Systemic capillary leak syndrome preceding plasma cell leukaemia

Kanjaksha Ghosh, Manisha Madkaikar, Yegneshwar Iyer, Anil Pathare, Farah Jijina, Dipika Mohanty

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

We report a patient with plasma cell leukaemia with systemic capillary leak syndrome, a rare disorder often associated with monoclonal gammopathy. In this patient, the manifestation of capillary leak syndrome antedated the diagnosis of plasma cell leukaemia by 5-6 months. During that time, he was repeatedly admitted to the hospital with weight gain, congestive cardiac failure, cough and anasarca in the presence of normal renal function, liver function and normal echocardiography. On presentation, a serum protein electrophoresis showed monoclonal IgG; the blood smear showed 60% plasma cells with a total count of 4.4 × 109/I. A bone marrow aspirate showed replacement of the normal marrow by sheets of immature plasma cells. His systemic capillary leak syndrome initially responded to decongestive therapy with terbutaline and aminophylline but later on he became refractory to them and responded to vincristine, doxorubicin and dexamethasone (VAD) combination therapy only transiently. Danocrine and oxypentiphylline, added during VAD chemotherapy, did not produce a durable response in capillary leak syndrome, which finally responded to autologous peripheral blood stem cell transplantation (PBSCT). After PBSCT, he remained free of capillary leak for 10 months without terbutaline, oxypentiphylline corticosteroids, aminophylline or danocrine. His disease relapsed without recurrence of the capillary leak. He died 15 months after PBSCT and 20 months after the diagnosis of plasma cell leukaemia.

Original languageEnglish
Pages (from-to)118-121
Number of pages4
JournalActa Haematologica
Volume106
Issue number3
DOIs
Publication statusPublished - 2001

Keywords

  • Capillary leak syndrome
  • Improved survival
  • Monoclonal gammopathy
  • Peripheral blood stem cell transplantation
  • Plasma cell leukaemia

ASJC Scopus subject areas

  • Hematology

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  • Cite this

    Ghosh, K., Madkaikar, M., Iyer, Y., Pathare, A., Jijina, F., & Mohanty, D. (2001). Systemic capillary leak syndrome preceding plasma cell leukaemia. Acta Haematologica, 106(3), 118-121. https://doi.org/10.1159/000046600