I. Overview
Aplastic anemia (AA), referred to as reanemia, is a group of syndromes in which there is a decrease in bone marrow hematopoietic tissue and hematopoietic failure, resulting in a decrease in peripheral blood whole blood cells. Clinically, it often presents with more severe anemia, bleeding and infection. There are more males than females and more young than old with primary reoccurrence. According to the speed of disease change and the severity of the disease, reoccurrence can be divided into acute and chronic forms by combining blood and bone marrow images. If there are pathogenic causes (such as drugs, chemicals, radiation, infection), it is secondary.
II. Diagnostic criteria
The diagnostic criteria of this disease are.
1. Decrease in whole blood cells and absolute value of reticulocytes.
2. Generally no hepatosplenomegaly.
At least one part of the bone marrow is hypoproliferative or severely hypoplasic (if active, there must be a significant decrease in megakaryocytes), and the bone marrow has an increase in small non-hematopoietic cells (if available, bone marrow biopsy and other examinations can show a decrease in hematopoietic tissue and an increase in adipose tissue). Other diseases that cause allogeneic cytopenia can be excluded. Such as paroxysmal sleep hemoglobinuria, refractory anemia in myelodysplastic syndrome, acute hematopoietic arrest, bone marrow fibrosis, acute leukemia, malignant histiocytosis, etc.
4. Generally speaking, anti-anemia drug treatment is ineffective.
III. Treatment plan
1.General treatment
Avoid triggering factors, do not use drugs that inhibit bone marrow, and do not use non-steroidal anti-inflammatory drugs. Strengthen isolation of severe patients, pay attention to skin, oral and vulva hygiene, and strengthen anti-inflammatory treatment in case of infection; transfusion of red blood cell suspension can be considered for patients with hemoglobin <60--70g/L and cardiopulmonary insufficiency; transfusion of platelet suspension in case of severe bleeding.
2.Drug treatment
(1) Androgens: High-dose androgens can stimulate bone marrow hematopoiesis and are the drug of choice for the treatment of chronic reoccurrence, and their efficacy often occurs 2-3 months after taking the drug. At present, the commonly used varieties and doses are as follows, and you can choose any one of them. Testosterone propionate 50–100mg/d, intramuscular injection, for more than 6 months; Stanozolol (Conilon) 2–4mg, 3 times a day, for 1–2 years; vigorous supplement ( 17-dehydromethyltestosterone) 15–30mg/d for more than 6 months; Androgel 40–80mg 3 times a day; Danazol 0.4–0.8/d for 6 months or more.
The main side effects of synthetic androgens are liver damage and sodium retention, and in children, accelerated skeletal maturation, which must be combined with adrenocorticosteroids. Oral medications such as Conlon are usually more hepatotoxic than protesterone, while androgenic effects are less severe than the latter. Androgenic side effects are reversible and can be reduced and disappeared with the reduction or discontinuation of the drug.
(2) Immunosuppressants may promote hematopoietic stem cell proliferation through cytotoxic immunosuppressive effects, removal of suppressive T lymphocytes that inhibit bone marrow hematopoiesis and promotion of growth factor synthesis and release through immune stimulation. It has become one of the main therapeutic measures for reblasts, especially acute reblasts. When applied, attention should be paid to protective isolation and supportive therapy.
3.Other treatments
(1) Cobalt chloride
Cobalt can inhibit cellular enzymes and make cells hypoxic, thus stimulating the kidney to increase the production of erythropoietin. 80–120mg/d for more than 3 months.
(2) Levamisole
May regulate cellular immunity by enhancing helper T-cell function. 50mg 3 times a day, 3 days a week for 2 months – 2 years.
(3) Adrenocorticotropic hormone
Helps hemostatic effect, may reduce bleeding, but not effective for visceral bleeding. Prednisone 20–30mg/d for several months.
IV. Prognosis assessment
The prognosis of reoccurrence depends on the type, the degree of bone marrow failure, the patient’s age and the early and late treatment. In recent years, there are several treatment methods for severe reoccurrence, but the overall effect is not satisfactory, about 1/3 – 1/2 patients die within a few months to a year. The main causes of death are infection and hemorrhage, especially cerebral hemorrhage. About 80% of patients with chronic reoccurrence are in remission after treatment, but there are still many patients with prolonged disease, and a few patients can recover completely.