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| IJCEM Copyright © 2008-All rights reserved. Published by e-Century Publishing Corporation, Madison, WI 53711 |
Int J Clin Exp Med 2012;5(3):262-266
Case Report
Myxofibrosarcoma with metastasis to the lungs, pleura, and mediastinum: a case
report and review of literature
Mihir Raval, Rekha Kallamadi, Dinesh Bande
Department of Internal Medicine, Sanford Hospital, Fargo, North Dakota; University of North Dakota School of Medicine, Grand Forks,
North Dakota; Roger maris cancer center, Sanford Health, Fargo, North Dakota, USA
Received December 11, 2011; accepted April 23, 2012; Epub June 15, 2012; Published June 30, 2012
Abstract: 84 year old male with past medical history of myelodysplastic syndrome (MDS) presented with progressive subcutaneous
and muscle bleed in the right forearm and arm. Workup revealed elevated aPTT- 71.8 seconds (normal 23 - 32 seconds) which was
persistently elevated after mixing study (37.1 seconds immediately and 51.1 seconds after 1 hour). Further laboratory work up revealed
low factor VIII level (3%) and elevated factor VIII inhibitor by Bethesda assay (3 units/ml of blood). Acquired hemophilia A (AHA)
diagnosis was established and patient was treated with recombinant factor VIIa to control the bleeding and also prednisone for
immunosuppression. Subsequent monitoring suggested reduction of factor VIII inhibitor - antibody levels to undetectable level in 3
days and increase of factor VIII level from 3% to 50% in 5 days. Despite of improvement in the laboratory values he continued to have
progression of his bleeding which involved posterior chest wall and also left arm. Due to the progression of the condition and prior
expressed wish family decided to stop the aggressive treatment and patient died nine days after the diagnosis. The case report
describes a rare presentation of AHA in MDS (With bone marrow cytogenetics abnormality) patient with fatal outcome. (IJCEM1112005)
Keywords: Factor VIII, acquired hemophilia A, rFVIIa, bethesda assay, myelodysplastic syndrome, aPTT, factor VIII inhibitor
Address all correspondence to:
Dr. Mihir Raval
Chief Resident -Internal Medicine
Department of Internal Medicine
University of North Dakota
Sanford Health System
801 Broadway North, Fargo, ND 58122-0170, USA.
Tel: 701-234-5934
E-mail: ravalmihir@gmail.com
Case Report
Myxofibrosarcoma with metastasis to the lungs, pleura, and mediastinum: a case
report and review of literature
Mihir Raval, Rekha Kallamadi, Dinesh Bande
Department of Internal Medicine, Sanford Hospital, Fargo, North Dakota; University of North Dakota School of Medicine, Grand Forks,
North Dakota; Roger maris cancer center, Sanford Health, Fargo, North Dakota, USA
Received December 11, 2011; accepted April 23, 2012; Epub June 15, 2012; Published June 30, 2012
Abstract: 84 year old male with past medical history of myelodysplastic syndrome (MDS) presented with progressive subcutaneous
and muscle bleed in the right forearm and arm. Workup revealed elevated aPTT- 71.8 seconds (normal 23 - 32 seconds) which was
persistently elevated after mixing study (37.1 seconds immediately and 51.1 seconds after 1 hour). Further laboratory work up revealed
low factor VIII level (3%) and elevated factor VIII inhibitor by Bethesda assay (3 units/ml of blood). Acquired hemophilia A (AHA)
diagnosis was established and patient was treated with recombinant factor VIIa to control the bleeding and also prednisone for
immunosuppression. Subsequent monitoring suggested reduction of factor VIII inhibitor - antibody levels to undetectable level in 3
days and increase of factor VIII level from 3% to 50% in 5 days. Despite of improvement in the laboratory values he continued to have
progression of his bleeding which involved posterior chest wall and also left arm. Due to the progression of the condition and prior
expressed wish family decided to stop the aggressive treatment and patient died nine days after the diagnosis. The case report
describes a rare presentation of AHA in MDS (With bone marrow cytogenetics abnormality) patient with fatal outcome. (IJCEM1112005)
Keywords: Factor VIII, acquired hemophilia A, rFVIIa, bethesda assay, myelodysplastic syndrome, aPTT, factor VIII inhibitor
Address all correspondence to:
Dr. Mihir Raval
Chief Resident -Internal Medicine
Department of Internal Medicine
University of North Dakota
Sanford Health System
801 Broadway North, Fargo, ND 58122-0170, USA.
Tel: 701-234-5934
E-mail: ravalmihir@gmail.com
