Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paroxystic nocturnal hemoglobinuria (PNH) is an acquired hemolytic anaemia related to an increase susceptibility of erythrocytes to complement-mediated lysis. PNH is a clonal disease of an hematopoietic stem cell which lost, by mutation, the ability to synthesized phospholipid anchor of membranous proteins, i.e. complement regulatory proteins: DAF, C8BP or CD59. The clinical features of PNH are hemoglobinuria episodes associated with chronic hemolytic anaemia or pancytopenia with active bone marrow or aplastic anaemia. The clinical course is marked by severe thrombotic complications (such as Budd-Chiari syndrome), hemorrhages or infections. The diagnosis is confirmed by in vitro hemolysis tests, and now by facs analysis of cell membrane expression of deficient proteins. Different treatments have been proposed with various results (corticosteroid therapy, androgens, chemotherapy...) but the only way to eliminate the abnormal clone appears to be related bone marrow allograft.
 ...
PMID:[Paroxysmal nocturnal hemoglobinuria]. 823 86

Forty-two cases of Coombs' positive or agglutinating immune-mediated hemolytic anemia (IMHA) in dogs were reviewed. Dogs ranged in age from 1 to 13 years, with a mean age of 6.4 +/- 3.4 years. The majority of dogs (74%) tested positive for IgG antibodies without complement. Spherocytosis was seen in 67% of the dogs, but hemoglobinemia and hemoglobinuria were found in only 10%. Marked bilirubinuria was found in all the dogs. A significant seasonal incidence was observed, with 40% of all cases diagnosed during the months of May and June. Severe anemia, with PCV < or = 20% was observed in 37 dogs (88%). Sixteen dogs (38%) had moderate to severe reticulocytosis and 12 dogs (29%) had mild reticulocytosis. Thus, the absence of reticulocytosis should not be used to rule out a diagnosis of IMHA. Concomitant mild to severe thrombocytopenia was observed in 28 dogs (67%). A mortality of 29% was observed during hospitalization. Risk of death was significantly increased in dogs without marked reticulocytosis, those with lower PCV, and dogs with serum bilirubin concentrations > or = 10 mg/dl. In severe cases of IMHA, rapid and aggressive supportive therapy is required.
 ...
PMID:Idiopathic immune-mediated hemolytic anemia in dogs: 42 cases (1986-1990) 832 Jan 40

The long-term effects of recombinant human erythropoietin (rhEPO) administration in two consecutive cases of paroxysmal nocturnal hemoglobinuria (PNH) with severe anemia are reported. In both patients, a 68-year-old woman and a 66-year-old man, a diagnosis of PNH was made on the basis of severe macrocytic anemia associated with hemoglobinuria, hemosiderinuria and positivity for the sucrose and Ham tests. Subcutaneous treatment with rhEPO, 150 U/Kg body weight daily, was followed in both cases by a progressive increase in hemoglobin concentrations, which thereafter were maintained above 10 g/dL with lower doses of rhEPO and without any relevant side effects for 32 and 29 months of continuous treatment, respectively. A clinical response was observed in spite of elevated baseline serum erythropoietin concentrations, appropriate to the degree of anemia in both patients. These results suggest that rhEPO may be appropriately and safely used in the long-term correction of anemia associated with PNH, and that the response to the pharmacologic doses of rHEPO administered was not dependent on the level of endogenous erythropoietin.
 ...
PMID:Recombinant human erythropoietin for long-term treatment of anemia in paroxysmal nocturnal hemoglobinuria. 864 43

Two hundred and three cases of PNH diagnosed (according to the National Unified Diagnosed Criteria, 1987) and treated from 1949 to 1992 in Peking Union Medical Hospital were analyzed retrospectively in an attempt to investigate the factors leading to delayed diagnosis. The presenting manifestation was anemia in 56.7% of the patients. Next was hemorrhage (24.6%). Hemoglobinuria was seen only in 12.8% of the cases at the onset. Ham test was persistently negative in 10.4% of the patients. Only one third of all the cases was correctly diagnosed as PNH within one year after onset. Aplastic anemia or anemia of unknown cause was first diagnosed in 60.1% of cases. The heterogeneity of the manifestations and the lack of sensitive and also specific laboratory tests were the factors leading to delayed diagnosis, but negligence of PNH in differential diagnosis seem to be a major the main cause. Constant awareness of the possibility and careful follow-up accompanied with repeated laboratory examinations in a suspected case are crucial for early diagnosis of PNH.
 ...
PMID:[Experiences in clinical diagnosis of paroxysmal nocturnal hemoglobinuria (PNH)]. 869 14

Babesiosis is a malaria-like illness transmitted by the tick Ixodes dammini. The disease is endemic to the Northeast coastal region and parts of the Midwest. Symptoms-which include fever, anemia, elevated liver function tests, and hemoglobinuria-may be especially severe in asplenic or immunocompromised patients. In rare cases, infection with Babesia may be associated with marked pancytopenia. Bone marrow biopsy may reveal hemophagocytosis and marrow histiocytosis. We report a severe case of babesiosis and hemophagocytic syndrome in an asplenic renal transplant patient.
 ...
PMID:Babesiosis and hemophagocytic syndrome in an asplenic renal transplant recipient. 878 22

We prospectively studied 50 Vietnamese patients with blackwater fever (BWF). All patients had fever and hemoglobinuria, 40 (80%) were jaundiced, 25 (50%) had hepatomegaly, 15 (34%) had splenomegaly, and 9 (18%) had hepatosplenomegaly. Twenty-one patients (42%) had impaired renal function, with creatinine clearances of < 50 mL/min/m2; however, only four (8%) developed oliguric renal failure, three (6%) of whom required dialysis. Forty-four patients (88%) developed anemia, which was severe (hematocrit, < 20% in 32 (64%). One patient died, representing a death rate for this once-feared disease that is considerably lower than that reported by earlier investigators. BWF was associated with quinine ingestion in 28 patients (56%), glucose-6-phosphate dehydrogenase (G6PD) deficiency in 27 (54%), and concurrent malaria infection in 16 (32%). There was no statistically significant difference in the severity of BWF associated with each of these three factors, as assessed by creatinine clearance and the hematocrit value on admission and by the number of units of blood transfused. There was considerable overlap in the occurrence of G6PD deficiency, quinine ingestion, and malaria, suggesting that these factors may interact and that it may not be justifiable to regard hemoglobinuria caused by G6PD deficiency as a separate syndrome.
 ...
PMID:Blackwater fever in southern Vietnam: a prospective descriptive study of 50 cases. 940 15

Severe and complicated malaria is an important cause of mortality in Plasmodium falciparum infection. We describe in this study the details of 532 cases of such syndromes admitted to hospital during an outbreak of malaria between September-December 1994. Increase in the annual rain fall, collection of water around Indra Gandhi Canal, forestation of shrubs around it and migration of labor, adaptation of Anopheles stephensi to desert climate and favorable breeding of An. culicifacies in the areas under impact of irrigation were presumptive causes of the outbreak in this region. Cerebral malaria (25.75%), hepatic involvement (11.47%), spontaneous bleeding (9.58%), hemoglobinuria (7.89%), severe anemia (5.83%), algid malaria (5.26%), ARDS (3%) and renal failure (2.07%) were the important manifestations. The overall mortality was 11.09%, which was high because of infection in the non-immune population of this area. Ignorance about the severity of this disease and lack of transportation facility was another important factor. Morality was highest in ARDS (81.25%) followed by severe anemia (70.97%), algid malaria (46.43%), renal failure (45.45%), jaundice (36.06%) and cerebral malaria (33.57%). Pregnancy was an important determinant increasing the mortality in female patients. Mortality was very high (82.35%) in those persons who presented with more than 3 syndromes together.
 ...
PMID:Severe and complicated malaria in Bikaner (Rajasthan), western India. 944 3

An asymptomatic 26-year-old woman with mild macrocytic anemia (11.6 g Hb/dl) was studied. All biochemical parameters, bone marrow histology and cytogenetics were normal. The Ham's and sucrose tests were negative. A flow cytometric analysis revealed that CD55 and CD59 staining was absent in 20% and 21% of the granulocytes, but erythrocytes and CD34-positive bone marrow cells were CD55 and CD59 positive. Seven months after the initial study, the patient suffered an episode of hemoglobinuria, with mild anemia, moderate thrombocytopenia and a weak positive sucrose lysis test. A new flow cytometric analysis disclosed an increased percentage of CD55 and CD59 negative granulocytes and a 25% of erythrocytes with an intermediate pattern of fluorescence after CD59 labelling. At fourteen months, a population of CD55-deficient erythrocytes was detected and the Ham's test became positive. The present report is, to our knowledge, the first case of smoldering paroxysmal nocturnal hemoglobinuria in a patient with no previous aplastic anemia, or evident pancytopenia. The diagnosis was established by flow cytometry of peripheral blood granulocytes, with apparently phenotipically normal progenitor cells in an early stage of the disease. Flow cytometry appears to be a useful tool in our knowledge of paroxysmal nocturnal hemoglobinuria evolution.
 ...
PMID:De novo smoldering paroxysmal nocturnal hemoglobinuria: a flow cytometric diagnosis. 958 88

The caval syndrome is a serious complication of chronic heartworm (Dirofilaria immitis) disease in dogs and cats. The syndrome is characterized by acute anorexia, respiratory distress, weakness, right-sided cardiac murmur, anemia, hemoglobinuria, hepatic and renal dysfunction, signs of forward and backward heart failure, and, possibly, disseminated intravascular coagulation (DIC). Retrograde migration of adult heartworms from the pulmonary arteries to the right ventricle, right atrium, and venae cavae causes disruption of the tricuspid apparatus. Valvular insufficiency, with concurrent pulmonary hypertension, reduces cardiac output thus resulting in forward and backward heart failure. Additionally, red blood cells are traumatized and hemolyzed as they flow through the mass of worms. Therapy consists of supportive care and the removal of the heartworm mass from the right ventricular inflow tract. Caval syndrome in dogs and cats is associated with high mortality rates and generally has a guarded to poor prognosis.
 ...
PMID:Canine and feline caval syndrome. 975 97

Malaria remains an overwhelming problem in tropical developing countries, with 300 to 500 million new cases and 1.5 to 3.5 million deaths per year. Malaria is a potentially life-threatening disease for travelers to the tropics. Imported malaria is an important clinical problem in nonendemic areas of the world because of increasing numbers of travelers, overseas workers, and immigrants from endemic areas. According to the World Health Organization's criteria, the recognition of one or more of the following clinical features should raise the suspicion of severe malaria: cerebral malaria (unrousable coma), severe anemia (hemoglobin <5 g/dL), renal failure (serum creatinine >3 mg/dL), pulmonary edema or adult respiratory distress syndrome, hypoglycemia (glucose <40 mg/dL), circulatory collapse or shock, disseminated intravascular coagulation, repeated generalized convulsions, acidosis (pH <7.25), macroscopic hemoglobinuria, hyperparasitemia (>5 percent of the erythrocytes infested by parasites), or jaundice (bilirubin >3 mg/dL). Although only a small proportion of patients with malaria develops severe manifestations, these patients require the most urgent and intensive care. Mortality among patients with cerebral malaria, even when treated in modern intensive care units, exceeds 30%, and when complicated by the adult respiratory distress syndrome, it may approach 80%. Among travelers, mortality remains a serious issue because of failure to obtain and use preventive measures, delay in seeking medical attention, and misdiagnosis.
 ...
PMID:Imported severe falciparum malaria in Israel. 977 25


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