Myelofibrosis, a myeloproliferative neoplasm characterized by bone marrow fibrosis, cytopenias, and extramedullary hematopoiesis, is a very challenging disease to treat, with patients experiencing progressive symptoms that can negatively impact their quality of life. In this excerpt of the transcript from their nursing continuing professional development (NCPD)-approved activity, Nursing Management of Myelofibrosis: Controlling Symptoms and Optimizing Patient Quality of Life, Alice Lynn, ANP, and Dawn Urbanovsky, RN, BSN, OCN®, both of The University of Texas MD Anderson Cancer Center, delve into a case study of a patient with myelofibrosis; in doing so, they share their perspectives on assessing disease characteristics and selecting treatments for patients with this debilitating disease.
Dawn Urbanovsky, RN, BSN, OCN®: Let's discuss when to treat myelofibrosis. It is important to determine when to start treatment. Research has shown that early treatment does not necessarily prolong survival. For patients who fall under the Dynamic International Prognostic Scoring System (DIPSS) risk score of low, observation is recommended, along with supportive therapy such as growth factors. It's important to evaluate if the patient's symptoms are really attributable to the disease or possibly to some other cause.
Alice Lynn, ANP: This brings us to our first case study, which will focus on assessing tumor burden. Ms. AJ is a postmenopausal 55-year-old woman with a past medical history of chronic obstructive pulmonary disease (COPD), hypertension, and diabetes. She presents with the following symptoms: increasing fatigue over the last six months, night sweats, decreased appetite, shortness of breath when walking up the stairs to her apartment, and abdominal discomfort and bloating. Dawn, what do you experience in caring for patients with myelofibrosis? How would you further direct your assessment of this patient with her symptoms?
Ms. Urbanovsky: With this patient, I think it would be important to ask more directed questions and ask her to describe the severity of her fatigue level and how it directly impacts her daily activities. I would ask her about the frequency and severity of her night sweats, and I would inquire more about her weight loss. For example, I'd want to know how many pounds she has lost, and over what period of time did the loss take place. I would want to know the extent of her abdominal discomfort and bloating.
Ms. Lynn: Upon further assessment, she reports that she has started taking one- to two-hour naps daily, and she has been having drenching night sweats. She reports a decrease in her appetite and also reports bloating, especially after a meal. A physical exam reveals multiple bruises on her arms, abdominal distention, a splenomegaly with the spleen measuring 10 cm below the costal margin on deep inspiration, hepatomegaly with the liver measuring 4 cm below the costal margin on deep inspiration, and 1+ bilateral ankle edema. Her laboratory results show a slightly elevated white blood cell count of 14,000; anemia with a hemoglobin of 10.2; and a normal platelet count of 250,000. She has no blasts present in the peripheral blood.
Chemistry tests show elevated levels of uric acid, lactate dehydrogenase (LDH), alkaline phosphatase, and bilirubin. Her bone marrow biopsy reveals megakaryocytic proliferation, which results in the overproduction of platelets. The megakaryocytic proliferation and atypia with grade 2 reticulin myelofibrosis are characteristic features of early phase myelofibrosis. There is a JAK2 mutation, and she is BCR-ABL–negative. She does not meet the criteria for other myeloproliferative neoplasms, myelodysplastic syndromes, or other myeloid neoplasms. Her cytogenetics are normal and do not show any evidence of deletion 13, deletion 20, or trisomy 8 or 9.
Ms. Urbanovsky: The patient's DIPSS score is Intermediate-1 based on her constitutional symptoms, the abnormal lab results, and the bone marrow biopsy results. With a DIPSS score of Intermediate-1 and constitutional symptoms, guidelines recommend treatment with ruxolitinib. With a platelet count of 250,000, the patient would be able to start ruxolitinib at 20 mg twice a day, and she should then be re-evaluated in four weeks to assess her tolerance of the therapy. If the platelet count is stable and the patient is tolerating the drug, then the dose may be increased to 25 mg twice daily. However, if the platelet count should drop below 50,000, the prescribing guidelines recommend holding the ruxolitinib and checking the platelet counts weekly. Once the platelet count has recovered to over 50,000, you can then resume the ruxolitinib at 5 mg twice a day and titrate the dosage up every two weeks as tolerated.
Ms. Lynn: Educating the patient on her disease symptoms and treatment can improve the communication between the patient and the care team, thus improving the patient's overall outcome.
About Ms. Lynn and Ms. Urbanovsky
Alice Lynn, ANP, is an advanced nurse practitioner at The University of Texas MD Anderson Cancer Center, where she specializes in the care and treatment of patients with cancer. She is also a member of the American Academy of Nurse Practitioners and the Oncology Nursing Society. Ms. Lynn has authored or coauthored several peer-reviewed publications focused on improving outcomes in patients with hematologic malignancies.
Dawn Urbanovsky, RN, BSN, OCN®, is an oncology nurse at The University of Texas MD Anderson Cancer Center, where she specializes in the treatment of patients with myeloproliferative neoplasms. She is also a member of the Oncology Nursing Society. Ms. Urbanovsky has served as a research nurse in several clinical trials focused on developing novel therapeutics to improve outcomes in patients with cancer.
Edits have been made to this excerpt for the sake of clarity and brevity. Any views expressed above are the speakers' own and do not necessarily reflect those of i3 Health.