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HELLP syndrome leading to a diagnosis of pregnancy.

ABSTRACT

HELLP syndrome (an acronym for hemolysis, elevated liver enzymes, and low platelets) is a life-threatening complication of pregnancy seen in association with either preeclampsia or eclampsia. HELLP syndrome is rarely diagnosed before 24 weeks of gestation. This atypical form of severe preeclampsia is associated with significant fetal and maternal mortality. The only effective treatment is delivery. We report a case of HELLP syndrome that proved to be a diagnostic clue to an early pregnancy.

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THROMBOTIC MICROANGIOPATHY is characterized by thrombocytopenia and microangiopathic hemolytic anemia. HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count occurring in association with preeclampsia) is one of the thrombotic microangiopathies seen in association with pregnancy. It is an atypical form of preeclampsia that is seen in about 10% of women with preeclampsia. (1) Most cases of HELLP syndrome are diagnosed either in the last trimester of pregnancy or postpartum. However, development of this syndrome before 24 weeks of gestation is rare. (2) We report a case in which pregnancy was recognized after the development of HELLP syndrome.

CASE REPORT

A 34-year-old woman with a history of hypertension presented with blurred vision in the left eye. The patient was alert, oriented, and did not appear to be in acute distress. Despite blood pressure of 235/158 mm Hg, her other vital signs were unremarkable. Funduscopic examination showed only "copper wiring" appearance of retinal vessels. Examination of the heart and lungs was unremarkable. The abdomen was minimally distended but not tender. There was no edema of the extremities, and results of neurologic examination were normal.

Laboratory values included white blood cell count of 13,700/[micro]L, hemoglobin 10 g/dL, platelet count 63,000/[micro]L, creatinine 1 mg/dL, and total bilirubin 0.8 mg/dL. The aspartate transaminase level was 51 U/L (upper normal, 39 U/L), but results of other liver function tests were normal. The serum lactate dehydrogenase was elevated at 621 U/L, reticulocyte count was 6%, and haptoglobin was undetectable. The prothrombin time, activated partial thromboplastin time, and fibrinogen were normal. Urinalysis revealed a moderate amount of protein, 5 to 10 red blood cells, and no evidence of infection. Examination of a peripheral blood smear showed numerous fragmented red cells, polychromasia, and thrombocytopenia.

The finding of thrombocytopenia with microangiopathic hemolytic anemia suggested possible thrombotic thrombocytopenic purpura. Arrangements were undertaken for plasma exchange. Meanwhile, a detailed menstrual history revealed irregular menstrual cycles and uncertainity regarding the date of the last menstrual period. Both urine and serum pregnancy tests were positive. Ultrasonography of the uterus showed a gravid uterus of estimated 10 weeks' gestation. Fetal heart sounds and fetal movements were absent. Misoprostol was used to empty the uterus of the missed abortion. Gestational age was revised to 21 weeks after pathologic examination of the macerated fetus. Examination of the placenta showed multiple infarcts. Tests for antinuclear, antiphospholipid, and anticardiolipin antibodies were negative. Thrombocytopenia and hemolysis resolved quickly after delivery, and liver function values normalized. Since hospital discharge, the patient has subsequently been well.

DISCUSSION

Thrombocytopenia and microangiopathic hemolytic anemia in a young woman may suggest a variety of conditions, including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, disseminated intravascular coagulation, lupus, HELLP syndrome, severe preeclampsia without HELLP syndrome, and acute fatty liver of pregnancy. The last three of these conditions are exclusive to pregnancy. Thrombocytopenia and microangiopathic hemolytic anemia are more severe in HELLP syndrome than in preeclampsia, and acute fatty liver of pregnancy manifests itself primarily as severe liver damage. (2)

Approximately, one third of HELLP syndrome cases are diagnosed postpartum, (3,4) and two thirds are identified ante partum. Of antepartum cases, about 20% are recognized beyond 37 weeks, 70% between 27 and 37 weeks, and only 10% before 27 weeks of gestation. The development of HELLP syndrome before 24 weeks of pregnancy is rare (2) but has been reported. (5) Maternal and perinatal mortality are significantly high at 24% and 30% to 40%, respectively. (2) We believe this is the first reported case of HELLP syndrome presenting with an unknown pregnancy.

CONCLUSION

This case illustrates the importance of eliciting a menstrual history in women with thrombocytopenia as a clue to the possibility of HELLP syndrome in early pregnancy. This case also confirms that even after a fetal death in utero, HELLP syndrome will not resolve until after all of the placental tissue has been removed. Prompt recognition and treatment of this syndrome by delivery can prevent serious maternal morbidity and mortality.

References

(1.) Weinstein L: Syndrome of elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982; 142:159-167

(2.) Magann EF, Martin JN Jr: Twelve steps to optimal management of HELLP syndrome. Clin Obstet Gynecol 1999; 3:532-550

(3.) Martin JN Jr, Magann EF, Blake PG, et al: Analysis of 454 pregnancies with severe preeclampsia/eclampsia HELLP syndrome using the 3-class system of classification. Am J Obstet Gynecol 1993; 68:386

(4.) Sibai BM, Ramadan MK, Usta I, et al: Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol 1993; 169:1000-1006

(5.) McMahon LP, Smith J: The Hellp syndrome at 16 weeks gestation: possible association with the antiphospholipid syndrome. Aust N ZJ Obstet Gynaecol 1997; 37:313-314

RELATED ARTICLE: KEY POINTS

* HELLP syndrome is an atypical form of severe preeclampsia or eclampsia.

* It usually occurs in the third trimester or postpartum and is rarely diagnosed before 24 weeks of gestation.

* This case report describes a young woman who had HELLP syndrome and an unknown pregnancy.

* It is important to elicit a detailed menstrual history in a young woman presenting with thrombocytopenia and anemia.

* Early diagnosis of HELLP syndrome and treatment by delivery can reduce the maternal morbidity and mortality.

From the Department of Medicine, Wright State University, Dayton, Ohio.

Reprint requests to Satheesh K. Kathula, MD, 2392 Miami Village Dr, Miamisburg, OH 45342.
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Title Annotation:hemolysis, elevated liver enzymes, and low platelets
Author:Magann, Everett F.
Publication:Southern Medical Journal
Geographic Code:1USA
Date:Aug 1, 2002
Words:989
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