Mycoplasma pneumoniae pneumonia in Helsinki 1962-1970. Epidemic pattern and autoimmune manifestations.
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Adolescent↗
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Autoimmune haemolytic anaemia complicating Mycoplasma pneumoniae infection.
Although a positive direct Coombs' test is common in patients with Mycoplasma pneumoniae infection, manifest haemolysis is rare. A case is presented in which M. pneumoniae infection was followed by severe autoimmune haemolytic anaemia with intravascular haemolysis and haemoglobinuria. The presence of cold haemagglutinins suggests that these autoantibodies caused the haemolysis. Corticosteroid treatment corrected the haemolysis, which was of brief duration. Published data indicate that the mycoplasma agent is capable of altering the I-receptors of normal erythrocytes by haemadsorption and production of a soluble haemolysin.
Adult↗
[Autoimmune hemolytic anemia with eosinophilia in elderly patient].
A 70-year-old woman was admitted to our hospital in November 1992 for evaluation of anemia. Physical examination revealed anemia, jaundice, swelling of axial and inguinal lymph nodes, and splenomegaly. Abnormal hematological findings were as follows: Hb of 3.9 g/dl, reticulocyte count of 58.2% (61.7 x 10(4)/microliters), hyperplasia of normal erythroblasts in bone marrow, and eosinophilia (21.0%, 2352/microliters) in peripheral blood. Routine laboratory examinations revealed polycolonal hypergammaglobulinemia 3.0 g/dl, a high level of serum LDH (797 IU/I) and a total bilirubin of 2.4 mg/dl (indirect, 1.6 mg/dl). The serum haptoglobin level was very low (< 5 mg/dl). Results of serological examinations were as follows: IgG of 3366 mg/dl, CH50 of 16.0 U/ml, positive Coombs test 2+, and positive tests for antinuclear antibody, rheumatoid factor, and cold agglutinin. CRP was negative. PHA-stimulated lymphocyte blast formation, NK activity, and ADCC activity were found to be suppressed, and the percentage of CD4-positive lymphocytes in peripheral blood was also low. An axillary lymph node biopsy revealed reactive lymphadenitis. No signs or history suggested allergy, collagen disease, or parasitic infection. Autoimmune hemolytic anemia (AIHA) complicated by immunologic abnormalities and eosinophilia was diagnosed. Oral prednisolone markedly reduced the hemolytic anemia, eosinophilia, lymph node swelling, and splenomegaly, but NK activity remained low.
Aged↗
An adult with aplastic crisis induced by human parvovirus B19 as an initial presentation of hereditary spherocytosis.
The association between aplastic crisis and human parvovirus (HPV) B19 infection is well described in patients with sickle cell anemia. This association has also been described, although much less frequently, in patients with hereditary spherocytosis (HS). However, most cases of aplastic crises in patients with HS and induced by HPV B19 have been reported in children or adolescents. In this paper, we describe an aplastic crisis induced by HPV B19 in an adult with HS. A 34-year-old female presented with presyncope, febrile sensation, and myalgia. The complete blood counts showed severe anemia. The peripheral blood smear revealed spherocytosis with reticulocytopenia and pancytopenia. The direct Coombs' test was negative; the osmotic fragility test was positive. In the bone marrow aspirates, a few giant pronormoblasts with deep blue cytoplasm, pseudopods, and intracellular inclusion bodies were observed. The patient was given eight units of packed red blood cells. HPV B19 infection was proven by the presence of IgM antibodies to HPV B19 and the detection of viral DNA using the PCR technique. To the best of our knowledge, this is the first report in Korea that describes an adult with aplastic crisis presenting initially with HS.
Adult↗
Minimal change nephrotic syndrome, lymphadenopathy and hyperimmunoglobulinemia after immunization with a pneumococcal vaccine.
A 67-year-old female was admitted to our hospital with eruption and cervical lymphadenopathy which occurred one week after pneumococcal vaccination. Polyclonal hyperimmunoglobulinemia (IgG 6,620 mg/dl) and mild plasma cell proliferation (6.4%) in a bone marrow specimen were found, but a lymph node aspiration biopsy showed no specific findings. Normochromic and normocytic anemia with a positive direct Coombs test were also confirmed. Short-term intensive steroid therapy was given, but the systemic eruption and lymphadenopathy continued. About 4 months after vaccination, she suffered from edema in her face and legs and visual disturbance. When massive proteinuria (10.4 g/day) was found, she was admitted to our ward. A renal biopsy specimen showed a minor glomerular abnormality with mild interstitial plasmacytic infiltration. An abdominal CT scan showed hepatosplenomegaly and para-aortic lymphadenopathy. Uveitis was also found by ophthalmoscopy. These abnormalities completely disappeared after intensive steroid therapy including pulse therapy. On the basis of her clinical course and laboratory findings, it was suggested that minimal change nephrotic syndrome might be induced after vaccination, possibly due to hypersensitivity syndrome.
Aged↗
Laboratory methods for diagnosis of autoimmune disease.
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Antibodies, Antinuclear↗
Systemic lupus erythematosus during penicillamine therapy for rheumatoid arthritis.
Six patients with rheumatoid arthritis developed a syndrome resembling lupus erythematosus while being treated with penicillamine. All patients had previous mucocutaneous reactions to chrysotherapy. Manifestations included pleurisy in five of six patients, rashes in three, nephritis in two, and neurologic disturbances in two; lupus erythematosus cells were found in five patients, antinuclear antibodies in all six, antideoxyribonucleic acid in three, positive Coombs' test results for three patients, and low C4 complement in five of the six. Symptoms were slow to resolve after penicillamine treatment was discontinued, and four of the patients needed corticosteroid therapy. A higher frequency than expected of HLA A 11 in three patients and B 15 in five patients was seen.
Adult↗
Bee sting envenomation resulting in secondary immune-mediated hemolytic anemia in two dogs.
Immune-mediated hemolytic anemia secondary to bee envenomation developed in 2 dogs. Clinical signs included lethargy, hematuria, ataxia, and seizures; 1 dog died. Clinicopathologic data included nonregenerative anemia, spherocytosis, positive results for Coombs' test, and occult hematuria. Treatment included oral administration of corticosteroids at immunosuppressive dosages and supportive care. The surviving dog initially responded to corticosteroids, but hemolysis recurred as the dosage was tapered. Hemolysis resolved with prolonged administration of corticosteroids. Bee venom contains hyaluronidase, histamines, and hemolysins that cause toxic and hemolytic effects. Envenomation should be considered in any dog with hemolytic anemia in which other causes are ruled out and exposure to bees is known.
Anemia, Hemolytic, Autoimmune↗
ACOG practice bulletin. Prevention of Rh D alloimmunization. Number 4, May 1999 (replaces educational bulletin Number 147, October 1990). Clinical management guidelines for obstetrician-gynecologists. American College of Obstetrics and Gynecology.
The reduction in the incidence of Rh D alloimmunization is a prototype for the effectiveness of preventive medicine. Some controversies remain, however, such as the use of anti-D immune globulin in patients with either threatened abortion or antenatal hemorrhage. Similarly, it may not be cost-effective either to screen all Rh D-negative patients with an indirect Coombs test at 24-28 weeks of gestation or to screen all postpartum patients for excessive fetomaternal hemorrhage.
Cost-Benefit Analysis↗
Classification of an intermediate group of patients with antiphospholipid syndrome and lupus-like disease: primary or secondary antiphospholipid syndrome?
OBJECTIVE: (1) To classify an intermediate group of patients (IntAPS) with antiphospholipid syndrome (APS) and lupus-like disease either as primary (PAPS) or secondary APS (SAPS) and to discuss 2 different classifications. (2) To compare patients of a division of rheumatology with either PAPS or SAPS. METHODS: Patients with APS and patients with systemic lupus erythematosus (SLE) followed at the Department of Rheumatology, University Hospital Bichat, Paris, from 1987 to 1996 were analyzed. A chart review and a standardized telephone interview in 1997 completed the data of this study. RESULTS: (1) We found a total of 108 patients with APS: 22 with PAPS, 69 with SAPS, and 17 with IntAPS. The group of IntAPS did not differ from PAPS in any clinical or laboratory signs with the exception of antibodies to dsDNA and to extractable nuclear antigen (ENA). Between IntAPS and SAPS, there were several significant differences in clinical signs of SLE (malar rash, discoid rash, arthralgia) and in laboratory values (leukocytopenia). (2) Comparison of PAPS and SAPS showed statistically significant differences for positive Coombs' test, leukocytopenia, lymphocytopenia, antinuclear antibodies, antibodies to dsDNA and to ENA, and hypocomplementemia. CONCLUSION: The mainstay of the diagnosis of APS is the clinical event of thrombosis or miscarriage in the presence of antiphospholipid antibodies. Less important are laboratory values, which may help to differentiate PAPS from SAPS in order to initiate adequate therapy (e.g., anticoagulation in the first and additional corticosteroids in the second). Patients with IntAPS are more likely to be integrated into the group of PAPS than in the group of SAPS; therefore, special exclusion criteria for PAPS are not appropriate.
Adult↗
[Autoimmune hemolytic anemia in chronic hepatitis C virus infection treated with alpha-interferon: a therapy-related event or extra-hepatic immunologic manifestation?].
We report a case of Coombs' positive autoimmune haemolytic anaemia occurring in a patient with chronic hepatitis C virus infection, after five months of alpha-interferon administration. Prednisolone treatment induced a complete remission of both clinical and hematological findings after two months, while Coombs' tests became negative after three months. This points out the possibility of an additional unusual extrahepatic autoimmune feature during the course of hepatitis C virus infection.
Anemia, Hemolytic, Autoimmune↗
[Autoimmune neutropenia accompanied by Tolosa-Hunt syndrome].
A 47-year-old woman presented with severe neutropenia accompanied by diplopia and orbital pain. Her bone marrow was normal except for the absence of segmented neutrophils. Because the administration of granulocyte colony-stimulating factor (G-CSF) at a dose of 1 microgram/kg/day was not sufficiently effective and neutropenia developed, the patient was admitted to our hospital. Physical examination revealed painful ophthalmoplegia and hypoalgesia in the first region of trigeminal nerve, suggestive of Tolosa-Hunt syndrome. Severe neutropenia was observed in both peripheral blood and bone marrow, together with mild anemia and thrombocytopenia. The life span of red cells and platelets was shortened. High PAIgG levels, a positive Coombs test, and a positive test for anti-NA1 antibody suggested that blood cells were being destroyed by an autoimmune mechanism. Corticosteroid hormone therapy preceded by the administration of G-CSF at 5 micrograms/kg/day was effective for both neutropenia and in improving the patient's neurological findings.
Autoimmune Diseases↗
[A boy with summer onset paroxysmal cold hemoglobinuria induced by Donath-Landsteiner antibody with anti-I specificity].
We encountered 4-year-old boy who developed paroxysmal cold hemoglobinuria (PCH) in the middle of August. He was admitted due to progressive jaundice and pallor following fever and nausea. Laboratory data revealed severe anemia, increased serum indirect bilirubin and LDH, and decreased serum haptoglobin. Direct/indirect Coombs tests were positive. These findings yielded a diagnosis of autoimmune hemolytic anemia. Serological test for syphilis was negative. The patient's symptoms and signs promptly subsided after treatment with prednisolone, which was started on the 2nd hospital day. The patient has been in a disease-free state for 16 months without any medication. After discharge, he was finally given a diagnosis of PCH because a Donath-Landsteiner test was positive. The antibody belonged to an IgM subclass and showed anti-I specificity. Considering that development of PCH is common in winter, this case was unique because it developed in August. We speculated that exposure to a cool air-conditioned atmosphere prior to hospitalization and the cooling mechanism of the body after admission were involved in the summer onset of PCH and its prolonged clinical course.
Air Conditioning↗
Evaluation of the enzyme test for the detection of clinically significant red blood cell antibodies during pregnancy.
In the Croatian transfusion medicine, no general agreement has yet been achieved whether red blood cell (RBC) Rhesus (Rh) antibodies detected during pregnancy only by enzyme tests can cause hemolytic disease of the newborn (HDN). Results of the detection of clinically significant RBC antibodies by low-ionic-strength additive solution antiglobulin test (LISS-IAT) and trypsin enzyme test in 22,947 pregnant women are presented. All pregnant women in whom clinically significant RBC antibodies (RBC-CSA) were detected by LISS-IAT and/or enzyme tests were followed and observed during pregnancy. The women who had enzyme-only anti-D antibodies in their serum were followed up during subsequent pregnancies. Out of 302 positive results obtained by both techniques, irregular clinically significant enzyme-only antibodies (anti-RhD and anti-RhE specificity) were detected in 14 (4.6%) pregnant women. None of 11 RhD positive newborns whose mothers had enzyme-only anti-D antibodies, had signs of HDN after delivery. In these 11 women, anti-D antibodies were detected by LISS-IAT in the first trimenon of subsequent pregnancy. Nine infants born from subsequent pregnancies to women who had previously had enzyme-only anti-D, had clinical signs of HDN. The authors concluded that there is no need for enzyme tests in prenatal testing because enzyme tests are not reliable in the prediction of HDN.
Blood Group Antigens↗
[Neonatal hyperbilirubinemia: evaluation and treatment].
We investigated 185 newborns with hyperbilirubinemia hospitalised in Neonatal Unit of Pediatric clinic during 1997. We identified an apparent cause for the jaundice in 76 newborns (41.0%), but in 109 newborns (58.8%) no cause for the jaundice was found. Contributing factors for this jaundice were: male sex, preterm bith, breast feeding, considerable weight loss, instrumental or induced delivery as well as genetic factors. Exchange transfusion was done in only 2 cases (1.08%) with Rh isoimmunisation and positive Coombs test, which is according to attitude that blood exchange transfusion as an expensive procedure with high mortality and morbidity has largely been replaced by phototherapy because of its relative freedom from complications together with its non-invasive nature. The average duration of hospitalisation of hyperbilirubinemic newborns lasted 4.8 days. We consider to make all efforts to short duration of hospitalisation time, because separation of newborn from the mother during phototherapy has a negative impact on early mother-infant social and emotional interaction.
Female↗
[Detection of light chains of warm-type autoantibodies in autoimmune hemolytic anemia with the aid of an autoanalyzer].
A method was evolved for detection of light chains of erythrocyte autoantibodies using a Technicon II AutoAnalyser with properly selected dilutions of bromelin and methylcellulose and anti-kappa and anti-lambda sera. It was demonstrated that the method is superior to the direct Coombs test because it made possible detection of one or both light chains in warm autoantibodies coating the erythrocytes of 20 patients with autoimmunohaemolytic anaemias in whom they could not have been detected by the manual method. It was found that in warm-type autoantibodies light chains can be detected always and that in some cases only one light chain is found.
Anemia, Hemolytic, Autoimmune↗
Sero- and molecular typing of Duffy blood group in Southeast Asians and Oceanians.
Duffy blood group was studied among malaria-endemic Thai and Indonesian populations: Hmong (n = 103), Akha (n = 218), Lisu (n = 44), Bugis (n = 95), Toraja (n = 77), Dani (n = 44), Mee (n = 80) and Irianese (n = 81). Phenotypes were studied by the ordinal indirect Coombs' test and genotypes were determined by the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) test. DNA analysis was used to type the Duffy blood group system. Deduced allele frequencies of Fya (0.958-1.0) based on the phenotypes were similar to those in other populations in Southeast Asia and Oceania. The study revealed the presence of Fya-antigens showing weak reactivity to antisera as well as a discrepancy between the genotype shown by the PCR-RFLP study and that predicted by the phenotype. The PCR-RFLP study also suggested the presence of an alternative genetic basis for the Fy(a- b-) phenotype, which differs from the African type.
Alleles↗
[Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine A and splenectomy].
A 19-year-old girl was admitted to our hospital because of general fatigue, headache and purpura. A number of her laboratory findings suggested hemolytic anemia and thrombocytopenia. Direct/indirect Coombs tests gave negative results. Although the patient had no neurological or renal abnormalities, peripheral blood smears showed marked red cell fragmentation, and therefore she was diagnosed as having thrombotic thrombocytopenic purpura (TTP). Fresh frozen plasma (FFP) was transfused daily. The thrombocytopenia and hemolysis immediately improved, but worsened again after reduction of the FFP transfusion. Plasma exchange was instituted every other day, but the patient's condition worsened. Palsy and consciousness disturbance developed, and finally she lapsed into a coma. Daily plasma exchange and methylprednisolone pulse therapy were performed, together with weekly vincristine therapy, and this led to a gradual improvement in the patient's condition. However, several attempts at weaning from plasma exchange resulted in exacerbation of the TTP activity. Therefore oral cyclosporine A was started and splenectomy was performed. After these interventions, despite transient relapse, the patient was successfully weaned off the FFP transfusion, and she is now in remission. Because in this case splenectomy and cyclosporine A resulted in sustained remission of TTP that was refractory to intensive plasma therapy, an autoimmune mechanism may have been involved in the pathogenesis.
Adult↗