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

Seven English Springer Spaniels (6 adult males and 1 female) with chronic hemolysis and sporadic intravascular hemolytic crises were determined to have a deficiency in erythrocyte phosphofructokinase (PFK) activity, a key regulatory enzyme of anaerobic glycolysis. Intermittent severe pigmenturia concomitant with weakness, lethargy, and anorexia were the major clinical signs and commonly were related to exercise or other stressful situations that caused panting or barking (hyperventilation). Pale or icteric mucous membranes, fever, mild hepatosplenomegaly, and muscle wasting sometimes were evident. Results of routine laboratory testing indicated a persistent marked bilirubinuria and reticulocytosis with normal PCV, to severe anemia and intermittent hemoglobinuria and hyperkalemia. Erythrocyte PFK activities were severely reduced to 8% to 22% of values for control dogs. The block of glycolysis at the PFK step caused a markedly diminished erythrocyte 2,3-diphosphoglycerate content, resulting in an increased hemoglobin-oxygen affinity and compensatory accelerated erythrocyte production. Phosphofructokinase-deficient erythrocytes had increased alkaline fragility in vitro and in vivo. Hemolytic crises were induced in vivo by hyperventilation that caused transient, mild alkalemia. Studies of family members of a PFK-deficient dog suggested an autosomal recessive mode of inheritance. Carrier dogs with half-normal erythrocyte PFK activities appeared clinically normal.
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PMID:Hemolysis caused by phosphofructokinase deficiency in English springer spaniels: seven cases (1983-1986). 295 37

The current increased participation in long distance running and endurance events brings new challenges to the physician. Adaptive changes of exercise must be differentiated from disease states. Left ventricular hypertrophy, Wenckebach rhythm, elevated creatine kinase (myocardial fraction), gastrointestinal bleeding, hematuria, hemoglobinuria, amenorrhea, and apparent anemia may all be exercise related. New syndromes have arisen--from "runner's high" to athletic amenorrhea. These dedicated athletes can be difficult patients. They are highly motivated and will continue training despite significant pain and danger of permanent damage. Speaking their language--carbohydrate loading, "the bonk," "slow twitch," "the wall"--is helpful in their treatment. Traditional health problems are less likely to bring the fitness addict to the physician than some new and peculiar disorders.
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PMID:Endurance athletes: physiologic changes and nonorthopedic problems. 353 54

Paroxysmal cold hemoglobinuria is a rare and potentially life-threatening acquired hemolytic anemia occurring either as an acute transient anemia following several different viral syndromes, or in a chronic idiopathic form. Episodic hemolysis in paroxysmal cold hemoglobinuria is usually associated with a biphasic (Donath-Landsteiner) IgG cold-reactive complement-fixing autohemolysin with anti-P specificity. Paroxysmal cold hemoglobinuria has not previously been associated with malignancy nor has it been clearly shown to be steroid-responsive. This report describes a patient with steroid-responsive autoimmune hemolytic anemia and immune thrombocytopenia (Evans' syndrome) associated with oat cell carcinoma of the lung and a unique biphasic anti-IgM autohemolysin. This case extends the spectrum of biphasic antibody-mediated immune cytopenias and widens both the clinical and the serologic definition of paroxysmal cold hemoglobinuria.
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PMID:Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria. 357 44

Anemia, hyperbilirubinemia, and reticulocytosis subsequent to viral infection were present in a 32-year-old woman. The direct antiglobulin test was negative, and no unexpected antibodies were detected in pretransfusion tests. Rosettes of red cells (RBCs) around neutrophils were observed in peripheral blood smears, and a Donath-Landsteiner (D-L) test was positive. However, the patient did not show the classic features of paroxysmal cold hemoglobinuria (PCH). There was no hemoglobinuria, and in vivo hemolysis was not precipitated by cold. The D-L antibody was IgG, but classic anti-P specificity was not apparent. Rather, protease- or neuraminidase-treated RBCs, as well as certain sialic acid deficient RBCs of uncommon MN phenotypes, were not hemolyzed in D-L tests. Further, D-L antibody activity could be inhibited by MN sialoglycoprotein. These data support a diagnosis of chronic D-L hemolytic anemia, caused by an anti-Pr-like biphasic hemolysin.
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PMID:Donath-Landsteiner hemolytic anemia due to an anti-Pr-like biphasic hemolysin. 376 34

Delayed hemolytic transfusion reactions usually occur as a result of a secondary immune response with maximal hemolysis occurring seven days posttransfusion. We report a delayed hemolytic transfusion reaction in which hemoglobinuria, anemia, and reticulocytosis developed four weeks after transfusion. The incriminated antibody, anti-C, was first detected eight weeks posttransfusion using enzyme-treated red blood cells. We conclude, that in all likelihood, this hemolytic transfusion reaction was due to a primary immune response, this case illustrates the importance of sequential testing in cases of suspected transfusion reactions.
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PMID:Delayed hemolytic transfusion reaction caused by a primary immune response. 680 51

Four patients experienced combined sickle cell disease and autoimmune hemolytic anemia within the past ten years. A fifth patient had positive direct antiglobulin test results without verified autoimmune hemolysis. Severely accelerated hemolysis was observed in four patients; anemia was severe, and the reticulocyte count rose into the 60% to 88% range. During the period of active autoimmune hemolysis, decline of the reticulocyte count rose into the 60% to 88% range. During the period of active autoimmune hemolysis, decline of the reticulocyte count into the 6% to 16% range was associated with rapid decrease in the hemoglobin level, requiring transfusion. All five patients were already alloimmunized by transfusions administered before onset of the autoimmune hemolytic anemia; two or more allospecificities were identified in four of five patients. The presence of autoantibody notably compromised compatibility testing; three patients experienced posttransfusion hemoglobinuria, and in vivo cross matching with 51Cr-labeled donor RBCs was employed on three occasions. All patients responded to corticosteroids; mercaptopurine was also administered to one patient. The direct antiglobulin test result reverted to negative in all patients after hospital discharge.
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PMID:Combined sickle cell disease and autoimmune hemolytic anemia. 724 97

We report the hematologic and clinical features of four adult patients (Pts.) with sickle cell anemia and iron-limited erythropoiesis. Two of the Pts. had spontaneous iron deficiency (chronic GI bleeding, low-grade hemoglobinuria). In the other two Pts. iron restriction was induced by periodic RBC aphereses as part of a pilot protocol designed to decrease intracellular HbS polymerization by MCHC reduction. Iron-limited erythropoiesis was defined by reduction in red cell indices (MCV range 60.4-67 fl) in the presence of low serum ferritin (range < 10-20 ng/ml). In these Pts. iron restriction did not cause clinically significant worsening of the anemia (Hb 7.8-9.0 g/dl). In two Pts. the anemia actually improved. Other hematologic effects of iron restriction were: decreased MCHC, reticulocyte count, RDW, and dense cells. A reduced hemolytic rate was suggested by a lowering of serum bilirubin and LDH. In one of the Pts. the 51Cr RBC T1/2 survival increased from 12 to 16 days. The intracellular HbS polymer fractions (fp) were determined at 25% O2 by Csat and with the use of the conservation of mass equation. The baseline fp values ranged from 0.48-0.53. After iron restriction they ranged from 0.33-0.48. The fp decreased even though iron-limited erythropoiesis also lowered the Hb F concentration in three of our Pts. In one of the two Pts. with induced iron depletion, hospitalization days for pain crises decreased from an average of 4.5 days/month (2 year baseline period) to an average of 0.5 days/month in the 3 year follow-up after iron depletion. The second patient with induced iron restriction experienced the rapid healing of a leg ulcer. Controlled iron restriction should be explored as a therapeutic strategy in selected SS patients.
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PMID:Improvement of sickle cell anemia by iron-limited erythropoiesis. 766 35

A four-year-old, spayed female toy fox terrier presented with hyperglycemia and severe anemia. A diagnosis of immune-mediated diabetes mellitus was made based upon the finding of beta-cell specific antibodies. Immune-mediated hemolytic anemia was diagnosed based on findings of a regenerative anemia, spherocytosis, hyperbilirubinemia, hemoglobinuria, and bilirubinuria. The anemia resolved following two months of immunosuppressive therapy. The diabetes was treated with insulin for four months, after which time treatment was no longer necessary. However, the dog remained positive for beta-cell antibodies which may be a predictive marker for the recurrence of diabetes mellitus in the future.
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PMID:Canine immune-mediated diabetes mellitus: a case report. 755 60

A 25-year-old man presented with a history of fever, chills and vomiting for three days. The parasite count was 207 ring-forms of P. falciparum per 1000 red cells. He developed hemoglobinuria and excreted hemoglobin in the urine 0.20-0.30 g/dl for 14 days during admission. Many blood transfusions were administered for correcting anemia. Although the malarial parasites disappeared one week after anti-malarial therapy, however, the fever and hemoglobinuria persisted. The Weil-Felix reaction OXK was positive with a titre of 1:40 on admission and increased to 1:160 on the second week. Chloramphenical and prednisolone were given for treatment of typhus fever and all symptoms subsided. Serum TCII levels were found to be increased and persisted high during the hemoglobinuria. The clearance of TCII was lower and increased relatively slowly to the normal level on day 30. On the other hand, TCII excretion in the urine was found to be increased during hemoglobinuria. These findings indicate that the catabolism and clearance of TCII in this patients is impaired with increased TCII excretion in the urine in parallel to the hemoglobinuria. Serum TCII level is, therefore, increased and persistently high in a patient with malaria and typhus fever infections with hemoglobinuria.
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PMID:Persistently elevated serum transcobalamin II in a patient with cerebral malaria and typhus infections. 762 77

A case of drug-induced immune hemolytic anemia is described. A 2 year old boy exhibited sudden anemia and hemoglobinuria after administration of minocycline (MINO). The specific immunoglobulin G antibody against MINO was demonstrated in the patient's serum by western blotting. This is a rare example where anti-minocycline immune complex-mediated hemolysis was responsible for an intravascular hemolytic process.
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PMID:Minocycline-induced hemolytic anemia. 787 87


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