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)

A 57-year-old female presented with general fatigue. She had neither lymphadenopathy nor hepatosplenomegaly. Laboratory data revealed anemia and leukopenia (1,500/microliters) with a differential count of 4.5% leukemic cells. The myelogram revealed 34.4% leukemic cells, of which diameter ranged from 20 to 28 microns. The diagnosis was acute myelogenous leukemia (FAB: M2) with myelodysplasia. Cytogenetic analysis revealed that the leukemic cells had chromosome abnormalities involving both diploidy and tetraploidy with structural rearrangement. Structural rearrangement included del(5) (q22q33), del(15) (q22q24), and t(3; 12) (q25;p13). Small dose aclacinomycin-A treatment was effective in reducing the number of leukemic cells in bone marrow, and both anemia and leukocytopenia were improved.
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PMID:[Acute myelogenous leukemia transformed from myelodysplastic syndrome with tetraploid chromosome constitution]. 160 14

Patients with typhoid or paratyphoid fever, admitted to 14 hospitals for infectious diseases during 1984-1987, were epidemically and clinically studied. Of the total number of 183 typhoid, 49 paratyphoid fever patients, those infected overseas was 44.3% and 71.4% respectively, giving an overall annual decrease, yet marking an increased ratio of overseas infection. Patients aged 20s-30s and males were dominant. One hundred and seventy six cases (96.2%) of typhoid and all the paratyphoid fever cases were bacteriologically diagnosed. The period from the onset to the diagnosis was around 14 days in most cases, but beyond 29 days in over 10% of the cases. We would like to emphasize that enteric fever, focusing on high fever, bradycardia, roseola, hepatosplenomegaly, leukopenia, elevated serum-GOT GPT and LDH, can be easily diagnosed by blood/stool culture before beginning chemotherapy. Intestinal bleeding was recognized in 24 cases (13.1%) of typhoid and 4 (8.2%) of paratyphoid fever, intestinal perforation in 2 (1.1%) and death in 1 (0.5%) of typhoid fever. CP was most commonly used in chemotherapy. Bacteriological relapse was recognized in 7/127 cases (5.5%) of typhoid, 6/48 (13.0%) of paratyphoid fever those followed beyond 3 weeks, though eradication was attained by retreatment. One strain of S. typhi resistant to CP.ABPC.KM.SM was isolated in 1986 from a patient infected overseas. New quinolones seem reliable in our preliminary studies.
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PMID:[Clinical research on patients with typhoid and paratyphoid fever (1984-1987). Research Group for Infectious Enteric Diseases, Japan]. 191 2

Two patients with benign cytophagic histiocytic panniculitis are presented and compared with the one benign and ten fatal cases previously reported. Benign-appearing histiocytes which engulf blood cells, nuclear debris, and platelets are the most characteristic feature of the disease. This cytophagia tends to be massive in the subcutaneous tissue and involves extracutaneous organs only in the fatal cases. The course is chronic in the benign form. Patients with the fatal form tend to have fever, hepatosplenomegaly, serosal effusions, ecchymoses, peripheral adenopathy, and mucosal ulcers. Anemia, leukopenia, elevated liver enzyme levels, and coagulopathy are present in almost all and hypocalcemia in many patients with a fatal course. Cytophagic histiocytic panniculitis may represent a form of regional histiocytosis primarily involving subcutaneous tissue. It is midway in the spectrum of the cytophagic histiocytoses.
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PMID:Cytophagic histiocytic panniculitis is not always fatal. 276 94

A 62-year-old man was admitted to our hospital with exertional dyspnea. On admission, neither hepatosplenomegaly nor lymphadenopathy were noted. Laboratory data revealed anemia (Hb, 4.8 g/dl), leukopenia (2,800 microliters) and a normal platelet count (21 X 10(4)/microliters). The immature blast cells in the peripheral blood were 15%, which increased to 32% during his clinical course. On cytochemical studies, the blast cells had no staining with peroxidase, alpha-naphthyl-butyrate esterase and PAS, although acid phosphatase was positive. More than 58% of the blasts were identified as being of megakaryocytic lineage by platelet peroxidase and by tests with monoclonal GP IIb/IIIa antibody. Bone marrow biopsy disclosed marked fibrosis. However, the patient constantly had normal counts of platelets ranging from 21 X 10(4) to 63 X 10(4)/microliters. This case provides evidence that the megakaryocytic leukemias can be categorized into two types, which are characterized by either undifferentiated or differentiated megakaryocytic leukemia cells.
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PMID:[Megakaryocytic leukemia with thrombocytosis]. 281 Jul 95

In a clinical and serological study performed on a large series of patients with different connective tissue disorders, only sera from patients with systemic lupus erythematosus (SLE) contained two precipitin systems (DA1 and DA2) directed against nuclear proteins present in a total extract of human spleen. DA1 antibody appeared in 5% of SLE patients and was always associated with DA2 antibody which was found in about 22% of SLE patients. Sera monospecific for DA1 and DA2 antibodies gave a speckled staining pattern on both rat kidney and HEp2 cells. DA1-positive patients showed a higher incidence of photosensitivity (p less than 0.001), hepatosplenomegaly (p less than 0.001), hemolytic anemia (p less than 0.05), CNS involvement, vasculitis, and diffuse proliferative glomerulonephritis than DA1-negative ones. DA2-positive patients showed an increased incidence of leukopenia (p less than 0.05) and thrombocytopenia (p less than 0.01) than controls. While several indirect evidences suggest that DA1 precipitin might be identical to the previously described MA antibody, DA2 precipitin appears as a new system in SLE. The serological association between DA1 and DA2 antibodies might be analogous to the well known ones between antibodies to Sm and nRNP and to SSA/Ro and SSB/La.
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PMID:Detection of two associated precipitating autoantibodies (DA1 and DA2) in sera from patients with systemic lupus erythematosus. 326 26

A 32-year-old male was hospitalized with high fever, pancytopenia and hepatosplenomegaly. No atypical cells were found in the peripheral blood. Bone marrow aspiration resulted in dry taps. Superficial lymph node swelling was not observed. He had been treated twice for high fever, hepatosplenomegaly and leukopenia that were very similar to the present illness, 13 and 3 years before the onset and hepatosplenomegaly had been noted by the patient for 13 years. The patient died after a rapid course of 20 days. Histiocytic medullary reticulosis (HMR) was diagnosed at the autopsy, which revealed atypical histiocytic infiltration showing erythrophagocytosis in the liver, spleen, left adrenal, and mesenterial and pulmonary hilar lymph nodes. This patient had shown the same clinical signs and hepatosplenomegaly 13 years before the onset of HMR, which suggest a possible latent stage and acute exacerbation of HMR.
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PMID:An autopsy case of histiocytic medullary reticulosis presenting with marked hepatosplenomegaly for 13 years before the onset. 341 86

Forty patients with brucellosis were hospitalized and clinical and laboratory findings of patients were recorded, treatment regimens were discussed. Twenty-five of the patients were males and 15 of the patients were females. Leukopenia 21.62%, anemia 27.02% and ESR elevation 83.87% were found. Fever and exhaustion were present all of the patients. Arthralgia, nonproductive cough, gastrointestinal symptoms, headache and night sweating were the other common symptoms. Hepatomegaly, splenomegaly, hepatosplenomegaly and ronchus were imported physical examination findings. First choice treatment solution was tetracycline-streptomycin combination and this combination succeed 89.19 in percent. Relapses were treated with rifampicin.
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PMID:[Brucellosis: clinical and laboratory findings and treatment in 40 patients]. 344 17

Four weeks after returning from a holiday on Ischia, a 53-year-old man fell ill with nocturnal sweating, fever, severe general malaise and hepatosplenomegaly. Sternal marrow and liver punctures revealed an infection with Leishmania donovani, i.e. Kala-Azar. Characteristic features were hyperplasia of the plasma cells in the bone marrow and liver, together with lymphoplasmocytoid cell wash-out and polyclonal IgG proteinaemia. Treatment with pentamidine (Lomidine) was unsatisfactory, while all manifestations of the disease regressed after two courses of sodium stibogluconate (Pentostam), although the hypergammaglobulinaemia and hyperproteinaemia disappeared only slowly. The plasma-cell hyperplasia in conjunction with the hypergammaglobulinaemia, which suggest multiple myeloma, is interpreted as a result of antibody formation by the chronic parasitic infection. Together with storage-cell hyperplasia it is also taken to be the cause of the peripheral leukopenia and thrombocytopenia.
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PMID:[Kala-azar originating in Ischia]. 365 55

Necrobiotic xanthogranuloma of the skin is associated with paraproteinemia and, often, with plasma proliferative disorders, including multiple myeloma. Other commonly recognized systemic abnormalities include hepatosplenomegaly, a highly increased erythrocyte sedimentation rate, and leukopenia. Fifteen of 16 patients (seven men and nine women with a mean age of 54 years) with this condition had ophthalmic manifestations. Thirteen patients had lesions affecting the skin of the eyelids and periorbital tissue; on casual examination these lesions resembled plane xanthoma. Unlike plane xanthoma, however, the lesions of necrobiotic xanthogranuloma were almost always indurated. Further, the lesions frequently became inflamed, leading to superficial ulceration. Deeper lesions occasionally involved the orbit. Yellow lesions were sometimes visible in the episcleral tissues where they were associated with recurrent symptoms of scleritis and episcleritis. Biopsy specimens of the skin lesions disclosed a distinctive pattern of subepidermal granulomatous masses with focal aggregates of histiocytes and giant cells surrounded by hyaline necrobiosis. Surgical excision of the eyelid lesions was often followed by recurrence and increased activity of the lesions. Low-dose chemotherapy is likely to produce a favorable response, with regard to both the skin lesions and the paraprotein abnormalities.
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PMID:Ophthalmic features of necrobiotic xanthogranuloma with paraproteinemia. 669 27

An autopsy case of a 42 year old man with the anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome) is reported. The patient was admitted because of a 1 month history of fatigue and fever. Physical examination showed hepatosplenomegaly. Laboratory data showed leukopenia, mild normocytic anemia, and high levels of serum lactate dehydrogenase and vitamin B12. Bone marrow aspirate revealed an elevated number of erythroblasts, with dyserythropoiesis (E/M = 3.7). After admission, thrombocytopenia progressed rapidly, but blast cells were not seen in the peripheral blood throughout the clinical course. On the 56th hospital day, the patient died of pneumonia. At autopsy, the spleen weighed 550 g and the liver 1800 g. Histologically, the white and red pulps of the spleen and the portal region and sinusoid of the liver were diffusely infiltrated by blast cells that were positive for anti-hemoglobin (Hb) antibody on immunoperoxidase staining. The bone marrow, the lymph nodes, the adrenal glands, the pancreas, and the heart were also infiltrated by the blast cells. This was thus considered to be a rare case of the anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome), the findings showing that Hb immunoperoxidase staining is useful for the diagnosis of this condition.
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PMID:Anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome) causing hepatosplenomegaly due to the infiltration of hemoglobin-bearing blast cells: an autopsy case. 755 Oct 2


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