Serologic profile in autoimmune hemolytic disease: pathophysiologic and clinical interpretations.
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Anemia, Hemolytic, Autoimmune↗
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[A case of UIP with autoimmune hemolytic anemia].
A 70-year-old male was admitted because of cough and sputum. Chest X-ray showed bilateral interstitial shadows, and usual interstitial pneumonitis was diagnosed by TBLB and BAL. He was followed without medication. The dyspnea increased and the abnormal shadow spread on chest X-ray. Autoimmune hemolytic anemia was newly diagnosed because the RBC count was 195 x 10(4)/mm3, Hb was 8.6 g/dl, and direct Coombs test and warm antibody were positive. In this case, there appears to be a relationship between deterioration of interstitial pneumonia and the on-set of autoimmune hemolytic anemia.
Aged↗
[Wilson disease, IgA glomerulonephritis and vascular purpura: an incidental association?].
BACKGROUND: IgA nephropathy may occur in adults with alcoholic cirrhosis. It has also been reported in children with alpha-1-antitrypsin deficiency. A case of IgA nephropathy associated with cirrhosis due to Wilson's disease is reported. CASE REPORT: A 10 year-old girl, was admitted for cirrhosis. She had suffered from ascitis and hematuria since the age of 6 years and vascular purpura since the age of 9 yr 6 mo. At admission, she had atrophic liver cirrhosis, ascitis splenomegaly and petechiae on her legs. There was microcytic anemia due to iron deficiency. The Coombs test was positive. There were hypoalbuminemia (2 g/dl) and polyclonal hyperglobulinemia (IgA 750 mg/dl, IgG 1670 mg/dl and IgM 250 mg/dl). Her serum concentrations of complement factors were normal and no immune complexes were detected in serum. She also showed biological changes due to kidney failure, hematuria and proteinuria. A liver biopsy showed micronodular cirrhosis with numerous plasma cells containing IgA. The search for an etiology showed ceruloplasmin levels of 5 mg/dl, elevated urinary copper excretion (> 150 micrograms/day) and above normal liver copper (255 micrograms/g dry weight); Kayser-Fleischer ring was also seen. The renal biopsy showed membranoproliferative glomerulonephritis, and immunofluorescent microscopy revealed C3 and IgA in the mesangial and subendothelial regions. The patient was given spironolactone and furosemide followed by triethylenetetramine dihydrochloride. CONCLUSIONS: The IgA nephropathy, vascular purpura and elevated serum IgA levels could be due to the liver changes. If so, they should be improved by a liver transplant, although our patient would be a better candidate for combined liver and kidney transplantation.
Child↗
Case report--a neonate with nonimmune hydrops fetalis.
A post-dated intra-uterine growth retarded male Malay baby was born to a 30-year-old mother gravida II by Caesarean section. Her previous pregnancy ended in still-birth. The baby was severely asphyxiated at birth. He was intubated and immediately admitted to the neonatal intensive care unit. He had anasarca, anaemia, purpura and firm, massive hepatosplenomegaly. X-rays revealed ascites and bilateral metaphysiitis of the long bones. The haemoglobin level was 5.0 gm/dl and PCV 18.3%. Coombs' test was negative. Prothrombin time (PT) and partial thromboplastin time (PTT) were prolonged. The baby and mother were positive for Venereal Disease Research Laboratory (VDRL) and the treponema pallidum haemagglutination assay (TPHA) tests. The baby was actively resuscitated but expired at three and a half hours of life due to overwhelming sepsis associated with severe anaemia and disseminated intravascular coagulation.
Adult↗
Unusual clinical features of neonatal anemia due to fetomaternal transfusion.
Fetomaternal transfusion occurs in about 50 percent pregnancies. However, it can cause severe neonatal anemia at birth. A one-day-old baby girl, small for gestational age, was born to a G1P1 woman without perinatal blood loss or obstetric complication. Pallor and mild tachypnea were noted after birth. Laboratory examination revealed severe anemia, (Hb: 4.8 gm/dl, Hct: 14.7%), reticulocytosis and normoblastemia. Direct coombs' test was negative. Brain echo examination was normal. Maternal peripheral blood smear stained with Kleihauer-Betke technique demonstrated presence of fetal cells. Following packed red blood cells transfusion, the baby's symptoms improved. The unusual clinical features were described and discussed. Kleihauer-Betke test should be performed in unexpected neonatal anemia after birth.
Anemia, Neonatal↗
[A case of Evans' syndrome in which CD4+CD45RA+ cells markedly decreased in its active phase].
A 38-year-old man was admitted to our hospital because of exertional dyspnea in February, 1990. The patient had already been diagnosed as having autoimmune hemolytic anemia (AIHA) in February, 1982 and treated with prednisolone (PSL) until January, 1988. The laboratory examination confirmed the relapse of AIHA (IgG-warm type) and additionally disclosed the marked decrease of CD4+CD45RA+/CD4+ ratio in peripheral blood lymphocytes. Thereupon, the patient was treated again with PSL and entered the remission in one month. Simultaneously, CD4+CD45RA+/CD4+ ratio also increased to the normal level. Afterwards, the remission had been maintained for about six months with a small dose of PSL. However, in September, 1990, the hemolysis relapsed with marked thrombocytopenia and decreased CD4+CD45RA+ ratio. Then the diagnosis was corrected to Evans' syndrome because PAIgG was highly elevated together with positive Coombs' test. Although danazol and azathioprine were administered in addition to PSL, the disease remained in active phase. Thus, splenectomy was carried out in March, 1992. Consequently, the patient entered the remission that has been maintained over six months. CD4+CD45RA+/CD4+ ratio was also normalized. These results suggest that CD4+CD45RA+ cells may play an important role in the pathogenesis of AIHA or Evans' syndrome.
Adult↗
[Silicosis associated with autoimmune hemolytic anemia].
A 60-year-old man with pneumoconiosis complained of general fatigue, exertional dyspnea, and anorexia. The patient had severe anemia, and laboratory examination revealed autoimmune hemolytic anemia with positive direct and indirect Coombs tests. After corticosteroid therapy, the anemia resolved markedly, and the antinuclear antibody test became negative. However, the silicotic shadow on chest X-ray film showed no remarkable change. The autoimmune hemolytic anemia in this case was probably due to an immunological disturbance caused by silicosis.
Anemia, Hemolytic, Autoimmune↗
[Complications of exchange transfusion in term and preterm newborns].
Exchange transfusion has an important role in the treatment of hyperbilirubinemia of the newborn. It is used in attempts to prevent kernicterus when bilirubin levels are high. We describe our experience in 203 exchange transfusions performed on 143 infants (81 males and 62 females) with hyperbilirubinemia during 1983-1992. In only 30% of cases was there a specific etiological diagnosis of the jaundice based on a positive Coombs test, G6PD deficiency, or the presence of sepsis or maternal diabetes; the rest were idiopathic. 57% of the neonates were premature (26-36 weeks of gestation). Premature neonates underwent more transfusions than full-term infants (1.6 vs 1.2). There was no direct death from exchange transfusion; morbidity was 6.3% (including bradycardia, apnea, thrombocytopenia, hypoglycemia and hyponatremia). Most complications occurred in preterm infants and those severely ill. All complications were treated immediately and there were no sequelae.
Exchange Transfusion, Whole Blood↗
[Five cases of hereditary high red cell membrane phosphatidylcholine hemolytic anemia in three families].
Five cases of hereditary high red cell membrane phosphatidylcholine hemolytic anemia in three families were described. All cases were clinically manifested by jaundice and splenomegaly. Hemolysis was evident from indirect hyperbilrubinemia, reticulocytosis and decrement of serum haptoglobin. Red blood cells showed morphological abnormalities such as poikylocytosis, anisocytosis and target cells on blood smears. Both direct and indirect Coombs' tests were negative. Ham test, sugar water test and hemoglobin electrophoresis showed no abnormalities. Osmotic fragility test showed decreased membrane fragility. Lipid analysis of red cell membrane showed increment of phosphatidylcholine content and decrement of sphingomyelin content, although plasma lipids were essentially normal. Influx and efflux of sodium through the red cell membrane were both increased. Splenectomy was performed without effect on one patient and the mother of other patients.
Adult↗
Infective endocarditis complicated with rapidly progressive glomerulonephritis: a case report.
A 26-year-old man who had suffered from intermittent chills and fever over a two month period was quite clear of heart or kidney involved developed acute deterioration of renal function. A new pansystolic murmur over the apex of the heart was heard on auscultation, and echocardiography clearly showed a vegetation about 0.7-0.9 cm in size on the atrial site of the mitral value. Laboratory investigation displayed normochromic anemia with negative Coombs' test. Immunological studies were positive for rheumatoid factor and circulating immune complex. High serum levels of erythrocyte sedimentation rate and C-reactive protein, nephritic sediment of urinalysis and negative blood cultures for bacteria, tuberculosis or fungus were also noted. Abdominal sonography showed normal kidney size, bilaterally. Renal biopsy revealed typical crescentic glomerulonephritis. After intravenous penicillin therapy for two weeks, the serum creatinine level recovered from 6.7 mg/dl to 2.0 mg/dl and circulating immune complex disappeared. In consideration of cardiac insufficiency and the potential risk for complications of the vegetation, the patient underwent mitral valve replacement. Four weeks after operation, all the abnormal data had resolved completely. These data suggested that infective endocarditis with rapidly progressive glomerulonephritis is curable by antibiotic therapy and surgical intervention.
Adult↗
[Chronic recurrent polyneuropathy in a patient with autoimmune hemolytic anemia associated with antibodies to gangliosides GM1, GA1, and GD1b].
We report a case of chronic recurrent polyneuropathy with warm-type autoimmune hemolytic anemia (AIHA) associated with antibodies to gangliosides GM1, GA1 and GD1b. There have been few reports of AIHA associated with polyneuropathy. A 39-year-old man was admitted in May 1991 because of severe hemolytic anemia. Direct Coombs' test was positive of IgG1 and complement type. The treatment was started with prednisolone, the hematological abnormalities disappeared after 6 months, but thereafter, he developed muscle weakness of all limbs and AIHA within 7 weeks. On neurological examination, he was unable to arise from a chair and could not stand, had muscle atrophy and numbness of the distal part of all limbs and areflexia. Cerebrospinal fluid was acellular and protein content was 117 mg/dl. Electrodiagnostic studies showed mild slowing of nerve conduction velocities associated with severe decrease in amplitudes of compound muscle action potential and multifocal conduction block. A sural nerve biopsy specimen showed moderate loss of small myelinated fibers, but not of unmyleinated fibers. AIHA was substantially improved by methylprednisolone (1,000 mg/day) for 3 days, but neurological symptoms remained unchanged. A month later, he was given 2 courses of intravenous gammaglobulin infusion (0.4 g/kg/day) for 5 days. He was able to walk after the second course. In May 1992, the mild motor-sensory polyneuropathy recurred and gammaglobulin infusion was effective for neurological symptoms. We detected the high level of anti-GM1 antibody titer in the patient's serum by ELISA, the level of the antibody titers correlated with neurological manifestation. A thin-layer chromatography with immunostaining showed the presence of anti-GM1, GA1 and GD1b antibodies in his serum. We grouped the reported cases of AIHA into cold and warm types, according to its hemolytic temperature, and characterized the neurological manifestations in each type. We also discussed the pathogenic role of anti-gangliosides antibodies in this case.
Adult↗
[Immunoblastic lymphadenopathy-like T cell lymphoma associated with erythroid hypoplasia and thrombocytopenia].
A 71-year-old female patient was admitted with generalized lymphadenopathy, anemia and thrombocytopenia. On admission a peripheral blood examination showed a red blood cell (RBC) count of 1.95 x 10(6)/microliter, hemoglobin (Hb) 5.0 g/dl, platelet count (Plt) 2.2 x 10(4)/microliter and no reticulocytes. A bone marrow aspiration specimen was hypocellular with a nuclear cell count 1.0 x 10(4)/microliter, erythroblasts less than 0.3% and no megakaryocytes. Serum examination showed polyclonal hypergammaglobulinemia and the results of the direct/indirect Coombs test indicated the existence of auto-antibodies. as immunoblastic lymphadenopathy-like T cell lymphoma was diagnosed based on the lymph node biopsy specimen. Cyclophosphamide, doxorubicin and etoposide obtained improvement of lymphadenopathy, hypergamma globulinemia, hypoplastic bone marrow and thrombocytopenia. On the other hand, the anemia did not improve and the ratio of erythroblasts in total bone marrow cells was remainde 0.5%. The detailed mechanism of this hypoplastic anemia is still unknown, however, our results imply some
Aged↗
Recurrent cold hemagglutinin disease following allogeneic bone marrow transplantation successfully treated with plasmapheresis, corticosteroid and cyclophosphamide.
A 10-year-old male with severe aplastic anemia following allogeneic BMT developed a hemolytic crisis on post-BMT day 67. The diagnosis of cold hemagglutinin disease was made based on the findings of anemia, reticulocytosis, positive direct Coombs test, and markedly elevated cold agglutinins. Anti-nuclear antibody and anti-DNA antibody were also increased. Plasmapheresis was effective as an emergency measure. Corticosteroid after plasmapheresis had a transient effect. At the second episode of hemolysis 6 months after BMT, immunosuppressive therapy with cyclophosphamide plus corticosteroid was successfully administered without negative effect on engraftment.
Anemia, Aplastic↗
Systemic lupus erythematosus: predictors of its occurrence among a cohort of patients with early undifferentiated connective tissue disease: multivariate analyses and identification of risk factors.
OBJECTIVE: To determine predictors of the occurrence of systemic lupus erythematosus (SLE) in patients with early (< or = 1 yr) undifferentiated connective tissue disease (CTD). METHODS: Analysis of a cohort of 213 patients with early undifferentiated CTD at entry, followed for 5 yrs at 11 tertiary centers. Baseline demographic, clinical, and laboratory data were compared using univariate and Cox multivariate regression analyses to identify possible predictive features for the subsequent occurrence of SLE. RESULTS: 143 of 213 patients had ascertainable clinical status at 5 yrs. By univariate analyses those who evolved to SLE (13%) were more likely to be younger, African-American, and to have alopecia, serositis, discoid lupus, positive Coombs' test, positive anti-dsDNA and anti-Sm antibodies, positive ANA (homogeneous pattern), and/or a false positive test for syphilis. Discoid lupus (relative risk = 15.8), serositis (4.1), ANA-homogeneous (4.8), and anti-Sm positivity (28.2) were retained as predictors of the occurrence of SLE in the Cox regression model. CONCLUSION: Some clinical and laboratory features in patients with early undifferentiated CTD can predict the subsequent occurrence of SLE.
Adult↗
[Hemolytic anemias observed in SLE].
Hemolytic anemia observed in patients with SLE include autoimmune hemolytic anemia (AIHA) and thrombotic microangiopathic hemolytic anemia (TMHA). AIHA is caused by anti-erythrocyte antibody, which can be detected by direct Coombs' test. Recently, anti-phospholipid antibody draw attention as a cause of AIHA in SLE. TMHA include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Patients with TTP show CNS symptoms and renal damage, while those with HUS develop renal insufficiency, but show minimal CNS symptoms. Clinical findings of TMHA are fever, hemolytic anemia, thrombocytopenia, CNS symptoms, and renal insufficiency, which are quite same as the findings of SLE itself. TMHA should be considered for differential diagnosis in acute exacerbation of SLE.
Anemia, Hemolytic↗
[Sarcoidosis in a patient with autoimmune hemolytic anemia].
A 65-year-old woman was admitted to our hospital because of severe anemia. A skin biopsy was done in January 1994 and sarcoidosis was diagnosed. Diffuse reticular shadows were seen in both lung fields on a chest X-ray film and mediastinal lymph node swelling was seen on a chest CT scan. She was followed as an outpatient and was not treated. She suddenly experienced vertigo and general fatigue in March 1995. Laboratory findings on admission were as follows: Hb 6.2 g/dl, MCV 115.9 fl, Ret 198%, LDH 732 IU/L, I-Bil 1.9 mg/dl, and Coombs' test was positive. Autoimmune hemolytic anemia was diagnosed, and she was treated with prednisolone (1 mg/kg). As of the time of this writing, she has no relapse of hemolytic anemia though prednisolone was discontinued 6 months ago.
Anemia, Hemolytic, Autoimmune↗
[Rapid onset of hemolytic anemia after allogeneic bone marrow transplantation from an unrelated ABO major mismatched donor].
A 46-year-old woman with chronic myelogenous leukemia received allogeneic bone marrow transplantation from an unrelated human leukocyte antigen (HLA) matched (but mixed lymphocyte culture (MLC) positive to graft-versus host disease (GvHD) donor. The blood type of the recipient was A type Rh (+) while the donor blood type was B type Rh (+). The patient received busulfan 8 mg/kg, cyclophosphamide 120 mg/kg, and total-body irradiation 10 Gy before bone marrow transplantation. Short-term administration of methotrexate and cyclosporin was given for prophylaxis of GvHD. The mononuclear cells harvested from the donor were concentrated by COBE Spectra before bone marrow transplantation. Although engraftment of transplanted bone marrow in the recipient was confirmed on day 11, the patient suffered from severe anemia on day 10. Since the direct Coombs' test to A type red blood cells was positive, and anti-A antibody titer increased 16-fold, we diagnosed her anemia as hemolytic anemia caused by ABO mismatched transplantation. In addition to hemolytic anemia, she had skin symptoms of acute GvHD grade II, microangiopathic hemolytic anemia, and died of multiple organ failure on day 44. This experience indicated that some allogeneic transplant recipients are at risk of severe hemolytic anemia in the early stage after unrelated ABO mismatched donor and that it is necessary to establish proper treatment and prophylaxis.
ABO Blood-Group System↗
[Acute hemolytic crisis as the initial manifestation of Wilson disease].
A 25-year-old woman who had delivered a normal child after a normal pregnancy four months ago, was suffering from a common cold. The latter was treated by tetracyclines. A few days later she developed an acute severe hemolysis. Moreover she had signs of hepatic failure which was characterized by a considerably reduced synthetic capacity of the liver but low serum aminotransferase levels and a proximal renal tubular disorder. With a negative Coombs test an autoimmune hemolytic anemia was unlikely. Therefore diagnostic procedures were directed at the cause of the liver injury. An increased urinary excretion of copper, strongly elevated levels of liver tissue copper and the detection of a Kayser-Fleischer ring by slit-lamp examination proved the diagnosis of Wilson's disease-presenting clinically only as severe hemolysis. Ceruloplasmin concentration in serum was in low normal range and not diagnostic.
Acute Disease↗