I. Autoimmune hemolytic anemia clinical pathway standard hospitalization process
(A) Applicable objects.
1. The first diagnosis of autoimmune hemolytic anemia (ICDC10.D59.102).
2. 1 month ≤ age < 18 years.
3. Warm antibody type.
4. Immunogenic.
(II) Diagnostic basis.
According to the Diagnostic and Efficacy Criteria for Hematological Diseases (edited by Zhang Zhinan and Shen Ti, Science Press, 2008, 3rd edition), Zhu Futang Practical Pediatrics (7th edition) (People’s Health Publishing House), and Clinical Diagnostic and Treatment Guide C Hematology Branch (edited by Chinese Medical Association, People’s Health Publishing House).
1.Medical history.
2.Clinical manifestations: anemia, hepatosplenomegaly, jaundice, hemoglobinuria.
3.Blood routine:The degree of anemia varies. Peripheral blood smear showed variable numbers of spherical red blood cells, juvenile red blood cells or red blood cell fragments, and increased reticulocytes.
4.Bone marrow examination shows juvenile erythrocyte hyperplasia, and occasionally erythrocytes with mild megaloblastic changes.
In regenerative crisis, the blood picture shows a decrease in whole blood cells and reticulocytes, and the bone marrow picture shows a decrease in hyperplasia.
6.The direct test of antiglobulin is positive, mainly anti-IgG and anti-complement C3 type, and occasionally anti-IgA type; the indirect test can be positive or negative.
(C) The choice of treatment plan.
According to “Zhu Futang Practical Pediatrics (7th edition)” (People’s Health Publishing House), “Clinical Diagnosis and Treatment Guide C Hematology Branch” (edited by Chinese Medical Association, People’s Health Publishing House).
1.Glucocorticoids as the first choice of treatment: can be given in regular doses or short courses of high doses.
2. Red blood cell transfusion: Severe anemia or symptoms of hypoxia require red blood cell transfusion. Use washed red blood cells as much as possible.
3.Intravenous infusion of gammaglobulin: for those with poor glucocorticoid effect.
(iv) The standard hospitalization day is within 14 days.
(V) Entry pathway criteria.
1.The first diagnosis must meet the ICD-10.D59.102 autoimmune hemolytic anemia (AIHA) disease code, and 1 month ≤ age < 18 years.
2.The blood test indexes meet the indications of needing hospitalization: hemoglobin <70g/L, or with obvious symptoms of hypoxia, or hemoglobin dropping too fast.
3.When the patient has other disease diagnoses at the same time, but does not need special treatment during hospitalization and does not affect the implementation of the clinical pathway process of the first diagnosis, he/she can enter the pathway.
(F) Definitive diagnosis and routine examination for hospital admission takes 2-3 days (working days).
1. Required examination items.
(1) Routine blood (including reticulocyte count), routine urine, routine stool + occult blood;
(2)Antiglobulin test, cold agglutinin test;
(3) liver and kidney function, electrolytes, coagulation function, complete set of hemolysis, pre-transfusion test, sedimentation, blood smear, blood group, autoimmune disease screening.
2.Checkup items that can be selected according to the patient’s condition.
(1)Infection-related pathogen examination;
(2) Relevant imaging examinations;
(3) Bone marrow morphology examination.
(vii) Treatment starts on day 1 of diagnosis.
(H) Treatment plan and drug selection.
1. Glucocorticoids as the first choice of treatment: observe the side effects of corticosteroids and treat them symptomatically.
(1) Conventional dose: Prednisone 1C2mg/Kg/d, divided into oral doses, and gradually reduce and stop after 2C4 weeks of medication.
(2) Short course of high dose administration: methylprednisolone 15C30 mg/Kg/d, or dexamethasone 0.5C1.0mg/kg/d, x3C5d after which the dose is reduced or discontinued.
2.Red blood cell infusion: infuse washed red blood cells as much as possible, 10mlC15ml/Kg/time.
3.Intravenous infusion of gammaglobulin: 0.4gC2.0g/Kg/d×1C5d, used when the effect of hormone is not good or combined when the condition is severe.
4. Extremely serious or cases with poor hormone or gammaglobulin effect may consider cyclosporine or other immunosuppressant (such as cyclophosphamide) treatment.
(ix) Discharge criteria.
Hemoglobin ≥ 70 g/L without red blood cell transfusion and without hypoxic symptoms and lasting more than 3 days.
(J) Variation and cause analysis.
1, After treatment, hemoglobin <70g/L and greater than 2 weeks, withdraw from the pathway.
2.The development of hemolytic crisis or aplastic crisis during treatment, withdraw from this pathway.
3. Final diagnosis of cold antibody autoimmune hemolytic anemia, withdraw from the pathway.
4. Final diagnosis of secondary autoimmune hemolytic anemia, withdraw from the pathway.