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PubMed2002

[Clinical features of hematological abnormality in systemic lupus erythematosus-related hematological disorders].

In order to investigate the clinical characteristics of hematological abnormality in patients with systemic lupus erythematosus (SLE) and inquire into the basis for differential diagnosis, the hematological data of 92 cases with lupus erythematosus-related hematological disorder (SLERHD) were retrospectively analyzed by use of SPSS/PC software. The results showed that these patients were short of specificity in clinical manifestation and hemogram, however, all cases possessed multiple SLE-related autoantibodies, increase of serum globulin level and varying extent dermal and arthral signs. The incidence of primary or initial symptom in the 92 cases was as follow: 65 anemia (72.8%), 39 purpura (42.4%), 17 hemolytic anemia (18.5%), 56 leukopenia (60.9%), 54 thrombocytopenia (58.7%), and 41 pancytopenia (44.6%). The bone marrow examinations showed that the cellularity of nucleated cells was mostly normal, and active proliferation in 57 cases (61.9%) and hypercellularity in 35 cases (38.1%); the G/E ratio was normal in majority, and G/E ratio > 3 in 59 cases (64.1%) and < 3 in 33 cases (35.9%) and G/E < 1 in 17 cases with hemolytic anemia Coombs' test positive; megakaryocyte counts were normal in 11 cases (11.9%), increase in 80 cases (86.9%) and lower than 7/marrow smear in 1 case (1.1%). Neutrophil alkaline phosphatase staining was negative in all of the cases. From above data it is concluded that patients with SLERHD are varied in clinical and blood pictures, but all patients are provided with multiple SLE-related autoantibodies, globulinemia and dermal and arthral signs. It is easy to identify SLERHD from aplastic anemia, myelodysplastic syndrome, idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia and Evans' syndrome by comprehensive and detailed clinical and laboratory examinations.

Adolescent↗
PubMed2002

[Simultaneous development of factor V inhibitor and autoimmune thrombocytopenia in a patient with dermatomyositis].

It has been previously demonstrated that aging, a history of malignancy or surgery, and exposure to bovine thrombin may be related to the presence of factor V inhibitor. However, dermatomyositis (DM) and autoimmune thrombocytopenic purpura (ATP) have rarely been associated with factor V inhibitor. Here we report a patient with factor V inhibitor accompanied by DM and ATP. In January 2000, a 77-year-old woman with DM was hospitalized because of susceptibility to bleeding. At the onset of DM, she had suffered from gastric leiomyosarcoma, and had undergone gastrectomy and splenectomy without the use of bovine thrombin. Thereafter, she had been treated with prednisolone until October 1999. On admission, prolongation of both APTT and PT was seen. Her factor V activity had fallen to 6%, and factor V inhibitor was positive at 8.9 Bethesda units. Moreover her platelet count had dropped to 1.0 x 10(9)/l. A bone marrow aspirate showed a cellular marrow with megakaryocytic hyperplasia, and the patient's PA-IgG level was elevated at 389 ng/10(7) cells. These findings suggested that ATP was also present. Additionally, the patient showed a positive result for Coombs test and consumption of complement. After a further course of steroid therapy, the patient's condition was markedly improved. This is a very rare case that showed factor V inhibitor and ATP simultaneously. Furthermore, the patient's clinical course suggests the relationship between the presence of factor V inhibitor and the reactivation of her collagen disease activity.

Aged↗
PubMed2003

[Autoimmune hemolytic anemia: a rare complication of ulcerative colitis].

Anaemia is a common problem in patients with ulcerative colitis, and its etiology is usually multifactorial. It can be produced by chronic blood loss, nutritional deficiencies, drugs such as salazopyrine, or it can be related to those chronic disease. However, ulcerative colitis is known to be associated with several immune disorders, as autoimmune haemolytic anaemia. Nevertheless, this rare complication can be found in 0.2 to 0.7% of patients affected by ulcerative colitis, and although 1.82% of patients with ulcerative colitis have a positive direct Coombs test without evidence of hemolysis. We report one new case of ulcerative colitis associated with autoimmune anaemia haemolytic, and a review the literature.

Aged↗
PubMed2003

Severe diffuse interstitial pneumonia due to Mycoplasma pneumoniae in a patient with respiratory insufficiency.

We report a 25-year-old man presenting with high fever, dyspnoea and somnolence. The presence of severe diffuse interstitial pneumonia with extrapulmonary symptoms, such as myositis and subclinical haemolysis, strongly suggested an infection by Mycoplasma pneumoniae. This diagnosis was supported by high titres of cold agglutinins and a positive Coombs test, and directly confirmed by specific IgM serological tests. After initiation of the appropriate antimicrobial treatment mechanical ventilation could be avoided and the patient showed a slow but complete clinical recovery. This diagnosis should be considered in any febrile patient with hypoxaemia and diffuse interstitial pneumonia, and rapid initiation of appropriate antibiotic treatment seems to be crucial for a favourable outcome.

Adult↗
PubMed2003

[Myelodysplastic syndrome accompanied by Evans syndrome].

Various cases of myelodysplastic syndrome (MDS) with diverse immunological disorders have been reported by many investigators. In this case report, we present a 70-year-old woman who had been diagnosed as having MDS and liver cirrhosis (LC) type C for 20 months, and who finally developed autoimmune phenomena against autologous blood cells. She was admitted to our hospital in order to evaluate her advanced anemia, thrombocytopenia and fatigue. The laboratory data at admission were as follows: hemoglobin 5.3 g/dl, red blood cell count 109 x 10(4)/microliter, white blood cell count 1,760/microliter, platelet count 4.3 x 10(4)/microliter and reticulocyte count 1.3%. The direct Coombs test was positive. In addition, anti-platelet antibody was positive, using the MPHA method. With these results, Evans syndrome secondary to MDS or LC was diagnosed. The mechanisms for the development of immunological disorders in patients with MDS or LC have not been fully elucidated. To our knowledge, this patient is one of the rare cases with MDS and Evans syndrome reported in the literature. During the generation process of autoantibodies, the role of HCV antigen in the pathogenesis of Evans syndrome was of interest in this patient.

Aged↗
PubMed1976

Adverse reactions to drugs: relationship to immunopathic disease.

Immunopathic disease resulting from drug treatment occurs when drugs interact with lymphoid cells and induce an immunological reaction. Drugs, being foreign to the body, are immunogenic, either as such or as haptens bound to carrier proteins. The immune response is usually innocuous or unnoticed but occasionally becomes pathogenic. The impact is especially obvious when the immunopathic response affects blood, skin, liver or kidney. Immunopathic responses and effector mechanisms of injury, whether to drugs or other antigens, are considered in terms of four types: Type I--anaphylactic and mediated by immunoglobulin (Ig) E antibody, and exemplified by immediate penicillin reactions; Type II--cytolytic and complement-associated, mediated by IgG antibody, and exemplified by haemolytic reactions; Type III--vasculonecrotic (Arthus reaction), mediated by immune complexes, and exemplified by serum sickness-like reaction; Type IV--delayed hypersensitivity involving T lymphocytes but no antibody, and exemplified by contact dermatitis. In addition, certain drugs induce true autoimmune reactions exemplified by reactions to procaine amide (lupus erythematosus) and alpha methyldopa (positive Coombs test result). Drug reactions must be interpreted in terms of modern immunology, with involvement of both the B and T lymphocyte systems. Inherited predisposition exists, probably dependent both on immune response genes and on the rate of enzymatic handling of drugs. Diagnosis depends on a carefully taken history of drug administration, recognition of clinical manifestations, and results of tests now available in departments of clinical immunology.

Aged↗
PubMed1992

[Use of anti-D (Rh) IgG or intramuscular polyvalent human immunoglobulin in the treatment of chronic autoimmune thrombocytopenic purpura].

The present study compares the effect of the intramuscular injection of low doses of IgG anti-D or human polyvalent immunoglobulin (Ig) on the platelet count of patients with CATP. Forty patients (14 children, 26 adults), 11 who had undergone splenectomy, were divided in the following groups of treatment: 20 patients received a single injection of 300 micrograms of IgG anti-D, 6 patients received the same dose as above plus 0.5 mg/kg daily of prednisone v.o and 14 patients received 640 mg of polyvalent Ig. Each patient was sequentially studied by measuring peripheral blood parameters, reticulocyte index, direct Coombs' test and C3-C4 determinations. Their blood group and Rh factor had been previously determined. The platelet response was evaluated as refractory (no response) and favorable (platelet increment over 50,000/microliters compared with initial platelet count). Patients with a favorable response over a month were considered as a prolonged remission. The results showed a favorable platelet response in 74% of the patients that received a single injection of IgG anti-D alone (one of the patients was Rh negative) or associated to prednisone, and 42.8% of the cases when polyvalent Ig was used. The patients who had not undergone splenectomy obtained better results than the group with splenectomy (62% vs 45%) and children showed a better response than adults (78.5% vs 46.1%). Forty five percent of prolonged remissions (including the Rh negative patient) were obtained with both schemes of IgG anti-D administration and only 28.5% when polyvalent Ig was used. The remissions were significantly longer with IgG anti-D (p < 0.01). The hematological and serological parameters did not show any significant modifications in all the cases and there was no adverse effects with the treatment. In conclusion, the intramuscular injection of immunoglobulins, especially IgG anti-D, produce an increase in the platelet count in some patients with CATP, several of them can obtain prolonged remissions, particularly children and patients that had not received immunoglobulins previously. This treatment is safe, ambulatory, easy to administer, and relatively inexpensive.

Adolescent↗
PubMed1962

Erythroblastosis fetalis, obstetrical management.

The anemia of erythroblastotic infants can be predicted antenatally by the indirect Coombs test, amniotic fluid pigment levels and maternal excretion of chorionic gonadotrophin. The only effective therapy for fetal anemia is delivery before the development of fetal hydrops. This may be accomplished by focusing attention on factors indicative of fetal anemia and by delivering potentially anemic fetuses as soon as they are sufficiently mature to escape the problems of prematurity.

Amniotic Fluid↗
PubMed1992

A family study of the antiphospholipid syndrome associated with other autoimmune diseases.

Antiphospholipid syndrome (APS) is an entity characterized by recurrent thrombotic events and may occur spontaneously or in the context of systemic lupus erythematosus (SLE). We describe an English Canadian family in whom the propositus, a woman with Graves' disease and SLE, was found to have a lupus anticoagulant and anticardiolipin antibody (aCL). A brother with deep vein thrombosis, pulmonary emboli, bilateral adrenal hemorrhage and thrombocytopenia, circulating anticoagulant and aCL had a positive antinuclear antibody and Coombs' test, but no other features of SLE. Fourteen members of 3 generations of this family underwent clinical assessments, serological testing and HLA typing. The propositus' mother had a family history of autoimmune thyroid disease and the father had aCL, but was asymptomatic. The thyroid disease and the SLE were associated with HLA-B8, DR3 haplotype. The aCL and the anticoagulant were associated with HLA-B60, DR4 haplotype. Both these haplotypes were present in the propositus. Among the other 4 carriers of the haplotype B60, DR4, 3 demonstrated significant titers of aCL. Our findings support the reported association between APS and the HLA haplotype DR4 in patients of English descent with SLE.

Adolescent↗
PubMed1992

[Cold agglutinin hemolytic anemia complicating mycoplasma pneumonia].

A case of Mycoplasma pneumonia complicated with severe hemolytic anemia, which occurred as a result of a high titer of cold agglutinin is presented. A 49 year-old male was admitted because of fever, jaundice and dyspnea. Chest x-ray showed diffuse small nodular infiltrates throughout both lung fields. Laboratory studies disclosed the following values: Hb 4.6 g/dl, Ht 13.9%, reticulocyte 11.5%, direct and indirect Coombs' test positive, haptoglobin 38 mg/dl, ESR 145 mm/hr, cold agglutinin titer 1:2,048 mycoplasma antibody titer 1:640, PPD negative. The diagnosis of autoimmune hemolytic anemia associated with Mycoplasma pneumonia was made, and treatment with minocycline and prednisolone observed striking clinical improvement. It was suggested that the cold exposure was possibly a major factor in the pathogenesis of hemolysis in this patient.

Anemia, Hemolytic, Autoimmune↗
PubMed1992

[Autoimmune hemolytic anemia induced by alpha-interferon therapy in a case of IgG-kappa type multiple myeloma].

A 47-year-old male case of IgG-kappa type multiple myeloma was treated with VMCP and recombinant human alpha-interferon (IFN-alpha 2a). The direct Coombs test was positive before treatment. Hemolytic anemia associated with massive hematuria was observed during the administration of 9 million IU IFN-alpha 2a per day for 2 weeks. The hemolytic symptoms rapidly improved after withdrawal of IFN-alpha 2a. This clinical course suggests that IFN-alpha as an immunomodulator was responsible for the progression of autoimmune hemolytic anemia in a case of multiple myeloma.

Anemia, Hemolytic, Autoimmune↗
PubMed1992

[Donath-Landsteiner hemolytic anemia. Physiopathological, diagnostic and therapeutic aspects].

Donath-Landsteiner hemolytic anemia accounts for one third of all immunologic hemolytic syndromes in pediatric patients. Diagnosis is suggested by results of the direct Coombs test which is positive with anti-C3d, evidence of erythrophagocytosis on admission blood smears, and results of the Donath-Landsteiner test. Anti-P specificity should be routinely looked for. Management, required once the diagnosis is established, is symptomatic. Warmed red blood cell concentrates should be used for blood transfusions. Exposure to cold should be avoided. Use of maintenance corticosteroid therapy is no longer acceptable.

Adult↗
PubMed2003

[An analysis of relapse and risk factors of autoimmune hemolytic anemia and Evans syndrome].

OBJECTIVE: To analyse the relapse rate and risk factors of autoimmune hemolytic anemia (AIHA) and Evans syndrome. METHODS: Fifty two cases of AIHA and Evans syndrome in remission being followed up for 1 - 14 years (median time 3.8 years) were analysed for relapse rate. The risk factors of relapse were analysed by case-control study. RESULTS: The total relapse rate of these AIHA and Evans syndrome patients was 57.7%, and the median remission duration to the first relapse was 9 months. The relapse rates in patients with negative Coombs test, warm autoantibodies and both of warm and cold autoantibodies were 30.8% (4/13), 54.0% (13/24) and 86.7% (13/15), respectively. The relapse rate in patients with cold antibody was the highest (P < 0.05). The relapse rate in patients with antibody titer >or= 100 was 92.9% (13/14) and was higher than that in patients with antibody titer < 100 [59.5% (13/22)] (P < 0.05). Patients treated with prednisone and cyclosporin relapsed less than those treated with prednisone alone, and the relapse was related to the therapy course of prednisone and CsA. CONCLUSION: Because of the high relapse rate, AIHA and Evans syndrome should be treated according to the class of autoantibodies, and with longer course of prednisone and cyclosporin and prophylaxis of infection.

Adolescent↗
PubMed2004

[Clinical analysis of 5 cases with acute intravascular hemolysis caused by puerarin].

OBJECTIVE: To explore the clinical characteristics of acute intravascular hemolysis caused by puerarin so as to help our prevention, diagnosis and treatment. METHODS: Analysis of 5 cases of acute intravascular hemolysis caused by puerarin was made and literature review conducted. RESULTS: All patients had the history of administering puerarin, with the pre-symptoms of acute hemolysis; the clinical characteristics of acute intravascular hemolysis were observed:sudden attacks of lumber and abdominal pain, chill, fever dyspnea, temporary conciousness loss, dark urine or hematuria, low hemoglobin, high reticular red blood cell, positive of Coombs' test. CONCLUSION: When sudden attacks of the above symptoms appear, the acute intravascular hemolysis should be taken into consideration first and the giving of the puerarin intravenously be immediately stopped Active and proper treatment should be made.

Adult↗
PubMed2004

[Hematological abnormality and clinical characteristics in systemic lupus erythematosus].

To investigate the hematological abnormality and clinical characteristics in systemic lupus erythematosus (SLE), the hematological data of 58 SLE and the curative effects of corticosteroid and immunosuppressive agents on SLE were retrospectively analysed by using SPSS/PC software. The results showed that the incidence of hematological abnormalities in 58 cases was as follows: 50 cases of hemogram abnormality (86.2%), 41 of anemia (70.7%), 34 of thrombocytopenia (58.7%), 37 of leukopenia (63.8%). Peripheral cytopenia of every cell lineage was common in SLE. The cell abnormalities of two or three lineages were seen in 41 cases (70.7%). The initial symptoms with hematological abnormality were found in 12 cases (20.7%), 7 out of 12 cases were erroneously diagnosed as hematology diseases (12.1%). In 30 out of 58 patients, the results of bone marrow examination showed that 23 had hyperplasia (76.7%) and 7 were hypoplasia. In 25 out of 38 cases, splenomegaly (65.8%) was found by B ultrasonography. In 25 patients with SLE receiving Coombs test, 3 were positive (12.0%). PAIg increased in 16 out of 22 cases of thrombocytopenia (72.7%). 26 cases of SLE with two or three lineage cytopenia in peripheral blood were treated by corticosteroid and immunosuppressive agent. The hemogram improved in all patients including 6 cases of bone marrow hypoplasia. It is concluded that the hematological abnormalities are frequent in SLE patients, which are short of specialty. The cytopenia of two or more lineage in peripheral blood is most common when bone marrow shows hyperplastic. The therapy with corticosteroid and immunosuppressive agents is efficacious.

Adolescent↗
PubMed2004

[Self-limited thrombotic microangiopathy associated with perianal abscess].

A 43-year-old female with Staphyloccocus-induced perianal abscess, was admitted to hospital because of a clinical picture of acute renal failure and thrombotic microangiopathy. Schistocytes, thrombopenia, a negative Coombs test and no detectable plasma haptoglobin were diagnostic for thrombotic microangiopathy. Antibiotics, surgical drainage, plasmapheresis and fresh frozen plasma were given with a favourable evolution. We review the prognostic factors determining recovery of renal function and hematological abnormalities.

Abscess↗
PubMed1997

Anti-Jk3 with no clinical evidence of HDN.

A sample was submitted to a reference lab from a 27-year-old Asian female, gravida 4 para 1, for antibody identification. Anti-Jk3 with an IgM component was identified. Subsequently, the antibody was eluted from the infant's cord and venous red blood cells. Normal bilirubin and hematocrit levels ruled out hemolytic disease of the newborn (HDN). Anti-Jk3 has been implicated in two cases of mild HDN. In this case, this noncomplement-binding antibody caused a positive direct Coombs test without clinical signs of HDN.

Journal Article↗
PubMed2004

[A woman suffering from systemic lupus erythematosus and acquired thrombotic thrombocytopenic purpura].

A 41-year-old woman with known systemic lupus erythematosus (SLE) was diagnosed with thrombotic thrombocytopaenic purpura (TTP). At the time of admission she was suffering from petechia, purpura and had neurological symptoms. At first a relapse of the SLE was suspected. Additional laboratory findings demonstrated haemolytic anaemia, thrombocytopaenia and high levels of fragmentocytes. After multiple plasmapheresis treatments and immunosuppressive therapy she recovered. TTP can be differentiated from other thrombotic microangiopathic syndromes by its normal levels of prothrombin time, partially activated thromboplastin time (APTT), fibrinogen and direct Coombs-test. Further investigation is needed to confirm the diagnosis by determination of the activity of Von Willebrand factor cleaving protease ADAMTS-13. In this patient, the ADAMTS-13 activity returned after plasmapheresis. This case demonstrates the importance of fast and appropriate laboratory testing in order to diagnose TTP quickly.

ADAM Proteins↗