Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction

We report the case of a 6-year-old boy diagnosed with acute promyelocytic leukemia (AML-M3V) when he presented with pallor, abdominal pain, anorexia, and fatigue. Induction chemotherapy was started according to the AML-BFM 98 protocol along with Vesanoid (ATRA, All-trans retinoic acid). On the sixth...

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Main Author: Farhat, Hussein (author)
Other Authors: Farah, Roula A. (author), Jalkh, Khalil S. (author), Sayad, Paul E. (author), Kadri, Adel M. (author)
Format: article
Published: 2011
Online Access:http://hdl.handle.net/10725/4044
http://dx.doi.org/10.1097/MBC.0b013e32834248e6
http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php
http://journals.lww.com/bloodcoagulation/Abstract/2011/03000/Acquired_protein_C_deficiency_in_a_child_with.12.aspx
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author Farhat, Hussein
author2 Farah, Roula A.
Jalkh, Khalil S.
Sayad, Paul E.
Kadri, Adel M.
author2_role author
author
author
author
author_facet Farhat, Hussein
Farah, Roula A.
Jalkh, Khalil S.
Sayad, Paul E.
Kadri, Adel M.
author_role author
dc.creator.none.fl_str_mv Farhat, Hussein
Farah, Roula A.
Jalkh, Khalil S.
Sayad, Paul E.
Kadri, Adel M.
dc.date.none.fl_str_mv 2011
2016-06-15T09:45:35Z
2016-06-15T09:45:35Z
2016-06-15
dc.identifier.none.fl_str_mv 0957-5235
http://hdl.handle.net/10725/4044
http://dx.doi.org/10.1097/MBC.0b013e32834248e6
Farah, R. A., Jalkh, K. S., Farhat, H. Z., Sayad, P. E., & Kadri, A. M. (2011). Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction. Blood Coagulation & Fibrinolysis, 22(2), 140-143.
http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php
http://journals.lww.com/bloodcoagulation/Abstract/2011/03000/Acquired_protein_C_deficiency_in_a_child_with.12.aspx
dc.language.none.fl_str_mv en
dc.relation.none.fl_str_mv Blood Coagulation & Fibrinolysis
dc.rights.*.fl_str_mv info:eu-repo/semantics/openAccess
dc.title.none.fl_str_mv Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
dc.type.none.fl_str_mv Article
info:eu-repo/semantics/publishedVersion
info:eu-repo/semantics/article
description We report the case of a 6-year-old boy diagnosed with acute promyelocytic leukemia (AML-M3V) when he presented with pallor, abdominal pain, anorexia, and fatigue. Induction chemotherapy was started according to the AML-BFM 98 protocol along with Vesanoid (ATRA, All-trans retinoic acid). On the sixth day of induction, he developed splenic and gallbladder infarcts. Splenectomy and cholecystectomy were performed while chemotherapy induction continued as scheduled. Four days later, he developed ischemic areas in the kidneys and ischemic colitis in the sigmoid colon. Hypercoagulation studies showed severe deficiency of protein C. Tests showed protein C 16% (reference range 70–140%), protein S 87% (reference range 70–140%), antithrombin III 122% (reference range 80–120%), prothrombin time 13.6 s (reference = 11.3), INR (international normalized ratio) 1.21, partial thromboplastin time 33 s (reference = 33), fibrinogen 214 mg/dl, D-dimer 970 μg/ml, factor II 98%, and that antinuclear antibody, antiphospholipid antibodies, mutation for factor II gene (G20210A), and mutation for Arg506 Gln of factor V were all negative (factor V Leiden). There was no evidence of clinical disseminated intravascular coagulation (DIC). He was treated with low molecular weight heparin and did well. He continues to be in complete remission 7 years later with normal protein C levels. Acquired protein C deficiency can occur in a variety of settings and has been reported in acute myelocytic leukemia. However, clinically significant thrombosis in the absence of clinical DIC, such as our case, remains extremely rare.
eu_rights_str_mv openAccess
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id LAURepo_4cc75adeecee2f32ceb51655d6a7c123
identifier_str_mv 0957-5235
Farah, R. A., Jalkh, K. S., Farhat, H. Z., Sayad, P. E., & Kadri, A. M. (2011). Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction. Blood Coagulation & Fibrinolysis, 22(2), 140-143.
language_invalid_str_mv en
network_acronym_str LAURepo
network_name_str Lebanese American University repository
oai_identifier_str oai:laur.lau.edu.lb:10725/4044
publishDate 2011
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repository.name.fl_str_mv
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spelling Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarctionFarhat, HusseinFarah, Roula A.Jalkh, Khalil S.Sayad, Paul E.Kadri, Adel M.We report the case of a 6-year-old boy diagnosed with acute promyelocytic leukemia (AML-M3V) when he presented with pallor, abdominal pain, anorexia, and fatigue. Induction chemotherapy was started according to the AML-BFM 98 protocol along with Vesanoid (ATRA, All-trans retinoic acid). On the sixth day of induction, he developed splenic and gallbladder infarcts. Splenectomy and cholecystectomy were performed while chemotherapy induction continued as scheduled. Four days later, he developed ischemic areas in the kidneys and ischemic colitis in the sigmoid colon. Hypercoagulation studies showed severe deficiency of protein C. Tests showed protein C 16% (reference range 70–140%), protein S 87% (reference range 70–140%), antithrombin III 122% (reference range 80–120%), prothrombin time 13.6 s (reference = 11.3), INR (international normalized ratio) 1.21, partial thromboplastin time 33 s (reference = 33), fibrinogen 214 mg/dl, D-dimer 970 μg/ml, factor II 98%, and that antinuclear antibody, antiphospholipid antibodies, mutation for factor II gene (G20210A), and mutation for Arg506 Gln of factor V were all negative (factor V Leiden). There was no evidence of clinical disseminated intravascular coagulation (DIC). He was treated with low molecular weight heparin and did well. He continues to be in complete remission 7 years later with normal protein C levels. Acquired protein C deficiency can occur in a variety of settings and has been reported in acute myelocytic leukemia. However, clinically significant thrombosis in the absence of clinical DIC, such as our case, remains extremely rare.PublishedN/A2016-06-15T09:45:35Z2016-06-15T09:45:35Z20112016-06-15Articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article0957-5235http://hdl.handle.net/10725/4044http://dx.doi.org/10.1097/MBC.0b013e32834248e6Farah, R. A., Jalkh, K. S., Farhat, H. Z., Sayad, P. E., & Kadri, A. M. (2011). Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction. Blood Coagulation & Fibrinolysis, 22(2), 140-143.http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.phphttp://journals.lww.com/bloodcoagulation/Abstract/2011/03000/Acquired_protein_C_deficiency_in_a_child_with.12.aspxenBlood Coagulation & Fibrinolysisinfo:eu-repo/semantics/openAccessoai:laur.lau.edu.lb:10725/40442021-03-19T10:00:54Z
spellingShingle Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
Farhat, Hussein
status_str publishedVersion
title Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
title_full Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
title_fullStr Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
title_full_unstemmed Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
title_short Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
title_sort Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction
url http://hdl.handle.net/10725/4044
http://dx.doi.org/10.1097/MBC.0b013e32834248e6
http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php
http://journals.lww.com/bloodcoagulation/Abstract/2011/03000/Acquired_protein_C_deficiency_in_a_child_with.12.aspx