Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Autoimmune lymphoproliferative syndrome (ALPS) is a disease of childhood characterized by typical clinical and laboratory findings. Here we describe an adult patient presenting with lymph node enlargement and splenomegaly. Pathological examination of an adenopathy supported the diagnosis of malignant T-zone lymphoma. The patient was treated accordingly. 3 years later his child was diagnosed with ALPS. Therefore the diagnosis of the father's disease was reconsidered. Review of the slides and functional tests led to the diagnosis of ALPS in both father and son. ALPS should be considered as a possible differential diagnosis in adult patients presenting with rare types of T-cell lymphomas.
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PMID:Revision of the diagnosis of T-zone lymphoma in the father of a patient with autoimmune lymphoproliferative syndrome type II. 1052 11