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Cases of autoimmune hemolytic anemia cases outlined the reasons for the diagnosis and clinical diagnostic laboratory tests do not treat
The disease produced by the immune dysfunction of autoantibodies, combined with the red blood cell surface or free in serum, the sensitized red blood cells, or activation of complement, premature destruction of red blood cell hemolytic anemia situation.(A), medical history and symptoms
medical history questions: Note: or without jaundice, blood transfusion; have painful foot cyanosis; the lymphatic system is suffering from cancer, myeloma, stomach cancer, autoimmune diseases (systemic lupus erythematosus, rheumatoid joints go far) mycoplasma, virus infection and syphilis and other diseases. whether to apply penicillin, quinidine, quinine, methyl dopa and other drugs. clinical symptoms: dizziness, fatigue, shortness of breath, palpitations; chills, fever, back pain, chest tightness (acute hemolysis).
(B), physical examination found
Appearance of anemia, skin, sclera jaundice, spleen slightly enlarged, some patients with hand, foot and cyanosis (cold antibody type).Blood: hemoglobin less positive cells are pigmented anemia; white blood cells, platelets normal; increased reticulocytes, small spherocytosis, showing immature red blood cells.
Bone marrow: hyperplasia to marked hyperplasia of immature red blood cells. Myeloid, megakaryocytic cell lines to normal. Red pigment of blood indirectly increased jaundice, seru


m haptoglobin decreased or disappeared, plasma free hemoglobin increased. Urine hemosiderin was negative.
Positive direct antiglobulin test, indirect antiglobulin test positive or negative (warm antibody type autoimmune hemolytic anemia).
Cold agglutinin test was positive (cold agglutinin syndrome.)
Hot and cold hemolysis test positive test positive in urine hemosiderin (paroxysmal cold hemoglobinuria).
(D), the differential diagnosis2. Paroxysmal nocturnal hemoglobinuria: Acid hemolysis test, venom factor hemolysis test positive test positive in urine hemosiderin.
3. Glucose -6- phosphate dehydrogenase deficiency: methemoglobin reduction test was positive; fluorescent spot test, nitro tetrazolium basket paper method: G6PD activity decreased.Cause clearly identified, the primary disease should be actively treated.
Glucocorticoids: hydrocortisone: 400 ~ 600mg / d, intravenous infusion, 3 ~ 5d after the switch to prednisone: 1mg/kg · d, orally, 7 ~ 10d improvement in condition, near normal hemoglobin, each weeks of prednisone decreasing dosage of 10 ~ 15mg, until the prednisone 20mg / d, regularly check hemoglobin and reticulocyte count 2 to 3 weeks, if the steady reduction of prednisone every 2.5mg, to 5 ~ 10mg / d, or every other day application of 10 ~ 20mg prednisone maintenance therapy for 6 months.
15mg) in order to maintain the improvement of hematology; or can not tolerate prednisone, immunosuppressive therapy, or have contraindications should consider splenectomy treatment. Splenectomy done before the best life and detained 51Cr red blood cell tests, to determine efficacy of splenectomy.
15mg) to maintain hematological improvements. Application of azathioprine: 50 ~ 200mg / d; cyclophosphamide: 50 ~ 150mg / d. Hematologic remission, the first reduction of corticosteroid dose, and then decreased to a maintenance dose of immunosuppressive agents, maintenance therapy 3 to 6 months. Observe during the treatment side effects such as bone marrow suppression.
Other: high-dose intravenous gamma globulin: 0.4 ~ 1.0g/kg, used in conjunction 5d, a small part of the IgG-mediated autoimmune hemolytic anemia have a certain effect, but the effects are short-lived. plasma exchange: for high antibody titers, glucocorticoid treatment of poor patients. blood transfusion: hemolytic crisis or severe anemia patients can lose the whole amount of blood or washed red blood cells.Looking for underlying disease, treatment of primary disease. Avoid cold stimulation, keep warm.
3 months.
Abstract: Objective cyclosporin A (CsA) in autoimmune hemolytic anemia (AIHA) and Evans syndrome, immunosuppressive therapy in the comprehensive role. Prospective study of CsA combined method of traditional programs (prednisone danazol (18 patients) and single-traditional program (26 patients) in treating Coombs test positive AIHA and Evans syndrome. Results CsA group was valid for 3 months 94.4%, the average onset time of 19.9d, complete remission rate was 88.9%, of which long-term follow-up of 12 patients only 1 recurrence within 1 year (recurrence rate 3.3%), 2 years, 2 cases of recurrence (recurrence rate (16.7% ), 3-year recurrence rate is still 16.7%; while the traditional scheme valid for 3 months was 92%, the average onset time of 23.2d, complete remission rate was 100%. two groups is efficient, no difference between the average onset time significant, and complete remission rate of CsA was significantly higher than the traditional program group, the recurrence rate within 3 years was significantly lower than traditional programs CsA group. Conclusion integrated with CsA immunosuppression in the treatment of AIHA and Evans syndrome can improve and consolidate for lack of efficacy . Number of pages: 3 page range :581-583 Key words: cyclosporin A Evans syndrome, autoimmune hemolytic anemia therapy subject classification: R556.62 [medicine, HEALTH "within SCIENCES" blood and lymphatic Department of Dise An Hemolytic An acquired hemolytic anemia] Related Articles: Topics related references (6) citations (9) coupled literature (166)


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