title: Systemic capillary leak syndrome preceding plasma cell leukaemia.
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 x 10(9)/l. 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 pentoxifylline, 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, pentoxifylline 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.
authors.affiliation: Institute of Immunohaematology (Indian Council of Medical Research), KEM Hospital Campus, Parel, Mumbai, India. kanjakshaghosh@hotmail.com
authors.forename: K
authors.initials: K
authors.lastname: Ghosh
authors.forename: M
authors.initials: M
authors.lastname: Madkaikar
authors.forename: Y
authors.initials: Y
authors.lastname: Iyer
authors.forename: A
authors.initials: A
authors.lastname: Pathare
authors.forename: F
authors.initials: F
authors.lastname: Jijina
authors.forename: D
authors.initials: D
authors.lastname: Mohanty
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000097:blood; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D019559:Capillary Leak Syndrome / Q000209:etiology*; D003131:Combined Modality Therapy; D003613:Danazol / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D006333:Heart Failure / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007952:Leukemia, Plasma Cell / Q000175:diagnosis / Q000188:drug therapy / Q000628:therapy; D008297:Male; D009363:Neoplasm Proteins / Q000097:blood; D010431:Pentoxifylline / Q000008:administration & dosage; D010950:Plasma Cells / Q000473:pathology; D011289:Preleukemia / Q000150:complications*; D012008:Recurrence; D014750:Vincristine / Q000008:administration & dosage; D015430:Weight Gain
chemical_list: D000911:Antibodies, Monoclonal; D007074:Immunoglobulin G; D009363:Neoplasm Proteins; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003613:Danazol; D010431:Pentoxifylline