Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of hepatosplenic candidiasis (HSC) are reported occurring after protracted episodes of neutropenia, induced by chemotherapy for acute leukemia in one case and drug hypersensitivity in the other. The disease presented with persistent or recurrent fever after correction of the neutropenia and with splenomegaly. The alkaline phosphatases were elevated. The diagnosis was strongly suggested by abdominal ultrasonography, CT scan and MRI, which showed multiple hepatosplenic defects. It was confirmed by serologic tests for candidiasis, the presence, in 1 case, of circulating candida antigens, and the rapid response to amphotericin B. The diagnosis of HSC should be considered in patients with persistent fever after an episode of neutropenia. Ideally, histologic confirmation is desirable, but this is often obtainable only by open liver biopsy, an aggressive procedure in such patients. Failing this, our 2 cases stress the diagnostic value of noninvasive imaging techniques, serological testing (in particular the discovery of circulating candida antigens) and the response to amphotericin B.
 ...
PMID:Hepatosplenic candidiasis: an overlooked cause of prolonged fever during recovery from an episode of neutropenia. 264 20

The clinical and hematological profile of 53 patients in whom primary myelofibrosis (PMF) had been diagnosed during the last 15 years was evaluated. Median age was 64 years (range 17-86). Thirty-five patients were males and 18 females. The most frequent symptoms were associated with the hypermetabolic state, anemia and splenomegaly. The latter was found in 96% of patients, while 83% had hepatomegaly and 9% had lymphadenopathy. Thirty-three patients had anemia at the time of diagnosis. The leukocyte and platelet counts were normal or moderately high in most cases. Myelemia was found in 83% of patients, with circulating erythroblasts in 72%. The most common biochemical abnormalities were the increased serum LDH (84%) and hypocholesterolemia (62%). Bone marrow aspirate was not analyzable in most cases. Bone marrow biopsy showed myelofibrosis in hypercellular phase in 22 patients, myelofibrosis without osteosclerosis in 17, and myelofibrosis with osteosclerosis in 14. The median survival of the series was 3.8 years; 34 patients had died at the time of the analysis. The major causes of death were infection, cardiovascular complications and hemorrhage. In 4 patients the evolution from PMF to acute leukemia was observed.
 ...
PMID:[Primary myelofibrosis: description of a series of 53 patients]. 266 65

The clinical and hematological profile, the response to therapy and the clinical evolution of 29 patients with acute T lymphoproliferative syndromes (ATLS) diagnosed during the last 5 years were evaluated. Mean age was 22.6 (SD 9.5) years. 24 patients were males and 5 females. Lymphadenopathy was present in 25 patients, hepatomegaly in 16 and splenomegaly in 14. Seventeen patients had a mediastinal mass. Anemia was present in 41% of patients and thrombocytopenia in 50%. The mean leukocyte count was 71 x 10(9)/l (SD 93). Bone marrow infiltration was found in 25 patients. The acid phosphatase reaction was positive, centrosomal type, in all cases except two. The immunophenotypic study classified the ATLS patients in the subtypes pre-T (10 cases), cortical thymic (15 cases), and mature thymic (4 patients). The cases with pre-T phenotype had a greater degree of bone marrow infiltration and lower frequency of mediastinal mass than the rest of patients. The rate of complete remissions (CR) was 83%. At the time of closing the study 12 patients had relapsed, with a median time of CR of 9.4 months. In addition, 11 patients had died, with a median survival of the series of 19 months. The results of the present study indicate that, despite some heterogeneity, those cases of ATLS with a more immature immunological phenotype (pre-T) have clinical and biological features more akin to acute leukemia, unlike those with a thymic phenotype (cortical or mature), which would be closer to lymphoblastic lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Acute T-cell lymphoproliferative syndromes. A study of 29 cases]. 269 96

Splenic erythropoiesis was demonstrated by surface counting of 59Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agnogenic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodysplastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombocythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II-III myelofibrosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis 1) is encountered in a variety of clinical conditions; 2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myeloproliferative disorders; 3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and 4) can be evaluated in a semiquantitative manner by surface counting.
 ...
PMID:Ferrokinetic study of splenic erythropoiesis: relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis. 275 9

Analysis of remission induction rates for 1,117 children 18 months to 10 years of age (group 1) and 90 infants less than 18 months of age (group 2) with acute lymphoid leukemia (ALL) and of duration of continuous complete remission (CCR) for 454 in group 1 and 33 in group 2 revealed that infants fared significantly worse in both measures of outcome (P = .03 and P less than .0001). To examine potential reasons for the poor prognosis of affected infants, clinical and biologic features of their ALL were compared. Infants had higher WBC counts (P less than .001), a higher incidence of massive splenomegaly (P less than .001), massive hepatomegaly (P less than .001), more central nervous system (CNS) disease at diagnosis (P less than .01), and lower platelet counts (P less than .001). Also, their blasts were less often PAS+ (P = .02). The incidence of non(T, B, pre-B), T and pre-B immunophenotypes of ALL did not differ significantly between the two groups. However, in patients with non(T, B, pre-B) ALL, the majority (51%) of infants had common ALL antigen (CALLA)-negative blasts, as compared with only 7% in group 1 (P less than .001). Furthermore, infants with non(T, B, pre-B) cell ALL who were less than 12 months of age were almost always CALLA- (18 of 21). The blasts of children from both groups usually expressed Ia-like antigens. These data illustrate that infants with ALL have extensive and bulky disease more often than do older children and are more often affected with a prognostically unfavorable phenotype of acute leukemia (AL) which expresses Ia-like antigens but is more often PAS- and CALLA-. We believe that these clinical and biological differences predict and explain in part the observed poor response to treatment of infants with ALL.
 ...
PMID:Clinical and biologic features predict a poor prognosis in acute lymphoid leukemias in infants: a Pediatric Oncology Group Study. 293 4

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

Clinical and laboratory features of seven patients with acute leukemia associated with the (4;11) chromosome translocation are presented. Leukemic blasts of these patients showed lymphoid morphology in 6 (although 1 was treated for monoblastic leukemia 3 years earlier) and monocytoid morphology in 1, were positive for TdT and HD 37 (CD 19) in 6 patients, whereas weak expression of CALLA was seen in only 1 patient and T-lineage-associated antigens in none. Leukemic blasts from four patients showed the simultaneous expression of B-lymphoid and myeloid antigens, suggesting leukemogenesis in a very early multipotent progenitor cell. In 2 patients an isochromosome of the long arm of No. 7 chromosome was found in the leukemic karyotypes in addition to t (4; 11) (q 21; q 23); in one instance present at diagnosis, in the other one occurring at relapse. In one other patient leukemia karyotype also demonstrated trisomy 8. Leukemic cells of three patients were investigated by molecular genetics and demonstrated immunoglobulin gene rearrangements for the Ig heavy chain sequences but not for the light chain constant regions and T cell receptor sequences. All patients were treated by intensive chemotherapy. Four of the 7 patients are in continuous complete remission. The longest event-free survival time (over 2 1/2 years) was seen in one patient who had also DOWN-syndrome. Including these 7 patients a clinical analysis of 71 patients with t (4; 11) acute leukemia was made, emphasizing the following characteristics at diagnosis: female sex (62%), age under 2 years (49%), leukocyte count over 100 X 10(9)/1 (61%), splenomegaly (80%), CNS-disease (11%). Survival of over 2 years was reported in less than 15% of the patients. It remains to be seen if risk-adapted treatment can alter the course of this early B-precursor acute leukemia with hitherto very bad prognosis.
 ...
PMID:Acute leukemia with chromosome translocation (4;11): 7 new patients and analysis of 71 cases. 349 35

Splenectomy is a valuable method of management of approximately 80 per cent of patients with hairy cell leukemia who have hypersplenic complications develop; operation can be performed with minimal morality and morbidity. Removal of the spleen also provides useful palliation in patients with chronic lymphocytic leukemia who have symptomatic splenomegaly or autoimmune hemolytic anemia; success is less predictable when operation is performed for thrombocytopenia. Splenectomy is linked with a high mortality and is of little benefit in patients with chronic granulocytic leukemia except when operation is performed as a prerequisite to bone marrow transplantation. Several patients with acute leukemia who have signs of splenic infarction and intra-abdominal bleeding develop had a short survival period after splenectomy.
 ...
PMID:The limited role of splenectomy in patients with leukemia. 385 56

Nineteen cases of canine acute leukemia were diagnosed during a 4-year period. Two main categories were identified on the basis of cytologic, hematologic, and clinical features: acute lymphoid leukemia and acute myelogenous leukemia. Clinical features included history of weight loss, anorexia, shifting limb lameness, and incoordination. Physical findings were characterized by hepatomegaly, splenomegaly, mild generalized lymphadenopathy, and pallor. Ocular lesions were found in 29% of dogs with acute myelogenous leukemia. Hematologic abnormalities included anemia, thrombocytopenia, pancytopenia, leukemia, and leukoerythroblastic reactions. Results of therapy were discouraging.
 ...
PMID:Clinicopathologic aspects of acute leukemias in the dog. 385 21

The 61 observations of primary thrombocythemia described in this report represent approximately 15% of the cases of polycythemia vera recorded by the authors over the same 18-year period. The group includes 35 females and 26 males, with a mean age of 62. The disease is usually discovered on routine blood tests (half of cases), and more rarely because of hemorrhagic or thrombotic manifestations. Splenomegaly is found in one-third of cases. Platelet count is permanently above 800 X 10(9)/l (mean : 1 500 X 10(9)/l); mild hyperleukocytosis (mean : 16 X 10(9)/l) with predominant neutrophil polynuclears is usual but myelemia is not constant (28% of cases) and always very moderate; red cell parameters are normal in three-fourths of cases, while the remaining patients have anemia, either due to iron depletion or not. Reticulinic myelofibrosis, usually minimal, is found in 40% of cases. Medullary karyotype is always normal, without chromosome Ph1. Platelet functional abnormalities are not constant and do not correlate with the magnitude of thrombocythemia. 51 patients (84%) received myelosuppressive therapy, mainly by busulfan or radioactive phosphorus. Most deaths were due to intercurrent causes and only one patient developed acute leukemia. 71% of patients are alive at five years and subsequent decrease in the actuarial survival curve is very gradual.
 ...
PMID:[Essential thrombocythemia. Clinical, biological study and developmental study of 61 cases]. 632 10


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>