Haemophagocytic Lymphohistiocytosis Presenting As HELLP Syndrome: a Diagnostic and Therapeutic Challenge

Overview

Journal BMJ Case Rep

Specialty General Medicine

Date 2017 Apr 24

PMID 28433984

Citations 13

Authors

Robert Noel Kerley

Raymond Michael Kelly

Mary Rose Cahill

Louise Clare Kenny

Affiliations

Soon will be listed here.

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, haematological disorder, which can be clinically challenging to diagnose and manage. We report a case of HLH in a previously healthy 33-year-old primigravida. The patient presented at 22 weeks gestation with dyspnoea, abdominal pain, anaemia, thrombocytopenia and elevated liver enzymes suggestive of HELLP syndrome.HELLP, a syndrome characterised by haemolysis, elevated liver enzymes and low platelets is considered a severe form of pre-eclampsia. Despite delivery of the fetus, her condition deteriorated over 3-4 days with high-grade fever, worsening thrombocytopenia and anaemia requiring transfusion support. A bone marrow biopsy showed haemophagocytosis and a diagnosis of HLH was made. Partial remission was achieved with etoposide-based chemotherapy and complete remission following bone marrow transplantation. Eleven months post-transplant, the disease aggressively recurred, and the patient died within 3 weeks of relapse.

Citing Articles

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Postpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis.

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Haemophagocytic lymphohistiocytosis in pregnancy.

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