Pregnancy and blood diseases

Hemoblastoses

The term "hemoblastoses" unites numerous tumors that form from hematopoietic cells and organs. These include leukemia (acute and chronic), lymphogranulomatosis, non-Hodgkin's lymphomas.

Acute lymphoblastic leukemia and acute myeloblastic leukemia are tumors of the hematopoietic tissue, which are characterized by infiltration of the bone marrow by immature blast cells without a tendency to differentiate into blood cells.

They are very rare in pregnant women. Sometimes pregnancy occurs in patients with acute lymphocytic leukemia, who fell ill in childhood and who achieved remission thanks to treatment. Acute myeloleukemia mainly affects adults, so this form of the disease is somewhat more common in pregnant women, although in general it is also a rare phenomenon. Sometimes leukemia first manifests itself during pregnancy.

The etiology is unknown. The disease is based on gene mutations, probably caused by ionizing radiation, chemical toxicants, viruses, or hereditary.

Diagnosis is based on cytomorphological examination of blood smears and bone marrow aspirate.

Pregnancy and childbirth usually aggravate the course of leukemia and can cause relapse in patients with prolonged remission. Significant deterioration of the condition, often maternal death most often occur after childbirth. Leukemia adversely affects the course of pregnancy. The frequency of spontaneous abortions, premature births, cases of intrauterine growth retardation, and perinatal mortality is significantly increased. Fetal death is usually associated with the death of the mother. In general, the prognosis for the outcome of pregnancy for the mother is unfavorable.

The tactics of pregnancy management in acute leukemia have not been finally determined. In our opinion, pregnancy should be terminated both early and late. Only if the disease is detected after 28 weeks can prolongation of pregnancy until the fetus is viable be justified. We consider chemotherapy of the underlying disease during pregnancy unacceptable.

There is another point of view, according to which chemotherapy should be prescribed during pregnancy, excluding the 1st trimester.

Termination of pregnancy and childbirth should be carried out conservatively, with careful prevention of bleeding and purulent-inflammatory complications.

Chronic leukemias are bone marrow tumors with differentiation of mature blood cells.

Chronic myelogenous leukemia is characterized by alternating remissions and exacerbations in the form of blast crises. A specific marker of the disease is the so-called Philadelphia chromosome, present in all leukemic cells.

Carrying out pregnancy is contraindicated: there is a high frequency of spontaneous termination of pregnancy, premature birth and perinatal mortality.

Specific treatment with busulfan is contraindicated in pregnant women, so if it is necessary to prescribe it as soon as possible, the pregnancy must be terminated. Expectant tactics and careful monitoring are possible only in the case of persistent remission of the disease, as well as in the late stages of pregnancy, when the viability of the fetus is already at stake.

The method of delivery is determined by the condition of the spleen: patients with splenomegaly (the vast majority of cases) should undergo a cesarean section; with a small spleen, delivery through the natural birth canal is possible.

In all cases of hemoblastosis, lactation is contraindicated.

Lymphogranulomatosis (Hodgkin's disease)

Lymphogranulomatosis (Hodgkin's disease) is an extramedullary hemoblastosis that affects the lymph nodes and lymphoid tissue of internal organs. It usually develops in people of reproductive age. It is much more common among pregnant women than leukemia.

The etiology and pathogenesis are not fully understood. Granulomas are formed in the lymph nodes, which contain giant (up to 80 µm in diameter) multinuclear Berezovsky-Sternberg cells and specific large mononuclear Hodgkin cells. Immunity is impaired, primarily the cellular link.

The international classification of lymphogranulomatosis is based on the number and location of affected lymph nodes and the presence of general clinical symptoms:

• Stage I - damage to one lymph node or one group of lymph nodes; 
• Stage II - damage to more than one group of lymph nodes located on one side of the diaphragm;
• Stage III - damage to the lymph nodes on both sides of the diaphragm or supradiaphragmatic lymph nodes and spleen;
• Stage IV - damage to the lymph nodes of internal organs (liver, kidneys, lungs, etc.) and bone marrow.

In each stage, a subgroup A (there are no general manifestations of the disease) or B (there is an increase in body temperature, night sweats, a decrease in body weight by 10% or more over six months) is distinguished.

Diagnosis is based on histological examination of biopsies of affected lymph nodes with the identification of pathognomonic Berezovsky-Sternberg cells.

Pregnancy has little effect on the course of lymphogranulomatosis, just as the latter has little effect on its course.

Taking into account, however, the urgent need for the earliest possible start of specific treatment (radiation or chemotherapy), pregnancy is permissible only in the case of stable remission (or even cure) of the disease. In the case of primary detection of lymphogranulomatosis or its relapse, termination of pregnancy is indicated both before 12 weeks and at later stages. If the disease is detected after 22 weeks, against the background of a satisfactory general condition of the woman, pregnancy can be prolonged by postponing the start of treatment until the postpartum period. Regardless of the stage and period (remission or relapse) of the disease, lactation should be stopped.

Delivery is carried out through the natural birth canal.

Children of women with lymphogranulomatosis are born healthy and subsequently develop normally.

Thrombocytopenia

Thrombocytopenia is a decrease in the number of platelets in the peripheral blood below 150*10 ⁹ /l due to limited production or increased destruction of platelets.

In the vast majority of cases, thrombocytopenia is acquired and caused by an autoimmune process, the influence of certain drugs (thiazide diuretics, estrogens, heparin, trimethoprim/sulfamethoxazole, antitumor agents) or ethanol, massive blood transfusion, artificial circulation. Approximately 3-5% of healthy women experience moderate thrombocytopenia during pregnancy (thrombocytopenia of pregnancy), which usually does not cause serious complications in the mother and fetus.

Of greatest clinical significance is chronic idiopathic thrombocytopenic purpura, an autoimmune disease that most often develops in women of reproductive age.

Prevalence in pregnant women is 0.01-0.02%.

The etiology and pathogenesis are based on the production of antiplatelet antibodies in the body, which, by binding to platelets, facilitate their elimination from the blood and destruction by spleen macrophages. Diagnosis is based on the anamnesis (development of the disease before pregnancy), exclusion of other causes that can lead to the development of thrombocytopenia. Antiplatelet antibodies are often detected, as well as antibodies to cardiolipin.

Pregnancy has little effect on the course of idiopathic thrombocytopenia, although cases of exacerbation of the disease during pregnancy are not uncommon. Most often, exacerbation occurs in the second half of gestation. Life-threatening bleeding is usually not observed. Quite often, it is during pregnancy that complete clinical and hematological remission occurs.

Antiplatelet antibodies can cross the placenta and cause thrombocytopenia in the fetus. However, no correlation has been established between the number of platelets in the mother's blood, the level of antiplatelet antibodies, and the degree of thrombocytopenia in the fetus and newborn. Perinatal mortality in idiopathic thrombocytopenia is significantly higher than in the general population, but its causes are not always associated with hemorrhagic complications.

Idiopathic thrombocytopenic purpura is not a contraindication to pregnancy, and its exacerbation in no case requires termination of pregnancy or early delivery. On the contrary, an exacerbation of the disease should be considered as a contraindication to active interventions, including induction of labor.

The tactics of pregnancy management consist of careful dynamic clinical and laboratory observation, treatment, waiting for the spontaneous onset of labor, and striving to deliver the baby through the natural birth canal. In the absence of hemorrhagic syndrome (petechiae, bruises on the skin, bleeding from the nose or gums, etc.) and the number of platelets in the peripheral blood is more than 50-10 ⁹ /l, special prenatal preparation is not carried out.

Treatment. The presence of hemorrhagic manifestations or a decrease in the platelet level to less than 50* 10 ⁹ /l (even in the complete absence of hemorrhages) requires the administration of corticosteroids.

Prednisolone per os is usually prescribed at a dose of 50-60 mg/day. After the platelet count has increased to 150*10 ⁹ /l, the prednisolone dose is gradually reduced to a maintenance dose (10-20 mg/day). Splenectomy during pregnancy is performed very rarely and only if glucocorticoid therapy is ineffective. In case of life-threatening bleeding, platelet concentrate is administered. Aminocaproic acid, fresh frozen (antihemophilic) plasma, and sodium etamsylate are used to prevent bleeding. Prophylactic administration of platelet concentrate is indicated in rare cases when a significant increase in the platelet count in the blood cannot be achieved by the time of delivery. The issue is decided by a hematologist in each individual case.

Thrombocytopathies

Thrombocytopathy is a hemostasis disorder caused by qualitative inferiority or dysfunction of platelets with a normal or slightly reduced number of them. The features of thrombocytopathy are a stable disorder of platelet properties, a discrepancy between the severity of hemorrhagic syndrome and the number of platelets in the peripheral blood, and a frequent combination with other hereditary defects.

A distinction is made between congenital (hereditary) and acquired thrombocytopagie. The former include disaggregation forms of pathology, deficiency or decreased availability of factor III (without significant disruption of platelet adhesion and aggregation), complex platelet dysfunctions in combination with other congenital anomalies. Acquired (symptomatic) thrombocytopathy is observed in hemoblastoses, _B12 -deficiency anemia, uremia, DIC syndrome and fibrinolysis activation, liver cirrhosis or chronic hepatitis, etc.

Pregnancy usually proceeds favorably, but childbirth may be complicated by bleeding.

The diagnosis is based on the study of the adhesive-aggregation properties of platelets, the reaction of release of intraplatelet substances, the number and morphological characteristics of platelets, and thromboplastin activity.

Treatment is symptomatic. Aminocaproic acid, ATP, magnesium sulfate, and riboxin are used. In case of life-threatening bleeding, the drug of choice is platelet concentrate (to avoid isosensitization of the patient, the donor should be selected according to the HLA system). If bleeding cannot be stopped, uterine extirpation is used.