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)

Burkitt lymphoma uncommonly presents as a acute leukemia. We describe the clinical course and findings of a 14-year-old female with Burkitt lymphoma who presented with acute leukemia. splenomegaly and an abdominal mass. She responded initially to prednisone alone and later achieved full remission with combination chemotherapy. Established morphologic criteria and clinical course were consistent with the diagnosis of Burkitt lymphoma (1), although B-cell determinants were not present on her tumor cells.
 ...
PMID:Acute leukemia in Burkitt lymphoma. 18 92

A total of 78 patients with acute leukemia [69 acute lymphoblastic leukemia (ALL) and 9 acute myeloblastic leukemia (AML)] survived more than 4 years. In the years between 1958 and 1967 the number of patients with ALL in Argentina who survived 4 years was 1%. This increased to 21% in the years between 1967 and 1972. The major cause of this increase was the initiation in 1967 of protocols of therapy on a national scale through a cooperative group known as GATLA. In AML patients the number of long-term survivals remained unchanged during both periods. A large percent of the long-term survivors had lower WBC, a higher platelet count, and no hepato- or splenomegaly, lymphadenopathy, or hemorrhagic manifestations at diagnosis. However, only for those patients with a WBC under 100,000/mm3 at diagnosis was there a significant prognostic implication (p less than 0.01). Patients with acute lymphoblastic leukemia who survive more than 4 years without relapse have about an 80% chance of long survival. For those who have lived 4 years with one or more lapses, there is only a 17% chance of long survival, and of these only those who develop extramedullar relapse (CNS or testicular) without bone marrow involvement have a chance of long survival. Patients who live 4 years in continuous complete remission have the same chance of very long survival regardless of previous therapy.
 ...
PMID:Long-term survival in acute leukemia in Argentina: a study of 78 cases. 26 4

Normal and pathologic reticulin networks colored black by silver nitrate can be automatically quantitated by electronic image analysis. By using this technique, different parameters can be obtained, such as the average density, the surface of network meshes, the thickness of the fibers, the complexity of the reticulum, and the heterogeneity of the myelofibrosis distribution. All of these parameters were obtained in 83 osteomedullar biopsies of blood diseases (primary splenomegaly, chronic myeloid leukemia, polycythermia vera, acute leukemia, and aplastic anemia). We have shown that there is no relation between the different parameters obtained and the medullary richness, hematopoietic center, or patient survival. On the other hand, the histomorphometric parameters can be used to distinguish acute leukemia and chronic myeloid leukemia myelofibrosis, while the parameters in primary splenomegaly are shown to be very heterogeneous.
 ...
PMID:[Quantitation of myelofibrosis in blood diseases by electronic image analysis (author's transl)]. 29 Sep 75

The authors report on 16 cases of either subacute (SMML) or chronic (CMML) myelomonocytic leukemia as well as chronic monocytic leukemia (CMoL). All these cases were oligoblastic and, according to their clinical course, they could be termed as smouldering leukemias. The chronic types affected mainly males. The diagnostic cytomorphological and cytochemical criteria are discussed. Erythro- and thrombocytopoiesis were distinctly less impaired than in acute leukemias (AL). The leucocyte count in the peripheral blood of the SMML cases was within the normal range. Hepato- and splenomegaly were markedly increased as compared to AL. According to our materials leukemic skin infiltrations were less frequent in CMoL, CMML and SMML than in acute monocytic leukemias. In each of the three types of leukemia discussed monocytic leukemic cells could be readily identified by cytochemical tests and usually showed fairly normal maturation. In accordance with these observations lysozyme levels in urine and serum usually were strongly increased. The patients in the CMML and CMoL groups showed a mean survival of more than 13 months (2 out of 7 are still alive), whereas the SMML patients survived an average of 8 months. Deaths were frequently due to advanced age rather than to leukemia. In other cases a terminal accumulation of blasts marked a transition to acute leukemia. During the smouldering phase of the disease no beneficial effect of combined chemotherapy could be noted. Supportive and symptomatic therapy might improve length and quality of survival.
 ...
PMID:[Subacute and chronic monocytic leukemia: diagnostic and clinical problems]. 29 89

In order to study the effect of oxymetholone therapy in advanced myelofibrosis, 11 patients (4 females, 7 males) were given, 3--5 mg per kg body weight, long-term oxymetholone treatment in a prospective multicenter study. Five cases had previously had a diagnosis of polycythemia vera. All patients had anemia initially, 4 leukocytopenia and 10 thrombocytopenia in addition. Hepato-splenomegaly was present in all cases but in varying degree. Five patients required regular blood transfusions before treatment. In 9 of the 15 courses given, there was normalization of the peripheral blood or substantial improvement (better than 3 g hemoglobin/dl or 50 X 10(9) platelets/1) after androgens. Significant effects were noted both on hemoglobin values and platelet counts. The need for blood transfusions ceased completely in all 5 cases. When oxymetholone treatment was reduced or interrupted 4 patients relapsed; 2 of them responded to a renewed course. The red cell counts returned to previous polycythemic values in one patient and another died from acute leukemia. The results of this study suggest that androgens might be of value in advanced cases of myelofibrosis with transfusion-requiring anemia or severe thrombocytopenia.
 ...
PMID:Oxymetholone treatment in myelofibrosis. 66 66

A group of 52 consecutive patients with polycythemia vera was submitted to long-term therapy with radioactive phosphorus (32P). Initial phase of therapy induced complete remissions (normalization of hematogram; spleen not palpable) in 45% of the patients, and partial remissions in the remaining 55%. During maintenance therapy of the complete remission group, mean remission time was about 3.5 years. Individual remission times ranged between 1 and 6 years. In the group of patients with incomplete remission, mean remission time increased with the progression of the disease due to gradual development of "spent" -polycythemia. In patients with splenomegaly, remission time was negatively correlated to spleen size. In both groups the increment of annual accumulated dose averaged 2.4 mCi 32P. When considering polycythemia related causes of death only, mean survival time attained 12 years after initial treatment with 32P. Acute leukemia occurred in 2 patients (4%).
 ...
PMID:32P-therapy in polycythemia vera. 80 69

Nine patients were selected according to the following criteria: 1. Hematological findings consistent with the diagnosis of myelofibrosis with myeloid metaplasia (MMM), except for an excess of blasts in the blood and bone marrow; 2. No previous (silent) phase of MMM. 3. No PH1 chromosome, and 4. No identifiable cause of secondary myelofibrosis. These patients had either an acute or subacute myelofibrosis. The onset of such symptoms as fever, bone pain, hemorrhage and mild splenomegaly was rapid. Terminal acute leukemia or more often progressive bone marrow biopsy showing myelofibrosis with persistence of differentiated myeloid tissue, particularly megacaryocytes. Isotopic studies (59Fe and 51Cr) showed splenic erythroid metaplasia, poor bone marrow 59Fe uptake and increased peripheral red blood cell destruction. This study confirms that malignant myelosclerosis is a well-defined syndrome which must be distinguished from: a) Acute transformation of typical agnogenic myeloid metaplasia even though it was previously undiagnosed (4 cases of MMM illustrating this possibility have been reported); b) Acute myeloblastic leukemia with myelofibrosis; and c) Myelofibrosis secondary to lymphomatous or carcinomatous bone-marrow invasion (2 cases with acute myelofibrosis appearing long after appropriate treatment have been reported).
 ...
PMID:[Acute or subacute myelofibrosis]. 95 Nov 81

The onset of postpolycythemic myeoloid metaplasia or spent polycythemia has been recognized for many years. As the result of many different series, the development of postpolycythemic myeolid metaplasia might be expected in from 15%-20% of patients with postpolycythemia vera. It appears that an etiologic role for sodium phosphate 32P may exist in this evolutionary pattern. About 70% of patients with PPMM will have symptoms with the onset of the syndrome. The major mechanisms producing symptoms result from (1) anemia, (2) pressure from massive splenomegaly, and (3) bleeding problems. Iron deficiency is a frequent cause of anemia in patients with PPMM. The major mechanism of anemia in these patients, however, relates to ineffective erythropoiesis and shortened red cell survival. Androgen trials for ineffective erythropoiesis seem worthwhile, although data on this point is too limited to draw any firm conclusions. A steroid trial for those patients with major hemolytic episodes is indicated. In those patients in whom adrenal steroid therapy fails to control major hemolysis, a consideration for splenectomy exists. Pressure-related manifestations secondary to massive splenomegaly have been treated with radiation therapy and oral alkylators. Although there is data to document amelioration of painful symptoms with associated shrinking of the spleen, long-term control of this problem has not been forthcoming. Again, patients who are medical failures in control of pressure-related manifestations may be considered for splenectomy. Bleeding problems may arise with PPMM secondary to thrombocytopenia, thrombocythemia, or qualitative platelet dysfunction. Adrenal steroids have met with some success in improving platelet counts in patients with life-threatening thrombocytopenia. Those patients who are medical failures with adrenal steroids in terms of thrombocytopenia might be candidates for splenectomy. Control of thrombocythemia has been observed with oral alkylator therapy and chlorambucil may have a special role in managing this complication. Qualitative platelet defects leading to severe bleeding are best managed with fresh platelet transfusions. Patients with PPMM in contrast to patients with agnogenic myeoloid metaplasia have a more lethal syndrome and shortened survivorship. Causes of death in patients with PPMM include cardiac problems, transition to acute leukemia, hemorrhage, and infection.
 ...
PMID:The evolution into and the treatment of late stage polycythemia vera. 125 Dec 24

Polycythemia vera (PV) is one of the myeloproliferative diseases, and, as such, is an example of clonal hematopoiesis. The progeny of a single, abnormal, hematopoietic stem cell gain a growth advantage over their normal counterparts resulting in overproduction of red cells generally accompanied by overproduction of granulocytes and platelets as well. There are a variety of nonspecific symptoms at onset related to the increased red cell mass and hematocrit accompanied by the more specific manifestations of pruritus, erythromelalgia, and hepatic, portal, and mesenteric vein thrombosis. Splenomegaly and hypertension are common. The laboratory hallmark is an increased red cell mass. There is also often an increase in white cell count, platelet count, and leukocyte alkaline phosphatase along with other findings reflecting the increased rate of turnover of hematopoietic cells. The bone marrow biopsy generally displays hypercellularity involving all three cell lines and absent iron stores. The diagnosis of PV depends on excluding spurious polycythemia in which there is a high hematocrit but a normal red cell mass and secondary polycythemia in which there is an increased red cell mass in response to tissue hypoxia or the inappropriate production of erythropoietin, generally by a tumor. In addition, one should try to establish the diagnosis in a positive fashion by a combination of studies of the blood and bone marrow. Phlebotomy and occasionally plateletpheresis should be used as acute therapy. Chronic therapy is guided by the knowledge that patients treated with phlebotomy alone have an increased rate of thrombotic complications particularly in older patients and those with previous thrombotic disease. Myelosuppressive therapy can reduce the incidence of these complications, but is commonly associated with an increased incidence of second malignancies, particularly acute leukemia. At present, hydroxyurea is the myelosuppressive agent of choice. Antiplatelet agents have a limited role except in the palliation of the syndrome of erythromelalgia. Median survival is approximately 10 years. As implied above, the causes of morbidity and mortality vary with the mode of chronic therapy which has been employed, leukemia being more common after myelosuppressive therapy and thrombotic complications being more common after therapy with phlebotomy alone. Ten percent to 50% of patients move into a spent phase followed by postpolycythemic myeloid metaplasia, irrespective of previous therapy employed. Eventually, the major problems may be cytopenias and massive splenomegaly.
 ...
PMID:Polycythemia vera. 158 7

A 63 year-old woman was referred to our hospital because of fever and increased number of blasts in the bone marrow. On physical examination she had slight hepatomegaly but no splenomegaly. Laboratory tests disclosed a hemoglobin level of 8.5 g/dl; a WBC count of 13,200/microliter with 26% blasts; a platelet count of 51,000/microliter. A bone marrow aspirate was normocellular with 74% blasts and 37% blasts were stained positive for myeloperoxidase. Cell surface markers for HLA-DR, CD10, CD19, CD13, CD33 were positive. Karyotype analysis revealed 46, XX, t (9q+; 22q-) and 45XX, -7, t (9q+; 22q-). Southern analysis showed rearrangement of immunoglobulin heavy chain but not T cell receptor beta gene. Rearrangements in M-BCR were not detected with 5' or 3' bcr probes. After 2 courses of chemotherapy, blasts decreased to 7% with recovery of normal elements and 11 out of 20 metaphases of the bone marrow cells were normal karyotype. These findings suggest that this case was de novo Ph1 positive acute leukemia which demonstrated both lymphoid and myeloid features.
 ...
PMID:[Biphenotypic acute leukemia with Ph1 chromosome, M-BCR-, myeloperoxidase+, and CALLA+]. 164 7


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