Pregnancy with aplastic anemia

Introduction to pregnancy with aplastic anemia Aplastic anemia is a group of syndromes characterized by impaired bone marrow hematopoietic stem cells or hematopoietic microenvironment caused by multiple causes. Pregnancy with aplastic anemia is rare. Pregnancy with aplastic anemia is a serious comorbidity in pregnancy. It is clinically characterized as anemia, hemorrhage, infection, etc., but it can be caused by anemia, bleeding and infection during pregnancy and childbirth. The child has an adverse effect. It is a serious blood disease that should be vigorously prevented during pregnancy. In the past 10 years, with the progress of the treatment of aplastic anemia, patients with aplastic anemia have symptoms of aplastic anemia after pregnancy or after pregnancy. Timely treatment, effective relief of symptoms, and no serious pregnancy complications, can safely pass through pregnancy and childbirth. Aplastic anemia is no longer a contraindication to pregnancy, and maternal mortality is also significantly reduced. basic knowledge The proportion of illness: 0.003% Susceptible population: pregnant women. Mode of infection: non-infectious Complications: heart failure, pregnancy-induced hypertension, sepsis

Pregnancy with aplastic anemia

(1) Causes of the disease

The cause of aplastic anemia is more complicated. Half of the patients are unexplained primary aplastic anemia, which occurs in young adults, accounting for more than 70% of all cases. A small number of women with aplastic anemia develop during pregnancy and give birth. After remission, re-emergence in pregnancy, animal experiments have shown that high doses of estrogen can inhibit bone marrow hematopoietic function, therefore, some people think that aplastic anemia is related to pregnancy, but most scholars believe that between pregnancy and aplastic anemia There is no necessary connection but accidental coincidence. Secondary aplastic anemia is often related to the following factors:

1. Physical, chemical factors Long-term exposure to benzene and its derivatives, organic phosphorus, pesticides, gasoline, arsenic, mercury and various ionizing radiation, such as X-rays, radionuclides, nuclear pollution, nuclear leakage, etc. can affect bone marrow hematopoiesis Function, the degree of damage depends on the dose, type, strength and extent of damage of the hematopoietic tissue, the length of time and the state of the body itself. Pregnant women often show strong sensitivity to these physical and chemical factors.

2. Drug factors It is reported that almost 50% of patients with aplastic anemia are related to drugs. The incidence of aplastic anemia is the highest in drug-induced blood diseases, the most serious condition, the mortality rate is about 50%, and the drug is stopped. It usually takes several months or even years to recover. The drug may directly inhibit the hematopoietic function of the bone marrow, or may have an unexpected atopic reaction due to sensitivity. The WHO survey results from 1968 to 1973 showed that it caused aplastic disease. There are 9 main drugs for anemia: namely, phenylbutazone, hydroxybutazone, chloramphenicol, indomethacin (indomethacin), sodiumoaurothiomate, trimethoprim (trimethoprim), sulfamethoxazole, phenytoin sodium.

3. Infection factors Various acute and chronic infections include bacterial, parasitic and viral infections. The problem of aplastic anemia after hepatitis has been reported since the first report in 1955. The research data indicates that aplastic Anemia occurs mostly in the stage of hepatitis improvement or recovery. It is common in hepatitis A and can also be seen in hepatitis B or hepatitis C. Physiological changes during pregnancy may also aggravate aplastic anemia, resulting in poor prognosis. It may also interfere with hepatocyte nuclear protein with virus. Synthetic, so that it can not be differentiated into mature cells.

4. Other factors Some patients with aplastic anemia have a certain relationship with immune mechanisms, and some are related to genetic factors, such as hereditary aplastic anemia (fanconi anemia) is a rare autosomal recessive hereditary disease, except for bone marrow. In addition to poor proliferation, it can be associated with a variety of congenital malformations and chromosomal abnormalities.

(two) pathogenesis

The main pathogenesis of aplastic anemia is abnormal immune response, reduced number and/or dysfunction of hematopoietic stem cells, and micro-environmental defects supporting hematopoiesis also involved in the development of aplastic anemia.

1. Abnormal immune response damage hematopoietic stem/progenitor cells Various studies have shown that the abnormal number and function of T lymphocytes and the cytokine secretion disorders caused by them are closely related to aplastic anemia, especially the incidence of severe aplastic anemia. In order to do bone marrow transplantation, the drug is first used to suppress immunity. After administration, the hematopoietic function of the patient before the bone marrow is recovered is restored; acute aplastic anemia can be effective for the treatment of various immunosuppressive agents such as anti-lymphocyte globulin and cyclosporine. In patients with aplastic anemia, especially in SAA bone marrow and peripheral blood, the number of CD8 T lymphocyte colonies increased, the number of T lymphocytes increased, the proportion of CD3 and CD T lymphocytes increased, while the CD4 T lymphocytes decreased, and the CD4 / CD8 ratio decreased. Lymphocytes are activated, immune-mediated aplastic anemia patients have specific, oligoclonal T lymphocyte proliferation against certain antigens, and abnormal T lymphocytes can directly inhibit the growth of bone marrow cells; or secrete multiple hematopoiesis Negative regulatory factors such as IL-2, macrophage inflammatory protein (MIP)-1a, tumor necrosis factor alpha, gamma interferon inhibit hematopoietic stem/progenitor cell hematopoiesis; Excessive production of hematopoietic regulatory factors such as tumor necrosis factor and , interferon can up-regulate the apoptosis of Fas and Fas-L pathways, and a large number of apoptosis of bone marrow CD34 cells is also an important cause of hematopoietic failure.

2. Hematopoietic Stem Cell Reduction or Defects A large number of experimental studies have confirmed that the lack or defect of hematopoietic stem cells is one of the main pathogenesis of aplastic anemia. The bone marrow CD34 cells in the aplastic anemia patients are significantly reduced; the bone marrow and blood mononuclear cells are proved by culture methods. The cell colony-forming unit (CFU-GM), the erythrocyte colony-forming unit (CFU-E), and the megakaryocyte colony-generating unit (CFU-Meg) were all reduced, the isogenic hematopoietic stem cell transplantation was successful, and the normal hematopoietic function was quickly restored. Lack of stem cells at the time of the barrier.

3. Hematopoietic microenvironment defects Hematopoietic microenvironment refers to the structural components supporting hematopoiesis in hematopoietic tissues. Hematopoietic cells proliferate and differentiate in reticular scaffolds formed by stromal cells. In vitro experiments show that bone marrow stromal cells culture fibroblasts in aplastic anemia patients The colony-generating unit (CFU-F) was also reduced; clinically, patients who could not restore hematopoiesis due to the input of syngeneic bone marrow also indicated that the lesions of these patients were not in stem cells.

Pregnancy with aplastic anemia prevention

Although aplastic anemia is not a contraindication to pregnancy, the risk of pregnancy is much greater than that of non-pregnant women. For pregnancy and childbirth in patients with aplastic anemia, it must be given enough attention and serious consideration. It is generally believed that Patients with aplastic anemia should not be treated with strict contraception and should not be pregnant.

Pregnancy with complications of aplastic anemia Complications, heart failure, pregnancy-induced hypertension, sepsis

1. Pregnant women's blood is relatively diluted, making anemia worse, prone to anemic heart disease, and even heart failure.

2. Due to the decrease in the number of platelets and the abnormality of the blood cells, as well as the increase in the brittleness and permeability of the blood vessel wall, it may cause bleeding in the nasal and gastrointestinal mucosa.

3. Due to the reduction of granulocytes in the surrounding blood, mononuclear cells and gamma globulin are reduced, and lymphoid tissue is atrophied, which makes the patient's defense function low and easy to merge.

4. Aplastic anemia is prone to pregnancy-induced hypertension, which further aggravates the condition. After delivery, the intrauterine placenta is more likely to cause infection and even cause sepsis.

Pregnancy with symptoms of aplastic anemia Common symptoms Whole blood cells reduce mucosal bleeding points Skin bleeding points trauma

1. Anemia is generally caused by progressive anemia, mainly due to bone marrow hematopoietic failure. A small number of patients may have ineffective erythropoiesis, that is, bone marrow still has certain hematopoietic function, but the generated young red blood cells have been released from the bone marrow to the surrounding blood. destroyed.

2. Bleeding is mainly caused by platelet production disorders, which can occur in the internal organs and brain of the skin, gums, nose, placenta, digestive tract.

3. Infection and postpartum hemorrhage and trauma are prone to birth or systemic infection, mainly due to decreased granulocytes and monocytes, decreased body defense function, and also associated with decreased gamma-globulin and lymphoid tissue atrophy. Postpartum infection is the leading cause of maternal death in aplastic anemia.

Examination of pregnancy with aplastic anemia

Peripheral blood

Almost all patients have a complete blood cell reduction, heavy white blood cells decreased to 1.0 × 10 ⁹ / L, lymphocyte proportion increased, more than 60%, neutrophils extremely reduced, extremely severe aplastic anemia, neutrophils less than 0.2 × 10 ⁹ / L, platelets can be less than 10 × 10 ⁹ / L, reticulocytes less than 1%, even 0, chronic, white blood cells mostly in 2.0 × 10 ⁹ / L, neutrophils 25% or so, platelets in (10 ~ 20) × 10 ⁹ / L, more than 1% reticulocyte, hemoglobin is more than 60g / L.

2. Bone marrow and bone marrow biopsy

It is the main basis for the diagnosis of aplastic anemia. Heavy aplastic anemia, macroscopic observation of bone marrow fluid has more oil droplets. In most cases, bone marrow hyperplasia is low, granules, red blood cells are reduced, megakaryocytes are not easy to find, non-hematopoietic cells such as plasma cells, tissue hobby Alkaline cells, reticular cells, lymphocytosis, bone marrow granulocyte hematopoietic cells account for less than 20% of the area, chronic aplastic anemia, sternal and spine hyperplasia, tibia hyperplasia, hyperplasia and heavy aplastic anemia, The active red blood cell line increased, and the young red blood cells increased, but the megakaryocytes decreased, the proportion of lymphocytes increased, and the bone marrow biopsy hematopoietic area was less than 50%.

According to clinical manifestations, choose ECG, B-ultrasound, biochemistry, liver and kidney function tests.

Diagnosis and diagnosis of pregnancy complicated with aplastic anemia

diagnosis

According to the patient's clinical manifestations and peripheral blood whole blood cell reduction, the 3 lines of bone marrow can be diagnosed with reduced proliferation. The diagnostic criteria and basis are as follows:

1. Peripheral blood presents a complete reduction in blood cells.

2. Bone marrow shows reduced or severe hyperplasia, non-hematopoietic cells, bone marrow biopsy showed decreased hematopoietic tissue and increased adipose tissue.

3. No liver and spleen, can exclude other diseases of whole blood cell reduction (such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome, acute hematopoietic stagnation, myelofibrosis, hypoproliferative leukemia and malignant histiocytosis, etc. ).

4. In addition to the rapid clinical manifestations of severe aplastic anemia, the absolute value of neutrophils is <0.5×109/L, the platelet<20×109/L and the absolute value of reticulocytes <15×10 ⁹ /L.

Differential diagnosis

1. Paroxysmal nocturnal hemoglobinuria (PNH) PNH is anemia mainly caused by chronic intravascular hemolysis, mainly characterized by anemia, less bleeding and infection, blood picture can be all poor, but reticulocytes increase, myeloproliferation Active, mainly erythroid hyperplasia, urinary hemosiderin test (Rous test), acid hemolysis test (Ham test) and venom factor hemolysis test were positive, CD55 and CD59 negative cells increased significantly (>10%) can be diagnosed .

2. Refractory anemia (RA) in myelodysplastic syndrome (MDS) may have a complete cytopenia (or 1 or 2 lineage reduction), but the myeloproliferation is active, showing typical pathological hematopoiesis and chromosomal changes, megakaryocytes The cells are not reduced.

3. Low-proliferative acute leukemia can be manifested as whole blood cell reduction. No primordial cells can be seen in peripheral blood smears. The bone marrow sometimes has hyperplasia and is easily diagnosed as aplastic anemia, but it is found in multiple sites and in the bone marrow. The original cells can be identified.

4. Acute hematopoietic stagnation can be manifested as poor blood picture, acute incidence, often have a clear incentive, bone marrow can be proliferative active, only 2 or 2 lines are reduced, megakaryocytes are not lacking, the cause can be relieved after removal.

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