Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have had the opportunity to study a case of Chediak-Higashi syndrome (CHS) in the accelerated phase that was associated with Epstein-Barr virus (EBV) infection. The clinical course of a 12-year-old boy was characterized by fever, lymphadenopathy, hepatosplenomegaly, and pancytopenia. However, in the terminal stage, the appearance of an atypical lymphoblastic leukocytosis was morphologically indistinguishable from acute lymphocytic leukemia, accompanied by benign histiocytosis with hemophagocytosis. Autopsy examination revealed an atypical lymphoid infiltration favoring EBV infection as the primary diagnosis. This case underscores the fatal consequences of EBV infection in CHS.
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PMID:Epstein-Barr virus infection in Chediak-Higashi syndrome mimicking acute lymphocytic leukemia. 216 46

A 5 year old German girl contracted visceral leishmaniasis during a vacation in Spain, either 32 or 20 month prior to the manifestation of disease. She presented with fever, hepatosplenomegaly and pancytopenia. A bone marrow aspirate proved the diagnosis. Therapy with a pentavalent antimony drug brought about immediate improvement. Visceral leishmaniasis has to be suspected in individuals with fever, hepatosplenomegaly and pancytopenia who have resided in endemic areas (Mediterranean countries, India, East Africa, South America) during the previous years. If untreated, visceral leishmaniasis runs a fatal course. Therefore, early diagnosis by morphological and serological means and specific therapy with pentavalent antimony drugs are mandatory.
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PMID:[Visceral leishmaniasis. Personal observation and review of epidemiology, clinical aspects and therapy]. 217 Jul 39

A 53-year-old woman was admitted to our hospital on Nov. 16, 1987, because of general fatigue. On admission, she had hepatosplenomegaly and her peripheral blood profile showed a white blood cell count (WBC) of 309 x 10(3)/microliters with immature neutrophils, a hemoglobin level (Hb) of 7.6 g/dl, platelet count (PLT) of 536 x 10(3)/microliters, neutrophilic alkaline phosphatase (NAP) score of 44. Both Vitamin B12 and LDH levels were high. The bone marrow showed marked myeloid hyperplasia. In a cytogenetic study, Ph1 was found in 3 of 8 metaphases and Ph1 with an additional abnormality of 8 trisomy was noted in 5 of 8 metaphases. She was diagnosed as having chronic myelogenous leukemia (CML) and treated by i.m. injection of interferon (IFN)-alpha at a daily dose of 6 x 10(6) U. Administration of IFN-alpha induced fever for a few days. WBC, PLT count and LDH level gradually decreased, and the NAP score and hepatosplenomegaly improved. She achieved remission in February, 1988. Administration of IFN-alpha was stopped in April, 1988, when the bone marrow showed hypocellularity and normal karyotype. She was treated with 20 mg of prednisolone daily from May until August, because of progressive pancytopenia. She had received no treatment until July, 1989. In May, 1989, the bone marrow again showed myeloid hyperplasia and Ph1 was found in all cells analyzed. Therefore, we resumed IFN-alpha treatment. It is interesting that remission of CML continues for more than one year after discontinuation of IFN-alpha in this case.
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PMID:[One-year remission of chronic myelogenous leukemia (CML) after discontinuation of interferon-alpha]. 221 81

We report a 47-year-old man diagnosed as angio-immunoblastic lymphadenopathy with dysproteinemia (AILD) with fibrosis of the bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph node. He was admitted to a hospital in May, 1980 because of general fatigue, cough, fever and systemic lymphadenopathy. The diagnosis of AILD was based on a biopsy of right cervical lymph node. His symptoms were improved but recurred with the addition of icterus and progressive pancytopenia with decrement of prednisolone. He was referred to our hospital in July, 1980 and his physical examination revealed generalized lymphadenopathy, icterus and hepatosplenomegaly. Hemogram showed pancytopenia, and needle biopsy of the bone marrow disclosed fibrosis. Sections from the lymph node showed AILD with proliferation of epithelioid cells. Administration of 60 mg/day of prednisolone improved the fever, lymphadenopathy and hepatosplenomegaly. However he died suddenly of acute respiratory failure on July 30. Autopsy showed fibrosis of bone marrow, lymph node, liver and spleen with infiltration of abnormal lymphocytes, and pulmonary aspergillosis.
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PMID:[Angio-immunoblastic lymphadenopathy with fibrosis of bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph nodes]. 221 92

We report a case of extranodal T-cell lymphoma with fever, hepatosplenomegaly, pancytopenia, and diffuse sinusoidal infiltration of the spleen, liver, and bone marrow by the tumor cells, mimicking malignant histiocytosis. This is the second case of T-gamma (T-cell suppressor) lymphoma resembling the case reported by Kadin et al. [N Engl J Med 304:648, 1981]. The lack of lymph node involvement in this case supports the theory that this type of lymphoma arises in the spleen. This paper draws attention to the extranodal T-cell lymphoma groups that mimic malignant histiocytosis and the need of immunophenotyping for a correct diagnosis. The causes for the absence of T-cell receptor gene rearrangement in T-cell tumors are discussed.
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PMID:Extranodal T-cell lymphoma mimicking malignant histiocytosis. 223 22

Disorders of the histiocyte-macrophage system in childhood comprise a manifold variety of clinical entities. The basic immunopathological mechanisms have still not been clarified in detail. We report on a 15 months old Yugoslavian boy of non-consanguineous parents, who developed persistent high temperatures and bloody diarrhoea at the age of 11 months, followed by a rapidly progressive dystrophy, hepatosplenomegaly and peripheral pancytopenia. An extensive virological, bacteriological and protozoal diagnostic program failed to reveal a pathological result. A bone marrow aspiration showed an extremely hypocellular marrow without infiltration of pathological cell populations. In spite of extensive supportive care and systemic steroid therapy, the patient died by bleeding complications due to severe untreatable thrombocytopenia. In post-mortem-biopsies a marked infiltration of RES-organs (lymph nodes, liver and spleen) by mature histiocytes, showing marked hemophagocytosis, was seen.
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PMID:[Hemophagocytic syndrome in childhood]. 226 13

The authors report on two examples of peripheral T-cell lymphoma associated with hemophagocytic syndrome. The latter represents a recently described entity, which is characterized by pancytopenia, hepatosplenomegaly, fever and rapid downhill clinical course. Microscopic examination of different tissue samples shows bone-marrow, liver and spleen infiltration by small-medium sized lymphoid elements, which display peripheral T-cell phenotype and are associated with an exuberant hyperplasia of hemophagocytosing, benign-looking histiocytes. The clinical and pathological findings, along with the main differential diagnoses, are discussed.
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PMID:[Peripheral T-cell lymphoma associated with hemophagocytic syndrome: a recently identified entity. Clinico-pathologic and immunohistochemical study of 2 cases]. 228 38

This rare fatal disease of infants and early childhood occurred in seven children from four families. Six children died during 2-4 weeks from the beginning of disease, the last one has survived two years with cytostatic treatment. The disease was characterized by intermittent fever, hepatosplenomegaly and progressive pancytopenia as well as hyperbilirubinemia, hyperlipemia and hypofibrinogenemia. In addition to substantial enlargement of the liver and spleen prevailing autoptic findings were infectious complications due to severe immune defect or signs of hemorrhagic diathesis. Lymphocytes and histiocytes phagocytizing blood cells were increased in bone marrow, liver, spleen, often brain and further organs. Problems of differential diagnosis and pathogenesis were discussed.
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PMID:[Familial hemophagocytic lymphohistiocytosis]. 235 Aug 12

59 year old female was admitted to Nagoya Memorial Hospital for anemia unknown etiology after the work up of the gastrointestinal tract. Peripheral blood count at admission was as follows: WBC 2,400/microliters, RBC 321 X 10(4)/microliters, Hb 9.8 g/dl, Ht 30.1%, Plt 8.2 X 10(4)/microliters, which showed pancytopenia with normocytic, normochromic anemia. She had no hepatosplenomegaly, vitamin B12 nor folate deficiency. Bone marrow was hyperplastic and showed trilineage megalodysplastic changes. The diagnosis of myelodysplastic syndrome (Refractory anemia) was made. Progenitor assay showed no colony formation of BFU-E but showed normal growth of CFU-GM colony and cluster. She had chromosomal abnormality of 47, XX, + 11. Administrated anabolic steroid, prednine and activated vitamin D3 were not effective and she died of brain hemorrhage in April 1987. Colony assay at this stage showed numerous leukemic clusters and no normal colonies. Re-performed chromosome assay showed 47, XX, + 11. There are only a few reports of trisomy-11 in a patient with MDS. Especially we could follow this case till her leukemic transformation by colony assay.
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PMID:[Abnormal cluster formation in a patient with myelodysplastic syndrome with trisomy-11--periodical approach by colony assay]. 236 44

A case of familial hemophagocytic limphohistiocytosis (FHLH) is presented in a 16 months old infant, with clinical picture of prolonged fever, cutaneous purpura, generalized lymphadenopathy and progressive hepatosplenomegaly and laboratory of severe pancytopenia, hypofibrinogenemia and hypertriglyceridemia, with elevation of the pre-beta fraction in the lipidogram, all this compatible with this disease. She also showed bone marrow and ganglionar biopsy infiltrated by histiocytic cells with hemophagocytosis. The patient received chemotherapy with cycles VP-16, vincristine (VCR) and intrathecal methotrexate (MTX), alternating every two-three weeks with VACP, during one year, with resolution of clinical and laboratory parameters after two months of this treatment without relapses. The survival from diagnosis is 39 months.
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PMID:[Familial hemophagocytic lymphohistiocytosis: survival of a case treated by polychemotherapy]. 236 5


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