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Hepatosplenic T cell lymphoma presenting as multiorgan failure
Puraskar Pateria1,2, Annalise Martin3, Tze Sheng Khor4
1Gastroenterology and Hepatology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
BMJ Case Reports
|March 24, 2019
Summary
A rare hepatosplenic T-cell lymphoma caused rapid multiorgan failure in a 59-year-old woman. Despite aggressive chemotherapy, the aggressive cancer progressed, leading to palliative care.
Area of Science:
- Hematology
- Oncology
Background:
- Hepatosplenic T-cell lymphoma (HSTCL) is an aggressive non-Hodgkin lymphoma.
- HSTCL primarily affects young males, with rare occurrences in older adults.
- Diagnosis can be challenging due to non-specific symptoms and rarity.
Observation:
- A 59-year-old woman presented with malaise, abdominal distention, and weight loss.
- Initial misdiagnosis as community-acquired pneumonia and chronic liver disease.
- Rapid deterioration into multiorgan failure requiring intensive care.
Findings:
- Liver and bone marrow biopsies confirmed hepatosplenic T-cell lymphoma.
- Poor response to first-line chemotherapy (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisolone).
- Disease progression despite salvage ifosfamide, carboplatin, etoposide (ICE) chemotherapy.
Implications:
- Highlights the importance of considering rare diagnoses in atypical presentations.
- Underscores the aggressive nature and poor prognosis of HSTCL, even in older patients.
- Emphasizes the challenges in treating refractory HSTCL and the need for novel therapeutic strategies.
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