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)

A 65-year-old black female with Sezary syndrome had generalized intractable pruritus, erythroderma, alopecia, onychogryphosis, lumphadenopathy and hepatomegaly. Abnormal lymphocytes with large, convoluted and grooved nuclei (Sezary cells) were identified in the skin and peripheral blood. A striking feature of her disease was severe, deforming arthropathy of the hands and knees, a clinical finding which has been described previously in only one patient with Sezary syndrome. At necropsy no associated lymphoma was found.
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PMID:Sezary syndrome with arthropathy. Report of a case. 5 75

The clinical symptoms, response to therapy, and prognosis of T-zone lymphoma were analyzed in 32 cases. This recently defined lymphoma entity developed relatively quickly with generalized lymphadenopathy and general malaise. Hepatomegaly and/or splenomegaly and skin efflorescence were frequent presenting symptoms. A few patients showed hyperimmune reactions and occasionally severe autoimmune hemolytic anemia. The erythrocyte sedimentation rate was usually markedly elevated. There was sometimes a polyclonal increase in serum immunoglobulin, sometimes a reduction. Blood and bone marrow smears from a few patients showed occasional atypical lymphocytes. A remarkable finding was the frequent involvement of lung or pleura (40.5% of the patients). The prognosis is unfavorable. Most of the patients were in stages III or IV at the time of diagnosis. Massive infiltration of organs, resistance to routine therapy, and decreasing resistance to infection resulted in death soon after diagnosis. The probability of survival was 0.48 in the first year after diagnosis. The prognosis for patients in stages I and II was clearly better than that for patients in stages III and IV.
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PMID:[T-zone lymphoma--clinical symptoms, therapy, and prognosis (author's transl)]. 31 15

Twenty-six cases of histologically proven malignant histiocytosis are presented. Patient ages ranged from 20 months to 82 years, and a 10:3 male preponderance was seen. Clinical presentation is similar to that of malignant lymphoma and leukemia; histologic differentiation is required. The frequency of intrathoracic involvement at the time of presentation is similar to that of non-Hodgkin's lymphoma. Retroperitoneal lymph node invasion was readily seen on five of the eight lymphograms performed. Hepatosplenomegaly was a common finding, and three of the patients had evidence of bone involvement during the course of their illness. While there are no radiographic findings specific to malignant histiocytosis, the presence of hepatomegaly should suggest this possibility. The establishment of the correct diagnosis is important to guide choices in chemotherapy.
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PMID:Radiographic manifestations of malignant histiocytosis. 97 May 32

Clinical features, response to treatment and prognosis of 50 cases of immunoblastic lymph-adenopathy are reviewed. Most of the patients presented with generalized lymphadenopathy, hepatomegaly and/or splenomegaly, and fever. Hyperergic reactions such as pruritus, skin rash or eosinophilia were frequent. Erythrocytic sedimentation rate was increased by differing amounts. In some cases there was a polyclonal increase in immunoglobulins, while in others there was a reduction. Proven hypersensitivity to a wide spectrum of drugs was present in nine cases. Prognosis is uncertain: almost half of the patients died within one to forty-two months, some perhaps as a result of massive chemotherapy and/or radiotherapy. Best management probably is symptomatic treatment alone or with small doses of corticoids or immunosuppressives, supplemented by antibiotics. It is concluded that immunoblastic lymphadenopathy represents a hyperimmune reaction and is not, despite the high death-rate, a true malignant lymphoma.
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PMID:[Immunoblastic adenopathy: clinical features, treatment and prognosis (author's transl)]. 113 24

The clinical and pathologic findings in 24 patients with "angio-immunoblastic lymphadenopathy with dysproteinemia" (AILD) are presented. The patients' ages ranged from 44 to 80 years, with a median age of 68 years. The disease has an acute onset. In many respects, the clinical presentation is suggestive of malignant lymphoma. Generalized lymphadenopathy was always present. Hepatomegaly was found in 20 patients, splenomegaly in 17, constitutional symptoms in 20 and skin rashes in nine. Twenty patients had anemia, with positive Coombs' test in eight of 14 tested. Polyclonal hypergammaglobulinemia was found in 17 of 22 patients. Two patterns of evolution were recognizable: (1) long survival (24 to 67 months) without treatment or after the administration of intensive combination chemotherapy; and (2) rapid progression (one to 19 months) regardless of the treatment given. Sixteen patients died; postmortem examination in 10 cases showed the cause of death to be attributable to severe infection in eight patients, to renal disease in one and to cardiovascular disease in one. No evidence of malignant lymphoma was seen in any of these autopsies. Histologically, the disease is systemic, with specific lesions in the lymph nodes. The spleen, liver, bone marrow, skin and lung are also involved, but the changes are less characteristic than in the lymph nodes. In the patients in whom sequential biopsies were performed, a trend toward restoration of the nodal architecture was observed. AILD is a clinical-pathologic entity in a spectrum of yet to be defined immune reactions. The clinical, laboratory and pathologic manifestations of AILD are consistent with an autoimmune disorder, in which a deficiency of the T-cell regulatory functions probably predisposes to an abnormal proliferative and autoaggressive reaction of the B-cell system. Surgical staging procedures do not appear to be indicated. Intensive cytotoxic treatment may be hazardous in some patients, precipitating their death, but long survival after such therapy has been observed in others. Supportive therapy and small doses of steroids appear to be a safer therapeutic approach.
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PMID:Angio-immunoblastic lymphadenopathy. Diagnosis and clinical course. 119 Feb 54

We report a case of malignant lymphoma in a 15-year-old male. He had erythema and nodular lesions, hepatomegaly, and splenomegaly with high fever for one month. Laboratory examination revealed a normal blood routine test, a few lymphomatic cells in the bone marrow, and histopathologic changes characteristic of malignant lymphoma in a skin biopsy. Two months later, a second bone marrow examination showed 90% prolymphoblasts. The peripheral blood count revealed WBC 108 x 10(9)/L; among these, prolymphoblasts accounted for 92%. The patient was diagnosed as acute lymphocytic leukemia, and died 2 days later. The diagnosis and transformation from malignant lymphoma to acute lymphocytic leukemia are discussed.
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PMID:[A case of acute lymphocytic leukemia transformed from malignant lymphoma]. 130 24

Multiple ultrasonographic (USG) evaluations of abdomen were performed in 97 patients: 30 with Hodgkin's disease (HD), 60 with non-Hodgkin lymphomas (NHL) and 7 with primary gastric lymphoma (PGL) before or during chemo- or X-ray therapy. In 33% of HD patients USG was normal, while in 63% splenomegaly, in 40% hepatomegaly and in 20% lymph node enlargement were observed. After therapy, in 57% USG was improved and in only 3% of patients worsening was observed. In NHL patients splenomegaly was observed in 70%, hepatomegaly in 60% and lymph node enlargement in 35%. During follow-up, in 49% of patients improvement and in 3% worsening was observed. In 5 patients with PGL no changes were observed, in further 2 patients in Stage IV stomach wall and infiltration of nearest lymph nodes was observed. USG evaluation of abdomen may be useful in staging and therapy monitoring of malignant lymphomas.
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PMID:Sonography in the diagnosis and therapy monitoring of lymphomas. 143 40

Hepatomegaly and deranged liver functions are common findings in reactive haemophagocytic syndrome (RHS). We report the findings of 12 fatal cases of RHS in which histological materials of the liver are available for study. The underlying diseases of these patients included lymphoma/leukaemia (6 cases), disseminated undifferentiated carcinoma of the ovary (1 case), disseminated nasopharyngeal carcinoma complicated by tuberculosis (1 case), adenovirus pneumonia (1 case), pneumococcal pneumonia (1 case), typhoid fever (1 case), and possible drug intoxication (1 case). Ten patients had involvement of the liver by the underlying disease process which contributed to the marked hepatic derangement. Non-specific reactive hepatitis, sinusoidal dilatation and steatosis resulting from systemic or local effects of the associated diseases and the haemophagocytosis also added to the high incidence of liver abnormalities. A diffuse Kupffer cell hyperplasia with haemophagocytosis is characteristic of the syndrome, as all the cases showed increased numbers of bland-looking histiocytes within the hepatic sinusoids and haemophagocytosis which was moderate to marked in 8 cases and mild in 4. Thus the finding of Kupffer cell hyperplasia with prominent haemophagocytosis in liver biopsy is indicative of an element of RHS and warrants clinical monitoring. Differential diagnoses of haemophagocytosis in liver are also discussed.
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PMID:Liver changes in reactive haemophagocytic syndrome. 147 7

Spontaneous lymphomas from a strain of hereditary cataract (CAC-nct/+) mice were examined by light and electron microscopy and by immunohistochemical reaction for the mouse heavy and light immunoglobulin chains. Lymphomas occurred in 28 out of 45 male cataract mice and in 34 out of 52 females at 25 to 65 weeks of age. All of the lymphoma-bearing mice showed an enlargement of the spleen and mesenteric lymph nodes, and some mice also had hepatomegaly. Morphologically, all tumors were composed of a mixed population of small and large cells. Neoplastic cells had features of follicular center cell lymphomas, such as scant to moderate amounts of cytoplasm and cleaved and/or round nuclei with a large nuclear-to-cytoplasmic ratio. Large cells were often admixed with small cells, and had vesicular nuclei with prominent nucleoli juxtaposed to the nuclear membrane. Intracytoplasmic eosinophilic inclusions were observed in occasional cells, but Golgi apparatus was poorly developed and rough-surfaced endoplasmic reticulum was scant, unlike those in plasma cells. C-particles were seen in all lymphoma-bearing mice by electron microscopy. Intracisternal A-particles were detected in some mice. Immunohistochemically, neoplastic lymphoid cells were positive for the kappa light chain and the surface/cytoplasmic immunoglobulin M. These results indicate that lymphoid cell neoplasms found in hereditary cataract mice originate from follicular center B cells.
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PMID:Spontaneous follicular center cell lymphomas of B cell origin in cataract mice. 158 91

Adult T-cell leukemia/lymphoma (ATLL) is an HTLV-I associated lymphoid malignancy frequently seen in Japan. Abdominal involvement in 40 patients with ATLL were assessed by ultrasonography and the findings seen in four clinical types, acute, chronic, lymphoma and smoldering, were compared. Splenomegaly was frequently found in the cases of acute and lymphoma types, and the sizes of the spleens measured by ultrasonography correlated well with the disease activity. Hepatomegaly was also found more frequently in acute and lymphoma types, and hepatosplenomegaly was proved to be due to the infiltration by ATL cells. Nodular lesions in spleen and liver and abdominal lymph node swelling were also found frequently in the lymphoma type but rarely in the other types. Ascites, pleural effusion, and pericardial effusion were found in the active stage of acute and lymphoma types. Ultransonography also could detect findings associated with therapies. Thus, ultrasonography studies were found to be very useful for assessing the clinical classification, examining various pathological conditions associated with ATLL, and monitoring the disease activity.
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PMID:Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types. 165 79


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