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PubMed · 8023604

[HELLP syndrome].

Abstract

The HELLP syndrome is a severe and life-threatening complication of preeclampsia with typical laboratory findings. The frequency of the disease is 1 to 150-300 live births in perinatal centers. The median gestational age at presentation is 34 weeks, however, the disease may also develop during the early postpartum period. Right upper quadrant pain is the most striking clinical symptom; in up to 15% of cases neither hypertension nor proteinuria is present on admission. For the detection of hemolysis determination of haptoglobin levels is the most suitable method. Coagulation disorders are more pronounced in patients with the HELLP syndrome as compared to those with preeclampsia without the HELLP constellation, however the full-blown syndrome of disseminated intravascular coagulation (DIC) is not a leading symptom but the consequence of delayed diagnosis and/or therapy of the primary disease. The course of the HELLP syndrome is unpredictable. On the one hand, complete reversal of symptoms under conservative treatment have been reported in individual cases, on the other hand, rapid, therapy-resistant deterioration of the disease had been observed in the majority of patients accompanied by severe complications (e.g. liver rupture). As a consequence the mother and the newborn need intensive care, and these women should be delivered in an obstetric intensive care unit. The maternal mortality reported from the international literature is 3.3%, and the perinatal mortality 22.6%.(ABSTRACT TRUNCATED AT 250 WORDS)

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BibTeXRIS

W Rath, W Loos, W Kuhn. 1994. [HELLP syndrome].. https://pubmed.ncbi.nlm.nih.gov/8023604/

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Fatal hemophagocytic syndrome after living-related liver transplantation: a report of two cases.

BACKGROUND: Hemophagocytic syndrome (HPS) is a serious hematological disorder caused by activated T lymphocytes in immunologically compromised patients. There is no report of HPS in liver transplant recipients. METHODS: Among 135 patients who underwent living-related liver transplantation between June 1990 and October 2000, HPS developed in two pediatric patients (1.5%) on the 15th and 134th postoperative day, respectively. The courses of these patients were evaluated. RESULTS: The cause of HPS was unknown in patient 1 and suspected to be Epstein-Barr virus infection in patient 2. The course of patient 2 was also complicated by posttransplant lymphoproliferative disorder. Both patients had high fever, pancytopenia, coagulopathy, and marked elevation of serum-soluble interleukin 2 receptor, serum ferritin, and urine beta2-microglobulin levels. The diagnosis was established based on clinical findings, laboratory data, and bone marrow biopsy. Both patients died in an acute course despite intensive care. CONCLUSIONS: HPS should be recognized as a severe hematological complication in liver transplant patients. Prompt institution of adequate treatment is necessary to prevent fatality.

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