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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the cases of 38 children with transfusion-associated HIV infection: 18 hemophiliacs and 2 patients with Von Willebrand disease, 6 with hemoglobinopathies, 8 with malignant diseases or aplastic anemia, 2 transfused during neonatal period and 2 during a surgical operation. Two groups with a different prognosis were found: In group A [hemophilia and Von Willebrand disease (n = 20)] 17 patients were asymptomatic or only with lymphadenopathy; 3 reached stage IV and none died. In group B [Others (n = 14)] 2 patients were asymptomatic, 4 reached stage IV and 8 died, 4 of them directly from AIDS. The difference between both groups was statistically significant. Prognosis of HIV infection is particularly severe in patients with leukemia, malignant tumors and aplastic anemia.
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PMID:[Infection by the HIV virus and transfusions. Study of 38 pediatric cases]. 259 49

The various etiologies of spontaneous hemarthrosis in adolescents and adults are reviewed: they include systemic diseases and local or regional disorders of the bones or joints. Among systemic diseases, the two main causes are coagulation disorders and hemoglobinopathies. Coagulation disorders may be either acquired (leukemia, thrombopenia, and hypoprothrombinemia induced by anticoagulant drugs with hemarthrosis being one of the major complications) or inherited (hemophilia which is not considered here, von Willebrand disease, and congenital thrombopathies). Hemoglobinopathies, particularly sickle-cell disease, are responsible for hemarthrosis in a few patients. Among local or regional disorders of the bones or joints, tumors such as hemangioma or synovial sarcoma are uncommon causes. Hemarthrosis is the main feature of pigmented villonodular synovitis. Hemarthrosis may occur in degenerative and metabolic diseases: while it is extremely rare in arthritis, it is frequently encountered in articular chondrocalcinosis which is the first diagnosis to consider when hemarthrosis occurs in an elderly patient. The search for an etiology, which is often difficult, should include a review of prior illnesses, a study of coagulation, and local clinical, radiological and biological investigations, with a study of the synovial fluid; in some instances, arthroscopy, synovial biopsy and even surgical exploration are required. Management includes rest, analgesics, antiinflammatory drugs and, above all, arthrocentesis which is essential for the prevention of articular damage and functional sequellae. Specific therapy is dependent on the etiology. In recurrent hemarthrosis, isotopic synoviorthesis may ensure lasting resolution of the effusion.
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PMID:[Spontaneous hemarthrosis in adolescents and adults, excluding hemophilia]. 629 20

100 patients with a modified Ivy bleeding time longer than 10 min in the presence of more than 80 x 10(9)/l blood platelets were studied retrospectively. 72 patients had repeated bleeding times between 10 and 20 min or one or more measurements exceeding 20 min, and were considered to have an unquestionable platelet dysfunction. 39 (54%) of these 72 patients were receiving large doses of antibiotics. Nearly one half of the patients on antibiotics had a bleeding tendency, but most of these patients had additional features that may have interfered with platelet function, or a coagulation defect. In the remaining 33 patients, the prolonged bleeding time was associated with von Willebrand's disease, liver disease, leukaemia/myeloproliferative disease, paraproteinaemia or aspirin ingestion. High doses of penicillin seem to be the most common cause of qualitative platelet disorders in general hospital practice.
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PMID:Prolonged bleeding time with adequate platelet count in hospital patients. 733 58

In the seventh national voluntary cross-sectional survey (in 1999) of Finnish patients with haemophilia A or B, type 3 von Willebrand disease or factor XIII deficiency, a plasma sample was received from 193 patients (67%). The samples were tested for hepatitis B and C, human immunodeficiency virus (HIV) and human T-cell leukaemia virus (HTLV) antibodies. Fifty-one percent of the patients were hepatitis C antibody positive and 34% hepatitis B core antibody positive. None of the patients had antibodies against HIV or HTLV. Eighteen percent of the patients had an elevated alanine aminotransferase activity. Abnormal alanine aminotransferase was significantly associated with hepatitis C seropositivity. No new seroconversions were detected among the haemophiliacs or patients with type 3 von Willebrand disease when compared with the last two surveys in 1993 and 1996, and there was no seroconversion in sole users of solvent/detergent-treated factor products. Currently, 32% of the patients use prophylactic factor treatment as their principal mode of therapy, particularly the younger patients with severe forms of the bleeding diseases.
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PMID:Viral markers and use of factor products among Finnish patients with bleeding disorders. 1113 80

Hyphema (blood in the anterior chamber) can occur after blunt or lacerating trauma, after intraocular surgery, spontaneously (e.g., in conditions such as rubeosis iridis, juvenile xanthogranuloma, iris melanoma, myotonic dystrophy, keratouveitis (e.g., herpes zoster), leukemia, hemophilia, von Willebrand disease, and in association with the use of substances that alter platelet or thrombin function (e.g., ethanol, aspirin, warfarin). The purpose of this review is to consider the management of hyphemas that occur after closed globe trauma. Complications of traumatic hyphema include increased intraocular pressure, peripheral anterior synechiae, optic atrophy, corneal bloodstaining, secondary hemorrhage, and accommodative impairment. The reported incidence of secondary anterior chamber hemorrhage, that is, rebleeding, in the setting of traumatic hyphema ranges from 0% to 38%. The risk of secondary hemorrhage may be higher in African-Americans than in whites. Secondary hemorrhage is generally thought to convey a worse visual prognosis, although the outcome may depend more directly on the size of the hyphema and the severity of associated ocular injuries. Some issues involved in managing a patient with hyphema are: use of various medications (e.g., cycloplegics, systemic or topical steroids, antifibrinolytic agents, analgesics, and antiglaucoma medications); the patient's activity level; use of a patch and shield; outpatient vs. inpatient management; and medical vs. surgical management. Special considerations obtain in managing children, patients with hemoglobin S, and patients with hemophilia. It is important to identify and treat associated ocular injuries, which often accompany traumatic hyphema. We consider each of these management issues and refer to the pertinent literature in formulating the following recommendations. We advise routine use of topical cycloplegics and corticosteroids, systemic antifibrinolytic agents or corticosteroids, and a rigid shield. We recommend activity restriction (quiet ambulation) and interdiction of non-steroidal anti-inflammatory agents. If there is no concern regarding compliance (with medication use or activity restrictions), follow-up, or increased risk for complications (e.g., history of sickle cell disease, hemophilia), outpatient management can be offered. Indications for surgical intervention include the presence of corneal blood staining or dangerously increased intraocular pressure despite maximum tolerated medical therapy, among others.
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PMID:Management of traumatic hyphema. 1268 17

The term 'monoclonal gammopathy of undetermined significance' denotes the presence of a monoclonal protein in patients without evidence of multiple myeloma, macroglobulinemia, amyloidosis or related plasma cell proliferative disorders. The disorder has been found in approximately 3% of persons older than 70 years and in approximately 1% of persons older than 50 years. A population-based study included 1384 patients from south-eastern Minnesota who had the disorder diagnosed at the Mayo Clinic from 1960 through 1994. Risk of progression was about 1% per year, but patients were at risk of progression even after 25 years or more of stable monoclonal gammopathy of undetermined significance. The risk for development of multiple myeloma was increased 25-fold; the risk of macroglobulinemia, 46-fold; and the risk of primary amyloidosis, 8.4-fold. Concentration and type of monoclonal protein were the only independent predictors of progression. The presence of a urine monoclonal protein and the reduction of one or more uninvolved immunoglobulins were not risk factors for progression. Monoclonal gammopathy of undetermined significance may be associated with various disorders, including lymphoproliferative diseases, leukemia, von Willebrand disease, connective tissue diseases and neurologic disorders.
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PMID:Monoclonal gammopathies of undetermined significance. 1261 97

Qualitative disorders of platelet function and production form a large group of rare diseases which cover a multitude of genetic defects that by and large have as a common symptom, excessive mucocutaneous bleeding. Glanzmann thrombasthenia, is enabling us to learn much about the pathophysiology of integrins and of how alphaIIb beta3 functions. Bernard-Soulier syndrome, an example of macrothrombocytopenia, combines the production of large platelets with a deficit or non-functioning of the major adhesion receptor of platelets, the GPIb-IX-V complex. Amino acid substitutions in GPIb alpha, may lead to up-regulation and spontaneous binding of von Willebrand factor as in Platelet-type von Willebrand disease. In disorders with defects in the MYH9 gene, macrothrombocytopenias are linked to modifications in kidney, eye or ear, whereas other inherited thrombocytopenias variously link a low platelet count with a propensity to leukemia, skeletal defects, learning impairment, and abnormal red cells. Defects of secretion from platelets include an abnormal alpha-granule formation as in the gray platelet syndrome (with marrow myelofibrosis), and of organelle biogenesis in the Hermansky-Pudlak and Chediak-Higashi syndromes where platelet dense body defects are linked to abnormalities of other lysosomal-like organelles including melanosomes. Finally, defects involving surface receptors (P2Y(12), TPalpha) for activating stimuli, of proteins essential for signaling pathways (including Wiskott-Aldrich syndrome), and of platelet-derived procoagulant activity (Scott syndrome) show how studies on platelet disorders are helping unravel the pathways of primary hemostasis.
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PMID:Qualitative disorders of platelets and megakaryocytes. 1610 44

Monoclonal gammopathy of undetermined significance (MGUS) is characterized by the presence of a monoclonal protein (M-protein) without evidence of multiple myeloma (MM), Waldenstrom's macroglobulinemia (WM), amyloidosis (AL), or a related plasma cell proliferative disorder. Agarose gel electrophoresis followed by immunofixation is recommended for recognition of an M-protein. Monoclonal gammopathy of undetermined significance is found in approximately 3% of persons > 70 years of age and in about 1% of those > 50 years old. In a series of 1384 patients from Southeastern Minnesota in whom MGUS was diagnosed at Mayo Clinic from 1960 through 1994, the risk of progression was 1% per year. This risk of progression continued even after > or = 25 years of a stable M-protein. The risk for developing MM, WM, or AL was increased 25-fold, 46-fold, and 8.4-fold, respectively. The concentration of the serum M-protein, abnormal serum free light-chain ratio, and the presence an immunoglobulin (Ig)M or an IgA M-protein were risk factors for progression. The presence of a urine M-protein or the reduction of > or = 1 uninvolved immunoglobulins was not a risk factor for disease progression. Patients must be monitored for progressive disease throughout their lives. Variants of MGUS consist of IgM MGUS, biclonal gammopathies, triclonal gammopathies, idiopathic Bence Jones (light-chain) proteinuria, and IgD MGUS. Monoclonal gammopathy of undetermined significance may be associated with many disorders, including lymphoproliferative diseases, leukemia, von Willebrand's disease, connective tissue diseases, and neurologic disorders. Epidemiologic and statistical methods must be used to evaluate these associations.
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PMID:Monoclonal gammopathy of undetermined significance. 1623 48

Inherited coagulopathies are bleeding disorders, which require treatment for life. Keeping an updated registry on these diseases is crucial for planning care, documenting prevalence of diseases and evaluating effectiveness of resources. We have analysed data from 26 treatment centres on coagulopathies in Brazil. Information included socio-demographic data, diagnosis of coagulopathies, severity of haemophilias A and B, presence and quantification of inhibitors in haemophilia, type of von Willebrand disease (VWD) and infection status for viral diseases. On 1 July 2007, there were 10 982 patients with inherited coagulopathies in Brazil, of which 6881 (62.7%) corresponded to haemophilia A, 1291 (11.7%) to haemophilia B, 2333 (21.2%) to VWD, 258 (2.4%) to other coagulopathies and 219 (2.0%) to undiagnosed bleeding disorders. Haemophilia A and B inhibitors were present in 9.9% and 1.9% of the patients, respectively. Human immunodeficiency virus infection was present is 6.5%, 4.8% and 1% of patients with haemophilia A, B and VWD, respectively. Hepatitis C virus infection was present in 34.9%, 29.7% and 12% of patients with haemophilia A, B and VWD, respectively. Infection by hepatitis B and human T-cell leukemia-lymphoma virus was also reported. This is the first report on the registry of patients with inherited coagulopathies in Brazil, supposed to be the third largest population of patients with haemophilia.
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PMID:Registry of inherited coagulopathies in Brazil: first report. 1897 55

A 5-year-old male cynomolgus monkey (Macaca fascicularis) with a clinical history of bleeding tendency, severe anaemia, thrombocytopenia and elevated serum concentration of liver-related enzymes was examined post mortem. Ecchymotic haemorrhages were present on the left eyelid and forehead. The liver, kidney and spleen were markedly enlarged and the kidneys had capsular petechiae. Microscopically, numerous atypical cells resembling myeloid cells were observed in the bone marrow, and myelofibrosis was present. Atypical cells were also present in the blood vessels of the liver, kidney, spleen, lymph nodes, lung, heart, bladder, adrenal gland and brain. Some neoplastic cells had oval or pleomorphic macronuclei and others were multinucleated. Immunohistochemically, the majority of the neoplastic cells had granular cytoplasmic expression of the megakaryocyte-associated antigens Von Willebrand Factor and CD61-IIIa, but were negative for myeloperoxidase. A diagnosis of acute megakaryocytic leukaemia (AMKL)-like disease was made. This would appear to be the first report of AMKL-like disease in non-human primates. This monkey was infected with simian retrovirus type D and it is possible that this viral infection was associated with the development of neoplasia.
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PMID:Acute megakaryocytic leukaemia (AMKL)-like disease in a cynomolgus monkey (Macaca fascicularis). 1915 98


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