
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PMID:Kikuchi's necrotizing lymphadenitis: a cause of fever of unknown origin and splenomegaly. 312 82

Sixty-nine percent of AIDS patients show evidence of splenomegaly on their plain chest film. This splenomegaly is not related to severity of AIDS, to intravenous drug abuse or to any specific infection or neoplasm but correlates well with severity of anemia, pancytopenia, and thrombocytopenia. Increasing splenomegaly usually indicates worsening of AIDS. The triad of splenomegaly, interstitial lung disease sparing the bases, and very thin chest wall soft tissues is highly specific for the diagnosis of AIDS. The sensitivity of the plain film for diagnosing AIDS can be increased by including AIDS in the differential diagnosis of all patients with splenomegaly, intrathoracic lymphadenopathy, cachexia, or bilateral interstitial lung disease of unknown cause.
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PMID:Splenic size on routine chest films in AIDS: diagnostic and prognostic significance. 336 27

Two male patients, aged 36 and 73 years respectively, gradually developed febrile pancytopenia with profound alteration of their general condition and major inflammatory repercussions. No superficial or deep lymph node enlargement was found initially. Patient n degree 2 had an enlarged spleen. In both cases histological examination of the bone marrow showed an extensive and apparently nonspecific myelofibrosis. The subsequent development of superficial lymphadenopathy provided a firm diagnosis of Hodgkin's disease with mixed cellularity. These two cases belong to the category of exceptional massive medullary forms of Hodgkin's disease described by Duhamel et al. in 1979.
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PMID:[Hodgkin's disease disclosed by myelofibrosis of primary appearance. Apropos of 2 cases]. 342 Mar 30

Two patients with hairy cell leukemia with massive splenomegaly and severe pancytopenia were treated with recombinant alpha-A interferon (IFN-alpha-2a). There was no significant response to a trial of IFN-alpha-2a (11 and 20 weeks) with respect to blood counts or spleen size. Subsequent treatment with 2'-deoxycoformycin (dCF) for 8 consecutive weeks (4 mg/m2/wk) resulted in normalization of spleen size and a normalization of peripheral blood counts and bone marrow in one patient. The second patient demonstrated a reduction in spleen size and improved blood counts following 9 weeks of dCF therapy but eventually became refractory. This demonstrates that dCF is non-cross-resistant with interferon and confirms the efficacy of dCF in nonsplenectomized patients.
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PMID:Response to 2'-deoxycoformycin after failure of interferon-alpha in nonsplenectomized patients with hairy cell leukemia. 348 58

Eight patients with hairy-cell leukaemia (HCL) complicated by pancytopenia were treated with low dose regimens of the adenosine deaminase (ADA) inhibitor 2'-deoxycoformycin (DCF). All patients had significant haematological and clinical improvement. One patient who had been splenectomized and five patients with mild to moderate splenomegaly achieved normal blood counts within 2 months, which have been maintained for up to 18 months. Complete remissions occurred in two patients and four patients had 50-95% marrow clearance of hairy cells. The initial DCF treatments produced a 1-3 g/dl fall in the haemoglobin levels and one patient had a temporary reduction in granulocyte and platelet counts. Five patients had nausea/vomiting, and/or lethargy following DCF, but there was no correlation between the plasma levels of deoxyadenosine and adenosine and the incidence or severity of these side effects. An increased incidence of infection and drug hypersensitivity may reflect the effects of DCF on the immune system. Low dose DCF is a highly effective agent in HCL.
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PMID:The treatment of hairy-cell leukaemia with 2'-deoxycoformycin. 348 71

A 41-year-old male presented with a prolonged febrile disease with marked splenomegaly. Bone marrow and liver biopsy smears and cultures for Leishmania were repeatedly negative and there was no hyperglobulinemia. Leishmania parasites were ultimately demonstrated only in the spleen at laparotomy, performed because of severe pancytopenia. The epidemiology of leishmaniasis in Israel is reviewed.
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PMID:Visceral leishmaniasis presenting as fever of unknown origin. 360 92

Because of a pancytopenia, moderately cellular bone marrow with hypoplastic erythropoiesis but with active granulo- and thrombopoiesis and a conspicuous increase in histiocytic cells, a patient was provisionally diagnosed as having histiocytic medullary reticulosis. However, Salmonella typhi was cultured from blood, urine and stool specimens. After 3 weeks the Widal reaction showed a titre of 1/320 for the H-antigen and 1/1,280 for the O-antigen. The patient was treated with chloramphenicol for 10 days and the fever, enlarged spleen and enlarged lymph nodes returned to normal.
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PMID:[Typhoid fever presenting as histiocytic medullary reticulosis: a case report]. 379 74


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