Essential thrombocythemia: A clonal disorder of hematopoietic stem cell

Singal, Usha; Prasad, Ananda S.; Halton, David M.; Bishop, Carter R.

doi:10.1002/ajh.2830140212

Cited by 22 publications

(5 citation statements)

References 10 publications

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“…Essential thrombocythemia (ET) is a rare clonal myeloproliferative disorder characterized by a sustained excessive proliferation of megakaryocytes in the bone marrow and an increased serum platelet level. ET has been associated with myeloproliferative syndromes like chronic myelogenous leukemia, polycythemia vera, myeloid metaplasia, and other secondary malignancies often described as a premalignant stage with a low risk of progression [1] , [2] . Currently, there are no clinical guidelines published regarding the perioperative management of ET patients.…”

Section: Introductionmentioning

confidence: 99%

Postoperative bleeding in essential thrombocytosis patients with colorectal cancer: Case report and literature review

Varela¹,

Nassr

Razak

et al. 2021

International Journal of Surgery Case Reports

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Introduction and importance Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by increased platelet count and a high risk of bleeding or thrombotic events due to platelet dysfunction. Patients with ET are treated according to their risk of complications with cytoreductive or anti-aggregant treatment. Neither guidelines for oncologic patients nor perioperative management of patients with ET have been determined. Case presentation A 41-year-old female patient with ET who had alternating constipation and diarrhea was referred after a screening colonoscopy diagnosing a locally advanced rectosigmoid junction colon adenocarcinoma with liver metastases. Systemic preoperative chemotherapy was indicated. The patient underwent laparoscopic low anterior resection plus volume-preserving right lobectomy of the liver. Postoperative bleeding of the internal iliac artery (IIA) associated with hematoma at the lower pelvic cavity was diagnosed and treated by interventional radiology; the patient was discharged without other complications 16 days after surgery. Clinical discussion ET has been related to the development of hematologic complications or second non-hematologic malignancies. A systematic review was conducted to seek guidance for the management of such patients in the perioperative period. Special perioperative care must be taken, and complications management should avoid further hemorrhages or cloth formation. Conclusion Under oncologic and hematological guidance, minimally invasive surgery and non-invasive management of complications are advised in the lack of published perioperative management guidelines of ET patients with colorectal cancer.

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Section: Introductionmentioning

confidence: 99%

Postoperative bleeding in essential thrombocytosis patients with colorectal cancer: Case report and literature review

Varela¹,

Nassr

Razak

et al. 2021

International Journal of Surgery Case Reports

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“…Essential thrombocythemia (ET) is a chronic myeloproliferative disorder characterized by the elevation of platelet count, which results from the clonal proliferation of a single neoplastic multipotent stem cell, affecting primarily megakaryocyte production [1,2]. ET was first described in 1934 [3], but firm guidelines for its diagnosis were established only in 1982 by the Polycythemia Vera Study Group [4].…”

Section: Introductionmentioning

confidence: 99%

Thrombotic complications in essential thrombocythemia with relatively low platelet counts

et al. 1997

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Essential thrombocythemia (ET) is often associated with thrombotic and hemorrhagic complications, mostly at platelet counts exceeding 600 x 10(9)/L. There are, however, a few reports of such complications in ET at considerably lower platelet levels and the therapeutic approach to affected patients with relatively low platelet counts is still controversial. In the present study, the first to directly address the issue of hemostatic manifestations at relatively low platelet counts, we have determined the lowest platelet counts associated with such manifestations in 56 consecutive ET patients. Clinical manifestations related to ET were recorded in 46 (82%) patients. Of the symptomatic patients, 32 (70%) had symptoms at platelet counts lower than 600 x 10(9)/L, 23 (50%) at counts lower than 500 x 10(9)/L, 10 (22%) at counts lower than 400 x 10(9)/L, and 6 patients (13%) at platelet counts as low as 300-350 x 10(9)/L. Severe complications occurred at platelet counts lower than 600 x 10(9)/L in 10 patients (22%), lower than 500 x 10(9)/L in 7 (15%), and at lower than 400 x 10(9)/L in 2 (4%). Thrombotic neurologic symptoms were the most common (31 patients, 67%), followed by peripheral vascular symptoms (17 patients, 37%); hemorrhagic complications were relatively rare (3 patients, 7%). In most cases, cessation or improvement of clinical manifestations was observed only after further reduction in platelet counts. In conclusion, thrombotic manifestations, including severe ones, are not uncommon in ET at relatively low platelet counts. We recommend that symptomatic patients with relatively low platelet counts be treated and the platelet counts further reduced well into the lower normal range.

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“…Essential thrombocythaemia (ET), a myeloproliferative disorder characterized by platelet counts persistently above 600 × 10 9 /l (Murphy et al 1986), has been recognized as a clonal disorder of the myeloid stem cell (Fialkow et al 1981;Singal et al 1983). About 2% of ET patients eventually progress to either acute myeloid (Chistolini et al 1990;Peterson et al 1994), lymphoid (O'Hea et al 1986;Murphy et al 1995;Krafts & Litz 1996), or megakariocytic leukaemia (Chistolini et al 1990;Patino-Sarcinelli et al 1996), especially following therapy with alkylating agents or radioactive phosphorus.…”

Section: Introductionmentioning

confidence: 99%