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Syndrome of hemolysis, elevated liver enzymes, and low platelet count: report of 4 cases.

Four cases of severe preeclamptic patients with the findings of hemolysis (H), elevated liver enzymes (EL) and a low platelet count (LP) were presented. Maternal mortality occurred in two of the four cases with one experiencing liver rupture. Intrauterine fetal death occurred in 3 cases. The entity has been termed the HELLP syndrome. The high maternal and perinatal mortality of our cases was mainly due to the severe coagulopathy associated with the HELLP syndrome. The practicing obstetrician must be knowledgeable about this severe consequence of toxemia in pregnancy and aggressive management is essential to improve maternal and perinatal outcome.

Adult↗

[Study of platelet count and phagocytic activity in experimental hemorrhagic shock. II. Evaluation of phagocytic activity of platelets].

The platelets phagocytic activity has been measured in rats during experimental haemorrhagic shock determining the per cent of phagocytic platelets and phagocytic index. The authors have the decrease of phagocytic activity of platelets during haemorrhagic shock. Disturbances of phagocytic activity of platelets have been accompanied by changes of their shape and formation platelet aggregates.

Animals↗

[The relationship among the counts of platelet, thrombopoietin and spleen index in patients with liver cirrhosis].

OBJECTIVE: To determine the reason of thrombocytopenia in patients with liver cirrhosis, we studied the relationship among platelet counts, serum thrombopoietin (TPO) level and spleen index. METHODS: Serum TPO, platelet counts and spleen index were measured in 71 cirrhotic patients. TPO was measured with ELISA method, spleen index were measured on ultrasonography by the same doctor. RESULTS: Platelet counts in patients with cirrhosis were lower than that of healthy group [(109.20+/-53.39) vs (169.63+/-26.60) x 10(12)/L, P<0.05]. Serum thrombopoietin level in patients with cirrhosis was similar to that of healthy group [(436.42+/-258.97) vs (412.63+/-132.80) pg/ml, P>0.05]. However, serum thrombopoietin level decreased as liver disease aggravated, [(526.13+/-317.44) pg/ml in Child-Pugh grade A, (445.22+/-214.90) pg/ml in grade B and (311.45+/-182.66) pg/ml in grade C, grade A vs. Grade C, P<0.05]. However, decline in platelet counts was accompanied with incline in spleen index coordinately. 35 of 71 cirrhotic patients had normal platelet counts whereas 36 of them had thrombocytopenia. Thrombopoietin levels were higher in non-thrombocytopenia group than in thrombocytopenia group [(529.43+/-282.64) vs. (351.27+/-228.25)pg/ml, P<0.01]; but spleen index of two groups showed no difference [(29.65+/-12.00) vs. (36.35+/-12.68) cm2, P>0.05]. Correlation was found between thrombopoietin level and platelet counts (r=0.252, P=0.025); no correlation was found between spleen index and platelet counts (r=-0.238, P=0.062). CONCLUSION: The decline serum TPO levels might play an important role for thrombocytopenia in patients with liver cirrhosis.

Adult↗

HELLP syndrome--a syndrome of hemolysis, elevated liver enzymes and low platelet count--complicating preeclampsia-eclampsia.

The course of preeclamptic/eclamptic patients may be complicated by HELLP syndrome, a syndrome of intravascular hemolysis (H), elevated liver enzymes (EL) and low platelet count (LP). These patients typically present at early third trimester with epigastric or right upper quadrant pain, nausea and vomiting. They may present without the clinical signs of preeclampsia (hypertension and proteinuria or edema), thus an initial wrong nonobstetric diagnosis is not uncommon. The most frequent maternal complication is intravascular coagulopathy (30%). Placental abruption and acute renal failure are also common. Ten cases of maternal deaths were reported among 295 cases reviewed in the English language literature, while the perinatal mortality rate was 226/1000. The grave prognosis for mother and fetus warrants physician awareness in order to accomplish early diagnosis and proper management. This paper is a review of the literature in English.

Eclampsia↗

Prolonged bleeding time with adequate platelet count in hospital patients.

100 patients with a modified Ivy bleeding time longer than 10 min in the presence of more than 80 x 10(9)/l blood platelets were studied retrospectively. 72 patients had repeated bleeding times between 10 and 20 min or one or more measurements exceeding 20 min, and were considered to have an unquestionable platelet dysfunction. 39 (54%) of these 72 patients were receiving large doses of antibiotics. Nearly one half of the patients on antibiotics had a bleeding tendency, but most of these patients had additional features that may have interfered with platelet function, or a coagulation defect. In the remaining 33 patients, the prolonged bleeding time was associated with von Willebrand's disease, liver disease, leukaemia/myeloproliferative disease, paraproteinaemia or aspirin ingestion. High doses of penicillin seem to be the most common cause of qualitative platelet disorders in general hospital practice.

Adolescent↗

[Maternal benefit of postpartum corticosteroid therapy in patients with HELLP (hemolysis elevated liver enzymes low platelets count) syndrome].

Our purpose was to assess the effects of corticotherapy prescribed after delivery on the kinetic of biological parameters of HELLP syndrome (hemolysis elevated liver enzymes low platelets count). Twenty patients in whom pregnancy was complicated by antepartum or postpartum Hellp Syndrome, were randomised to receive, after delivery, either 12 mg of Dexamethasone every 12 hours for two doses (Group C, n=10), or a placebo (Group T, n=10). Biological parameters of the Hellp syndrome were analysed just before the first bolus of either treatment (H0), then after 24, 36, 48 and 72 hours. The corticotherapy was more efficient than placebo, in the correction of the biological parameters of the hellp syndrome. It permitted a rapid correction of the thrombopenia (from the 24th hour) but its effect on hemolysis marker was later (from the 36th hour). The kinetic of the hepatic cytolysis marker was not modified by corticotherapy. Finally, the administration of corticosteroids does not reduce maternal morbidity related to HELLP Syndrome.

Adult↗

Intrahepatic cholangiocarcinoma masquerading as the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count) in pregnancy: case report.

A young woman was seen at 26 weeks' gestation with a clinical profile consistent with the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count). The patient underwent cesarean delivery and was found to have a massive tumor involving the liver. After correction of the coagulopathy, a liver biopsy specimen demonstrated an intrahepatic cholangiocarcinoma. The patient died 3 weeks after diagnosis.

Adult↗

The spectrum of severe preeclampsia: comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome classification.

OBJECTIVE: This study was undertaken to explore the spectrum of maternal disease with a triple classification system of HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome and compare these classes with severe preeclampsia without HELLP syndrome. STUDY DESIGN: In this retrospective analytic study the pregnancies of 777 patients with class 1, 2, or 3 HELLP syndrome were compared and contrasted with those of 193 women with severe preeclampsia but without HELLP syndrome. RESULTS: Eclampsia, epigastric pain, nausea and vomiting, significant proteinuria, major maternal morbidity, and stillbirth increased as HELLP syndrome worsened from class 3 to class 1. In contrast, headache and diastolic hypertension were more common among the significantly heavier patients with severe preeclampsia without HELLP syndrome. Approximately half of pregnancies complicated by class 1 HELLP syndrome exhibited significant maternal morbidity, compared with only 11% of those complicated by severe preeclampsia without HELLP syndrome. Although a significant trend was apparent in increasing levels of lactate dehydrogenase, aspartate aminotransferase, and uric acid as HELLP syndrome worsened, there was considerable variation within groups. CONCLUSION: Laboratory and clinical indices of disease severity in patients with severe preeclampsia or eclampsia generally were highest with class 1 HELLP syndrome and were lowest when HELLP syndrome was absent. Class 3 HELLP syndrome is considered a clinically significant transitional group.

Aspartate Aminotransferases↗

Neonatal outcome in severe preeclampsia at 24 to 36 weeks' gestation: does the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome matter?

OBJECTIVE: Our purpose was to compare neonatal outcome after preterm delivery of infants whose gestation was complicated by the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome, partial HELLP syndrome, or severe preeclampsia. STUDY DESIGN: We reviewed the maternal and neonatal charts from 269 consecutive pregnancies complicated by the HELLP syndrome or severe preeclampsia managed at our perinatal center. The HELLP syndrome was defined by previously published laboratory criteria. Viable pregnancies were divided into 3 groups: HELLP syndrome, partial HELLP syndrome (at least 1, but not all 3, features of the HELLP syndrome), and severe preeclampsia (no features of the HELLP syndrome). Results were compared by means of chi2 analysis and Student t test where appropriate. Logistic regression was used to evaluate outcome variables at different gestational ages. RESULTS: There were no significant differences in complications among the 3 groups at each gestational age. There was, as expected, a significant decrease in morbidity and mortality rates with advanced gestational age. CONCLUSIONS: In severe preeclampsia, neonatal morbidity and death are related to gestational age rather than to the presence or absence of the HELLP syndrome. Whether expectant management is safe for women with the HELLP syndrome requires further study.

Adult↗

HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome versus severe preeclampsia: onset at < or =28.0 weeks' gestation.

OBJECTIVE: Our purpose was to determine whether the onset of the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome in women at < or =28.0 weeks' gestation is associated with an increased risk of adverse maternal and perinatal outcomes in comparison with the risk for women with severe preeclampsia but without the HELLP syndrome at a similar gestational age. STUDY DESIGN: Sixty-four patients with either the HELLP syndrome (n = 32) or severe preeclampsia but absent HELLP syndrome laboratory test results (n = 32), admitted at < or =28.0 weeks' gestation between July 1, 1992, and April 30, 1999, were studied. Maternal and perinatal outcomes were compared between the 2 groups. Statistical analysis was performed by the Student t test and the Fisher exact test. RESULTS: There were no significant differences between the 2 groups regarding African-American race (59% vs 75%), nulliparity (50% vs 56%), or the use of corticosteroids (59% vs 78%). There were no maternal deaths. One woman with the HELLP syndrome had a liver hematoma. The rate at which transfusion of blood products was required was significantly greater in women with the HELLP syndrome than in those with severe preeclampsia only (25% vs 3%; P <.05). There were no significant differences between the 2 groups with respect to eclampsia (16% vs 13%), abruptio placentae (6% vs 9%), disseminated intravascular coagulopathy (13% vs 0%), pulmonary edema (13% vs 6%), acute renal failure (3% vs 0%), pleural effusion (3% vs 3%), or ascites (6% vs 16%). No significant differences were found between the 2 groups with respect to neonatal death (11% vs 17%), respiratory distress syndrome (78% vs 86%), or composite neonatal morbidity. CONCLUSIONS: Except for the need for transfusion of blood products in women with the HELLP syndrome, onset at < or =28.0 weeks' gestation is not associated with an increased risk of adverse maternal or neonatal outcomes in comparison with the risk for women with severe preeclampsia but without the HELLP syndrome at a similar gestational age.

Adult↗

Simple surrogate index of the fibrosis stage in chronic hepatitis C patients using platelet count and serum albumin level.

This study was conducted to develop a simple surrogate index comprised of routinely available laboratory tests to reflect the histological fibrosis stage. Clinical characteristics and laboratory data from 368 and 249 consecutive patients with chronic hepatitis C, a training cohort and a validation cohort, respectively, were retrospectively evaluated. Platelet (Plt) count and albumin (Alb) level contributed to the discrimination of the respective fibrosis stages. We derived the fibrosis index (FI), FI = 8.0-0.01 x Plt (10(3)/microliter) - Alb (g/dl), from a multiple regression model. FI significantly correlated with the histological fibrosis stage in both the initial and validation cohort at rho=0.691 and rho=0.661, respectively (Spearman's rank correlation coefficient, p<0.0001). The sensitivity and positive predictive value of FI at a cutoff value < 2.10 for predicting fibrosis stage F0-1 were 66.8% and 78.8% in the initial cohort and 68.5% and 63.6% in the validation cohort, respectively. Corresponding values of FI at a cutoff value > or = 3.30 for the prediction of F4 were 67.7% and 75.0% in the initial cohort and 70.8% and 81.0% in the validation cohort. The fibrosis index comprised of platelet count and albumin level reflected the histological fibrosis stage in patients with chronic hepatitis C.

Adult↗

Acute lymphocytic and myelomonocytic leukemia associated with low platelet counts and a 21q- marker chromosome.

A terminal deletion of the long arm of one chromosome 21 at band q21 was found in two patients with acute leukemia and a low platelet count: one case of acute myelomonocytic leukemia and one case of acute lymphocytic leukemia. The segment deleted from chromosome 21 could not be found translocated to any other chromosome of the complement. The results indicate that the 21q- marker chromosome may be due to a true deletion and that the marker is no specific for primary thrombocythemia or other myeloproliferative disorders associated with thrombocytosis.

Chromosome Deletion↗

Vascular reactivity in patients with preeclampsia and HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome.

OBJECTIVE: Early structural and functional changes in the systemic vasculature have been proposed to play a major pathogenetic role in preeclampsia and HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. The aim of the study was to determine vascular reactivity in patients with preeclampsia with and without HELLP syndrome with respect to those in healthy pregnant control subjects. STUDY DESIGN: Forearm blood flow was measured by strain gauge plethysmography with the venous occlusion technique in 12 hypertensive patients with HELLP syndrome, in 8 patients with preeclampsia without HELLP syndrome, and in 8 healthy normotensive pregnant control subjects. To determine vascular reactivity the forearm blood flow was measured at baseline and after forearm occlusion for a period of 5 minutes (reactive hyperemia). The investigations were repeated 4 to 6 months post partum. Forearm vascular resistance was calculated as the ratio of mean arterial pressure to forearm blood flow. RESULTS: Mean arterial pressure at rest was elevated in patients with preeclampsia (116 +/- 20 mm Hg) and in patients with HELLP syndrome (110 +/- 16 mm Hg) with respect to healthy pregnant control subjects (86 +/- 10 mm Hg; P <.05). Forearm blood flow at rest was not statistically different in patients with preeclampsia (5.1 +/- 2.6 mL/min per 100 mL) and with HELLP syndrome (4.7 +/- 1.5 mL/min per 100 mL) with respect to pregnant control subjects (5.9 +/- 3.1 mL/min per 100 mL); however, forearm vascular resistance at rest was elevated in patients with preeclampsia (25.9 +/- 9.5 units; P <.05) and in patients with HELLP syndrome(24.6 +/- 6.9 units; P <.05) with respect to healthy control subjects (17.0 +/- 6.1 units). During reactive hyperemia the peak forearm blood flow, which is an indicator of maximal vasodilatory capacity, was impaired in patients with preeclampsia (21.9 +/- 8.2 mL/min per 100 mL; P <.05) but not in patients with HELLP syndrome (37.4 +/- 17.5 mL/min per 100 mL) and healthy control subjects (44.9 +/- 15.0 mL/min per 100 mL). Consequently, minimum forearm vascular resistance was higher in women with preeclampsia (6.1 +/- 1.9 units) than in both women with HELLP syndrome (3.5 +/- 1.6 units) and the control subjects (2.8 +/- 2.4 units). CONCLUSION: Despite similarly elevated forearm vascular resistances at rest in patients with HELLP syndrome and in patients with preeclampsia, forearm vascular resistance during reactive hyperemia did not differ significantly from that in healthy control subjects in the women with HELLP syndrome but was increased in women with preeclampsia. Vasodilatory reactivity thus is reduced in preeclampsia but not in HELLP syndrome, which suggests different alterations of the vasculature.

Adult↗