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)

An infant was vaccinated at the age of 3 days with BCG vaccine. At the age of 3 years 10 months he developed an infection by Salmonella typhimurium. The infection persisted with recurrent episodes of fever, peri-nephritic abscess, abscesses of lymph nodes, hepatomegaly, splenomegaly and paravertebral and retro-peritoneal abscesses, from which Salmonella were isolated. At the age of 6 years and 2 months later. At post-mortem examination there were widespread histiocytic nodules in many organs, from which Mycobacterium bovis BCG were cultured. One previous case has been reported from Czechoslovakia. The mother of that child was the sister of the father of the child reported here. It was not possible to investigate the possibility of abnormalities of humoral or cellular immunity in the family.
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PMID:Fatal generalized BCG histiocytosis. 32 38

We have studied immunologic reactivity to leukemia-associated antigens in patients with chronic myelocytic leukemia (CML) treated with chemotherapy and adjunctive immunotherapy. All patients were immunologically competent as measured by skin test reactivity to dinitrochlorobenzene. Immunotherapy consisted of allogeneic irradiated leukemic myeloblasts injected intradermally, with BCG vaccine (Research Foundation, Chicago, Ill.) given by multiple puncture at the same site. 10(9) cells plus BCG were given weekly for 4 wk, and 10(8) cells plus BCG were given at monthly intervals thereafter. Eight patients judged clinically to be in the stable phase of their disease developed circulating antibody against the immunizing blast cells demonstrable by cytotoxicity and immunofluorescence assays. The antibody also showed reactivity against a panel of myeloblasts (12 paients) but not against the corresponding remission lymphocytes (five patients) or normal lymphocytes (20 donors). In two cases the antibody showed reactivity against the patient's own leukemic blasts. Seven of these eight patients have maintained a steady clinical course ranging from 20 to 40 mo, while one entered the blastic phase and died. Six patients were judged to be in the aggressive phase of CML because of progressive leukocytosis and splenomegaly or increasing myeloblastosis; five died an average of 16 mo after diagnosis. Humoral antibodies were not detected in these patients after repeated courses of BCG and allogeneic leukemic cells. We conclude that specific active immunotherapy of patients with CML can abet the production of humoral antibody against blast cell antigens and that this response may be impaired during the aggressive phase of the disease.
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PMID:Antibody responses to leukemia-associated antigens during immunotherapy of chronic myelocytic leukemia. 106 Apr 71

In a 3-year-old girl, who was admitted to our hospital with marked splenomegaly, a swollen abdomen and progressive loss of weight, the difficulty in establishing the correct diagnosis, namely a very rare generalized infection with Mycobacterium avium (serum type III), and the lengthy course of the disease are demonstrated. Norcardiosis, sarcoidosis and BCG-granulomatosis were excluded. From the spleen, lymphatic nodes, gastric juice, stools and urine of the child, who had not been vaccinated with BCG vaccine, masses of acid-fast bacilli were isolated. The generalisation of the disease was promoted by an immunological deficiency. At autopsy an atrophic thymus was discovered. The extensive clinical and immunological investigations pointed to a partial deficiency of the cell-mediated immune systeme. The patient died 8 months after admission.
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PMID:[Generalized mycobacterium avium infection in an infant (author's transl)]. 123 56

In this case report a rare adverse event (mild toxic epidermic necrolysis (Lyell)) was described. Incorrect a purified protein derivative (PPD) administration involves false negative tuberculin test (TT): BCG vaccine was injected even though high immunization to BCG. Mild, benign epidermic necrolysis, fever, mononucleosis-like syndrome, splenomegaly and lymphadenopathy were observed. Evaluation of white blood cells was done by automatic (simultaneously two analysers: Baker 900 plus and Technicon, Bayer) and by microscopic methods and revealed high lymphocyte activation (blastic transformation), lymphocytosis and high eosinophilia.. Wiener and coworkers describe interleukin-2-induced dermatotoxicity resembling toxic epidermal necrolysis. Further the most common side effects of IL-2 are skin eruptions and eosinophilia. Careful analysis yielded the conclusions that Lyell's syndrome may be fatal consequence of inappropriate revaccination, hyperergia--delayed type hypersensitivity and massive IL-2 release. Successful dexamethasone therapy confirms this observation.
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PMID:[Mild form of Lyell's syndrome as an consequence of inappropriate BCG revaccination--case report]. 1569 Jul 9