[Symptomatic hemolytic anemia with positive Coomb's test in osteomyeloreticulosis].
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A case with autoimmune hemolytic anemia (AIHA) induced by interferon-alpha (IFN-alpha) is presented. A 40-year-old male who had a previous history of autoimmune hemolytic anemia, agranulocytosis and thrombocytopenia was admitted to our hospital because of chronic C type hepatitis. Liver biopsy was performed, which diagnosed chronic active hepatitis and IFN-alpha was administrated at a dose of 3 Meg unit per day. 11 days after the initiation of the therapy he developed hemolytic anemia, but Coombs tests were negative. Although IFN was withdrawn 15 days later, anemia became progressively more serious. 20 days later, both direct and indirect Coombs tests became positive. He was diagnosed as AIHA and treated with methylprednisolone pulse therapy, then he recovered soon afterward. Further analysis of Coombs tests revealed that he had both cold type and warm type (IgG) autoantibodies which was the same type of antibodies for AIHA he suffered 10 years ago. In conclusion, latent AIHA may be reactivated by the treatment with IFN-alpha.
We report a case of Coombs positive autoimmune hemolytic anemia occurring in a patient with chronic hepatitis C, never treated with interferon-alpha. Prednisone treatment induced the complete remission of both clinical and hematological findings after 2 months. The indirect Coombs test turned negative, while the direct Coombs test remained weakly positive. Autoimmune hemolytic anemia during chronic hepatitis C has been reported to develop only after or simultaneously with interferon-alpha therapy. After discarding other possible causes such as drugs, infectious, neoplastic, lymphoproliferative diseases, and essential mixed cryoglobulinemia, we made the hypothesis of a correlation between autoimmune hemolytic anemia and hepatitis C virus infection.
Autoimmune hemolytic anemia has been rarely described in patients with chronic hepatitis C during interferon-alpha therapy. We report a case of Coombs-positive autoimmune hemolytic anemia, occurring 9 months after the onset of monotherapy with interferon-alpha-2b (3 million units thrice in week) for chronic hepatitis C. Interferon withdrawal and prednisone treatment induced a complete remission of hematological findings after 3 months. During a follow-up period of 30 months after cessation of interferon, hemoglobin levels stabilized about 16 g/dL, and the indirect Coombs test turned negative, while the direct Coombs test remained weakly positive; besides, serum alanine aminotransferase values were normal and hepatitis C virus-RNA remained undetectable. In our patient, clinical and laboratory findings suggested a correlation between autoimmune side effects of interferon-alpha and autoimmune hemolytic anemia.
In ABO incompatible infants, a strong association between Coombs test positivity and hyperbilirubinaemia in the first few days of life has been recorded in all samples studied. A remarkable variability between ethnic groups and between different series of infants from the same group has been also observed. A discriminant analysis carried out on several maternal and neonatal variables in two samples of ABO incompatible infants has shown that the Coombs test is the most important predictor of jaundice. Gestational age and birth weight in white and birth weight in black infants gave also important and independent contributions as discriminating variables. Among ABO incompatible black infants the incidence of hyperbilirubinaemia was higher than among ABO incompatible white infants. Discriminant analysis suggests the genetic and environmental factors which predispose to jaundice the ABO incompatible black infants may act mainly through immunological and developmental mechanisms accounted for by Coombs test, gestational length and birth weight.
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During 1968-1973 510 Rh-negative, non-Rh-immunized primi- and multigravidae giving birth to Rh-positive infants, regardless of the ABO constellation, received 250 mug immunoglobulin anti-D post partum. In serological follow-ups using the indirect Coombs' test and the papain method more than six months after childbirth, Rh-antibodies were detected in two cases with papain but not at all with the indirect Coombs' test. Seventy women gave birth to Rh-positive babies in subsequent pregnancies. Twenty-six of these non-Rh-immunized women were given an intramuscular injection of 250 mug anti-D five to ten weeks before delivery and were included in a series comprising a total of 131 Rh-negative women who had received immunoglobulin anti-D in the estimated 32nd-34th week of pregnancy. Bilirubin and haemoglobin were determined in the neonates on cord blood and serum. The infants whose mothers had received immunoglobulin anti-D during pregnancy showed no signs of haematological abnormalities related to the administration of immunoglobulin anti-D. The determination of cord blood haemoglobin or bilirubin and serum bilirubin revealed no significant difference between Rh-positive infants and Rh-negative ones. Antibodies by the papain method were detected 41 of the women at the time of delivery (22 Rh-positive babies and 19 Rh-negative ones). There was no correlation between the time at which immunoglobulin anti-D was administered and the detection of antibodies at the time of delivery, regardless of the Rh group of the infant. The indirect Coombs' test was positive only in ten of the 48 Rh-negative infants examined. 250 mug immunoglobulin anti-D provides effective post partum prophylaxis, and the same dose administered to Rh-negative pregnant women prior to delivery did not cause any detectable haemolytic damage to the fetus.
INTRODUCTION: There are two types of autoimmune haemolytic anemia, idiopathic or associated to other disorders (infections, thyroidal diseases, cancer.). The treatment of this particular anemia depends on the primary or secondary feature. EXEGESIS: We report the case of a 76-year old woman, presenting an autoimmune haemolytic anemia with a positive direct Coombs test in IgG. The corticodependance of this anemia after 6 months of treatment had indicated a splenectomy. During surgery, a gastric stromal tumor was discovered. After removal of the tumor and splenectomy, the haematological symptomatology quickely disappeared and induced the negativation of the Coombs test within a post operative period going from 7 to 14 months, without any immunosuppressor treatment. CONCLUSION: Splenectomy does not induce the negativation of a Coombs test in an autoimmune haemolytic anemia, someaning that there is a link between the anemia and the tumor. This observation is completed by a review the literature.
Hydrops fetalis (HF) consists of an abnormal accumulation of fluid in two or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema. Almost all observed cases of HF are of the nonimmune type, the causes of which remain undetermined in 15% of patients. We report a newborn infant with nonimmune hydrops fetalis (NIHF) and congenital hypothyroidism. The infant's mother was healthy and there were no malformations of the placenta or umbilical cord. The infant did not show any structural abnormalities of his central nervous, cardiovascular, gastrointestinal, or urinary tract systems, and there was no evidence of anemia, infectious disease, or inborn error of metabolism. An immune-based process was unlikely, because the blood group of the mother and infant was A-positive and results of an indirect Coombs test in the mother and a direct Coombs test in the infant were negative. The patient's condition gradually improved with mechanical ventilation, repeated thoracocentesis, and total parenteral nutrition. By day 5 of age the skin edema, pericardial effusion, and ascites disappeared, but accumulation of significant amounts of chylous pleural fluid persisted. Because of lethargy, FT4 and thyroid-stimulating hormone levels were obtained and showed hypothyroidism. Thyroid hormone supplementation was then started, and within 4 days the infant became more vigorous and was weaned from mechanical ventilation. After 7 days, the chylothorax resolved completely as the serum thyroxine level normalized. No reaccumulation of pleural effusion was noticed. The infant started to gain weight and was discharged from the hospital at 35 days of age. A possible pathophysiologic association between congenital hypothyroidism and NIHF is discussed. NIHF may be caused by lymphatic congestion attributable to an impairment of lymphatic flow and a delayed return of lymph to the vascular compartment. There could be a possibility that because of thyroid hormone deficiency in this patient, there was reduced adrenergic stimulation of the lymphatic system. This could result in a sluggish flow of the lymph with engorgement of the lymphatic system, leakage of lymph into the pleura and the interstitial spaces, and the production of chylothorax with NIHF. Animal studies demonstrate a direct relationship between lymph flow rate or lung liquid clearance and adrenergic receptor activity in the lymphatic system. These observations support our hypothesis that deficient adrenergic activity in congenital hypothyroidism might lead to chylothorax with NIHF in the fetus. We speculate that thyroid hormone may play a role in the regulation of adrenergic receptors in the lymphatic system and lungs, thus modulating both the lymphatic flow rate and lung liquid clearance, and facilitating the resolution of chylothorax. Examination of thyroid functions should be included in the investigation of fetuses and neonates with NIHF of an obscure origin.