Myeloproliferative disorders （MPD） are clonal haematopoietic stem cell disorders characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased numbers of mature and immature ce...Myeloproliferative disorders （MPD） are clonal haematopoietic stem cell disorders characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased numbers of mature and immature cells in the peripheral blood. MPDs are classified into five categories： polycythemia vera （PV）, essential thrombocythaemia （ET）, idiopathic myelofibrosis （IMF）, chronic myelogenous leukaemia （CML） and atypical MPD. The atypical MPD includes chronic myelomonocytic leukaemia, juvenile myelomonocytic leukaemia, chronic neutrophilic leukaemia, chronic eosinophilic leukaemia, chronic basophilic leukaemia, hypereosinophilic syndrome, systemic mastocytosis, atypical CML and unclassifiable cases.1-3 Apart from the Philadelphia chromosome and BCR-ABL fusion gene as the characteristic genetic abnormality of CML, the molecular pathogenesis of most MPDs such as PV, ET and IMF has not been described. Nonspecific cytogenetic abnormalities were found at diagnosis in numerous patients including deletions of the long arms of chromosome 20 and chromosome 13,展开更多
Therapeutic options for gastric variceal bleeding in the presence of extensive portal vein thrombosis associated with a myeloproliferative disorder are limited.We report a case of a young woman who presented with gast...Therapeutic options for gastric variceal bleeding in the presence of extensive portal vein thrombosis associated with a myeloproliferative disorder are limited.We report a case of a young woman who presented with gastric variceal bleeding secondary to extensive splanchnic venous thrombosis due to a Janus kinase 2 mutation associated myeloproliferative disorder that was managed effectively with partial splenic embolization.展开更多
Rationale:Chronic myeloid leukaemia is a myeloproliferative disorder due to clonal hyperproliferation of myeloid cells within the bone marrow.It can present both pro-and anti-thrombotic states.CML has different presen...Rationale:Chronic myeloid leukaemia is a myeloproliferative disorder due to clonal hyperproliferation of myeloid cells within the bone marrow.It can present both pro-and anti-thrombotic states.CML has different presentations within the gastrointestinal tract.Patient’s concern:A 40-year-old non-diabetic and non-hypertensive male complained of abdominal pain with nausea and emesis for 1 day.Besides,he had a history of abdominal distension and fever for 1 day.Diagnosis:Acute small bowel gangrene due to chronic myeloid leukaemia.Intervention:A limited resection of small intestine with ileostomy and mucus fistula.Outcome:After 3 months following surgery the patient underwent stoma closure.The patient was followed up for more than 3 years postoperatively.During the follow-up,the patient was asymptomatic without any recurrence of the disease.Lesson:Chronic myeloid leukaemia should be considered as one of the causes for small intestine gangrene when there is increased leukocyte count,splenomegaly without evidence of atherosclerotic occlusion or systemic emboli from the heart.展开更多
文摘Myeloproliferative disorders （MPD） are clonal haematopoietic stem cell disorders characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased numbers of mature and immature cells in the peripheral blood. MPDs are classified into five categories： polycythemia vera （PV）, essential thrombocythaemia （ET）, idiopathic myelofibrosis （IMF）, chronic myelogenous leukaemia （CML） and atypical MPD. The atypical MPD includes chronic myelomonocytic leukaemia, juvenile myelomonocytic leukaemia, chronic neutrophilic leukaemia, chronic eosinophilic leukaemia, chronic basophilic leukaemia, hypereosinophilic syndrome, systemic mastocytosis, atypical CML and unclassifiable cases.1-3 Apart from the Philadelphia chromosome and BCR-ABL fusion gene as the characteristic genetic abnormality of CML, the molecular pathogenesis of most MPDs such as PV, ET and IMF has not been described. Nonspecific cytogenetic abnormalities were found at diagnosis in numerous patients including deletions of the long arms of chromosome 20 and chromosome 13,
文摘Therapeutic options for gastric variceal bleeding in the presence of extensive portal vein thrombosis associated with a myeloproliferative disorder are limited.We report a case of a young woman who presented with gastric variceal bleeding secondary to extensive splanchnic venous thrombosis due to a Janus kinase 2 mutation associated myeloproliferative disorder that was managed effectively with partial splenic embolization.
文摘Rationale:Chronic myeloid leukaemia is a myeloproliferative disorder due to clonal hyperproliferation of myeloid cells within the bone marrow.It can present both pro-and anti-thrombotic states.CML has different presentations within the gastrointestinal tract.Patient’s concern:A 40-year-old non-diabetic and non-hypertensive male complained of abdominal pain with nausea and emesis for 1 day.Besides,he had a history of abdominal distension and fever for 1 day.Diagnosis:Acute small bowel gangrene due to chronic myeloid leukaemia.Intervention:A limited resection of small intestine with ileostomy and mucus fistula.Outcome:After 3 months following surgery the patient underwent stoma closure.The patient was followed up for more than 3 years postoperatively.During the follow-up,the patient was asymptomatic without any recurrence of the disease.Lesson:Chronic myeloid leukaemia should be considered as one of the causes for small intestine gangrene when there is increased leukocyte count,splenomegaly without evidence of atherosclerotic occlusion or systemic emboli from the heart.
