Eltrombopag Use in Patients With Chronic Myelomonocytic Leukemia (CMML): A Cautionary Tale

Ramadan, Hanadi; Duong, Vu H.; Ali, Najla Al; Padron, Eric; Zhang, Ling; Lancet, Jeffrey E.; List, Alan F.; Komrokji, Rami

doi:10.1016/j.clml.2016.02.009

Cited by 30 publications

(17 citation statements)

References 7 publications

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“…In lower-risk CMML, treatment of anemia can rely on Erythropoiesis Stimulating Agents (ESAs) with MDS regimens [65], whereas thrombopoietin agonists seem more difficult to use, and still require prospective evaluation [66]. In patients with proliferative features, hydroxyurea remains a standard of care [2].…”

Section: Treatmentmentioning

confidence: 99%

CMML: Clinical and molecular aspects

et al. 2017

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Section: Treatmentmentioning

confidence: 99%

CMML: Clinical and molecular aspects

et al. 2017

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“…Early studies with the thrombopoietin analogue eltrombopag for thrombocytopenia in CMML have also shown an increased incidence of “proliferative” features like leukocytosis, monocytosis and circulating blasts, warranting close scrutiny. [33] All patients with splenic rupture presented with spleen related pain and intraabdominal hemorrhage and in these patients an emergent splenectomy was a lifesaving procedure. Similar to myelofibrosis, elective splenectomy was also very effective in alleviating spleen related mechanical and constitutional symptoms (85% vs 67%).…”

Section: Discussionmentioning

confidence: 99%

Splenectomy in patients with chronic myelomonocytic leukemia: Indications, histopathological findings and clinical outcomes in a single institutional series of thirty‐nine patients

et al. 2018

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In a 28-year period, 39 (7%) patients with chronic myelomonocytic leukemia (CMML) (median age 66 years, 64% male) underwent a splenectomy at our institution. Primary indications for splenectomy were refractory thrombocytopenia (36%), progressive spleen related symptoms (33%), emergent splenectomy for splenic rupture (21%), refractory anemia (8%), and prior to allogeneic stem cell transplant (3%). Eleven (28%) patients had anemia at the time of splenectomy, of which 3 (27%) were autoimmune. The median time to splenectomy from CMML diagnosis was 6 months (0-40); perioperative morbidity and mortality rates were 43% and 13%, while the median postsplenectomy survival was 25 months (11-38). Durable remission in spleen related symptoms, thrombocytopenia, complications from splenic rupture, and anemia were achieved in 85%, 50%, 62%, and 21% of patients, respectively. Perioperative morbidity (n = 30) included infections/sepsis in 6 (20%), intraabdominal bleeding in 4 (13%), venous thromboembolism (VTE) in 3 (10%), and acute lung injury in 2 (7%) patients. The median duration of hospital stay was 6 days (1-25), with 5 deaths occurring secondary to respiratory failure (n = 2), multiorgan dysfunction (n = 2) and hemorrhagic shock (n = 1). There was no difference in overall survival between CMML patients that underwent splenectomy, in comparison to those that did not. Unlike in myelofibrosis, portal hypertension was not an indication for splenectomy and no patients developed post-splenectomy thrombocytosis. In conclusion, apart from being a lifesaving emergent modality in the event of splenic rupture, splenectomy has an important palliative role in patients with CMML, with significant and durable improvements in spleen related symptoms and refractory cytopenias.

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“…In patients with CMML, thrombocytopenia commonly occurs and is due to dysplasia of megakaryocytes [ 1 ]. However, although eltrombopag can be used to treat low blood platelet counts, it has only been used in clinical trials, due to safety concerns, as some patients with MPN-CMML on eltrombopag who transformed to AML [ 10 ]. For patients who are clinically stable and at low risk, clinical observation with supportive treatment is recommended, as a small percentage of patients with CMML demonstrate no disease progression for several years [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of Synchronous Bone Marrow Chronic Myelomonocytic Leukemia (CMML) and Nodal Marginal Zone Lymphoma (NMZL)

et al. 2018

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Patient: Male, 67Final Diagnosis: Nodal marginal zone lymphoma and chronic myelomonocyticSymptoms: Cervical lymphadenopathy and leukocytosisMedication: —Clinical Procedure: —Specialty: OncologyObjective:Rare co-existance of disease or pathologyBackground:Leukemias and lymphomas can arise from myeloid or lymphoid stem cells. Combined myeloid leukemia and non-Hodgkin’s lymphoma (NHL), either synchronous or metachronous, rarely occur in the same patient. This report is of a 67-year-old man with a synchronous diagnosis of both bone marrow chronic myelomonocytic leukemia (CMML) and nodal marginal zone lymphoma (NMZL), which a peripheral low-grade B-cell NHL.Case Report:A 67-year-old Caucasian man, who was a long-term cigarette smoker, presented with a five-year history of leukocytosis and cervical lymphadenopathy. He had no symptoms of night sweats, fever, or weight loss. Review of his medical records showed a progressively increasing leukocytosis with a peak of 58×109/L. Computed tomography (CT) imaging of the chest and abdomen showed lymphadenopathy, including enlarged cervical, axillary, mediastinal, and retroperitoneal lymph nodes. Bone marrow biopsy and histology showed CMML. Lymph node biopsy and histology showed NMZL. The patient was treated for NMZL with weekly intravenous rituximab infusions. Although his CMML was stable, the patient requested an evaluation for treatment with hematopoietic allogeneic stem cell transplantation (ASCT). At the time of this report, the patient remains asymptomatic.Conclusions:The synchronous occurrence of bone marrow CMML and NMZL in a single patient is rare and may be attributed to a genetic mutation common to both. There are no current treatment guidelines for this group of patients, and treatment strategies should be individualized to provide an optimum outcome or symptomatic improvement.

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Eltrombopag Use in Patients With Chronic Myelomonocytic Leukemia (CMML): A Cautionary Tale

Cited by 30 publications

References 7 publications

CMML: Clinical and molecular aspects

CMML: Clinical and molecular aspects

Splenectomy in patients with chronic myelomonocytic leukemia: Indications, histopathological findings and clinical outcomes in a single institutional series of thirty‐nine patients

A Case of Synchronous Bone Marrow Chronic Myelomonocytic Leukemia (CMML) and Nodal Marginal Zone Lymphoma (NMZL)

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