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A 5-year-old girl developed acute lymphoblastic leukemia (T-ALL) 15 months after being diagnosed with autoimmune hemolytic anemia (AHA), while AHA was in partial remission. AHA was mediated by warm antibodies. Because AHA could not be controlled during the induction therapy of ALL, she was administered immunoglobulin G and plasmapheresis was performed. Hepatomegaly dissappeared in the 4th month. However, anemia requiring blood transfusion, positive direct Coombs' test, and splenomegaly dissappeared in the 13th month of the leukemia treatment; reticulocytosis and decreased haptoglobin level persisted. AHA exacerbated in the 24th month of the ALL therapy. Prednisolone was started but the family refused to continue the therapy. This case presents some features that were not reported before, such that ALL was preceded by AHA and involved T-cell lineage, AHA was mediated by warm antibodies, and the two disorders took place in childhood. 相似文献
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Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a distinct entity with an aggressive course. Drugs, autoimmunity, and viruses have been implicated in its etiology. Without treatment, liver dysfunction is progressive and fatal. Although successful immunosuppressive treatment has been reported, a few patients have undergone liver transplantation with recurrence of the primary disease in the allograft. We report, an 18-year-old boy with progressive GCH with AHA without recurrence in the allograft following deceased donor (DD) liver transplantation. 相似文献
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TOORU KUDOH NOBUO NAGATA NOBUHIRO SUZUKI SHUJI NAKATA SHUNZO CHIBA TOMOYA TAKAHASHI 《Pediatrics international》1994,36(6):701-704
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. 相似文献
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Autoimmune hemolytic anemia 总被引:6,自引:0,他引:6
Agarwal B 《Indian journal of pediatrics》1998,65(5):663-668
Immune hemolytic anemia can be either isoimmune or autoimmune. Autoimmune hemolytic anemias (AIHA) consist of group of disorders
whose common characteristics are the presence of an antibody which in turn causes short red blood cell (RBC) life. The rate
and site of hemolysis and hence the clinical manifestations depends on the type of antibody attached and its propensity to
fix complement. Antibodies of the IgG class are most commonly responsible for AIHA in chidren. Rh erythrocyte antigen is involved
in more than 70% of cases. Since the antibody has its maximal activity at 37°C, the resultant hemolysis is called warm antibody
induced hemolytic anemia. This is a severe life threatening condition, the clinical features are : sudden onset of pallor,
jaundice and dark urine. The cornerstone of diagnosis is a positive Coomb’s antiglobulin test in the presence of hemolysis.
Coomb’s test has false negative and false positive rates in about 2–4% and 8% of all cases respectively. The modalities for
treatment of warm AIHA include blood transfusion, steroid therapy, intravenous gammaglobulin, plasma-pheresis and splenectomy.
The choice depends on the severity of the disease and child’s response to therapy. 相似文献
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Objective To study the clinico-hematological profile and treatment outcome in children suffering from auto immune hemolytic anemia (AIHA).
Methods Twelve children were diagnosed with auto immune hemolytic anemia over a period of four years. Direct antiglobulin test was
positive in all the cases. Other causes of hemolytic anemia like thalassemia syndromes, hereditary spherocytosis, G6PD deficiency
were excluded by appropriate tests. The children were followed up for 6 months to 4 years.
Results The age ranged from 7 mth to 9 yr with a mean age of 4.51 yr. All patients had pallor as the presenting complaint followed
by splenomegaly (83.3%), jaundice (66.7%), fever (50%) and bleeding manifestations (16.7%). 9 patients had primary disease
and 3 had secondary disease. Tubercular infection was seen in 2 patients with secondary disease. Jaundice was seen equally
in both the groups. Oral prednisolone produced remission in 83.3% cases. 4 patients (3 in primary and one in secondary group)
had relapse after initial response. All responded to a second course of steroids but had subsequent relapses and developed
a chronic course.
Conclusion Autoimmune hemolytic anemia is an uncommon cause of hemolytic anemia in children. Tubercular infection is an underlying pathology
in cases of secondary autoimmune hemolytic anemia. Although oral steroids induce remission in most of the cases, relapses
are common. 相似文献
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Infantile cytomegalovirus-associated autoimmune hemolytic anemia 总被引:2,自引:0,他引:2
Murray JC Bernini JC Bijou HL Rossmann SN Mahoney DH Morad AB 《Journal of pediatric hematology/oncology》2001,23(5):318-320
Autoimmune hemolytic anemia (AIHA) is a hematologic disorder that is rarely seen in infants and young children. Most cases are associated with viral or bacterial infection, but the immunologic events leading to hemolysis are poorly understood. We describe two infants with severe cytomegalovirus (CMV)-associated warm antibody AIHA. One case was immunohematologically analyzed and showed suggestive evidence that endogenous anti-CMV IgG antibodies were the pathogenic antibodies leading to hemolysis, implicating a possible causal relationship between AIHA and CMV infection. Both patients were ultimately treated with intravenous CMV immune globulin, with subsequent improvement. These cases suggest that investigation for the presence of CMV in infantile AIHA is warranted and that CMV immune globulin should be considered as a therapeutic option. 相似文献
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Naithani R Agrawal N Mahapatra M Kumar R Pati HP Choudhry VP 《Pediatric hematology and oncology》2007,24(4):309-315
The clinical and hematological profile and treatment outcome of children with warm autoimmune hemolytic anemia (AIHA) were assessed using retrospective case record analysis. There were 26 (17 idiopathic; 9 secondary) patients with a median age of 11 years. Pallor (100%), fever (39%), and jaundice (59%) were the main presenting complaints. Jaundice was much more common in idiopathic (70%) compared to secondary (44%). Direct antiglobulin test was negative in 3 patients. Oral prednisolone produced remission in 81% patients. Four patients relapsed after a median period of 7 months (2 months to 2 year) after response. All responded to a second course of steroids in median 14 days. One child required cyclosporin A in addition. No correlation was found between response and parameters such as age, sex, jaundice, low pretreatment hemoglobin, reticulocyte count, total leukocyte count, platelet count, subtype of AIHA, and hepatosplenomegaly. Relapse correlated with increased duration between the onset of symptoms and treatment. This study indicates that oral prednisolone is an effective therapy for autoimmune hemolytic anemia. In refractory cases cyclosporine A may be useful. 相似文献
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