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Haemophagocytic lymphohistiocytosis presenting as HELLP syndrome: a diagnostic and therapeutic challenge
- Source :
- BMJ Case Reports. :bcr-2017
- Publication Year :
- 2017
- Publisher :
- BMJ, 2017.
-
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.
- Subjects :
- Adult
HELLP Syndrome
Pediatrics
medicine.medical_specialty
Abdominal pain
HELLP syndrome
medicine.medical_treatment
Lymphohistiocytosis, Hemophagocytic
Article
Diagnosis, Differential
03 medical and health sciences
Fatal Outcome
0302 clinical medicine
Pregnancy
hemic and lymphatic diseases
Biopsy
medicine
Humans
Etoposide
Bone Marrow Transplantation
Chemotherapy
030219 obstetrics & reproductive medicine
medicine.diagnostic_test
business.industry
General Medicine
medicine.disease
Haemolysis
medicine.anatomical_structure
030220 oncology & carcinogenesis
Female
Bone marrow
medicine.symptom
business
Rare disease
medicine.drug
Subjects
Details
- ISSN :
- 1757790X
- Database :
- OpenAIRE
- Journal :
- BMJ Case Reports
- Accession number :
- edsair.doi.dedup.....c3c6b6dadb337f7b15f1c62eaff9e389