Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT
Aim. To show the peculiarity of clinical manifestation,diagnostic and therapeutic approach of misdiagnosed chronic myeloproliferative disorder (CMPD), which has debuted by hepatic thrombosis. Patients and methods. We present the results of clinical description, observation data and treatment resul...
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PAGEPress Publications
2014-08-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | https://mjhid.org/index.php/mjhid/article/view/1712 |
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| author | Maria Dmitrievna Sanatko |
| author_facet | Maria Dmitrievna Sanatko |
| author_sort | Maria Dmitrievna Sanatko |
| collection | DOAJ |
| description | Aim. To show the peculiarity of clinical manifestation,diagnostic and therapeutic approach of misdiagnosed chronic myeloproliferative disorder (CMPD), which has
debuted by hepatic thrombosis.
Patients and methods. We present the results of clinical description, observation data and treatment results in a patient with Budd-Chiari syndrome as manifestation of latent primary (idiopathic) myelofibrosis. Standard clinical and laboratory analyses, cytomorphological examination of bone marrow, ultrasound Doppler investigation of portal vessels have been used.
Results. We observed a patient with primary myelofibrosis (PM), which started with Budd-Chiari syndrome. The hematological disorder was not recognized properly due to the latent course of the disease and thrombosis at background. The appropriate treatment of PM was not prescribed. The diagnosis of PM was verified, and moreover we have found hereditary thrombophilic factors. Only after verification of the diagnosis anticoagulant and cytostatic therapy was started. The result was positive: we observed a resolution of clinical symptoms, normalization of blood counts and partial restore of blood flow in hepatic veins. But discontinuation of the antithrombotic therapy provoked rethrombosis of the same localization. The resumption of anticoagulant therapy led to positive clinical effect again.
Conclusion. The described clinical case shows that the Budd-Chiari syndrome development can be the consequence of the existing myeloproliferative disorder.
Hereditary thrombophilia factors and very important in maintenance of the thrombotic state in CMPD patients. Therefore special attention should be paid not only to cytoreductive therapy for normalization of blood counts, but also to perpetual intake of anticoagulants particularly in case of thrombotic complication of such localization. |
| format | Article |
| id | doaj-art-b8c8e15fd1c74b82a00ccfea28ecec5e |
| institution | OA Journals |
| issn | 2035-3006 |
| language | English |
| publishDate | 2014-08-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-b8c8e15fd1c74b82a00ccfea28ecec5e2025-08-20T01:47:47ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062014-08-011Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENTMaria Dmitrievna Sanatko0Hematology Research CenterAim. To show the peculiarity of clinical manifestation,diagnostic and therapeutic approach of misdiagnosed chronic myeloproliferative disorder (CMPD), which has debuted by hepatic thrombosis. Patients and methods. We present the results of clinical description, observation data and treatment results in a patient with Budd-Chiari syndrome as manifestation of latent primary (idiopathic) myelofibrosis. Standard clinical and laboratory analyses, cytomorphological examination of bone marrow, ultrasound Doppler investigation of portal vessels have been used. Results. We observed a patient with primary myelofibrosis (PM), which started with Budd-Chiari syndrome. The hematological disorder was not recognized properly due to the latent course of the disease and thrombosis at background. The appropriate treatment of PM was not prescribed. The diagnosis of PM was verified, and moreover we have found hereditary thrombophilic factors. Only after verification of the diagnosis anticoagulant and cytostatic therapy was started. The result was positive: we observed a resolution of clinical symptoms, normalization of blood counts and partial restore of blood flow in hepatic veins. But discontinuation of the antithrombotic therapy provoked rethrombosis of the same localization. The resumption of anticoagulant therapy led to positive clinical effect again. Conclusion. The described clinical case shows that the Budd-Chiari syndrome development can be the consequence of the existing myeloproliferative disorder. Hereditary thrombophilia factors and very important in maintenance of the thrombotic state in CMPD patients. Therefore special attention should be paid not only to cytoreductive therapy for normalization of blood counts, but also to perpetual intake of anticoagulants particularly in case of thrombotic complication of such localization.https://mjhid.org/index.php/mjhid/article/view/1712chronic myeloproliferative disordersBudd-Chiari syndromeportal hypertensionthrombophilia. |
| spellingShingle | Maria Dmitrievna Sanatko Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT Mediterranean Journal of Hematology and Infectious Diseases chronic myeloproliferative disorders Budd-Chiari syndrome portal hypertension thrombophilia. |
| title | Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT |
| title_full | Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT |
| title_fullStr | Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT |
| title_full_unstemmed | Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT |
| title_short | Ph-NEGATIVE CHRONIC MYELOPROLIFERATIVE DISORDER (IDIOPATHIC MYELOFIBROSIS) – AS ONE OF THE REASONS OF THE BUDD-CHIARI SYNDROME. CLINICAL CASE OF A YOUNG PATIENT |
| title_sort | ph negative chronic myeloproliferative disorder idiopathic myelofibrosis as one of the reasons of the budd chiari syndrome clinical case of a young patient |
| topic | chronic myeloproliferative disorders Budd-Chiari syndrome portal hypertension thrombophilia. |
| url | https://mjhid.org/index.php/mjhid/article/view/1712 |
| work_keys_str_mv | AT mariadmitrievnasanatko phnegativechronicmyeloproliferativedisorderidiopathicmyelofibrosisasoneofthereasonsofthebuddchiarisyndromeclinicalcaseofayoungpatient |