May-Heglin anomay is an autosomal dominant disorder characterized by thrombocytopenia, giant platelets, and leukocyte inclusions (Chatwani, 1992). Urato and Repke (1998) described such a woman who was delivered vaginally. Despite a platelet count of 16,000/�L, she did not bleed excessively. he neonate inherited the anomaly but also had no bleeding despite a platelet count of35,000/�L. A systematic review of26 studies containing 75 pregnancies in 40 women reported four cases of postpartum hemorrhage, 34 cases of neonatal thrombocytopenia, and two fetal deaths (Hussein, 2013).
The primary form-also termed idiopathic thrombocytopenic purpura (ITP)-is usually caused by a cluster of IgG antibodies directed against one or more platelet glycoproteins (Konkle, 2015). Antibody-coated platelets are destroyed prematurely in the reticuloendothelial system, especially the spleen. Although not proven, the disorder is probably mediated by autoantibodies directed at platelet-associated immunoglobulins-P AlgG, P AIgM, and P lA.In adults, immune thrombocytopenia most often is a chronic disease that rarely resolves spontaneously.
As shown in Table 56-5, secondary forms of immunemediated chronic thrombocytopenia appear in association with systemic lupus erythematosus, lymphomas, leukemias, and several systemic diseases. Approximately 2 percent of thrombocytopenic patients have positive serological tests for lupus, and in some cases, levels of anticardiolipin antibodies are high. Finally, approximately 10 percent of HIV -positive patients have associated thrombocytopenia (Scaradavou, 2002).
Diagnosis and Management. Only a few adults with primary immune thrombocytopenia recover spontaneously, and for those who do not, platelet counts usually range from 10,000 to 100,000/�L (George, 2014). Evidence does not suggest that pregnancy increases the risk of relapse in women with women with active disease. hat said, it is certainly not unusual years to have recurrent thrombocytopenia during pregnancy.
Although this may be from closer surveillance, hyperestrogen emia has also been implicated.
Therapy is considered if the platelet count is below 30,000 to 50,000/�L (American College of Obstetricians and Gyne cologists, 20 16c). Primary treatment includes corticosteroids or intravenous immune globulin (lYlG) (Neunert, 201r1).
Initially, prednisone, 1 mg/kg daily, is given to suppress the phagocytic activity of the splenic monocyte-macrophage sys tem. IYlG given in a total dose of 2 g/kg during 2 to 5 days is also efective.
In pregnant women with no response to corticosteroid or IVIG therapy, open or laparoscopic splenectomy may be efective. In late pregnancy, cesarean delivery may be necessary for surgical exposure. Improvement usually follows splenectomy in 1 to 3 days and peaks at approximately 8 days. Cytotoxic agents are typically avoided in pregnancy due to teratogenicity risks. Azathioprine and rituximab, however, which are used in nonpregnant persons with ITP, have been used for other conditions in pregnancy. Finally, the thrombopoietin agonist romplostim has stimulated responses in some patients (Decrooq, 2014; Imbach, 2011; Kuter, 2010).