OVERVIEW
OVERVIEW
Patients with acquired hemophilia A spontaneously produce autoimmune antibodies that neutralize or inactivate F VIII activity, causing a decrease in F VIII levels and leading to clinically serious and life-threatening bleeding complications. The disease is aggressive and has a high mortality rate.
Whether medical insurance
Yes, it is
Department
Hematology
Synonyms
Acquired hemophilia A, Spontaneously acquired factor VIII inhibitors
Clinical symptoms
Generalized skin and mucous membrane bleeding, hematuria, black stools, menorrhagia and postpartum hemorrhage.
Dangers
Excessive bleeding may cause anemia, and a few patients may have joint bleeding or deformity, or even disability.
Examination
Activated partial thromboplastin time (APTT), mixed correction test, FⅧ activity and inhibitor test, etc.
Diagnosis
Diagnosis is made on the basis of symptoms such as bleeding from the skin and mucous membranes, combined with APTT, mixed correction test, FⅧ activity and inhibitor test.
Treatment principle
Aggressively treat the underlying disease, control bleeding, and focus on immunosuppression, immunomodulation, and other treatments.
Curative
Treatment may improve symptoms.
Dietary advice
Reasonable diet, pay attention to balanced nutrition, choose soft and easily digestible food.
Etiology
Epidemiology
Acquired hemophilia A occurs most often in adults, especially pregnant or postpartum women, people with immune disorders, and older adults without obvious underlying medical conditions. The prevalence is equal in men and women and is rare in children.
Causes
The disease is associated with autoimmune disorders, drug allergies, malignant tumors, pregnancy or post-partum, bronchial asthma, skin diseases, chronic enteritis, and graft-versus-host disease.
Symptoms and Diagnosis
Typical symptoms
Acute, widespread, spontaneous hemorrhage occurs suddenly in patients with no previous history of hemorrhage, and the degree of bleeding is more severe than that of hemophilia A patients, with 8% fatal hemorrhage. The symptoms include generalized skin and mucous membrane hemorrhage, various visceral hemorrhages may cause hematuria, black stools, menorrhagia and postpartum hemorrhage, and even intracranial hemorrhage, and joint hemorrhage and deformity may occur in a few patients.
Diagnostic basis
The patient has no previous history of bleeding disorders.
Clinical symptoms are usually dominated by sudden severe bleeding with hematuria, black stools and muscle bleeding.
The diagnosis is made in combination with normal prothrombin time (PT) and thrombin time (TT), marked prolongation of APTT, and inability to be corrected by mixed correction test.
Treatment
Treatment guidelines
Actively treat the primary disease and control bleeding. Long-term suppression or removal of autoantibodies to prevent immune memory reaction and inhibit antibody production.
Drug therapy
Inhibition or removal of autoantibodies: the efficacy is obvious for patients with acquired hemophilia, preferred immunosuppressant, recommended prednisone, cyclophosphamide, azathioprine and cyclosporine A.
2. Anti-CD20 monoclonal antibody combined with glucocorticoids, or combined with cytotoxic drugs, most patients can get remission or partial remission, currently recommended as the second-line treatment for acquired hemophilia A.
Other treatments
If antibody titers are low, consider 1-desamino-8-D-arginine vasopressin (DDAVP) or infusion of F VIII products. If the antibody titer is high, FⅧ products are ineffective and hemostatic therapy with plasminogen complex (PCC) or recombinant activated FⅦ (rFⅦa) products should be used.
Prognosis
With effective treatment, bleeding can be controlled and the morbidity and mortality reduced, but some patients will have recurrence.
Nursing care
Daily care
1. Pay attention to rest, combine work and rest, and live a regular life.
2 Perform appropriate sports and exercise to help protect the joints, but avoid strenuous exercise, pay attention to protection during activities, and once there is slight bleeding should be treated in a timely manner.
3. Choose soft-bristled brush to prevent bleeding gums.
4. Choose loose and soft clothes to prevent skin bleeding caused by friction.
Diet regulation
Choose soft and easy-to-digest food, avoid rough and hard food, and pay attention to balanced nutrition. If combined with gastrointestinal bleeding, fasting, liquid diet or semi-liquid diet should be given according to the situation, and moderate food temperature is suitable.