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)

Three cases of juvenile chronic myeloid leukemia (JCML) are reported. The patients were aged 3-4.5 years and presented with generalized lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia, elevated white blood cell count with monocytosis, and high fetal hemoglobin level. Philadelphia chromosome was absent in two cases studied. The bone marrow showed myeloid hyperplasia with increased monocytoid cells and blasts. Biopsy or postmortem material available in two cases revealed malignant infiltration of lymph nodes, liver, spleen, lungs, intestines, and skin. The neoplastic cells ranged from cells with irregular nuclei possessing nuclear grooves to large blastic cells with round to lobulated nuclei and prominent nucleoli. They showed weak staining for acid phosphatase and nonspecific esterase and exhibited the immunophenotype EBM11+KiM1+KiM6+KiM8+CD4+HLADR+ S-100 protein+. The neoplastic cells of JCML therefore share features of dendritic cells and mononuclear phagocytes. The authors' findings show that JCML is a unique histiocytic malignancy in which S-100 protein is a useful marker.
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PMID:Juvenile chronic myeloid leukemia. A malignancy of S-100 protein-positive histiocytes. 317 74

An established cloned human renal carcinoma line RC-1, which has been continuously maintained in culture for several years and which produces erythropoietin, was injected s.c. into BALB/c athymic mice and produced tumors. Tumorigenicity was directly correlated with the number of RC-1 cells inoculated. Tumor cell histology resembled the original patient-derived tumor. Tumor-bearing mice developed hepatosplenomegaly and significant reticulocytosis with elevated hemoglobin and hematocrit values that were proportional to tumor mass. In addition, red cell mass and blood volume of nude mice increased over 100% as compared to control mice or to animals bearing nonrelevant neoplasms. Large amounts of immunoreactive erythropoietin could be extracted from the nude mouse RC-1 tumors. These results indicate that the RC-1 cell line is tumorigenic and produces biologically active erythropoietin in vivo in athymic mouse hosts, thus providing a reproducible model to study ectopic erythropoietin production and its regulation in vivo.
Cancer Res 1988 Jun 15
PMID:Erythropoietin-induced polycythemia in athymic mice following transplantation of a human renal carcinoma cell line. 337 Jun 40

The early phase of the disease in 30 children referred to this Clinic with the diagnosis of rheumatic disease and whose condition was ultimately diagnosed as malignancy was analysed. A correct diagnosis was established after a period of 1-22 months. thus acute leukemia, non-Hodgkin's lymphoma, Hodgkin's disease and solid tumours were diagnosed in 13, 7, 3, 7 consecutive patients. It was demonstrated that in an early stage of neoplastic diseases, signs may occur which are typical also of rheumatic diseases. Fever, arthralgia or arthritis, hepatosplenomegaly, lymphadenopathy and signs of cardiac involvement predominated in the early picture of the disease. The need for diagnostic investigations ruling out neoplastic diseases has been emphasized. They should be performed even in children fulfilling the diagnostic criteria for rheumatic diseases. Attention has been called to the importance of carefully performed hematological and morphological analysis.
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PMID:Differential diagnosis of neoplastic and rheumatic diseases in children. 348 89

A 66-year-old man with a 6-month history of sweating at night, generalized lymphadenopathy, hepatosplenomegaly, and paraproteinemia was diagnosed to have a Stage IV mantle zone lymphoma (MZL), which behaved aggressively. The neoplasm rapidly disseminated to extranodal sites--the skin, lungs, pleural cavity, and the central nervous system. The neoplasm did not respond to initial double-agent chemotherapy, but it did partially respond to multi-agent chemotherapy. In addition, the neoplasm had histopathologic features not reported previously in MZL--vascular invasion, massive extranodal infiltration, high mitotic count, and convoluted nuclei.
Cancer 1987 Dec 01
PMID:High-grade mantle zone lymphoma. 367 7

A number of cases of sinusoidal hematolymphoid malignancy ("malignant histiocytosis") in which early lymph node biopsies have not allowed clear separation from benign histiocytic disorders have been observed. Nine such cases in which only a follow-up lymph node biopsy documented their malignant nature are reported. Seven of the nine patients were under 21 years of age; there was an even sex distribution. All had localized or generalized lymphadenopathy and three had hepatosplenomegaly at initial presentation. Histologically, the initial lymph node biopsy specimens showed an atypical sinusoidal proliferation, often with hemophagocytosis, but without sufficient atypia to allow a diagnosis of malignancy. The analysis of the follow-up biopsies, performed from 6 weeks to 5 years after the initial biopsy, showed a progression in histology with an increase in atypia, a greater tendency to efface lymph node architecture, and a decrease in hemophagocytosis. All patients were treated with chemotherapy. Six patients ultimately died; at autopsy, these patients showed a pattern of organ involvement typical of what has been described for malignant histiocytosis.
Cancer 1986 Oct 15
PMID:Sinusoidal hematolymphoid malignancy ("malignant histiocytosis") presenting as atypical sinusoidal proliferation. A study of nine cases. 375 90

Cancer is the second leading cause of death in children and, among cancers, lymphoma is the third most frequently diagnosed type. The majority of these are non-Hodgkin's lymphomas (NHL). Although NHL have been grouped in a variety of confusing ways, recent research in cytogenetics and immunology, coupled with a better understanding of the normal pathways of lymphoid differentiation, has greatly lessened this confusion. Burkitt's lymphoma (BL) is a type of B-cell NHL which is sub-classified as either endemic (African) or non-endemic (American). Kinetic studies have demonstrated a doubling time of only 24 h. As a result of this extremely rapid growth, symptomatic complaints occur early in the disease course. Structures contiguous to the primary become compressed and metabolic derangements occur. Signs of chronic debilitation such as cachexia, diarrhea, generalized lymphadenopathy, and weight loss usually are not found. Thirteen cases of American BL have been treated since 1980 at the Texas Children's Hospital and form the basis of this review. Ten patients had obstructive abdominal symptoms including constipation and pain, and 3 had masses in the neck, maxilla, or tonsil. Systemic manifestations included ascites, jaundice, fever, and hepatosplenomegaly. Two patients ultimately developed central facial nerve paralysis.
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PMID:Burkitt's lymphoma. 381 93

The clinical and pathologic findings in 13 cases of malignant lymphoma involving the prostate gland were reviewed. The lymphomas tended to occur in elderly men with a mean age of 60 years (range, 30-86 years) and were clinically manifested by prostatic enlargement with urinary obstruction. In only one of the patients was there clinical suspicion of lymphoma before surgery. Seven patients had primary extranodal lymphoma of the prostate, with a variety of histologic subtypes, including small cell lymphocytic (one patient), diffuse small cleaved cell (two patients), diffuse mixed small and large cell (two patients), diffuse large non-cleaved cell (one patient), and high-grade diffuse small non-cleaved cell (undifferentiated non-Burkitt's) (one patient). At the time of presentation, none of these patients had hepatosplenomegaly, inguinal lymphadenopathy, abnormal complete blood counts, or elevated serum acid phosphatase levels. Six other patients with previously documented malignant lymphoma at other sites had prostatic involvement 2 to 60 months (mean, 14 months) after the primary diagnosis. Histologically, these secondary prostatic lymphomas included diffuse small cleaved cell (two patients), diffuse mixed small and large cell (one patient), diffuse large non-cleaved cell (two patients), and large cell immunoblastic, polymorphous type (T-cell by immunotyping) (one patient). The mean survival was 14 months for all patients (range, 2-44 months), with no apparent difference between primary and secondary involvement. One patient remains alive 44 months after secondary prostatic involvement with diffuse large non-cleaved cell lymphoma. Although malignant lymphomas involving the prostate are rare, they should be included in the differential diagnosis of lower urinary tract obstruction, particularly in patients with a previous history of lymphoma.
Cancer 1985 Dec 15
PMID:Malignant lymphomas involving the prostate. A study of 13 cases. 384 Apr 6

Between 1971 and 1981, 699 children were diagnosed to have acute lymphoblastic leukemia (ALL) in Hungary. 34 of these children had received prednisolone therapy prior to the establishment of the diagnosis. The most frequent presumptive diagnoses that prompted steroid treatment were aplastic conditions and arthritic disorders. Leukemia was diagnosed when the presenting symptoms reappeared usually several weeks after the initiation of steroid therapy and often following withdrawal of the drug. Initial leukemic burden, as judged by leukocyte count and hepatosplenomegaly, was smaller in these patients than in other children with leukemia at the time of diagnosis. Although they entered remission at the same rate as the other patients, the length of continuous complete remission was significantly shorter in the prednisolone pretreated group. It appears that prolonged prednisolone therapy given before remission induction imparts a distinct unfavorable prognosis.
Cancer 1985 Apr 15
PMID:The adverse effect of prolonged prednisolone pretreatment in children with acute lymphoblastic leukemia. 385 64

A retrospective review of all 115 infants less than 1 year of age with acute lymphoblastic leukemia (ALL) entered on a consecutive series of recent Children's Cancer Study Group (CCSG) leukemia protocols was undertaken to examine in detail the outcome and clinical course of a large group of similarly treated infants. In comparison to the 4,392 children older than 1 year, entered on the same studies, infants had a significantly (P = .0001) increased incidence of leukocytosis, hepatosplenomegaly, meningeal leukemia at presentation, hypogammaglobulinemia, and failure to achieve complete remission (CR) status by day 14 of induction therapy. In contrast, lymphadenopathy, non-L1 French-American-British (FAB) morphology, mediastinal mass, and T cell leukemia were not more frequently observed. Ninety percent of these infants successfully completed the induction phase of therapy. With a median follow-up of 35 months, life table estimate of disease-free survival is only 23% at 4 years. Identical disease-free survival rates for infants were observed in each of the individual studies reviewed. Excessive toxicity resulting in limitation of therapy delivered was not a causative factor for the disappointing outcome of these patients. Rather, early disease recurrence, characterized by bone marrow relapse (55%) and CNS (22%) relapse, was the major factor responsible for the extremely poor prognosis of this patient group. Identical CNS relapse rates were observed in those patients who received cranial irradiation as part of CNS prophylaxis (21.8%) and in those patients who did not receive cranial radiotherapy (24%). Results of salvage therapy for patients who experienced systemic or extramedullary relapse were dismal. Debilitating neuropsychologic sequellae, presumably related to CNS irradiation, have been observed in 50% of the small number of long-term survivors. Infants less than 1 year of age with ALL present with a constellation of features which predict a poor outcome and constitute the group of children with ALL at greatest risk for treatment failure.
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PMID:Acute lymphoblastic leukemia in infants less than one year of age: a cumulative experience of the Children's Cancer Study Group. 386 94

A 38-year-old black man with giant lymph node hyperplasia (GLH), osteoblastic lesions, and the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) was treated at the University of Chicago Hospitals. The patient had hepatosplenomegaly and generalized peripheral lymphadenopathy. Endocrinologic abnormalities included decreased testosterone with elevated luteinizing hormone and follicle-stimulating hormone, as well as hyperprolactinemia and possible hypothyroidism. Biopsy of a right femoral lymph node revealed GLH, and an osteoblastic pelvic lesion showed a marked lymphoplasmacytic infiltrate. By immunohistochemical techniques, plasma cells in the lymph node and osteoblastic lesion were polyclonal. A polyclonal hypergammaglobulinemia was present. The lymph node T-lymphocyte population showed a decreased helper-to-suppressor cell ratio. Other findings included thickening of the skin, finger clubbing, and anasarca. A severe sensory-motor polyneuropathy was the major factor contributing to the patient's death. The association of GLH, osteoblastic bone lesions, and the POEMS syndrome has been noted previously in Japan; however, the authors are unaware of reports on Western patients who had this combination of clinical and laboratory findings.
Cancer 1985 Jul 01
PMID:Giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki's) syndrome. 389 Oct 64


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