Contributions
Abstract: PB2313
Type: Publication Only
Background
Myelofibrosis (MF), polycytemia vera (PV), and essential thrombocytosis (ET) are BCR-ABL negative chronic myeloproliferative diseases (CMPD) which may transform to acute myeloid leukemia or myelodysplastic syndrome. Furthermore, lymphoid malignancies are more prevalent in CMPD than normal population. Non-hematological malignancies are also increased in CMPD and those are related to several different etiologic factors.
Aims
Our aim is to evaluate secondary malignancies in our CMPD patients.
Methods
BCR-ABL negative CMPD patients diagnosed and followed up in Ege University Hematology department between 2000 and 2017 are retrospectively evaluated. Patients diagnosis, time of diagnosis, age, gender, mutational status regarding JAK-2, MPL and calreticulin, treatment, treatment side effects, physical examination findings, follow-up time and outcome are recorded. Patients who developed secondary malignancy are evaluated about their cancer treatment and outcome.
Results
There are 264 CMPD (138 female, 126 male) patients investigated. Distribution according to diseases are: 153 ET, 77 PV, 26 MF and 8 other CMPD. During follow-up, secondary malignancy occurred in 5 of our patients (1.89%):
Patient 1: A male post-polycytemia MF patient, diagnosed at the age of 68. JAK-2 mutation is positive. He has been followed up since 10 years and receiving hydroxyurea 500-1500 mg per day. At the seventh year of CMPD, he was diagnosed as squamous cell skin cancer. After surgical resection, he is still in remission.
Patient 2: A male ET patient, diagnosed at the age of 63. JAK-2, MPL and calreticulin mutations are negative. He has been followed up since 7 years. He received only hydroxyurea 1000-1500 mg per day for 6 years, hydroxyurea and anagrelide 1 mg per day combination for the last 1 year. At the fifth year of CMPD, he was diagnosed as squamous cell skin cancer on his ear. He treated only with cryotherapy and still in remission.
Patient 3: A female ET patient, diagnosed at the age of 54. JAK-2, MPL and calreticulin mutations are negative. She has never received cytoreductive treatment. She has been followed up since 7 years. At the fifth year of CMPD, she was diagnosed as licen planus and after local therapies, her lesion is still stable.
Patient 4: A male ET patient, diagnosed at the age of 54. JAK-2 mutation is positive. He has been followed up since 12 years. He received hydroxyurea for sometime after diagnosis but than swithced to anagrelide 2-4 mg per day since 10 years. At the eleventh year of CMPD, he was diagnosed as thyroid papillary carcinoma. After surgery,he was treated with radioactive iodine and now in remission. Patient 5: A female ET patient, diagnosed at the age of 43 after ischaemic cerebrovascular event. JAK-2, MPL and calreticulin mutations are negative. She has been followed up since 14 years. One year after diagnosis she received only hydroxyurea but during last 13 years she has switched to anagrelide 0.5-2 mg per day treatment. At the sixth year of CMPD, she was diagnosed as invaziveductal carcinoma and after mastectomy she received hormonotherapy. She is still in remission.
Conclusion
There are a number of evidences supporting that secondary malignancies are increased in CMPD patients compared to normal population. Although several factors, such as hydroxiurea, are blamed for that increase, the etiologic connection is yet unclear. Skin cancers have been the leading secondary malignancy (60%) in our study group. It is wise to keep in mind the reality of secondary cancers, during CMPD patients hematological management.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Anagrelide, Hydroxyurea, Myeloproliferative disorder, Second malignancy
Abstract: PB2313
Type: Publication Only
Background
Myelofibrosis (MF), polycytemia vera (PV), and essential thrombocytosis (ET) are BCR-ABL negative chronic myeloproliferative diseases (CMPD) which may transform to acute myeloid leukemia or myelodysplastic syndrome. Furthermore, lymphoid malignancies are more prevalent in CMPD than normal population. Non-hematological malignancies are also increased in CMPD and those are related to several different etiologic factors.
Aims
Our aim is to evaluate secondary malignancies in our CMPD patients.
Methods
BCR-ABL negative CMPD patients diagnosed and followed up in Ege University Hematology department between 2000 and 2017 are retrospectively evaluated. Patients diagnosis, time of diagnosis, age, gender, mutational status regarding JAK-2, MPL and calreticulin, treatment, treatment side effects, physical examination findings, follow-up time and outcome are recorded. Patients who developed secondary malignancy are evaluated about their cancer treatment and outcome.
Results
There are 264 CMPD (138 female, 126 male) patients investigated. Distribution according to diseases are: 153 ET, 77 PV, 26 MF and 8 other CMPD. During follow-up, secondary malignancy occurred in 5 of our patients (1.89%):
Patient 1: A male post-polycytemia MF patient, diagnosed at the age of 68. JAK-2 mutation is positive. He has been followed up since 10 years and receiving hydroxyurea 500-1500 mg per day. At the seventh year of CMPD, he was diagnosed as squamous cell skin cancer. After surgical resection, he is still in remission.
Patient 2: A male ET patient, diagnosed at the age of 63. JAK-2, MPL and calreticulin mutations are negative. He has been followed up since 7 years. He received only hydroxyurea 1000-1500 mg per day for 6 years, hydroxyurea and anagrelide 1 mg per day combination for the last 1 year. At the fifth year of CMPD, he was diagnosed as squamous cell skin cancer on his ear. He treated only with cryotherapy and still in remission.
Patient 3: A female ET patient, diagnosed at the age of 54. JAK-2, MPL and calreticulin mutations are negative. She has never received cytoreductive treatment. She has been followed up since 7 years. At the fifth year of CMPD, she was diagnosed as licen planus and after local therapies, her lesion is still stable.
Patient 4: A male ET patient, diagnosed at the age of 54. JAK-2 mutation is positive. He has been followed up since 12 years. He received hydroxyurea for sometime after diagnosis but than swithced to anagrelide 2-4 mg per day since 10 years. At the eleventh year of CMPD, he was diagnosed as thyroid papillary carcinoma. After surgery,he was treated with radioactive iodine and now in remission. Patient 5: A female ET patient, diagnosed at the age of 43 after ischaemic cerebrovascular event. JAK-2, MPL and calreticulin mutations are negative. She has been followed up since 14 years. One year after diagnosis she received only hydroxyurea but during last 13 years she has switched to anagrelide 0.5-2 mg per day treatment. At the sixth year of CMPD, she was diagnosed as invaziveductal carcinoma and after mastectomy she received hormonotherapy. She is still in remission.
Conclusion
There are a number of evidences supporting that secondary malignancies are increased in CMPD patients compared to normal population. Although several factors, such as hydroxiurea, are blamed for that increase, the etiologic connection is yet unclear. Skin cancers have been the leading secondary malignancy (60%) in our study group. It is wise to keep in mind the reality of secondary cancers, during CMPD patients hematological management.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Anagrelide, Hydroxyurea, Myeloproliferative disorder, Second malignancy