Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera
Polycythaemia vera (PV) is one of the chronic myeloproliferative neoplasms, which are collectively characterised by clonal proliferation of myeloid cells with variable morphologic maturity and haematopoietic efficiency. PV is distinguished clinically from other myeloproliferative neoplasms by the p...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
European Medical Journal
2021-07-01
|
| Series: | European Medical Journal Hematology |
| Online Access: | https://www.emjreviews.com/hematology/article/recurrent-episodes-of-angioedema-as-presenting-feature-of-jak2-positive-myeloproliferative-disorder-consistent-with-polycythaemia-vera-j060121/ |
| _version_ | 1797356566802857984 |
|---|---|
| author | Laith Al-Azrai Ala’a Abdullah Bani Mustafa Adi Yousef al-Wahadneh |
| author_facet | Laith Al-Azrai Ala’a Abdullah Bani Mustafa Adi Yousef al-Wahadneh |
| author_sort | Laith Al-Azrai |
| collection | DOAJ |
| description |
Polycythaemia vera (PV) is one of the chronic myeloproliferative neoplasms, which are collectively characterised by clonal proliferation of myeloid cells with variable morphologic maturity and haematopoietic efficiency. PV is distinguished clinically from other myeloproliferative neoplasms by the presence of an elevated red blood cell mass because of uncontrolled red blood cell production. This is accompanied by increased white blood cells and platelet production, which is because of abnormal clone of haematopoietic stem cells with increased sensitivity to the different growth factors for maturation. PV can present with variable symptoms because of impaired oxygen delivery caused by slugging of blood, such as headaches, dizziness, vertigo, tinnitus, visual disturbances, and angina pectoris. Some patients present with bleeding complications (1%); another 1% of patients present with thrombotic complications. It is uncommon for patients with myeloproliferative disorders to present with features of angioedema. There are many reported cases in the literature that describe the relationship between the occurrence of angioedema and lymphoproliferative diseases; however, there are no reported cases describing instances of myeloproliferative neoplasm with angioedema. In this article, the authors have studied a case of a 53-year-old male who presented with recurrent episodes of features that are suggestive of angioedema. He was diagnosed with JAK2-positive myeloproliferative disorder consistent with PV; this is the first reported case in Jordan. |
| first_indexed | 2024-03-08T14:29:31Z |
| format | Article |
| id | doaj.art-de59b9fbd7b749178cea8c80f6088ee7 |
| institution | Directory Open Access Journal |
| issn | 2053-6631 |
| language | English |
| last_indexed | 2024-03-08T14:29:31Z |
| publishDate | 2021-07-01 |
| publisher | European Medical Journal |
| record_format | Article |
| series | European Medical Journal Hematology |
| spelling | doaj.art-de59b9fbd7b749178cea8c80f6088ee72024-01-12T15:17:12ZengEuropean Medical JournalEuropean Medical Journal Hematology2053-66312021-07-0110510910.33590/emjhematol/20-00274Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia VeraLaith Al-Azrai0Ala’a Abdullah Bani Mustafa1Adi Yousef al-Wahadneh2Department of Internal Medicine, Queen Alia Military Hospital, Amman, JordanDepartment of Internal Medicine, King Hussein Medical Hospital, Amman, Jordan; Department of Internal Medicine, Ministry of Health, Dr Jameel al Totanji Hospital, Amman, JordanDepartment of Internal Medicine, Ministry of Health, Dr Jameel al Totanji Hospital, Amman, Jordan Polycythaemia vera (PV) is one of the chronic myeloproliferative neoplasms, which are collectively characterised by clonal proliferation of myeloid cells with variable morphologic maturity and haematopoietic efficiency. PV is distinguished clinically from other myeloproliferative neoplasms by the presence of an elevated red blood cell mass because of uncontrolled red blood cell production. This is accompanied by increased white blood cells and platelet production, which is because of abnormal clone of haematopoietic stem cells with increased sensitivity to the different growth factors for maturation. PV can present with variable symptoms because of impaired oxygen delivery caused by slugging of blood, such as headaches, dizziness, vertigo, tinnitus, visual disturbances, and angina pectoris. Some patients present with bleeding complications (1%); another 1% of patients present with thrombotic complications. It is uncommon for patients with myeloproliferative disorders to present with features of angioedema. There are many reported cases in the literature that describe the relationship between the occurrence of angioedema and lymphoproliferative diseases; however, there are no reported cases describing instances of myeloproliferative neoplasm with angioedema. In this article, the authors have studied a case of a 53-year-old male who presented with recurrent episodes of features that are suggestive of angioedema. He was diagnosed with JAK2-positive myeloproliferative disorder consistent with PV; this is the first reported case in Jordan.https://www.emjreviews.com/hematology/article/recurrent-episodes-of-angioedema-as-presenting-feature-of-jak2-positive-myeloproliferative-disorder-consistent-with-polycythaemia-vera-j060121/ |
| spellingShingle | Laith Al-Azrai Ala’a Abdullah Bani Mustafa Adi Yousef al-Wahadneh Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera European Medical Journal Hematology |
| title | Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera |
| title_full | Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera |
| title_fullStr | Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera |
| title_full_unstemmed | Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera |
| title_short | Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera |
| title_sort | recurrent episodes of angioedema as presenting feature of jak2 positive myeloproliferative disorder consistent with polycythaemia vera |
| url | https://www.emjreviews.com/hematology/article/recurrent-episodes-of-angioedema-as-presenting-feature-of-jak2-positive-myeloproliferative-disorder-consistent-with-polycythaemia-vera-j060121/ |
| work_keys_str_mv | AT laithalazrai recurrentepisodesofangioedemaaspresentingfeatureofjak2positivemyeloproliferativedisorderconsistentwithpolycythaemiavera AT alaaabdullahbanimustafa recurrentepisodesofangioedemaaspresentingfeatureofjak2positivemyeloproliferativedisorderconsistentwithpolycythaemiavera AT adiyousefalwahadneh recurrentepisodesofangioedemaaspresentingfeatureofjak2positivemyeloproliferativedisorderconsistentwithpolycythaemiavera |