Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty consecutive patients with blastic chronic myelogenous leukemia were evaluated clinically, morphologically, biochemically, and therapeutically. Forty-five patients had a preceding stable phase (38 Ph'+, 7 Ph'-); five patients presented with de novo Ph+ blast crisis. The most frequent clinical signs of impending blast crisis were weakness, fatigue, increasing splenomegaly, anemia, thrombocytopenia, marrow fibrosis, and a rising neutrophil alkaline phosphatase. Fever (unrelated to infection), skin infiltration, lymphadenopathy, hepatomegaly, thrombocytosis, and basophilia were much less common. The development of aneuploidy occurred in less than one-half of the total group. Myeloblastic morphology at blastic transformation was most frequent with occasional lymphoblastic, promyelocytic, and undifferentiated cases seen. Terminal deoxynucleotidyl transferase was present in one-third of the patients, but had no clear-cut relationship to the morphology. Response to treatment was generally disappointing (two complete and 15 partial remissions in 45 treated patients).
 ...
PMID:Blastic transformation in chronic myelogenous leukemia: experience with 50 patients. 27 33

Two cases of atypical Kawasaki disease are reported. Case 1 was a five-month-old male infant admitted to this hospital with a 10-day's history of high fever. On examination, he appeared ill-looking and only hepatomegaly was noted. Laboratory studies showed leukocytosis, thrombocytosis, elevated ESR and pleocytosis in CSF. He was treated as sepsis with meningitis. Sudden death occurred on the eighth day of admission, and left coronary artery aneurysm with thrombosis was noted at autopsy. Case 2 was a four-month-old male infant referred to our hospital with fever and cervical lymphadenopathy of 11 day's duration, and unresponsive to antibiotics. Skin rash had developed after oxacillin injection. Echocardiogram, performed on the third day of admission, disclosed a 5-8 mm aneurysm of the left coronary artery and a 4 mm aneurysm of the right coronary artery. Before a specific diagnostic test for Kawasaki disease becomes available, we suggest that a possible diagnosis of Kawasaki disease and echocardiographic evaluation should be considered in case of (1) presence of partial criteria of Kawasaki disease with thrombocytosis; and/or (2) young infants with prolonged unexplained fever.
 ...
PMID:Atypical Kawasaki disease: report of two cases. 151 14

During the twenty-nine-year period from June 1959 to October 1988, 32 infants and children with histology-proved hepatoblastoma were seen at the Department of Pediatrics, National Taiwan University Hospital (NTUH). The age at diagnosis ranged from four days to six years, with a mean of one year and eight months. Most of the patients (87.5%) were under the age of three. There was no sex predilection (16 males, 16 females). The most common presenting signs were an upper abdominal mass (71.9%) and/or abdominal distention (62.5%). Physically, all the patients revealed hepatomegaly, with or without mass. Laboratory abnormalities included primarily thrombocytosis (64.3%), elevated serum aspartate aminotransferase (75.0%), serum alanine aminotransferase (62.5%), cholesterol (78.3%) and alphafetoprotein (92.3%). According to the classification of Ishak and Glunz, 56.3% of these belonged to the epithelial type; the others belonged to the mixed type. During the follow-up period, 6 patients with complete resection of the tumor remained alive from 2 years to 17 years and 4 months. Twenty-two patients died from 8 days to 16 months after diagnosis. Four patients were lost to follow-up. The two-year survival rate was 21.4% (6/28). Complete resection of the tumor was the key treatment for achieving survival.
 ...
PMID:Hepatoblastoma in infancy and childhood: a clinical and pathological study of 32 cases. 164 97

Vasculitis may accompany rheumatoid arthritis. One must distinguish between vascular involvement associated with the pathogenesis of rheumatoid arthritis, isolated digital vasculitis, and the syndrome of clinical rheumatoid vasculitis. The cause of clinical rheumatoid vasculitis is unknown. High titers of rheumatoid factor, cryoglobulins, diminished circulating complement, an increased prevalence of HLA-DR4, and the pathologic findings suggest an immune etiology. However, similar, but perhaps less pronounced, abnormalities occur in uncomplicated rheumatoid arthritis, and these findings are not universal in complicating vasculitis. Classic cutaneous clinical manifestations include ischemic ulcers, digital gangrene, and palpable purpura. Mononeuritis multiplex is another classic presentation of rheumatoid vasculitis. Small digital infarctions may accompany other manifestations in clinical vasculitis or may occur alone as isolated digital arteritis, in which case the prognosis is relatively favorable. Weight loss, pleuritis, pericarditis, ocular inflammation, splenomegaly, hepatomegaly, and Felty's syndrome have also been reported in association with rheumatoid vasculitis. Although renal involvement has been considered unusual in rheumatoid vasculitis, several studies suggest that this may be more common than previously recognized. Ideally, a biopsy or an angiogram confirms the diagnosis of rheumatoid vasculitis, but often the diagnosis rests upon the clinical picture. In general, blind biopsies are not helpful, although one series indicated that a blind rectal biopsy may be an exception to this rule. An elevated erythrocyte sedimentation rate, increased C-reactive protein level, anemia, thrombocytosis, hypoalbuminemia, and a positive rheumatoid factor are common laboratory findings. Leukocytosis, hypergammaglobinemia, leukocytopenia, an elevated creatinine level, and minimal abnormalities of the urinary sediment also occur in patients with rheumatoid vasculitis. However, these abnormalities overlap in patients with uncomplicated rheumatoid arthritis, and their role in distinguishing rheumatoid vasculitis from uncomplicated rheumatoid arthritis is limited. Other immunologic tests have no established clinical role in diagnosing rheumatoid vasculitis. Therapy depends upon the clinical manifestation of rheumatoid vasculitis. Uncomplicated rheumatoid arthritis deserves appropriate therapy, and general attention to nutrition, cessation of tobacco, and control of blood pressure are indicated for all patients. Isolated digital vasculitis generally requires no more than the usual treatment for uncomplicated rheumatoid arthritis. Appropriate dermatologic management is indicated for ischemic ulcers. Most clinical experience in managing more symptomatic rheumatoid vasculitis has focused on glucocorticosteroids, D-penicillamine, and cytotoxic immunosuppressive drugs.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Vasculitis associated with rheumatoid arthritis. 218 61

Primary myelofibrosis terminating in megakaryoblastic crisis is uncommon. A case with this condition is reported. The patient, a 62-year-old female, having had primary myelofibrosis for 13 years and a splenectomy 4 years before, was admitted because of high fever, hepatomegaly, thrombocythemia and leukocytosis. On admission, blasts appeared in the peripheral blood and thereafter gradually increased in number. The blasts were proven to be of megakaryocytic lineage. To our knowledge this is the third case of primary myelofibrosis terminating in megakaryoblastic crisis to be reported in Japan.
 ...
PMID:Primary myelofibrosis terminating in megakaryoblastic crisis. 223 72

A case of chronic myelogenous leukemia (CML) of 10-year survival in described. A 44-year old male was admitted to our hospital because of general malaise, abdominal fullness and fever in February, 1977. On physical examination, giant splenomegaly and hepatomegaly were detected. Peripheral blood examination revealed leukocytosis without hiatus leukemia , normochromic macrocytic anemia and thrombocytosis. NAP rate and score were 16% and 22. Cytogenetic analysis of PB without stimulator revealed 46, XY, Ph1. Then he was diagnosed as having a typical type of Ph1-positive CML. He had been successfully treated over 9 years by intermittent administration of busulfan. However, anemia suddenly progressed in February, 1986 followed by leukopenia and thrombocytopenia. Hemorrhage was not detected by the examination. Though he had been received blood transfusion, the anemia progressed rapidly. He was died of cachexia on 4th of August, 1987. The postmortem examination revealed bone marrow aplasia with no signs of blast crisis nor myelofibrosis. Secondary hemochromatosis was seen in the liver, spleen, pancreas and some other organs.
 ...
PMID:[Bone marrow aplasia without blast crisis in a case of CML of 10-year survival]. 279 87

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
 ...
PMID:Essential thrombocythemia. Clinical characteristics and course of 61 cases. 336 70

Fifty-four patients aged 60 years or older with a diagnosis of chronic myelogenous leukemia were referred to University of Texas M. D. Anderson Hospital between 1965 and 1982. Patients in this age group had a significantly shorter median survival than that of the 249 patients younger than 60 seen during the same period (26 vs 42 months; P = .01). Old age was associated with a higher incidence of poor performance status, hepatomegaly, and anemia. Fourteen other patient characteristics were correlated with poor prognosis, including black race, weight loss, symptoms, hepatomegaly, splenomegaly, anemia, thrombocytopenia or thrombocytosis, increased peripheral blast cells and promyelocytes or basophils, increased blasts or basophils in the bone marrow, decreased megakaryocytes, and additional cytogenetic abnormalities. A multivariate analysis that accounted for the interactions of these factors identified old age as being of primary adverse prognostic significance in patients with chronic myelogenous leukemia, suggesting a biologic difference in the disease in older patients. The poor prognosis in elderly patients receiving present available therapy justifies promising and well tolerated investigational approaches such as interferons in patients in this age group.
 ...
PMID:Old age: a sign of poor prognosis in patients with chronic myelogenous leukemia. 347 69

We present a patient complaining of abdominal pain during eighteen months, localized in left subcostal region, weight loss, asthenia and anorexia for the previous 5 months and fever one month before his admission. He presented painful hepatomegaly, leukocytosis, with an increased percentage of mature and inmature neutrophils, anemia, elevated erythrocyte sedimentation, thrombocytosis, hypergammaglobublinemia and dissociated cholestasis. Complementary exams demonstrated the presence of a poorly vascularized occupying process, localized in the left hepatic lobe. Following laparotomy and biopsy-resection of the process in hepatic left lobe, staining revealed isolated, Gram + filaments and histopathological study showed multiple micro-abcesses displaying actinomyces-like characteristics. There was no evidence of any other pathological process except for previous history of dental abscesses and extractions, which could represent the primary focus for hematogenous dissemination. There was satisfactory response to erythromycin.
 ...
PMID:[Primary hepatic actinomycosis]. 361 61

The prognostic importance of patient pretreatment clinical and laboratory features was investigated in a group of 303 patients with Philadelphia chromosome-positive benign-phase chronic myelogenous leukemia. Intensive chemotherapy was given to 97 patients, and 78 underwent an early elective splenectomy. The overall median survival time, dated from hospital admission, was 39 months. Patient characteristics associated with shortened survival were age 60 years or older, black race, the presence of hepatomegaly, splenomegaly, symptoms, weight loss, and poor performance status. Adverse blood and bone marrow parameters were anemia, thrombocytosis or thrombocytopenia, a high proportion of peripheral blasts plus promyelocytes or of basophils, a high proportion of marrow blasts or basophils, decreased marrow megakaryocytes, and cytogenetic abnormalities in addition to the Philadelphia chromosome. Several of these factors were interrelated. A multivariate regression analysis demonstrated that the combination blood basophilia, race, additional cytogenetic abnormalities, age and marrow basophilia had the strongest predictive relationship to survival time. This resulted in a model segregating patients into low-, intermediate-, and high-risk groups, with median survivals of 53, 39, and 25 months, respectively. Another model was derived that did not include the marrow features and identified splenomegaly and platelet counts as adding to the prognosis prediction by blood basophilia, race, and age. Evaluation of the effect of therapy, after adjusting for differences in prognostic characteristics, showed that intensive chemotherapy was associated with survival prolongation among patients at intermediate and high risk of death. We conclude that a combination of pretreatment factors identifies different risk subcategories in patients with chronic myelogenous leukemia and is helpful in assessing overall prognosis and treatment effect.
 ...
PMID:Chronic myelogenous leukemia: a multivariate analysis of the associations of patient characteristics and therapy with survival. 386 97


1 2 3 4 Next >>