Is acquired hemophilia refractory?

summary

Acquired hemophilia (AHD) is a kind of haemorrhagic disease caused by the production of specific autoantibodies to f Ⅷ. Although there are often serious life-threatening bleeding, if the diagnosis is correct and the treatment is timely, it will achieve effective hemostatic effect. The determination of treatment depends on the severity of bleeding and antibody characteristics. Is acquired hemophilia refractory? Let's talk about it

Is acquired hemophilia refractory?

Because there is no prospective study, it is difficult to evaluate the pros and cons of each scheme. An important principle is to prevent bleeding. Minimal injury, intramuscular injection, arterial blood collection and any invasive procedure should be avoided. Aspirin or NSAIDs should also be avoided. The determination of treatment depends on the antibody titer and the severity of bleeding. If there is only slight bleeding and the antibody titer is less than 5bu, 1-deamino-8-d-arginine vasopressin (DDAVP) or human f Ⅷ product can be used; If the antibody titer is greater than 5bu, or the infusion of human f Ⅷ product is invalid, activated prothrombin complex (APCC) or prothrombin complex (PCC) or recombinant activated f Ⅶ (RF Ⅶ a) product or pig f Ⅷ product can be used (Fig. 1).

DDAVP: mechanism of action: increase f Ⅷ: C, endogenous f Ⅷ release can neutralize low titer autoantibodies; It may also bind to vasopressin V2 receptor on microvascular endothelial cells, resulting in camp increase, inducing VWF release from endothelial cells, increasing plasma VWF level, inhibiting the binding of F Ⅷ and vWF, blocking f Ⅷ autoantibody dose: 0.3 μ Intravenous DDAVP was only effective in some patients with antibody titer less than 3bu.

Plasma dialysis and immunoadsorption: plasma dialysis and immunoadsorption can be used when antibodies must be removed quickly, especially when patients have severe bleeding. There are several methods of plasma dialysis and in vitro immunoadsorption, such as the use of Sepharose bound staphylococcal protein A, antibodies against human heavy chain and light chain, double filtration plasma dialysis combined with immunosuppressive therapy to remove f Ⅷ antibody.

matters needing attention

Acquired hemophilia must follow the doctor's advice, conventional treatment, usually in the diet should pay attention to eat more blood food, eat more protein rich food, eat more vitamin C containing vegetables and fruits, such as jujube, kiwi, banana and so on, so that the body get enough nutrition, enhance the body's own immunity, so as to resist the disease.