Treatment of postpartum thrombotic microangiopathy with plasma exchange using cryosupernatant as replacement

Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are rare but acknowledged problems of pregnancy and the postpartum period. These diseases together with thrombotic angiopathy are associated with high maternal and fetal mortality and severe long-term morbidity. We describe four women with postpartum HUS/TTP treated with plasma exchange cryosupernatant fraction of plasma (CFP) as replacement fluid. Anuria or oliguria at the beginning of treatment and thrombocytopenia [thromb- (29-68) x 109/L] were common features. Two of the patients developed a prolonged and more difficult clinical condition affecting the central nervous system and the liver and their platelet counts remained low despite the plasma exchange. The renal and hepatic function of all of the patients recovered fully. This small analysis lends weight to early plasma exchange with cryosupernatant as part of the treatment of postpartum HUS/TTP and suggests that persistent thrombocytopenia is a signal of more serious disease.     

Postpartum hemorrhagic shock resulting in thrombotic thrombocytopenic purpura–hemolytic uremic syndrome

Thrombotic microangiopathies may be initiated by a number of antecedent events. When presented with postpartum hemorrhage and unexplained thrombocytopenia, it is prudent to consider microangiopathic hemolytic anemia in the differential diagnosis. A 25-year-old woman, gravida 2, para 1, had an uncomplicated repeat Cesarean delivery at 38 weeks' gestation. She subsequently had an exploratory laparotomy for hemoperitoneum resulting from a left uterine artery laceration. On postoperative day 3, she developed thrombotic thrombocytopenic purpura–hemolytic uremic syndrome and was treated with plasma exchange therapy and dialysis. It is critical that clinicians consider this potentially fatal disease in the differential diagnosis when hemorrhagic shock is associated with unexplained thrombocytopenia, so that appropriate and early treatment may lead to a favorable outcome.     

Thrombotic Microangiopathy in a Pregnant Woman With Kidney Transplantation: A Case Report

BackgroundThe differential diagnosis of thrombotic microangiopathy (TMA) in pregnancy includes common conditions, such as preeclampsia. In women with kidney transplantation, additional causes of TMA must be considered.CaseA 22-year-old primigravid woman with a transplanted kidney presented with fetal growth restriction, hypertension, acute kidney injury, and hemolysis at 28 weeks gestation. While her clinical presentation was initially consistent with preeclampsia, hemolysis persisted beyond 1 week postpartum. Diagnoses of TMA associated with tacrolimus and antibody-mediated rejection were considered. An elevated tacrolimus level likely contributed to her TMA and a decrease in dosage improved her clinical picture and laboratory markers.ConclusionWe report the case of a pregnant kidney transplant recipient with TMA. A multidisciplinary approach is required to optimize the maternal health outcomes in this complex population.     

Successful Maternal and Fetal Outcome in a Pregnancy Complicated by Thrombotic Thrombocytopenic Purpura in the First Trimester

Thrombotic thrombocytopenic purpura is an uncommon but severe hematologic condition affecting women in their reproductive years. Of approximately 70 cases diagnosed during pregnancy documented in the literature, only 20% had a successful maternal-fetal outcome. In our case study, a woman developed thrombotic thrombocytopenic purpura on her sixth week of gestation. She responded to a combination of glucocorticosteroids, plasma infusion and exchange. Relapses at 17 and 20 weeks were managed with glucocorticosteroids, plasma exchange and intravenous dextran. She delivered a term infant and had an uneventful postpartum course. Modern medical management of thrombotic thrombocytopenic purpura can lead to a successful maternal-fetal outcome even when initially diagnosed in the early first trimester.     

Esophageal achalasia in pregnancy

Achalasia is rare motor disorder of esophageal smooth muscle. It has been linked to malnutrition during pregnancy leading to maternal and fetal mortality. We report a case of achalasia with intrauterine fetal death who succeeded in following pregnancy with good fetal and maternal outcome after operative treatment. A 34-year-old pregnant woman had intrauterine fetal death at 27 gestational weeks due to severe and persistent maternal malnutrition during pregnancy. Achalasia was diagnosed postpartum and myotomy-fundoplasty was performed. She succeeded in normal delivery with a healthy baby 21 months after the operation. This case suggests the effect of severe achalasia of early onset on poor fetal outcome, and the efficacy of surgical myotomy for the improvement of following pregnant outcome.     

Síndrome ELLP,un diagnóstico diferencial complicado

HELLP syndrome (Hemolysis, Elevated Liver enzymes, and Low Platelet count) is a thrombotic microangiopathy of pregnancy. This syndrome may be incomplete, with manifestations of only a few of its parameters: EL (elevated liver enzymes), ELLP (elevated liver enzymes and thrombocytopenia) and LP (thrombocytopenia alone). ELLP syndrome is a difficult differential diagnosis in which the main causes of thrombocytopenia in pregnancy and other diseases must be excluded. In current clinical practice, the management of ELLP is similar to that of complete HELLP syndrome. However, maternal and fetal morbidity is lower in ELLP syndrome, which may suggest the need to evaluate different protocols for these two variants of the same disease.     

Imitators of severe preeclampsia

There are several obstetric, medical, and surgical disorders that share many of the clinical and laboratory findings of patients with severe preeclampsia-hemolysis, elevated liver enzymes, and low platelets syndrome. Imitators of severe preeclampsia-hemolysis, elevated liver enzymes, and low platelets syndrome are life-threatening emergencies that can develop during pregnancy or in the postpartum period. These conditions are associated with high maternal mortality, and survivors may face long-term sequelae. Perinatal mortality and morbidity also remain high in many of these conditions. The pathophysiologic abnormalities in many of these disorders include thrombotic microangiopathy, thrombocytopenia, and hemolytic anemia. Some of these disorders include acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and acute exacerbation of systemic lupus erythematosus. Because of the rarity of these conditions during pregnancy and postpartum, the available literature includes only case reports and case series describing these syndromes. Consequently, there are no systematic reviews or randomized trials on these subjects. Differential diagnosis may be difficult due to the overlap of several clinical and laboratory findings of these syndromes. It is important that the clinician make the accurate diagnosis when possible because the management and complications from these syndromes may be different. For example, severe preeclampsia and acute fatty liver of pregnancy are treated by delivery, whereas it is possible to continue pregnancy in those with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and exacerbation of systemic lupus erythematosus. This review focuses on diagnosis, management, and counseling of women who develop these syndromes based on results of recent studies.     

Antepartum mastitis: a case report

Background: Acute mastitis commonly occurs in the postpartum period. It has been reported only rarely in the antepartum period.Case: A 14-year-old patient presented at 29 weeks gestation with her symptoms and examination consistent with bilateral mastitis that had worsened over 2 months. She had evidence of systemic infection. She was treated with parenteral antibiotics and local skin care. She gradually improved and delivered a healthy infant at term.Conclusion: The management of antepartum mastitis can be derived from experience with puerperal mastitis. It must include early recognition, a search for predisposing factors and causative organisms, and aggressive treatment. Such an approach can lead to successful pregnancy outcome with minimal fetal or maternal morbidity.     

Hemolytic uremic syndrome in twin pregnancy at 32 weeks gestation with HELLP syndrome. Case report]

Renal failure occurring in pregnancy or post partum is an unusual but well-described complication. Acute renal failure seems to be associated more often with HELLP syndrome rather than with pre-eclampsia or chronic hypertension. Probable overlapping of HELLP and hemolytic uremic syndrome in pregnancy or postpartum should be taken into consideration when treating pregnant women who show signs of proteinuria, hypertension, hematuria, increase of reticulocytes, decrease of haptoglobin with thrombocytopenia and microangiopathic hemolytic anemia. Our case refers to a 32 year old woman at 32 weeks gestation in twin pregnancy who presented with HELLP syndrome and renal failure. Immediately postpartum oliguria was noted and the laboratory analyses suggested the coexistence of HELLP and hemolytic uremic syndrome. In patients with gestosis and/or HELLP syndrome presenting oliguria combined with a decrease of hemoglobin level not due to intraoperative hematic leaks it is always necessary to ask for haptoglobin dosage. In treating hemolytic uremic syndrome it is very important to use a high dosage of plasma and sometimes plasmapheresis. HELLP syndrome contributes to various complications which are sometimes responsible for kidney or maternal mortality. In treating these patients early diagnosis combined with a specific treatment can considerably reduce kidney and maternal mortality.     

Choledochal cysts diagnosed in pregnancy: a case report and review of treatment options.

An 18-year-old woman at 18 weeks' gestation presented with abdominal pain and jaundice. After extensive evaluation, she was diagnosed as having a choledochal cyst. Ten days after percutaneous drainage, she underwent complete excision and Roux-en-Y hepaticojejunostomy anastomosis. At term, she delivered vaginally without complications. Although choledochal cysts are rare in pregnancy, it is important for the obstetrician to be familiar with the presentation and the best treatment modalities available. Delay in diagnosis and inappropriate treatment can result in maternal morbidity and fetal mortality.     

Successful management of pregnancy-associated thrombotic thrombocytopenic purpura by monitoring ADAMTS13 activity

Thrombotic thrombocytopenic purpura (TTP) during pregnancy is very rare and is caused by an absent or severely depleted ADAMTS13 (a disintegrin-like and metallopeptidase with thrombospondin type 1 motif, 13). A 37-year-old multigravida woman developed TTP with severe anemia and thrombocytopenia at 22 weeks' gestation. ADAMTS13 activity was markedly decreased to 3% and ADAMTS13 inhibitor was positive, leading to a definitive diagnosis of TTP. She was successfully treated by plasmapheresis six times, resulting in symptomatic relief. Close follow up with periodic ADAMTS13 measurement facilitated plasmapheresis at appropriate points at a minimum frequency during pregnancy. Because of intrauterine growth retardation from 28 weeks' gestation, an elective cesarean section was performed at 30 weeks' gestation. After delivery, the mother and child showed no appreciable problem. To our knowledge, this is the first report of successful management for pregnancy-associated TTP by monitoring ADAMTS13 activity during pregnancy and the postpartum period.     

Pregnancy complicated by concurrent primary hyperparathyroidism and arrhythmia.

Primary hyperparathyroidism during pregnancy results in a high rate of fetal complications and maternal morbidity. Maternal hypercalcemia in pregnancy results in fetal hypercalcemia, which leads to suppression of fetal parathyroid function. Spontaneous abortion and stillbirth can occur, and the loss of maternal calcium after birth leads to neonatal hypocalcemia. It is essential to detect primary hyperparathyroidism during pregnancy because early diagnosis and management can decrease the rate of fetal and maternal complications. We present the case of a 27-year-old gravida 1, para 0 woman whose pregnancy was complicated by hyperparathyroidism and arrhythmia. The patient complained of dyspnea and palpitations in the seventh and 15th weeks of gestation. Electrocardiography showed ventricular premature contraction bigeminy and trigeminy in association with hypercalcemia (3.3 mmol/L). A parathyroidectomy in the second trimester revealed parathyroid adenoma. Hypercalcemia and arrhythmia resolved completely and the patient delivered a term baby without any maternal or fetal complications. The simultaneous occurrence of arrhythmia with ventricular premature contractions and hyperparathyroidism in pregnancy is rarely reported. Palpitations and dyspnea due to arrhythmia may be associated with primary hyperparathyroidism in pregnancy and should be considered in the differential diagnosis. In the management of symptomatic primary hyperparathyroidism during pregnancy, surgical intervention is preferable in the second trimester when organogenesis is completed and the risk of spontaneous abortion is low.     

Premature labor in a women with perforating appendicitis at 36 weeks of gestation. A case report

BACKGROUND: Acute appendicitis during pregnancy is a serious complication because of the difficult diagnosis and increased perinatal morbidity and mortality. CASE: A 29-year-old woman underwent cesarean section and an appendectomy because of clinical symptoms of an acute abdomen and fetal distress. CONCLUSION: Acute appendicitis in pregnancy is rare but requires special attention to maternal and fetal morbidity and mortality. In this case, acute appendicitis and peritonitis were associated with uterine contractions and fetal distress. How extrauterine infections may mediate uterine contractions and fetal distress remains unsolved.     

Thrombotic thrombocytopenic purpura in a patient with acquired immunodeficiency syndrome at 28 weeks gestation

An increased incidence of thrombotic thrombocytopenic purpura has been reported among human immunodeficiency virus-infected patients or those with acquired immunodeficiency syndrome (AIDS). Despite this association, hitherto only a single case of thrombotic thrombocytopenic purpura has been reported in an HIV-infected patient during pregnancy. We describe a young multiparous patient with long-standing AIDS who presented with lower abdominal pain. Following findings of thrombocytopenia, microangiopathic hemolytic anemia, renal failure, low-grade fever, and mental status changes, thrombotic thrombocytopenic purpura was diagnosed after initially considering the diagnosis of severe preeclampsia, and the patient was delivered by cesarean section. The patient required multiple plasma exchanges during a protracted postpartum course, and was discharged in good health. This first report of thrombotic thrombocytopenic purpura and AIDS in pregnancy demonstrates that when encountering preeclampsia in patients with AIDS, thrombotic microangiopathy should be strongly considered. In addition, these patients may exhibit an attenuated response to plasma exchange therapy and despite previous reports in nonobstetric patients, a favorable outcome is attainable in these critically ill patients.     

Choledochal cysts diagnosed in pregnancy: a case report and review of treatment options

An 18-year-old woman at 18 weeks' gestation presented with abdominal pain and jaundice. After extensive evaluation, she was diagnosed as having a choledochal cyst. Ten days after percutaneous drainage, she underwent complete excision and Roux-en-Y hepaticojejunostomy anastomosis. At term, she delivered vaginally without complications. Although choledochal cysts are rare in pregnancy, it is important for the obstetrician to be familiar with the presentation and the best treatment modalities available. Delay in diagnosis and inappropriate treatment can result in maternal morbidity and fetal mortality.     

Kikuchi disease during pregnancy

Background Kikuchi disease is a rare but distinctive type of necrotizing lymphadenitis. It has a self-limiting clinical course and usually affects the cervical lymph nodes in young women. Although Kikuchi disease occurs most often in young women, it is rare during pregnancy.Case A 28-year-old woman, primigravida, 8 weeks pregnancy, presented initially for antenatal care. Last 4 years, she had chronic cough, weight loss and enlarged left supraclavicular lymph node. The diagnosis was for Kikuchi disease and she received prednisolone for 4 months and had no symptom. She had an enlarged left supraclavicular node again at her first antenatal care. She received no treatment for Kikuchi disease during pregnancy and remained symptom free. The antenatal care course was unremarkable. The maternal outcome was complicated with immediate postpartum hemorrhage; however, the baby was healthy.Conclusion This is the fourth reported case of Kikuchi disease developing during pregnancy. When Kikuchi disease occurs in a pregnant woman, there is neither any effect of disease on the pregnant woman nor the fetus.     

Term extrauterine pregnancy in a Nigerian mother: a complication of uterine dehiscence

Abdominal pregnancy is an uncommon but life-threatening form of ectopic pregnancy. It is associated with high maternal/fetal morbidity and mortality. We present a rare case of term abdominal pregnancy resulting from anterior uterine wall dehiscence, in a 36-year-old woman with three previous caesarean sections. The diagnosis was made at laparotomy for the fourth “caesarean section”.     

Rapid growth of a fetal sacrococcygeal teratoma in an HIV-infected woman: a case report

BACKGROUND: Sacrococcygeal teratoma, the most common congenital neoplasm of the newborn, associated with fetal hydrops and high morbidity and mortality related to the secondary effects of the tumor mass, is of unknown etiology. Prompt diagnosis and early treatment have proven to be effective. CASE: A 24-year-old woman, gravida 3, para 2, at 385/7 weeks' gestation, with a pregnancy complicated by HIV diagnosed during pregnancy, seizure disorder and tobacco use, presented with premature rupture of membranes. Ultrasound examination at 17 weeks' gestation showed normal fetal anatomy. Cesarean delivery was complicated by difficulty delivering a live infant with a large sacral mass. Successful surgical excision of a 650-g mass and stabilization of the infant occurred in the neonatal period. CONCLUSION: This is the first case report to describe a rapidly growing sacrococcygeal teratoma in a neonate from a pregnancy complicated by HIV. Ultrasound in the first and second trimesters identified no fetal abnormalities of the spine. Further research concerning sacrococcygeal teratoma and HIV in pregnancy is necessary for prompt and early diagnosis and treatment of antepartum and peripartum complications.     

A case of acute inflammatory demyelinating polyradiculoneuropathy in early pregnancy.

Acute or chronic inflammatory demyelinating polyradiculoneuropathy is a rare disorder that presents with weakness, hyporeflexia, and sensory loss. Treatment consists of immunosuppression and/or plasma exchange. A woman in the first trimester of pregnancy presents with flaccid quadriplegia and numbness. Electromyography demonstrated a demyelinating polyneuropathy with active denervation. The diagnosis of acute inflammatory demyelinating polyradiculoneuropathy was made. She was treated with corticosteroids and plasmapheresis. Despite slow improvement, she decided to terminate the pregnancy at 18 weeks' gestation. At 1 year postpartum she is still in remission. Inflammatory demyelinating polyradiculoneuropathy should be considered in the differential diagnosis of every pregnant woman with new onset peripheral neuropathy. It is suggested that relapse occurs three times more common during pregnancy. Plasmapheresis is the recommended treatment.     

Paroxysmal nocturnal hemoglobinuria in pregnancy

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thrombotic events. We report a case of a pregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up during the pregnancy, the delivery and the postpartum period. The obstetric literature on women with PNH is reviewed, the maternal and fetal risks are evaluated and the management of pregnancies and deliveries in such patients are discussed. During the pregnancy our patient was hypertransfused and used anticoagulation treatment. A healthy child was delivered in week 37 by cesarean section because of premature rupture of the membranes, unsuccessful induction and intrauterine infection. Because of bleeding problems a hysterectomy also had to be performed. In the postpartum period the patient developed her second episode of a liver vein thrombosis. She recovered gradually and 18 months after the delivery her disease is now in a stable phase. The literature shows a high maternal morbidity and mortality among pregnant PNH patients. Fetal wastage and prematurity rate are also high. Pregnancy in patients with PNH represents a high-risk situation for both the mother and the child and should not be recommended. A pregnant PNH woman should be followed closely by both obstetricians and hematologists.