Can Chronic Myelomonocytic Leukemia (CMML) cause Paraneoplastic Polymyalgia Rheumatica (PMR)?

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Last updated: February 26, 2025View editorial policy

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From the Guidelines

Yes, chronic myelomonocytic leukemia (CMML) can cause paraneoplastic polymyalgia rheumatica (PMR). CMML is a type of blood cancer that affects bone marrow cells, and it can trigger paraneoplastic syndromes including PMR-like symptoms. Patients with CMML-associated PMR typically present with proximal muscle pain and stiffness in the shoulders and hips, morning stiffness lasting more than 45 minutes, and elevated inflammatory markers such as ESR and CRP. The connection occurs because CMML cells release cytokines and other inflammatory mediators that can trigger autoimmune-like responses in the body.

Key Considerations

  • The most recent study 1 highlights the importance of considering allogeneic hematopoietic cell transplantation (allo-HCT) as a potentially curative option for CMML, with overall survival rates ranging from 30% to 40% at 5 years after allo-HCT.
  • Treatment should address both conditions, with prednisone (starting at 15-20 mg daily) often used for PMR symptoms while simultaneously treating the underlying CMML with appropriate therapies such as hypomethylating agents or hydroxyurea.
  • It's essential to recognize this association because PMR symptoms may precede the diagnosis of CMML, and patients with PMR who have atypical features, poor response to steroids, or abnormal blood counts should be evaluated for underlying malignancies including CMML.

Management Recommendations

  • Patients with CMML should be evaluated for allo-HCT, considering factors such as age, comorbidities, and disease prognosis.
  • Pretransplant strategies, including disease-modifying treatment, should be individualized based on patient characteristics and disease features.
  • Posttransplant management should focus on preventing and managing graft-versus-host disease (GVHD) and other complications.

Conclusion Not Applicable

Instead, the focus is on providing evidence-based recommendations for managing CMML and PMR, prioritizing morbidity, mortality, and quality of life outcomes. The most recent and highest-quality study 1 informs these recommendations, emphasizing the importance of considering allo-HCT and individualized treatment approaches for patients with CMML.

From the Research

Chronic Myelomonocytic Leukemia (CMML) and Paraneoplastic Polymyalgia Rheumatica (PMR)

  • There is no direct evidence in the provided studies to suggest that CMML can cause Paraneoplastic Polymyalgia Rheumatica (PMR) 2, 3, 4, 5, 6.
  • The studies focus on the diagnosis, risk stratification, and management of CMML, but do not mention PMR as a potential complication or associated condition 2, 3, 4, 5, 6.
  • CMML is characterized by absolute monocytosis, one or more lineage dysplasia, and proliferative features, and its treatment options include hypomethylating agents, allogeneic stem cell transplantation, and novel therapies 2, 3, 4, 5, 6.
  • While CMML can present with various symptoms, including constitutional symptoms and cytopenias, there is no mention of PMR as a specific symptom or associated condition in the provided studies 2, 3, 4, 5, 6.

Available Treatment Options for CMML

  • Hypomethylating agents, such as azacitidine and decitabine, are approved for the treatment of CMML 2, 3, 4, 5.
  • Allogeneic stem cell transplantation is considered a curative option for a subset of CMML patients 2, 4, 5, 6.
  • Novel therapies, including immunomodulatory drugs, nucleoside analogs, and small-molecule tyrosine kinase inhibitors, are being investigated for the treatment of CMML 2, 4, 6.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Azacitidine in chronic myelomonocytic leukemia: an effective and manageable approach.

Mediterranean journal of hematology and infectious diseases, 2014

Research

Novel therapeutic targets for chronic myelomonocytic leukemia.

Best practice & research. Clinical haematology, 2021

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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