A 67-Year-Old Man with Primary Myelofibrosis - Episode 1

Overview: A 67-Year-Old Man with Primary Myelofibrosis

Raajit Rampal, MD, PhD, reviews the case of a 67-year-old man with primary myelofibrosis and shares his initial impressions.

Case: A 67-Year-Old Man With Myelofibrosis

Initial presentation

RF is a 67 y/o man who visits his primary care physician for his yearly checkup. He reports having fatigue and bone pain for the last few months. More recently, he’s been complaining of abdominal discomfort which he thinks is probably related to the big Thanksgiving dinner party, he and his wife hosted.

He enjoys spending time working on antique cars in his garage, but his wife says he becomes tired after just an hour.

RF has not noticed any changes in his eating habits, but his wife mentions he doesn’t eat as much as he used too. Upon examination, RF has lost close to 13 lbs. since this last yearly exam.

PMH:

Primary myelofibrosis diagnosed about 1 year ago

Diabetes (controlled with diet and exercise)

COPD

SMH: Smoked 3 packs/day but quit 5 years ago; he drinks occasionally

PE: abdominal exam reveals spleen palpable 5cm below left coastal margin, visible bruising

Initial Labs/Workup:

Platelet count: 184 x 109/L

Hgb: 10g/dL

WBC: 34 x 109/L

Bone Marrow Biopsy

Shows an increase in megakaryocytes

Bone Marrow Fibrosis

Megakaryocyte Atypia

Molecular testing

JAK-V617F mutation: positive

Initial treatment:

Patient was started on ruxolitinib 10mg BID when initially diagnosed

Current Presentation/Labs:

One year later, RF reports increasing fatigue and abdominal pain)

Current Labs:

Platelet count: 57 x 109/L

Hgb: 8g/dL

WBC count: 29 x 109/L

Raajit Rampal, MD, PhD: Hi, I'm Dr Raajit Rampal from Memorial Sloan Kettering Cancer Center [New York, NY], and I will be talking to you today about the case of a 67-year-old man with myelofibrosis. To begin, we'll start with a case presentation.

RF is a 67-year-old man who visits his primary care physician for his yearly checkup. He reports having fatigue and bone pain for the last few months. More recently, he's been complaining of abdominal discomfort, which he thinks is possibly related to the big Thanksgiving dinner party he and his wife hosted. He enjoys spending time on antique cars in his garage but his wife says he's become tired after just an hour of work. RF has not noticed any changes in his eating habits but his wife mentions he doesn't eat as much as he used to. Upon examination, RF has lost close to 13 pounds since his last yearly exam. His medical history is remarkable for a diagnosis of primary myelofibrosis, which was made about 1 year ago; diabetes, which is controlled with diet and exercise; and COPD [chronic obstructive pulmonary disease]. His other social history includes that he smoked 3 packs a day but quit 5 years ago. He drinks alcohol occasionally. Upon physical examination, the exam is notable for a spleen that is palpable to 5 cm right below the left costal margin as well as visible bruising. Laboratory evaluation demonstrates a platelet count of 184,000, hemoglobin level of 10, white blood cell count of 34,000. A bone marrow examination is performed, which shows an increase in megakaryocytes and megakaryocytopoiesis as well as bone marrow fibrosis. Molecular testing demonstrates the presence of a JAK2 V617F mutation. Based on the patient’s symptoms and weight loss, he was started on ruxolitinib 10 mg twice daily when he was initially diagnosed. One year later, RF reports increasing fatigue and abdominal pain. His current labs demonstrate a platelet count of 57, 000, a hemoglobin level of 8, a white blood cell count of 29,000.

To review and summarize the patient's case before we further delve into data and management questions: we have a patient with primary myelofibrosis who was initially observed but now has symptoms such as early satiety, unanticipated weight loss, and fatigue. These are often characteristics of myelofibrosis in symptomatic patients, with fatigue being the most common of these complaints. The patient’s blood count demonstrates that he did have preserved platelet count, but has some mild anemia and leukocytosis. His bone marrow exam demonstrated findings consistent with myelofibrosis including the presence of fibrosis, clustered megakaryocytes and megakaryocytopoiesis, and the JAK2 mutation. Based on the patient’s symptom profile and based upon his labs, he would be deemed to be likely intermediate risk, and therefore it is reasonable to consider starting therapy in this patient. He was started on ruxolitinib 10 mg twice daily, due to his spleen and symptom burden.

Transcript edited for clarity.