Kasabach-Merritt phenomenon: a single centre experience.

Hdl Handle:
http://hdl.handle.net/10147/206379
Title:
Kasabach-Merritt phenomenon: a single centre experience.
Authors:
Ryan, Clodagh; Price, Vicotria; John, Philip; Mahant, Sanjay; Baruchel, Sylvain; Brandao, Leonardo; Blanchette, Victor; Pope, Elena; Weinstein, Miriam
Affiliation:
Department of Haematology, Mercy University Hospital, Grenville Place, Cork,, Ireland.
Citation:
Eur J Haematol. 2010 Feb 1;84(2):97-104. Epub 2009 Nov 3.
Journal:
European journal of haematology
Issue Date:
31-Jan-2012
URI:
http://hdl.handle.net/10147/206379
DOI:
10.1111/j.1600-0609.2009.01370.x
PubMed ID:
19889011
Abstract:
OBJECTIVE: Kasabach-Merritt phenomenon (KMP) can lead to life-threatening bleeding, and its optimum treatment has not been established. We review the experience of managing KMP in a single institution. METHODS: A retrospective chart review on all children with KMP treated at the Hospital for Sick Children, Toronto, over an 18 yr period was carried out. RESULTS: All 15 patients had profound thrombocytopenia and hypofibrinogenemia at presentation, half had bleeding symptoms, and three had cardiac failure. All patients received corticosteroids. Five responded to steroids alone, given for an average of 13 wk, increasing platelets to >20 x 10(9)/L at a mean of 6.2 d and fibrinogen >1 g/dL at 25.6 d. Ten patients received at least one other therapeutic modality in addition to steroids, including vincristine, interferon, anti-platelet agents and pentoxifylline. Five patients received vincristine, for a mean of 6 wk, with two patients responding. Eight patients received interferon, for a mean of 4 months, with two patients responding. Overall, the mean time to increasing platelets >20 x 10(9)/L was 56 d, to >150 x 10(9)/L was 88 d and fibrinogen >1 g/dL 49 d. Ten patients showed a partial response to embolisation, with a mean of 2.8 procedures performed. Thrombotic complications occurred in 7%. Twelve patients remain alive, with relapse in six patients, all treated successfully. One patient died, and two patients have been lost to follow-up. CONCLUSION: KMP is a rare condition, with significant morbidity and mortality. The therapeutic approach should include a multidisciplinary team and consensus on guidelines.
Language:
eng
MeSH:
Adrenal Cortex Hormones/*administration & dosage; Antineoplastic Agents, Phytogenic/administration & dosage; Female; Hematologic Diseases/blood/*drug therapy/*mortality; Humans; Infant; Infant, Newborn; Interferons/administration & dosage; Male; Pentoxifylline/administration & dosage; Platelet Aggregation Inhibitors/administration & dosage; Platelet Count; Retrospective Studies; Vincristine/administration & dosage
ISSN:
1600-0609 (Electronic); 0902-4441 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorRyan, Clodaghen_GB
dc.contributor.authorPrice, Vicotriaen_GB
dc.contributor.authorJohn, Philipen_GB
dc.contributor.authorMahant, Sanjayen_GB
dc.contributor.authorBaruchel, Sylvainen_GB
dc.contributor.authorBrandao, Leonardoen_GB
dc.contributor.authorBlanchette, Victoren_GB
dc.contributor.authorPope, Elenaen_GB
dc.contributor.authorWeinstein, Miriamen_GB
dc.date.accessioned2012-01-31T16:39:23Z-
dc.date.available2012-01-31T16:39:23Z-
dc.date.issued2012-01-31T16:39:23Z-
dc.identifier.citationEur J Haematol. 2010 Feb 1;84(2):97-104. Epub 2009 Nov 3.en_GB
dc.identifier.issn1600-0609 (Electronic)en_GB
dc.identifier.issn0902-4441 (Linking)en_GB
dc.identifier.pmid19889011en_GB
dc.identifier.doi10.1111/j.1600-0609.2009.01370.xen_GB
dc.identifier.urihttp://hdl.handle.net/10147/206379-
dc.description.abstractOBJECTIVE: Kasabach-Merritt phenomenon (KMP) can lead to life-threatening bleeding, and its optimum treatment has not been established. We review the experience of managing KMP in a single institution. METHODS: A retrospective chart review on all children with KMP treated at the Hospital for Sick Children, Toronto, over an 18 yr period was carried out. RESULTS: All 15 patients had profound thrombocytopenia and hypofibrinogenemia at presentation, half had bleeding symptoms, and three had cardiac failure. All patients received corticosteroids. Five responded to steroids alone, given for an average of 13 wk, increasing platelets to >20 x 10(9)/L at a mean of 6.2 d and fibrinogen >1 g/dL at 25.6 d. Ten patients received at least one other therapeutic modality in addition to steroids, including vincristine, interferon, anti-platelet agents and pentoxifylline. Five patients received vincristine, for a mean of 6 wk, with two patients responding. Eight patients received interferon, for a mean of 4 months, with two patients responding. Overall, the mean time to increasing platelets >20 x 10(9)/L was 56 d, to >150 x 10(9)/L was 88 d and fibrinogen >1 g/dL 49 d. Ten patients showed a partial response to embolisation, with a mean of 2.8 procedures performed. Thrombotic complications occurred in 7%. Twelve patients remain alive, with relapse in six patients, all treated successfully. One patient died, and two patients have been lost to follow-up. CONCLUSION: KMP is a rare condition, with significant morbidity and mortality. The therapeutic approach should include a multidisciplinary team and consensus on guidelines.en_GB
dc.language.isoengen_GB
dc.subject.meshAdrenal Cortex Hormones/*administration & dosageen_GB
dc.subject.meshAntineoplastic Agents, Phytogenic/administration & dosageen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHematologic Diseases/blood/*drug therapy/*mortalityen_GB
dc.subject.meshHumansen_GB
dc.subject.meshInfanten_GB
dc.subject.meshInfant, Newbornen_GB
dc.subject.meshInterferons/administration & dosageen_GB
dc.subject.meshMaleen_GB
dc.subject.meshPentoxifylline/administration & dosageen_GB
dc.subject.meshPlatelet Aggregation Inhibitors/administration & dosageen_GB
dc.subject.meshPlatelet Counten_GB
dc.subject.meshRetrospective Studiesen_GB
dc.subject.meshVincristine/administration & dosageen_GB
dc.titleKasabach-Merritt phenomenon: a single centre experience.en_GB
dc.contributor.departmentDepartment of Haematology, Mercy University Hospital, Grenville Place, Cork,, Ireland.en_GB
dc.identifier.journalEuropean journal of haematologyen_GB
dc.description.provinceMunster-

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