Blood ; 22 : Little data exist on the clinical presentation, management and treatment of aplastic anemia AA in pregnancy. The aim of this study is to describe the clinical presentation, management and outcomes of patients with AA in pregnancy based on a single centre, North American experience. Patients with AA during pregnancy managed at Mount Sinai Hospital, a tertiary care centre in Toronto, Canada, were retrospectively identified through the Special Pregnancy Program database from to , inclusive. Between and , 24 pregnancies were identified in 12 women with AA.
Fanconi's anemia pregmancy a rare, inherited disease that leads Anemia aplastic pregnancy aplastic anemia. But some people with myelodysplastic syndrome have empty marrow that's difficult to distinguish from aplastic anemia. Pregnancy in Fanconi anaemia with bone marrow failure: a case report and review of the literature. Aplastif injury can be caused pregnwncy radiation therapy and cytotoxic Anemia aplastic pregnancy, whereas indirect damage involves immunoeffector pathways, which are responsible for idiopathic cases and present in those preceded by a history of hepatitis. This pathological process is associated with significant maternal and neonatal morbidity and mortality. Telomere length in leukocyte subpopulations of Anemia aplastic pregnancy with aplastic anemia. Hemorrhage and sepsis are the major reasons for death in pregnant women with AA. Therapy is indicated in symptomatic disease, Cowgirl catalogs and very severe cases, and patients classified as nonsevere in whom severe cytopenia of Porn for free gals least one cell line requiring transfusions is present.
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Certain inherited conditions can damage the stem cells and lead to aplastic anemia. Factors that can temporarily or permanently injure bone marrow and Anemia aplastic pregnancy blood cell production include:. Between and people are diagnosed with aplastic anemia each year in the U. Earlier case reports have proposed pregnancy termination as an alternative approach. There are no clear guidelines for the Anemia aplastic pregnancy of aplastic anemia during pregnancy. Symptoms and Anemia aplastic pregnancy course of myelodysplastic syndrome vary for each patient depending on which blood cells are affected. Mayo Clinic Marketplace Check out these best-sellers and special offers on books Anemia aplastic pregnancy newsletters from Mayo Clinic. Note: Blood consists of a liquid portion called plasma and a solid portion the formed elements that includes red blood cells, white blood Hiv rapid test, and platelets. Aplastic anemia can be mistaken for a condition called myelodysplastic syndrome. Author information Article notes Copyright and License information Disclaimer. Pregnancy and severe aplastic anemia: causal relation or coincidence?
Although aplastic anemia was first recognized by Ehrlich in , the pathogenesis of aplastic anemia has remained elusive.
- Although aplastic anemia was first recognized by Ehrlich in , the pathogenesis of aplastic anemia has remained elusive.
- Normally, the bone marrow produces a sufficient number of new red blood cells RBCs , white blood cells WBCs , and platelets for normal body function.
Although aplastic anemia was first recognized by Ehrlich in , the pathogenesis of aplastic anemia has remained elusive. The prevalence of aplastic anemia in pregnancy is rare. Aplastic anemia is a subtype of anemia characterized by pancytopenia and a hypocellular bone marrow. This condition can be due to chemicals, drugs, infections, irradiation, leukemia, and inherited disorders. The treatment involves immunosuppressive therapy with antithymocyte globulin and cyclosporine and bone narrow transplantation [ 1 ].
The relationship between pregnancy and aplastic anemia remains controversial. There is universal agreement that pregnancy complicated by aplastic anemia is a serious condition [ 2 ]. The risk to the mother is mainly in the form of hemorrhage and sepsis, while the fetus may suffer from growth restriction and even intrauterine death.
All along with these, maternal infections may lead to the development of chorioamnionitis and resultant preterm labor and birth [ 3 ]. In the literature, fetal thrombocytopenia, placentomegaly, and severe oligohydramnios have also been reported. This high incidence is because the hospital is a tertiary care referral unit with good hematology and blood bank support. She did not have any medical or surgical problems in the past, and her antenatal investigations had been normal.
Bone marrow biopsy was suggestive of aplastic anemia. Injection dexamethasone was given for fetal lung maturation. A multidisciplinary team consisting of obstetricians, anesthesiologists, hematologists, and neonatologists planned on offering an elective cesarean section under general anesthesia for severe IUGR and fetal compromise.
Pre-operatively, her hemoglobin was 8. There were no abnormal intraoperative findings, and surgery was uneventful. Post-operatively, she developed fever, was started on cefotaxime and gentamicin, progressing on to imipenem and teicoplanin, which was changed to amphotericin and voriconazole because of mild infusional toxicity.
Definitive treatments of aplastic anemia like allogenic bone marrow transplant, Antithymocyte globulin ATGAM , and Cyclosporin were offered to the patient, but she could not avail these because of financial constraints. The baby was discharged back home in a good condition. Routine blood count revealed pancytopenia with hemoglobin 8. With a suspected aplastic anemia, she underwent bone marrow aspirate and trephine biopsy which showed a hypoplastic marrow confirming the diagnosis.
Historically, there were no risk factors for aplastic anemia. Clinical examination was normal except for mild pallor. Ultrasound examination revealed a normal live fetus consistent with gestational age. She and her family were explained of the potential risks to the patient and the fetus, and she chose to continue the pregnancy. She was booked as a high-risk pregnancy and managed jointly by a multidisciplinary team consisting of obstetricians and hematologists. She was discharged well on the eighth postnatal day with hemoglobin of 8.
Aplastic anemia is a serious hematological disorder characterized by pancytopenia, bone marrow hypocellularity, and absence of underlying malignant or myeloproliferative disease [ 4 , 5 ]. Aplastic anemia is known to increase the antenatal complications. On the literature review, the rate of preterm birth was Although previously cited complications are commonly encountered in cases of aplastic anemia, no such complications accompanied our case. Postpartum hemorrhage is an important complication among patients with the diagnosis of aplastic anemia due to decreased platelet count.
Intrauterine growth retardation complicated one of our cases. In cases of aplastic anemia, vaginal birth is preferred, and cesarean section is performed only for obstetric indications. In our case, cesarean section was performed electively, because of severe preeclampsia with IUGR and absent end diastolic flow.
In general, treatment for aplastic anemia includes withdrawal from offending drugs, supportive care, and some form of definitive therapy. However, BMT is contraindicated during pregnancy because it requires high-doses of immunosuppressive agents or radiation therapy, which would be toxic to the fetus. Although case reports have suggested a promising result with antithymocyte immunoglobulin ATGAM or cyclosporine therapy during pregnancy, there is currently little agreement on the universal use of these therapies.
The role of androgens is not clear, and androgen treatment may cause the virilization of female fetuses. The efficacy of corticosteroids or granulocyte colony-stimulating factor is also equivocal. Overall, current evidence does not favor the routine use of any drug therapy in the treatment of pregnancy-associated aplastic anemia [ 7 ].
Earlier case reports have proposed pregnancy termination as an alternative approach. However, experience from pregnancy following organ transplant shows that cyclosporine is apparently not teratogenic. Though it is excreted in milk, fetal growth and development were found to be normal [ 8 ]. Supportive therapy in the form of repeated blood and platelet transfusions are given to keep hemoglobin above Aplastic anemia is a rare complication of pregnancy.
Pregnancies complicated by intrauterine growth retardation, preterm labor, stillbirth, and spontaneous abortion can be successfully followed till term if appropriate diagnostic tests and treatment modalities are employed. In our cases, in spite of good supportive care, we had a case of maternal mortality, but fetal outcome was good.
Multidisciplinary team approach, intensive targeted therapy, and good financial support are necessary to achieve an optimal outcome. National Center for Biotechnology Information , U. J Obstet Gynaecol India. Published online Aug Author information Article notes Copyright and License information Disclaimer.
Corresponding author. Received Dec 14; Accepted Jun This article has been cited by other articles in PMC. Introduction Although aplastic anemia was first recognized by Ehrlich in , the pathogenesis of aplastic anemia has remained elusive.
Discussion Aplastic anemia is a serious hematological disorder characterized by pancytopenia, bone marrow hypocellularity, and absence of underlying malignant or myeloproliferative disease [ 4 , 5 ]. Conclusion Aplastic anemia is a rare complication of pregnancy. References 1. Pregnancy associated aplastic anemia—a series of 10 cases with review of literature. Pregnancy associated aplastic anemia: maternal and fetal outcome. J Obstet Gynaecol Res. Aplastic anemia during pregnancy: variable clinical course and outcome.
Aplastic anemia: pathogenesis, diagnosis, treatment and prognosis. N Eng J Med. Pregnancy and severe aplastic anemia: causal relation or coincidence?
Br J Haematol. Pavithran K, Thomas M. Pregnancy associated aplastic anemia. J Assoc Physicians India. Supportive management of pregnancy-associated aplastic anemia. Int J Gynecol Obstet. Aplastic anemia in pregnancy: treatment with cyclosporine and granulocyte-colony stimulating factor.
Fortunately, aplastic anemia has a low maternal mortality due to treatment. Supportive management of pregnancy-associated aplastic anemia. Supportive therapy in the form of repeated blood and platelet transfusions are given to keep hemoglobin above Myelodysplasia is a condition in which the bone marrow makes too many faulty blood cells. This drop in the blood oxygen level triggers the release of erythropoietin, which travels via the blood to the red bone marrow and stimulates red blood cell production. Aplastic anemia and related bone marrow failure states.
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Aplastic anemia during pregnancy: a review of obstetric and anesthetic considerations
Aplastic anemia is a hematologic condition occasionally presenting during pregnancy. This pathological process is associated with significant maternal and neonatal morbidity and mortality. Obstetric and anesthetic management is challenging, and treatment requires a coordinated effort by an interdisciplinary team, in order to provide safe care to these patients.
In this review, we describe the current state of the literature as it applies to the complexity of aplastic anemia in pregnancy, focusing on pathophysiologic aspects of the disease in pregnancy, as well as relevant obstetric and anesthetic considerations necessary to treat this challenging problem.
A multidisciplinary-team approach to the management of aplastic anemia in pregnancy is necessary to coordinate prenatal care, optimize maternofetal outcomes, and plan peripartum interventions. Conservative transfusion management is critical to prevent alloimmunization. Although a safe threshold-platelet count for neuraxial anesthesia has not been established, selection of anesthetic technique must be evaluated on a case-to-case basis. Aplastic anemia AA is a life-threatening disorder 1 that tends to worsen during pregnancy.
This disorder consists of pancytopenia as a result of hypocellular bone marrow in the absence of an abnormal infiltrate or bone-marrow fibrosis.
Therapy during the peripartum period is also approached in the context of a review of recent literature.
Acquired AA is an uncommon disorder characterized by progressive pancytopenia caused by altered bone-marrow function.
Incidence is estimated to be one to two cases per million per year. Typically, this disorder affects young adults who present with peripheral pancytopenia in the absence of other hematological diseases. Therapy is indicated in symptomatic disease, severe and very severe cases, and patients classified as nonsevere in whom severe cytopenia of at least one cell line requiring transfusions is present.
Although the hematologic stem-cell HSC compartment is affected in all types of AA, in the acquired form, the damage is extrinsic and involves direct and indirect mechanisms. Direct injury can be caused by radiation therapy and cytotoxic agents, whereas indirect damage involves immunoeffector pathways, which are responsible for idiopathic cases and present in those preceded by a history of hepatitis.
The antigens responsible for autoimmunization remain elusive; however, autoantibodies have been identified in serum of patients with AA. In addition to individual susceptibility, T-cell dysregulation is necessary for AA to develop. Paroxysmal nocturnal hemoglobinuria PNH has been considered a late clonal disease occurring in patients recovering from AA, and sometimes these two disorders overlap.
Complement inhibitors, such as the monoclonal antibody eculizumab, have proven useful for treatment of PNH, highlighting the underlying mechanism of red-blood-cell destruction and bone-marrow suppression.
This therapy has been used with success in pregnancy. The first report of AA was published by Ehrlich in Incidentally, his patient was pregnant and died 1 month after delivery, due to postpartum hemorrhage.
Hemorrhage and sepsis are the major reasons for death in pregnant women with AA. Pathophysiological mechanisms underlying the association between AA and pregnancy have not been clearly elucidated. It has been postulated that hormonal influences may contribute to worsening of blood counts in pregnant patients with AA, but the exact mechanism and causes are still unclear. The factors responsible for the observed thrombocytopenia in pregnant patients with AA are yet to be definitively elucidated.
Principles of AA treatment during pregnancy include identification of any underlying cause and treatment of cytopenias, while minimizing maternal and fetal side effects of therapy. Termination should be considered if a triggering factor causing bone-marrow suppression, such as drug reaction or infection, is detected, and the medication cannot be discontinued or the microorganism cannot be adequately treated if pregnancy continues. Management of AA starts with accurate diagnosis, definition of severity, and a comprehensive assessment, followed by supportive treatment.
Immunosuppressant agents are excreted in breast milk, and some practitioners do not recommend breastfeeding while on those medications in the context of HSCT; however, reports of successful immunosuppression in breastfed infants are encouraging, and the informed decision to recommend breastfeeding must be individualized.
Fertility prospects must be discussed with the patient before making the decision to proceed with HSCT in the postpartum period.
The optimal treatment for AA depends on different factors, including patient age, neutrophil count, and presence of comorbid conditions.
In the nonobstetric population, mild cases can be either observed or treated with specific colony-stimulating factors, antithymocyte globulin ATG , cyclosporine, and methylprednisolone. In his series, four patients were treated during the postpartum period and only one before delivery, receiving the medication at 23 weeks.
The baby was delivered at 36 weeks, weighing 1, g. The patient died 2 months postpartum as a result of an episode of pneumonia without recovery from her AA. There are no reports of fetal adverse effects attributable to ATG in humans, and low birth weight might be the result of comorbid conditions, rather than drug toxicity. If corticosteroids are used, those unable to cross the placenta, such as prednisone, prednisolone, and hydrocortisone, are preferred, in order to minimize fetal brain exposure and the slight association of orofacial malformations.
Because cyclosporine has not been shown to be consistently effective, it should be carefully considered by the treating physician. Alloantibodies to both types of antigens are responsible for PTR. When treating pregnant patients with AA, clinicians encounter significant ethical challenges. Therapy for maternal AA may put the fetus at risk secondary to exposure to medications, whereas maternal complications occurring if AA is left untreated may also affect the developing fetus.
The biomedical ethical principles of autonomy, beneficence, nonmaleficence, and justice provide a guideline to make therapeutic decisions in these cases. From the legal point of view, it is clear that the rights of the mother prevail over those of the fetus. Pregnancies complicated by AA require a multidisciplinary-team approach.
Collaboration between high-risk obstetricians, hematologists, anesthesiologists, and transfusion-medicine specialists is necessary. Antepartum management includes frequent monitoring for clinically significant depletion of blood cell lines, with the goal of being conservative in terms of transfusion practices. Fetal growth surveillance should be performed by 28 weeks of gestation, and antenatal testing should also be offered by 30—32 weeks, due to the high prevalence of growth restriction.
Mode of delivery should be carefully considered. Multiple transfusions in patients with AA can lead to significant HLA alloimmunization, especially if nonleukocyte reduced blood products have been used. HLA-matched platelets can be expensive and have limited availability.
Addressing permanent or long-acting reversible contraception is of critical importance, given that AA does not appear to decrease fertility, but is likely to progress or relapse with subsequent pregnancies.
Another relevant aspect to consider in the context of AA is the risk of neonatal thrombocytopenia in mothers exposed to platelet transfusions. Neonatal alloimmune thrombocytopenia occurs when maternal antibodies directed against HPAs cross the placenta.
Clinically significant neonatal thrombocytopenia associated with HLA alloimmunization has been suggested; 76 , 77 however, one prospective study was unable to find this association. The anesthetic management of a pregnant patient with diagnosis of AA requires coordinated interaction with other care teams, including the blood bank, hematology, obstetrics, interventional radiology, nursing staff, and neonatology.
The presence of severe thrombocytopenia, which per se confers poor prognosis in the context of AA, 9 , 80 , 81 puts the patient at risk for surgical bleeding, and may contraindicate the use of neuraxial techniques for labor and delivery. This review also suggests that the risks of placing and removing epidural catheters are equivalent.
Overall, in light of the heterogeneous etiology of thrombocytopenia in retrospective studies and case reports published to date, and recognizing that some patients might have low platelet counts, but normal or even enhanced platelet function, no recommendation regarding a specific threshold of platelet count can be made, and experience of anesthesiologists as well as institutional protocols must be considered to make the decision to perform a neuraxial block in a patient with AA. The role of the anesthesiologist in management of anemic parturients with AA is critical, and encompasses preoperative assessment, determining need for transfusion, and devising strategies to optimize oxygen consumption:demand ratio.
The preoperative assessment must include evaluation of the degree of anemia and determination of signs of cardiovascular compensatory response and compromised organ perfusion. Other aspects related to anesthetic care of AA patients deserve mention, since neuraxial techniques may be contraindicated. The anesthesia-care provider must be ready to use general anesthesia if the patient is to undergo cesarean section.
Postoperative pain relief with a multimodal approach is reasonable to facilitate early ambulation and breastfeeding. Additionally, inadequate postpartum pain control has been associated with chronic pain and depression. Systemic analgesia can be obtained with inhaled nitrous oxide and intravenous IV opioids. Nitrous oxide administration requires special equipment and patient cooperation. Few good-quality studies have addressed labor analgesia with nitrous oxide, and research on effectiveness, satisfaction, and adverse effects is still needed.
Remifentanil has been used for labor analgesia based on its short context-sensitive half-life. The use of IV patient-controlled analgesia PCA with remifentanil has been associated with hypoxic episodes, as well as cardiac and respiratory arrest during labor. IV PCA with fentanyl is another alternative for labor analgesia. AA is a complex disorder that warrants a comprehensive multidisciplinary-team approach, in order to devise an obstetric, hematological, anesthetic, and neonatal plan and anticipate complications during the peripartum period.
Conservative transfusion strategies are necessary to avoid complications related to alloimmunization. Close monitoring of fetal well-being and adequate growth has to be carried out by the maternofetal specialist, and planning for delivery needs to be discussed, with the vaginal route being the preferred mode. Anesthetic management has to be individualized, and should include considerations related to the degree of blood cell line compromise, as well as possible complications that have an impact on the anesthetic technique.
Multimodal labor and postoperative analgesic techniques, including but not limited to the use of systemic opioids, should be considered. Effective contraception is important in light of progression and relapse of AA during pregnancy. National Center for Biotechnology Information , U. Int J Womens Health. Published online Feb Author information Copyright and License information Disclaimer.
This work is published and licensed by Dove Medical Press Limited. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Abstract Aplastic anemia is a hematologic condition occasionally presenting during pregnancy.
Keywords: aplastic anemia, platelets, high-risk obstetrics, obstetric anesthesia, pregnancy. Background Aplastic anemia AA is a life-threatening disorder 1 that tends to worsen during pregnancy. Review of literature Acquired AA is an uncommon disorder characterized by progressive pancytopenia caused by altered bone-marrow function. Table 1 Classification of aplastic anemia based on severity. Open in a separate window.
Figure 1. Pathophysiologic mechanisms of aplastic anemia. Abbreviation: HLA, human leukocyte antigen. Pathophysiology in pregnancy The first report of AA was published by Ehrlich in Maternal and fetal considerations in aplastic anemia in pregnancy Treatment of aplastic anemia in pregnancy Principles of AA treatment during pregnancy include identification of any underlying cause and treatment of cytopenias, while minimizing maternal and fetal side effects of therapy.
Perinatal implications of aplastic anemia Pregnancies complicated by AA require a multidisciplinary-team approach. Anesthetic implications of aplastic anemia The anesthetic management of a pregnant patient with diagnosis of AA requires coordinated interaction with other care teams, including the blood bank, hematology, obstetrics, interventional radiology, nursing staff, and neonatology.
Conclusion AA is a complex disorder that warrants a comprehensive multidisciplinary-team approach, in order to devise an obstetric, hematological, anesthetic, and neonatal plan and anticipate complications during the peripartum period.