Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Infectious mononucleosis as a manifestation of primary Epstein-Barr virus infection occurs uncommonly in adults over age 40. While fever is almost universal, older patients with the disease often present without lymphadenopathy, pharyngitis, splenomegaly, lymphocytosis or atypical lymphocytes. Jaundice and hepatomegaly occur more commonly in older patients than in adolescents and create diagnostic confusion. Often, infectious mononucleosis in this age group is confused with lymphoma, leukemia or biliary obstruction, or is classified as "fever of unknown origin."
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PMID:Infectious mononucleosis in older adults. 224 52

Nine out of 10 patients with primary lymphoma of the liver presented in a manner that did not suggest a tumour. The initial diagnoses were chronic active hepatitis in three cases and "granulomatous cholangitis", inflammatory pseudotumour, and anaplastic carcinoma in one case each. Moreover, extensive haemorrhagic necrosis in three cases initially suggested the Budd-Chiari syndrome. All the tumours were diffuse non-Hodgkin's lymphomas like the 50 cases reported previously, but they differed from most of these in that nine were of T cell phenotype. Five were pleomorphic small T cell, two T zone, and two T lymphoblastic lymphomas: only one was centrocytic and of B cell lineage. This report extends the range of clinical manifestations (diffuse hepatomegaly without a tumour), histological appearances (resemblance to chronic inflammatory or vascular liver diseases) and phenotype (of T cell lineage) of primary lymphoma of the liver: these features seemed to be related in this series. Recognition is important as prognosis remains favourable in appropriately treated cases. Although the appearances of the liver biopsy specimens may be difficult to interpret, the destructiveness of the infiltrate is an important clue to the diagnosis.
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PMID:Primary lymphoma of the liver: clinical and pathological features of 10 patients. 226 72

The authors report a case of systemic mastocytosis in a 65 year-old woman. The clinical picture was generalized abdominal and extra abdominal lymph nodes, ascites, splenomegaly, and hepatomegaly. As gastro-intestinal symptoms led to suspect a digestive malignant lymphoma; the patient underwent an exploratory laparotomy. The diagnosis of systemic mastocytosis was established by demonstrating an excessive number of mast cells in the cutaneous and extracutaneous tissues (liver, mesenteric lymph, nodes, spleen and bone marrow). Bone marrow biopsy revealed extensive myelofibrosis.
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PMID:[Systemic mastocytosis. A case report]. 236 87

An autopsy case of malignant lymphoma of the central nervous system which showed extracranial disseminations was presented. A 50-year-old man developed mental and physical slowness over one year prior to admission followed by dementia and consciousness disturbance without general physical symptoms. Physical examination on admission showed no lymph node enlargement, hepatomegaly, splenomegaly, or abdominal mass. Neurological examination revealed mild dementia, left positive Babinski and Chaddock reflexes, and bilateral positive frontal lobe signs. CT scan revealed low density areas with contrast enhancement in the white matter of the bilateral parietal lobes adjacent to the trigon of lateral ventricles. Without any therapy, the low density area in the left cerebral hemisphere on CT scan disappeared and the low density area in the right cerebral hemisphere became unenhanced. Any other lesions except brain were found despite of the extensive systemic examinations including scintigrams, echograms, gastrointestinal examinations, body CT scan, aspiration of bone marrow, and lymphography. Primary intracranial malignant lymphoma was suspected and treated with steroid without any response. Subsequent radiation therapy made a transient improvement. But a few months later, the brain lesions gradually worsened, followed by general physical deterioration with diarrhea, pleural fluids, and ascites. Cytologic study of cerebrospinal fluid revealed neoplastic lymphocytes with atypical nuclei containing conspicuous nucleoli and mitosis, which were identified as B cell type malignant lymphoma by analysis using monoclonal antibody. The patient died of cardiac failure about two years after the initial symptom.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsy case of primary intracranial malignant lymphoma showing extracranial disseminations]. 239 Mar 65

This review describes the transmission, clinical picture and immunological abnormalities of HIV infection in children in general, and the special problems of AIDS in African children. The review begins with a thorough introduction to the epidemiology of AIDS. Transmission to children generally involves vertical transmission by placental transfer or transmission of HIV via transfusion of blood and blood products, or by contaminated needles. Casual transfer is unknown, and only a few cases of transmission via breast milk are known. The clinical picture of HIV infection in infants and children differs from that in adults in 3 important aspects: earlier onset, different clinical presentation and existence of AIDS embryopathy. The average onset was 5 months of age. The most common symptoms in young children are chronic interstitial pneumonitis without demonstrable etiology, hepatomegaly, failure to thrive, adenopathy, diarrhea, oral or perineal thrush, eczema and thrombocytopenia. The common opportunistic infections are pneumocystis carinii pneumonia, cytomegalovirus, Epstein-Barr virus, Cryptosporidium diarrhea, pyogenic infections of the middle ear and gram-negative septicemia. Several infections seen in adult AIDS cases are rare in children: mycobacterium avium-intracellulare, toxoplasma gondii, hepatitis B, as well as Kaposi's sarcoma, malignant lymphoma and cardiac abnormalities. The AIDS embryopathy or HIV dysmorphic syndrome is characterized by immunological abnormalities, growth failure, and craniofacial dysmorphism, particularly microcephaly, prominent box-like forehead, hypertelorism, flattened nasal bridge, obliquity of the eyes, blue sclerae and patulous lips. AIDS in African children is extremely difficult to diagnose because of similarities between the presenting symptoms and those commonly seen in sick children there, many of whom are also immune compromised. Where serotesting is available, the picture is complicated by cross reaction between the test agents and some factor found in sera from malaria patients. Seropositivity in some areas is high, increased by the prevalence of transfusion and injection treatments. Diagnosis is made more difficult by lack of laboratory facilities and difficulties in follow-up for pediatric patients. The CDC definitions of AIDS and ARC, and the WHO/CDC definitions of AIDS are appended.
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PMID:Human immunodeficiency virus infection in childhood. 245 15

An acute terminal phase of a Bence Jones kappa plasma cell myeloma developed 22 months after chemotherapy is presented. The patient's symptoms were fever, cytopenias, adenomegalies and hepatomegaly. A lymph node biopsy showed an immunoblastic polymorphic kappa B cell lymphoma, expressing CD30 antigen. After new polychemotherapy the patient died because of bleeding and infection. Autopsy revealed a decrease in the tumour with defective immunophenotype. The few reported cases have been reviewed with emphasis on clinical aspects, prognosis and morphology, The significance of CD30 positivity (activation marker) in a high-grade lymphoma is discussed.
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PMID:Malignant B cell immunoblastic lymphoma expressing CD30 antigen in the terminal phase of a multiple myeloma. 256 14

Reported are two cases of a malignant lymphoma that presented a liver injury in which death was brought on by hepatic failure. In both instances on admission, liver injury and hepatomegaly were noticed. Abdominal ultrasonography and computerized tomography, however, showed no SOL in the liver or any clear lymph node mass, though a bright echo and a diffuse low density was observed in one of the patients (Case 1). In spite of treatment that included prednisolone, the liver injury in both patients progressed towards hepatic failure. Severe jaundice increased rapidly in Case 1, and a disseminated intravascular coagulopathy complicated by jaundice was observed in the other patient (Case 2). Both autopsies revealed severe lymphoma cell infiltration into the liver, mainly in Glisson's capsule, and diffuse hepatic cell necrosis. Diagnostic imaging of the diffuse lymphoma infiltration into the liver proved very difficult, so that it was necessary to rely on a differential diagnosis, employing laboratory tests and a liver biopsy.
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PMID:[Two fatal cases of hepatic failure due to a malignant lymphoma presenting a liver injury]. 264 66

The clinical and hematological profile, the response to therapy and the clinical evolution of 29 patients with acute T lymphoproliferative syndromes (ATLS) diagnosed during the last 5 years were evaluated. Mean age was 22.6 (SD 9.5) years. 24 patients were males and 5 females. Lymphadenopathy was present in 25 patients, hepatomegaly in 16 and splenomegaly in 14. Seventeen patients had a mediastinal mass. Anemia was present in 41% of patients and thrombocytopenia in 50%. The mean leukocyte count was 71 x 10(9)/l (SD 93). Bone marrow infiltration was found in 25 patients. The acid phosphatase reaction was positive, centrosomal type, in all cases except two. The immunophenotypic study classified the ATLS patients in the subtypes pre-T (10 cases), cortical thymic (15 cases), and mature thymic (4 patients). The cases with pre-T phenotype had a greater degree of bone marrow infiltration and lower frequency of mediastinal mass than the rest of patients. The rate of complete remissions (CR) was 83%. At the time of closing the study 12 patients had relapsed, with a median time of CR of 9.4 months. In addition, 11 patients had died, with a median survival of the series of 19 months. The results of the present study indicate that, despite some heterogeneity, those cases of ATLS with a more immature immunological phenotype (pre-T) have clinical and biological features more akin to acute leukemia, unlike those with a thymic phenotype (cortical or mature), which would be closer to lymphoblastic lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute T-cell lymphoproliferative syndromes. A study of 29 cases]. 269 96

The frequency, pattern, extent and clinical features of bone marrow involvement in 94 cases of non-Hodgkin's lymphoma, classified according to the International Working Formulation, were reviewed. In 30% of cases there was bone marrow involvement by lymphoma at the time of diagnosis. Marrow involvement was most frequently found in the follicular small cleaved cell (57%), diffuse mixed small and large cell (56%), and lymphoblastic (56%) lymphomas. A paratrabecular pattern of marrow involvement tended to occur in low or intermediate grade lymphomas. High grade lymphomas tended to show an interstitial or diffuse pattern of marrow involvement. The results of marrow aspiration and biopsy were of equal value and complementary to each other. In 43% of cases with marrow lymphoma there was peripheral blood involvement at the time of diagnosis. Blood involvement was most frequently found in the lymphoblastic lymphoma (70%). In general, the degree of blood involvement was related to the extent of marrow involvement. Patients with marrow involvement often presented with "B" symptoms and hepatomegaly. Abnormalities in at least one of the blood counts were quite common (93%). A normal blood count made marrow involvement unlikely but not impossible. Other common laboratory findings included hyperimmunoglobulinemia (47%) and elevated serum levels of lactate dehydrogenase (56%). Patients with marrow involvement showed a significantly higher frequency of hepatomegaly and abnormal blood counts, as compared to those with negative marrows (p less than 0.05 and less than 0.001, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Bone marrow involvement in non-Hodgkin's lymphoma. 276 11

The retrovirus-associated adult T-cell leukemia/lymphoma (ATL) has not been previously documented in Taiwan. Five cases identified recently by the authors are reported. Three of the patients were women, and their ages ranged from 36 to 60 years. The most important diagnostic clue was the observation of polylobated lymphoid cells in the peripheral blood. Other variably observed significant features included hypercalcemia, cutaneous eruptions, osteolytic bone lesion, hepatomegaly, and lymphadenopathy. Surface marker studies revealed that the leukemic or lymphoma cells were T-helper cells. Histopathologic examination revealed one case of pleomorphic type and three cases of medium-sized cell type. No tissue was available for study in one case. The diagnosis of ATL was confirmed by the indirect immunofluorescence test on MT-1 cell for antibodies to adult T-cell leukemia virus-associated antigen (ATLA). Three patients were dead within 6 months, and two patients had been in clinical remission for 7 and 10 months, respectively. These two latter cases were similar to the so-called smoldering type of ATL. Two descendents among nine relatives of the patients were also positive for anti-ATLA (22%). Two husbands were negative. Four of the five patients lived in the same county in northeastern coastal Taiwan, which suggested a possible clustering of ATL in that region.
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PMID:Adult T-cell leukemia/lymphoma in Taiwan. A clinicopathologic observation. 286 99


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