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Systemic capillary leak syndrome preceding plasma cell leukaemia
1Institute of Immunohaematology (Indian Council of Medical Research), KEM Hospital Campus, Parel, Mumbai, India. kanjakshaghosh@hotmail.com
Acta Haematologica
|November 20, 2001
Summary
Systemic capillary leak syndrome preceded plasma cell leukaemia diagnosis by months. Autologous stem cell transplant offered a durable response for capillary leak syndrome, though not for the underlying plasma cell leukaemia.
Area of Science:
- Hematology
- Oncology
- Internal Medicine
Background:
- Systemic capillary leak syndrome (SCLS) is a rare disorder.
- SCLS is often associated with monoclonal gammopathies.
Observation:
- A patient presented with SCLS symptoms preceding plasma cell leukaemia (PCL) diagnosis by 5-6 months.
- Initial SCLS symptoms included weight gain, congestive heart failure, cough, and anasarca with normal organ function.
- Diagnosis of PCL was confirmed by serum protein electrophoresis showing monoclonal IgG and peripheral blood smear with 60% plasma cells.
Findings:
- SCLS initially responded to terbutaline and aminophylline but became refractory.
- Vincristine, doxorubicin, and dexamethasone (VAD) therapy provided only transient SCLS response.
- Danocrine and pentoxifylline did not yield durable SCLS response.
- Autologous peripheral blood stem cell transplantation (PBSCT) achieved a sustained SCLS remission for 10 months.
Implications:
- This case highlights the complex relationship between SCLS and PCL.
- PBSCT may be a viable therapeutic option for SCLS refractory to conventional treatments in PCL patients.
- Further research is needed to understand the pathophysiology and optimize treatment strategies for SCLS associated with hematologic malignancies.