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MYELOPROLIFERATIVE NEOPLASMS: TREATMENT APPROACH AND OUTCOMES, THE DREXEL UNIVERSITY EXPERIENCE
Author(s):
Sukhdeep Kaur
Affiliations:
internal medicine,Drexel university/Hahnemann hospital,Philadelphia,United States
(Abstract release date: 05/21/15) EHA Library. Kaur S. 06/12/15; 102613; PB1928 Disclosure(s): Drexel university/Hahnemann hospital
internal medicine
Sukhdeep Kaur
Sukhdeep Kaur
Contributions
Abstract
Abstract: PB1928

Type: Publication Only

Background
Myeloproliferative neoplasms are a group of clonal disorders that arise from a transformation in a hematopoietic stem cell. These disorders consist of essential thrombocytosis (ET), primary myelofibrosis (PMF) and polycythemia vera (PCV). Several therapeutic agents have been used in the past to treat these disorders. Treatment strategies for these patients must consider the possibility of long-term survival, morbidity from thrombotic complications, transformation into myelofibrosis with myeloid metaplasia or acute myeloid leukemia, and the effect of specific therapies on the incidence of leukemic transformation and on pregnancy.

Aims
At Drexel University, a significant number of patients were treated with busulfan and were thought to have a more favorable clinical course and increased survival in comparison to other agents. In our study we analyzed the outcomes of patients treated in the practice of I. Brodsky Associates diagnosed with ET, PCV and PMF, who received a variety of treatment modalities, and compared their clinical courses to determine if there is a superior treatment

Methods
This study is a retrospective cohort study in which we examined the medical records of patients treated for the diagnoses of ET, PCV and PMF at Hahnemann Hospital-Drexel University College Medicine in the practice of I. Brodsky Associates from January 1960 to December 2013. The following variables were measured and compared: age of the patient; sex of the patient; demographics; cytogenetics; family history; baseline hemoglobin level, hematocrit level, platelet count and WBC count; JAK 2 V617F mutation status; initial erythropoietin level, red cell mass, oxygen saturation, presence of splenomegaly and B12 level; Bone marrow biopsy results; thrombohemorrhagic complications; transformation to acute leukemia, progression to myelofibrosis and development of  secondary malignancies; and treatment with busulfan, aspirin with or without clopidogrel, anegralide, hydroxyurea, and phlebotomy


Results
One hundred ninteen patients charts were reviewed. Twenty-four patients were given aspirin. One progressed to myelofibrosis (4%), seven patients were noted to have recurrent thrombotic episodes in the form of TIA, CVA, DVT/PE, and MI (29%). Thirty four patients were given Busulfan. Four patients progressed to myelofibrosis with MM (11%) and one patient progressed to CML (3%). Six patients were noted to have thrombotic events in the form of CVA, MI, subclavian vein thrombosis and carotid artery stenosis (17%). Twenty six patients were given hydroxyurea. One patient’s course was complicated by Hodgkin’s lymphoma (4%), four patients progressed to acute leukemic blast crises (15%), and three patients progressed to myelofibrosis (11%). Eight patients were noted to have multiple thrombotic events in the form of CVA, DVT, TIA, splenic vein thrombosis, and renal artery stenosis (31%). Twenty four patients were given Busulfan and Hydroxyurea  together during their course of treatment. Fourteen patients were noted to have multiple thrombotic events in the form of PVT, SMV thrombosis, PE, TIA, Budd Chiari syndrome and LE arterial thrombosis (58%). Two patients progressed to CML blast crises (8%) and three patients developed myelofibrosis with MM (12%).  Eleven patients were treated with only phlebotomy. Five patients progressed to myelofibrosis with MM (45%). Two patients were noted to have thrombotic events that included MI or TIA (18%)

Summary
Our study set out to discover if this unique treatment correlated with improved survival and less treatment toxicity. Busulfan and hydroxyurea given together proved to have the lowest rate of progression to leukemia and myelofibrosis when compared to other standard therapies. Patients treated with hydroxyurea and intermittent busulfan, were shown to have the best long-term outcomes. This suggests that physicians should include the use of busulfan in treating myeloproliferative neoplasms

Keyword(s): Busulfan, Essential Thrombocytemia, Hydroxyurea, Polycythemia vera

Session topic: Publication Only
Abstract: PB1928

Type: Publication Only

Background
Myeloproliferative neoplasms are a group of clonal disorders that arise from a transformation in a hematopoietic stem cell. These disorders consist of essential thrombocytosis (ET), primary myelofibrosis (PMF) and polycythemia vera (PCV). Several therapeutic agents have been used in the past to treat these disorders. Treatment strategies for these patients must consider the possibility of long-term survival, morbidity from thrombotic complications, transformation into myelofibrosis with myeloid metaplasia or acute myeloid leukemia, and the effect of specific therapies on the incidence of leukemic transformation and on pregnancy.

Aims
At Drexel University, a significant number of patients were treated with busulfan and were thought to have a more favorable clinical course and increased survival in comparison to other agents. In our study we analyzed the outcomes of patients treated in the practice of I. Brodsky Associates diagnosed with ET, PCV and PMF, who received a variety of treatment modalities, and compared their clinical courses to determine if there is a superior treatment

Methods
This study is a retrospective cohort study in which we examined the medical records of patients treated for the diagnoses of ET, PCV and PMF at Hahnemann Hospital-Drexel University College Medicine in the practice of I. Brodsky Associates from January 1960 to December 2013. The following variables were measured and compared: age of the patient; sex of the patient; demographics; cytogenetics; family history; baseline hemoglobin level, hematocrit level, platelet count and WBC count; JAK 2 V617F mutation status; initial erythropoietin level, red cell mass, oxygen saturation, presence of splenomegaly and B12 level; Bone marrow biopsy results; thrombohemorrhagic complications; transformation to acute leukemia, progression to myelofibrosis and development of  secondary malignancies; and treatment with busulfan, aspirin with or without clopidogrel, anegralide, hydroxyurea, and phlebotomy


Results
One hundred ninteen patients charts were reviewed. Twenty-four patients were given aspirin. One progressed to myelofibrosis (4%), seven patients were noted to have recurrent thrombotic episodes in the form of TIA, CVA, DVT/PE, and MI (29%). Thirty four patients were given Busulfan. Four patients progressed to myelofibrosis with MM (11%) and one patient progressed to CML (3%). Six patients were noted to have thrombotic events in the form of CVA, MI, subclavian vein thrombosis and carotid artery stenosis (17%). Twenty six patients were given hydroxyurea. One patient’s course was complicated by Hodgkin’s lymphoma (4%), four patients progressed to acute leukemic blast crises (15%), and three patients progressed to myelofibrosis (11%). Eight patients were noted to have multiple thrombotic events in the form of CVA, DVT, TIA, splenic vein thrombosis, and renal artery stenosis (31%). Twenty four patients were given Busulfan and Hydroxyurea  together during their course of treatment. Fourteen patients were noted to have multiple thrombotic events in the form of PVT, SMV thrombosis, PE, TIA, Budd Chiari syndrome and LE arterial thrombosis (58%). Two patients progressed to CML blast crises (8%) and three patients developed myelofibrosis with MM (12%).  Eleven patients were treated with only phlebotomy. Five patients progressed to myelofibrosis with MM (45%). Two patients were noted to have thrombotic events that included MI or TIA (18%)

Summary
Our study set out to discover if this unique treatment correlated with improved survival and less treatment toxicity. Busulfan and hydroxyurea given together proved to have the lowest rate of progression to leukemia and myelofibrosis when compared to other standard therapies. Patients treated with hydroxyurea and intermittent busulfan, were shown to have the best long-term outcomes. This suggests that physicians should include the use of busulfan in treating myeloproliferative neoplasms

Keyword(s): Busulfan, Essential Thrombocytemia, Hydroxyurea, Polycythemia vera

Session topic: Publication Only

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