The Syndrome of Idiopathic Myelofibrosis

Varki, Ajit; Lottenberg, Richard; Griffith, Rogers C.; Reinhard, Edward H.

doi:10.1097/00005792-198311000-00002

Cited by 108 publications

(8 citation statements)

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“…Data on the duration of follow-up were provided in nine studies [1][2][3][4]6,8,10,13,15]; on late surgical morbidity, in four [8,9,13,15], and on the median survival, in three [6,10,13]. Defined criteria were used to evaluate the benefit of splenectomy in seven studies [3,8,[10][11][12][13]151. None of the latter reported whether this benefit was transient or sustained and only two [3,6] specified the outcome of splenectomy in each patient.…”

Section: Literature Reviewmentioning

confidence: 99%

“…The rate of immediate operative complications (atelectasis, pneumonia, hemorrhage, thromboembolism, and subphrenic infections) was 25% (3) to 83% (4) with an average of 45.4% (95% CI: 39.6-51.1%). Data on the late surgical morbidity (thromboembolism and massive enlargement of the liver) were reported in four studies, as from 12 (8) to 23% (13) with an average of 16.6% (95% CI: 9.9-22.5%) ( Table V).…”

Section: Morbidity and Mortality After Splenectomy In Patients With Ammmentioning

confidence: 99%

“…However, the indications for surgery are subject to controversy. This ambiguity is due to the unpredictable prognosis of individual patients with AMM [11,13] and to the observation that the manifestations considered as indications for splenectomy in AMM are also associated with an increased operative mortality. Consequently, patients with symptoms that could possibly be relieved by splenectomy are poor surgical risks, while asymptomatic, good risk patients are not usually considered for surgical treatment [ …”

Section: Introductionmentioning

confidence: 99%

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The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia

J¹,

Gilon²,

Penchas³

1990

American J Hematol

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The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or transfusion requirements in patients with AMM. We employed formal decision analysis to determine the relative value of medical and surgical treatment of advanced AMM. Results of data synthesis showed that splenectomy in AMM is associated with an operative mortality of 13.4% (95% confidence intervals (CI): 9.5-17.2%), an early morbidity of 45.3% (CI: 39.6-51.1%), and a late morbidity of 16.3% (CI: 9.9-22.5%). Almost all patients with portal hypertension and painful splenomegaly, but only about half of those with thrombopenia and anemia were reported to have experienced relief in their symptoms or signs after splenectomy. We found no evidence that splenectomy affects survival in AMM. We concluded that splenectomy in advanced AMM is a palliative procedure that carries a substantial risk. It may be considered for symptomatic patients after they have been informed about the operative mortality, morbidity, and chances of palliation. Decisions about treatment of advanced AMM should be guided predominantly by the patient's preferences.

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Section: Literature Reviewmentioning

confidence: 99%

Section: Morbidity and Mortality After Splenectomy In Patients With Ammmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia

J¹,

Gilon²,

Penchas³

1990

American J Hematol

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“…Prolonged bleeding time [10], thrombocyto penia [1,2] and less often thrombocytosis [11] have all been reported. Abnormalities of platelet aggregation are common, particu larly lack of platelet reponsiveness to epi nephrine, which has been associated partly with decreased alpha-adrenergic receptor …”

Section: Discussionmentioning

confidence: 99%

“…The operation has been associated with substantial morbidity and mortality due to enhanced thrombotic as well as hemorrhagic tendencies [2,3], especially in patients with inapparent disseminated intravascular coag ulation [1]. In order to better define the role of the spleen in impaired hemostasis, we studied retrospectively the influence of splenectomy on hemostatic tests in 10 pa tients with AMM.…”

Section: Introductionmentioning

confidence: 99%

Influence of Splenectomy on Hemostasis in Agnogenic Myeloid Metaplasia

Brenner¹,

Tavori²,

Tatarsky³

1987

Pathophysiol Haemos Thromb

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The effect of splenectomy on hemostatic tests was studied in 10 patients with agnogenic myeloid metaplasia. Prolonged partial thromboplastin time and abnormal prothrombin consumption were found before splenectomy and were normalized postoperatively in the majority of patients. Prolonged bleeding time and prothrombin time were normalized in some patients. While platelet counts and fibrinogen levels rose significantly following splenectomy, platelet aggregation remained impaired. Thrombocytosis and increased fibrinogen level after splenectomy in patients with agnogenic myeloid metaplasia can enhance a thromboembolic tendency.

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Extramedullary hematopoiesis. A cause of severe generalized lymphadenopathy in agnogenic myeloid metaplasia

Williams¹

1985

Archives of Internal Medicine

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Although microscopic evidence of extramedullary hematopoiesis in lymph nodes is a frequent finding in autopsy studies of patients with agnogenic myeloid metaplasia, clinically significant lymphadenopathy is a rare occurrence. In this article we describe an unusual case of severe generalized lymphadenopathy and lymphedema secondary to extramedullary hematopoiesis in multiple lymph nodes. With the use of low-dose radiotherapy, both the lymphadenopathy and lymphedema resolved.

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The Syndrome of Idiopathic Myelofibrosis

Cited by 108 publications

References 0 publications

The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia

The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia

Influence of Splenectomy on Hemostasis in Agnogenic Myeloid Metaplasia

Extramedullary hematopoiesis. A cause of severe generalized lymphadenopathy in agnogenic myeloid metaplasia

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