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Appendiceal Disease Peri-Transplant
S296
Abstracts / Biol Blood Marrow Transplant 22 (2016) S19eS481
Figure 1. TEG during defibrotide therapy with corresponding abnormal RCT. A) At initial diagnosis of SOS B) Post transfusion with TEG normalization prior to defibrotide therapy C) Coagulopathy that developed day +13 demonstrated by abnormal TEG and RCT D) Corrected TEG but persistent abnormal RCT following plasma transfusion
Introduction: Defibrotide is being used with increasing frequency in the prevention and treatment of sinusoidal obstructive syndrome (SOS). The mechanism of action of defibrotide includes fibrinolytic, anti-thrombotic, antiischemic, and anti-inflammatory properties. At Phoenix Children’s Hospital our practice is to optimize hemostasis during defibrotide therapy in order to prevent possible lifethreatening bleeding. Coagulopathy is historically identified by routine coagulation testing (RCT), which includes elevated international normalized ratio (INR), prothrombin time (PT) and activated partial thromboplastin time (aPTT). Study guidelines suggest maintaining INR 1.5, platelets >30 k/uL and fibrinogen 150 mg/dL with transfusions during defibrotide therapy. However, there are times when a coagulopathy can’t be corrected despite transfusion therapy or when therapy is limited by fluid overload. In these cases, we have used thromboelastography (TEG), a functional assay that can assess interaction of clotting mechanisms, to evaluate the overall hemostatic balance and guide initiation of defibrotide therapy. Case Description: Our patient is a 6 year old boy with desmoplastic medulloblastoma who underwent autologous stem cell transplant. On Day +9 he was diagnosed with SOS (hyperbilirubinemia, ascites, hepatomegaly, increased weight) and a significant coagulopathy (INR 1.8, aPTT 47.6 sec, platelet 22 k/uL). His initial TEG was abnormal (R 23.7 min, K 9.6 min, and Angle 23.3 ). After plasma and platelet transfusions, his TEG normalized but his INR and aPTT remained prolonged. Despite these prolonged RCTs, defibrotide was started. Over the course of his defibrotide, attempts were made to correct the abnormal RCTs with blood products. However, his abnormal RCTs persisted despite multiple transfusions while TEG parameters remained normal or corrected with transfusion. By day +17, his abnormal RCTs began to resolve with improvement of the SOS. During his course of defibrotide, he had no bleeding complications. Discussion: TEG evaluation in the setting of SOS provided reassuring data on hemostasis that allowed utilization of defibrotide despite prolonged RCTs. TEG has been reported in the literature to be a more accurate predictor for risk of
bleeding given its ability to monitor the interaction of cell surfaces with procoagulant and anticoagulant factors. In this case, TEG parameters remained normal or corrected with transfusion and patient remained free of bleeding complications. Abnormal RCTs persisted despite multiple transfusions and did not provide as valuable information on risk of bleeding. Overall, our experience would suggest that TEG should be considered along with RCTs in assessing bleeding risk and transfusion needs in the setting of defibrotide therapy. Further studies using TEG during defibrotide therapy are needed to help further define its role.
435 Appendiceal Disease Peri-Transplant Zachary Wright 1, Michael A. Wiggins 2, John S. Renshaw 3, Alexander W. Brown 3, Michael B. Osswald 3. 1 SAMMC, FSH, TX; 2 Hematology/Oncology, San Antonio Military Medical Center, Fort Sam Houston, TX; 3 Hematology/Oncology, San Antonio Military Medical Center, JBSA-Fort Sam Houston, TX Appendiceal disease peri-transplant Appendicitis and other appendiceal disease are relatively rarely reported complications during stem cell transplantation for hematopoetic malignancies and there is not much guidance on management in the published literature. Over the past year we have taken care of five patients with appendiceal disease either pre and post stem cell transplantation and report on their management and outcomes. Management of appendicitis and other appendiceal disease peri-transplant is dictated by clinical situation. Case 1 had a mucinous adenoma of the appendix discovered on his final restaging CT pre-transplant; pre-emptive, elective appendectomy delayed his transplant by 5 weeks without complication. Case 2 developed appendicitis during neutropenic phase of induction/consolidation chemotherapy pre-transplant and did not respond fully to conservative therapy with antibiotics prompting laparoscopic appendectomy. Case 3 developed clinical appendicitis with an
Abstracts / Biol Blood Marrow Transplant 22 (2016) S19eS481
S297
Table 1 Case
Primary Hematologic Diagnosis
Clinical Appendiceal diagnosis
Timing relative to transplantation
Treatment
Outcome
1 21 M 2 49M
PTCL NOS
Mucinous Adenoma
Day -42 pre Auto SCT in CR1
ALL
Appendicitis
Day e 108 pre Allo SCT in CR1
Transplant delayed 5 weeks. No complications Recovered post appendectomy. Transplant complicated by VOD, Died TRM Day+42
3 55M
Myelofibrosis
Appendicitis with appendicolith
Day + 106 post Allo SCT
Immediate laparoscopic Appendectomy IV antibiotics with delayed (3 weeks) laparoscopic appendectomy IV antibiotics
4 33M 5 58M
NHL w/CNS relapse
Appendicitis with C. diff infection Mucositis with possible appendicitis
Day + 9 post Auto SCT in CR2
Myeloma
Day +8 post Auto SCT in PR1
appendicolith 3 1/2 months post allogenic transplantation with successful, non-operative management and planned elective appendectomy when off immunosuppression. Cases 4 and 5 had CT radiographic evidence of appendicitis early post transplantation and recovered successfully with intravenous antibiotics and supportive care suggesting standard regimen related mucositis as the etiology with no further therapy needed.
TRANSPLANT DATA MANAGEMENT
436 Improving the Reporting of Cytogenetics and Molecular Markers Lindsay Dozeman. Internal Medicine, Division of Hematology, Oncology, and Blood and Marrow Transplantation, University of Iowa Hospitals and Clinics, Iowa City, IA
IV antibiotics. Non-operative IV antibiotics Supportive care
Recovered. Planned elective appendectomy when off immunosuppression Recovered without complication Recovered without complication
Improving the Reporting of Cytogenetics and Molecular Markers Background: In 2013 CIBMTR began requesting cytogenetics and molecular markers data to be reported on the Pre-TED for all patients. We found that organizing the data entry process improved our reporting accuracy of this complex data. We also found that utilizing a split screen feature for recipients with multiple transplants assured greater reporting accuracy among transplants. Methods: Our data team downloaded the CIBMTR Data Back to Centers (DBtC) files to create the cytogenetics database template. Hover tips were programmed to provide data staff with hints about commonly associated diseases with each marker. A Help Portal was built to support the team’s questions and need for information when reporting difficult cases. Conclusion: By utilizing a variety of features to support cytogenetics and molecular markers data entry, our team has been able to accurately capture this complex data for analyses as well as assure quality data is being reported to the CIBMTR.
Figure 1. Cytogenetics database template
Abstracts / Biol Blood Marrow Transplant 22 (2016) S19eS481
Figure 1. TEG during defibrotide therapy with corresponding abnormal RCT. A) At initial diagnosis of SOS B) Post transfusion with TEG normalization prior to defibrotide therapy C) Coagulopathy that developed day +13 demonstrated by abnormal TEG and RCT D) Corrected TEG but persistent abnormal RCT following plasma transfusion
Introduction: Defibrotide is being used with increasing frequency in the prevention and treatment of sinusoidal obstructive syndrome (SOS). The mechanism of action of defibrotide includes fibrinolytic, anti-thrombotic, antiischemic, and anti-inflammatory properties. At Phoenix Children’s Hospital our practice is to optimize hemostasis during defibrotide therapy in order to prevent possible lifethreatening bleeding. Coagulopathy is historically identified by routine coagulation testing (RCT), which includes elevated international normalized ratio (INR), prothrombin time (PT) and activated partial thromboplastin time (aPTT). Study guidelines suggest maintaining INR 1.5, platelets >30 k/uL and fibrinogen 150 mg/dL with transfusions during defibrotide therapy. However, there are times when a coagulopathy can’t be corrected despite transfusion therapy or when therapy is limited by fluid overload. In these cases, we have used thromboelastography (TEG), a functional assay that can assess interaction of clotting mechanisms, to evaluate the overall hemostatic balance and guide initiation of defibrotide therapy. Case Description: Our patient is a 6 year old boy with desmoplastic medulloblastoma who underwent autologous stem cell transplant. On Day +9 he was diagnosed with SOS (hyperbilirubinemia, ascites, hepatomegaly, increased weight) and a significant coagulopathy (INR 1.8, aPTT 47.6 sec, platelet 22 k/uL). His initial TEG was abnormal (R 23.7 min, K 9.6 min, and Angle 23.3 ). After plasma and platelet transfusions, his TEG normalized but his INR and aPTT remained prolonged. Despite these prolonged RCTs, defibrotide was started. Over the course of his defibrotide, attempts were made to correct the abnormal RCTs with blood products. However, his abnormal RCTs persisted despite multiple transfusions while TEG parameters remained normal or corrected with transfusion. By day +17, his abnormal RCTs began to resolve with improvement of the SOS. During his course of defibrotide, he had no bleeding complications. Discussion: TEG evaluation in the setting of SOS provided reassuring data on hemostasis that allowed utilization of defibrotide despite prolonged RCTs. TEG has been reported in the literature to be a more accurate predictor for risk of
bleeding given its ability to monitor the interaction of cell surfaces with procoagulant and anticoagulant factors. In this case, TEG parameters remained normal or corrected with transfusion and patient remained free of bleeding complications. Abnormal RCTs persisted despite multiple transfusions and did not provide as valuable information on risk of bleeding. Overall, our experience would suggest that TEG should be considered along with RCTs in assessing bleeding risk and transfusion needs in the setting of defibrotide therapy. Further studies using TEG during defibrotide therapy are needed to help further define its role.
435 Appendiceal Disease Peri-Transplant Zachary Wright 1, Michael A. Wiggins 2, John S. Renshaw 3, Alexander W. Brown 3, Michael B. Osswald 3. 1 SAMMC, FSH, TX; 2 Hematology/Oncology, San Antonio Military Medical Center, Fort Sam Houston, TX; 3 Hematology/Oncology, San Antonio Military Medical Center, JBSA-Fort Sam Houston, TX Appendiceal disease peri-transplant Appendicitis and other appendiceal disease are relatively rarely reported complications during stem cell transplantation for hematopoetic malignancies and there is not much guidance on management in the published literature. Over the past year we have taken care of five patients with appendiceal disease either pre and post stem cell transplantation and report on their management and outcomes. Management of appendicitis and other appendiceal disease peri-transplant is dictated by clinical situation. Case 1 had a mucinous adenoma of the appendix discovered on his final restaging CT pre-transplant; pre-emptive, elective appendectomy delayed his transplant by 5 weeks without complication. Case 2 developed appendicitis during neutropenic phase of induction/consolidation chemotherapy pre-transplant and did not respond fully to conservative therapy with antibiotics prompting laparoscopic appendectomy. Case 3 developed clinical appendicitis with an
Abstracts / Biol Blood Marrow Transplant 22 (2016) S19eS481
S297
Table 1 Case
Primary Hematologic Diagnosis
Clinical Appendiceal diagnosis
Timing relative to transplantation
Treatment
Outcome
1 21 M 2 49M
PTCL NOS
Mucinous Adenoma
Day -42 pre Auto SCT in CR1
ALL
Appendicitis
Day e 108 pre Allo SCT in CR1
Transplant delayed 5 weeks. No complications Recovered post appendectomy. Transplant complicated by VOD, Died TRM Day+42
3 55M
Myelofibrosis
Appendicitis with appendicolith
Day + 106 post Allo SCT
Immediate laparoscopic Appendectomy IV antibiotics with delayed (3 weeks) laparoscopic appendectomy IV antibiotics
4 33M 5 58M
NHL w/CNS relapse
Appendicitis with C. diff infection Mucositis with possible appendicitis
Day + 9 post Auto SCT in CR2
Myeloma
Day +8 post Auto SCT in PR1
appendicolith 3 1/2 months post allogenic transplantation with successful, non-operative management and planned elective appendectomy when off immunosuppression. Cases 4 and 5 had CT radiographic evidence of appendicitis early post transplantation and recovered successfully with intravenous antibiotics and supportive care suggesting standard regimen related mucositis as the etiology with no further therapy needed.
TRANSPLANT DATA MANAGEMENT
436 Improving the Reporting of Cytogenetics and Molecular Markers Lindsay Dozeman. Internal Medicine, Division of Hematology, Oncology, and Blood and Marrow Transplantation, University of Iowa Hospitals and Clinics, Iowa City, IA
IV antibiotics. Non-operative IV antibiotics Supportive care
Recovered. Planned elective appendectomy when off immunosuppression Recovered without complication Recovered without complication
Improving the Reporting of Cytogenetics and Molecular Markers Background: In 2013 CIBMTR began requesting cytogenetics and molecular markers data to be reported on the Pre-TED for all patients. We found that organizing the data entry process improved our reporting accuracy of this complex data. We also found that utilizing a split screen feature for recipients with multiple transplants assured greater reporting accuracy among transplants. Methods: Our data team downloaded the CIBMTR Data Back to Centers (DBtC) files to create the cytogenetics database template. Hover tips were programmed to provide data staff with hints about commonly associated diseases with each marker. A Help Portal was built to support the team’s questions and need for information when reporting difficult cases. Conclusion: By utilizing a variety of features to support cytogenetics and molecular markers data entry, our team has been able to accurately capture this complex data for analyses as well as assure quality data is being reported to the CIBMTR.
Figure 1. Cytogenetics database template