Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 58-year-old woman presented with a history of premature onset of menopause, longstanding hepatosplenomegaly, monoclonal gammopathy, lower limb polyneuropathy of recent onset, diabetes mellitus, excessive perspiration and leg edema. Polyneuropathy and excessive perspiration improved following a course of prednisone and melphalan. The clinical and pathophysiological features fit the rare entity known as POEMS syndrome.
Isr J Med Sci 1986 Dec
PMID:An unusual case of POEMS syndrome. 357 Jul 37

A 66-year-old man with a 6-month history of sweating at night, generalized lymphadenopathy, hepatosplenomegaly, and paraproteinemia was diagnosed to have a Stage IV mantle zone lymphoma (MZL), which behaved aggressively. The neoplasm rapidly disseminated to extranodal sites--the skin, lungs, pleural cavity, and the central nervous system. The neoplasm did not respond to initial double-agent chemotherapy, but it did partially respond to multi-agent chemotherapy. In addition, the neoplasm had histopathologic features not reported previously in MZL--vascular invasion, massive extranodal infiltration, high mitotic count, and convoluted nuclei.
Cancer 1987 Dec 01
PMID:High-grade mantle zone lymphoma. 367 7

CT examination of a 53-year-old patient with endocarditis and clinical signs of hepatosplenomegaly revealed an atypical vascular morphology in the hilum of the spleen, for which no diagnosis could be established. Angiography indicated the presence of a symptom-free splenoportal fistula, the histological study of which suggested its congenital genesis. The paper describes the morphology, as ascertained by CT, of the splenic vessels characterised by changed haemodynamics due to a hilar splenoportal fistula.
Digitale Bilddiagn 1986 Dec
PMID:[CT morphology of the splenic vessels in splenoportal fistula]. 381 52

Double heterozygosis condition for hemoglobin variants induce clinical syndromes known as intermediate thalassemias. Their diagnosis is often of certain difficulty because of their low frequency and heterogeneity of clinical expressions. We report a case of a 4 year child admitted to our medical center with a story of hepatosplenomegaly. An appropriate hematological study on patient's family permitted a diagnosis of double heterozygosis for Hb Lepore and beta-thalassemia. Results of hematological investigation are reported.
Quad Sclavo Diagn 1985 Dec
PMID:[Hematologic characterization and analysis of genetic transmission in a case of double heterozygosity Hb Lepore/beta-thalassemia]. 383 15

The clinical and pathologic findings in 13 cases of malignant lymphoma involving the prostate gland were reviewed. The lymphomas tended to occur in elderly men with a mean age of 60 years (range, 30-86 years) and were clinically manifested by prostatic enlargement with urinary obstruction. In only one of the patients was there clinical suspicion of lymphoma before surgery. Seven patients had primary extranodal lymphoma of the prostate, with a variety of histologic subtypes, including small cell lymphocytic (one patient), diffuse small cleaved cell (two patients), diffuse mixed small and large cell (two patients), diffuse large non-cleaved cell (one patient), and high-grade diffuse small non-cleaved cell (undifferentiated non-Burkitt's) (one patient). At the time of presentation, none of these patients had hepatosplenomegaly, inguinal lymphadenopathy, abnormal complete blood counts, or elevated serum acid phosphatase levels. Six other patients with previously documented malignant lymphoma at other sites had prostatic involvement 2 to 60 months (mean, 14 months) after the primary diagnosis. Histologically, these secondary prostatic lymphomas included diffuse small cleaved cell (two patients), diffuse mixed small and large cell (one patient), diffuse large non-cleaved cell (two patients), and large cell immunoblastic, polymorphous type (T-cell by immunotyping) (one patient). The mean survival was 14 months for all patients (range, 2-44 months), with no apparent difference between primary and secondary involvement. One patient remains alive 44 months after secondary prostatic involvement with diffuse large non-cleaved cell lymphoma. Although malignant lymphomas involving the prostate are rare, they should be included in the differential diagnosis of lower urinary tract obstruction, particularly in patients with a previous history of lymphoma.
Cancer 1985 Dec 15
PMID:Malignant lymphomas involving the prostate. A study of 13 cases. 384 Apr 6

Seven cases of early congenital syphilis have been recorded in the past 10 years in the Mersey Regional Health Authority. Antenatal serology was initially negative in five mothers, who were either incubating or acquired the infection later, and treatment had probably failed in two women given erythromycin for syphilis during pregnancy. Serology should be repeated later in pregnancy in those at high risk. Social factors that define this group include women who book for antenatal care late in pregnancy, have a past history of sexually transmitted disease, and have multiple consorts. Clinical signs in the infant such as failure to thrive, hepatosplenomegaly, symmetrical rash, rhinitis, and osteochondritis should alert the clinician to the possibility of congenital syphilis. Adequate management of mother and baby requires close liaison between the genitourinary physician, microbiologist, obstetrician, and paediatrician. Penicillin remains the treatment of choice.
Arch Dis Child 1985 Dec
PMID:Early congenital syphilis still occurs. 384 73

The liver-spleen scintigrams of eight patients with the acquired immunodeficiency syndrome (AIDS) were reviewed to characterize the abnormal findings and to assess the diagnostic utility of scintigraphy in evaluating these patients for hepatic and splenic disease. Hepatosplenomegaly was present on the scintigrams of six patients. Additional findings included a solitary hepatic defect in a patient with Kaposi sarcoma and liver metastasis, multiple hepatic defects in a patient with multiple hemangiomas, decreased splenic activity in a patient with lymphoma, and markedly diminished splenic activity in a patient with thrombocytopenic purpura. Hepatosplenomegaly is the most common abnormal scintigraphic finding in AIDS patients. However, the presence of other hepatic or splenic parenchymal scintigraphic abnormalities suggests an underlying secondary pathologic process. Liver-spleen scintigraphy can be a reliable and valid adjunct to the diagnosis of occult hepatic or splenic disease in AIDS patients.
AJR Am J Roentgenol 1985 Dec
PMID:Liver-spleen scintigraphy in patients with acquired immunodeficiency syndrome. 387 22

A case of systemic mastocytosis is reported with an observation period of 20 years. During these two decades multiple manifestations of the disease appeared including urticaria pigmentosis, episodic histamine release, gastro-intestinal involvement and hepatosplenomegaly. The most extraordinary, and possibly unique phenomenon, has been the development of a massive proliferation of large mastocytomas mainly, but not exclusively, limited to the lower extremities. For different reasons (mechanical disability, bleeding, cosmesis), these tumors have required repeated admissions for surgical removal. The most successful technique has involved use of the ultrasonic scalpel.
J Surg Oncol 1985 Dec
PMID:Systemic mastocytosis with extensive large cutaneous mastocytomas: surgical management. 393 76

We present the clinical, pathologic, and metabolic findings of an adult woman with debilitating coronary artery disease and hepatosplenomegaly who was discovered to have multiorgan infiltration by sea blue histiocytes. A diagnosis of sea blue histiocyte (SBH) syndrome was made and no further workup performed. The patient suffered from progressive heart failure and sepsis following coronary artery bypass surgery and died 9 months after presentation. Tissues examined at autopsy showed pronounced infiltrates of both granular sea blue histiocytes and foamy, vacuolated histiocytes, which were morphologically compatible with Niemann-Pick cells. Ultrastructural examination of these cells revealed lamellar myelin-like figures as described in Niemann-Pick (N-P) disease. Fibroblast enzyme assay studies and liver lipid analyses performed after the patient's death revealed pronounced sphingomyelinase deficiency and a lipid profile diagnostic of N-P disease, type B. This case adds further support to the claim that some cases of apparent SBH syndrome actually represent a type of N-P disease.
Am J Hematol 1985 Dec
PMID:Adult Niemann-Pick disease masquerading as sea blue histiocyte syndrome: report of a case confirmed by lipid analysis and enzyme assays. 407 13

BALB/c mice neonatally injected with (BALB/c X C57BL/6)F1 hybrid spleen cells develop host-versus-graft disease (HVGD) with immunopathological features characteristic of either systemic lupus erythematosus (SLE), or immunoblastic lymphadenopathy (IBL) in man. HVGD mice manifest polyclonal hypergammaglobulinemia with various autoantibodies, generalized lymphadenopathy, hepatosplenomegaly, immune complex glomerulonephritis and evolve in the end to malignant lymphoma. The necessary prerequisite for HVGD induction between donor and host can be summarized as follows: Histoincompatibilities in the H-2 region between donor and host are needed; predominant F1 donor cells needed for HVGD induction are, if not sole, steroid resistant, nylon wool nonadherent and Thy-1 positive T cells; the role of donor T cells is not only to present H-2 complex to the host, but also to interact with and proliferate in the host; strain differences are found for the susceptibility of HVGD induction in the host. It has been found that HVGD evolves to a malignant lymphoma of host T cell origin. The HVGD mouse model may, therefore, contribute to the understanding of the cell to cell interactions at work in the pathogenesis of IBL, as well as SLE, in man.
Clin Exp Immunol 1985 Dec
PMID:Autoimmune disease and malignant lymphoma associated with host-versus-graft disease in mice. 408 49


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