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)

The case of a 57-year-old man with lymphadenopathy fever, splenomegaly, and polyclonal gammopathy is described. The difficult clinical course, the short lived response to cytotoxic treatment, the frequent infections, the immunologic abnormality, and the histologic findings in lymph node biopsy and postmortem material justified our diagnosis of angioimmunoblastic lymphadenopathy with dysproteinemia, a new entity described recently.
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PMID:Angioimmunoblastic lymphadenopathy: a case study. 99 Oct 79

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a disease of unknown etiology and pathogenesis. It has the features of hyperimmunity and immune deficiency, and its behavior resembles malignant lymphoma. We report a review of 16 patients with AILD seen at Ramathibodi Hospital from 1982 to 1986. Thirteen patients had fever and seven had pruritus and rashes. Lymphadenopathy was found in all 16 cases; generalized in 14 and localized in 2. Hepatomegaly was present in 14 patients while only 7 had splenomegaly. Laboratory findings included autoimmune hemolytic anemia, lymphocytosis and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in 5 cases, and bone marrow showed the characteristic features of the disease in 9 cases. Two patients went on to develop diffuse lymphocytic, poorly differentiated lymphoma. Fourteen patients were treated with prednisolone initially. Five responded with complete recovery, eight responded with partial recovery, and one died with extensive involvement of the disease. Six of the patients that recovered partially were later treated with cyclophosphamide, vincristine and prednisolone. One patient recovered completely and two partially. Three died from extensive involvement. Two patients with malignant lymphoma were treated by combination chemotherapy. One case went to complete remission while the other died from infection. One patient was lost to follow up before any treatment was started.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia in Thailand. 332 19

A 13-year-old girl presented with doughy swelling of both hands, a pruritic maculopapular rash, fever, malaise, lymphadenopathy and splenomegaly. Examination of an enlarged cervical lymph node revealed typical histopathological features of AILD. Partial remission was achieved by treatment with prednisolone. During the next 4 years 3 flare-ups of the disease could be controlled by corticosteroids, one going along with an enlargement of the right tonsil, histologically proven as an AILD manifestation, the other two with generalized lymphadenopathy. At the age of 17 years, an acute reduction of visual acuity occurred in both eyes in the absence of lymphadenopathy or cutaneous manifestations. Plasma viscosity was elevated in connection with high levels of IgM and IgG. Fundoscopy revealed papilledema compatible with hyperviscosity syndrome. Plasmapheresis resulted in a slow recovery of vision when suddenly a bilateral loss of vision occurred. MRI at this time revealed a lesion within the optic chiasm and additional high-signal lesions in parietal white matter of brain. All of these regions showed enhancement after i.v. application of Gd-DTPA. Again, high-dose corticosteroids were introduced and a partial restoration of vision could be achieved. This was paralleled by an improvement of the changes on followup MRI examinations. The last examination ten months after onset of CNS complications revealed a single small hyperintense residual area positioned in left parietal white matter. Enhancement of contrast medium was absent. These cerebral and retinal complications are so far undescribed complications of AILD which occurred in a childhood case.
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PMID:CNS complications in a girl with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). 889 75

A 66-year-old woman was admitted with high fever, systemic lymphadenopathy, hepatosplenomegaly and pancytopenia. Bone marrow aspiration showed infiltration of atypical lymphoid cells and hemophagocytic histiocytes. The findings of lymph node biopsy were compatible with angioimmunoblastic T-cell lymphoma (AILD). A diagnosis of lymphoma-associated hemophagocytic syndrome (LAHS) was made. Treatment with the THP-COP regimen achieved clinical remission except for mild splenomegaly, but relapse of LAHS was confirmed two years after diagnosis. The patient's condition deteriorated rapidly, and she died of multi-organ failure one month later. Autopsy revealed extended, diffuse infiltration of lymphoma cells in almost all organs. Numerous macrophages showing phagocytosis of erythrocytes and nucleated cells were found in the adrenal glands, lungs, bone marrow, spleen and liver. Epstein-Barr virus genomes were not detected by in situ hybridization, but cytotoxic molecules were immunostained with TIA-1 and granzyme B in the lymphoma cells. Elevated serum levels of sIL-2R, IFN-gamma, IL-6 and M-CSF were found at the onset and relapse of lymphoma. Overproduction of these cytokines was considered to have contributed to the pathogenesis of HPS.
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PMID:[Angioimmunoblastic T-cell lymphoma associated with hemophagocytic syndrome at onset and relapse]. 1120 Nov 51