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)

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