An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia
Mastocytosis is a myeloproliferative neoplasm characterized by clonal expansion of abnormal mast cells, ranging from the cutaneous forms of the disease to mast cell leukemia. In a significant proportion of patients, systemic mastocytosis (SM) coexists with another hematologic malignancy, termed syst...
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| Language: | English |
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Wiley
2014-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2014/526129 |
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| author | Theodoros Iliakis Niki Rougkala Panagiotis T. Diamantopoulos Vasiliki Papadopoulou Fani Kalala Konstantinos Zervakis Nefeli Giannakopoulou Polixeni Chatzinikolaou Georgia Levidou Eleftheria Lakiotaki Penelope Korkolopoulou Efstratios Patsouris Eleni Variami Nora-Athina Viniou |
| author_facet | Theodoros Iliakis Niki Rougkala Panagiotis T. Diamantopoulos Vasiliki Papadopoulou Fani Kalala Konstantinos Zervakis Nefeli Giannakopoulou Polixeni Chatzinikolaou Georgia Levidou Eleftheria Lakiotaki Penelope Korkolopoulou Efstratios Patsouris Eleni Variami Nora-Athina Viniou |
| author_sort | Theodoros Iliakis |
| collection | DOAJ |
| description | Mastocytosis is a myeloproliferative neoplasm characterized by clonal expansion of abnormal mast cells, ranging from the cutaneous forms of the disease to mast cell leukemia. In a significant proportion of patients, systemic mastocytosis (SM) coexists with another hematologic malignancy, termed systemic mastocytosis with an associated hematologic nonmast cell lineage disorder (SM-AHNMD). Despite the pronounced predominance of concomitant myeloid neoplasms, the much more unusual coexistence of lymphoproliferative diseases has also been reported. Imatinib mesylate (IM) has a role in the treatment of SM in the absence of the KITD816V mutation. In the setting of SM-AHNMD, eradicating the nonmast cell malignant clone greatly affects prognosis. We report a case of an adult patient with SM associated with B-lineage acute lymphoblastic leukemia (B-ALL). Three cases of concurrent adult ALL and mastocytosis have been reported in the literature, one concerning SM and two concerning cutaneous mastocytosis (CM), as well as six cases of concomitant CM and ALL in children. |
| format | Article |
| id | doaj-art-7a22de0bf7de461e984066d1a35bd387 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-7a22de0bf7de461e984066d1a35bd3872025-02-03T01:26:26ZengWileyCase Reports in Medicine1687-96271687-96352014-01-01201410.1155/2014/526129526129An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic LeukemiaTheodoros Iliakis0Niki Rougkala1Panagiotis T. Diamantopoulos2Vasiliki Papadopoulou3Fani Kalala4Konstantinos Zervakis5Nefeli Giannakopoulou6Polixeni Chatzinikolaou7Georgia Levidou8Eleftheria Lakiotaki9Penelope Korkolopoulou10Efstratios Patsouris11Eleni Variami12Nora-Athina Viniou13Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceDepartment of Pathology, National and Kapodistrian University of Athens, Athens, GreeceDepartment of Pathology, National and Kapodistrian University of Athens, Athens, GreeceDepartment of Pathology, National and Kapodistrian University of Athens, Athens, GreeceDepartment of Pathology, National and Kapodistrian University of Athens, Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceHematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street, 11527 Athens, GreeceMastocytosis is a myeloproliferative neoplasm characterized by clonal expansion of abnormal mast cells, ranging from the cutaneous forms of the disease to mast cell leukemia. In a significant proportion of patients, systemic mastocytosis (SM) coexists with another hematologic malignancy, termed systemic mastocytosis with an associated hematologic nonmast cell lineage disorder (SM-AHNMD). Despite the pronounced predominance of concomitant myeloid neoplasms, the much more unusual coexistence of lymphoproliferative diseases has also been reported. Imatinib mesylate (IM) has a role in the treatment of SM in the absence of the KITD816V mutation. In the setting of SM-AHNMD, eradicating the nonmast cell malignant clone greatly affects prognosis. We report a case of an adult patient with SM associated with B-lineage acute lymphoblastic leukemia (B-ALL). Three cases of concurrent adult ALL and mastocytosis have been reported in the literature, one concerning SM and two concerning cutaneous mastocytosis (CM), as well as six cases of concomitant CM and ALL in children.http://dx.doi.org/10.1155/2014/526129 |
| spellingShingle | Theodoros Iliakis Niki Rougkala Panagiotis T. Diamantopoulos Vasiliki Papadopoulou Fani Kalala Konstantinos Zervakis Nefeli Giannakopoulou Polixeni Chatzinikolaou Georgia Levidou Eleftheria Lakiotaki Penelope Korkolopoulou Efstratios Patsouris Eleni Variami Nora-Athina Viniou An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia Case Reports in Medicine |
| title | An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia |
| title_full | An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia |
| title_fullStr | An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia |
| title_full_unstemmed | An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia |
| title_short | An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia |
| title_sort | adult patient with systemic mastocytosis and b acute lymphoblastic leukemia |
| url | http://dx.doi.org/10.1155/2014/526129 |
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