Expert James Foran, MD, discusses the challenging case of an 87-year-old patient diagnosed with blastic plasmacytoid dendritic cell neoplasm (BPDCN), its diagnosis, and treatment options, highlighting the unique considerations for elderly individuals.
James M. Foran, MD: I’m Dr James Foran, a medical oncologist at the Mayo Clinic Cancer Center and professor of medicine based in Jacksonville, Florida. I chair the acute leukemia and myeloid neoplasm disease group in the cancer center. I’m going to discuss a patient and a plan for a patient with a blastic plasmacytoid dendritic cell neoplasm, or BPDCN.
An 87-year-old man was referred from a dermatologist for progressive and persistent cutaneous nodules first noted only 3 weeks earlier, so a relatively short interval. On systems review, he had fatigue. Over the past 3 months, he’s experienced about a 5-kg weight loss, so significant weight loss. Remarkably for an 87-year-old man, he had very little in the way of past medical history or comorbidities, apart from recurrent sinusitis in the past. His examination was notable for multiple purpuric nodules, some of them large and raised up to 5 cm, on his arms, legs, and torso. He did not have any clinical adenopathy or hepatosplenomegaly. Because of his fatigue and weight loss, his performance status was ECOG 1.
He had a [complete blood count] that showed a white blood cell count of 14.1. The differential showed 12% myeloid blasts, 32% neutrophils, 16% monocytes, and 40% lymphocytes. Hemoglobin was 8.9 g/dL and platelet count was 54. His skin had purpuric nodules. On the peripheral blood smear, he had blast cells with large, round, and slightly irregular nuclei. The blast cytoplasm stained grayish blue, but there were no granules or Auer rods.
He went for a bone marrow biopsy. The bone marrow biopsy showed 40% blasts by morphology, and the marrow was hypercellular at 80% cellular with an interstitial infiltrate. Immunohistochemistry was positive in the blasts for CD123, CD4, CD56, and TCL1. Flow cytometry showed CD4, CD56, CD123, CD34, and negative T and B lineage markers. Cytogenetics showed a diploid male karyotype, 46XY and 20 metaphases. He underwent a lumbar puncture that did not indicate any pleocytosis or [central nervous system] involvement. This patient has a diagnosis of BPDCN and was started on tagraxofusp therapy.
When I’m thinking about the case in my initial presentation, this is an older patient with few comorbidities and a short-term history of fatigue and weight loss, so constitutional symptoms, cutaneous nodules, peripheral blood and bone marrow involvement. This is a difficult situation with the diagnosis of an acute leukemia and BPDCN, a rare but acknowledged manifestation of acute leukemia that confers a poor prognosis. Historically with multiagent chemotherapy, patients can achieve remissions if they are fit for such therapy, but the remissions tend to be short, with median survival of around 8 to 16 months. The first order of business in a person with worsening performance status, cutaneous nodules, and active leukemia is to try to induce remission.
That is going to be the plan for this patient. There are different ways to do that, of course. But this is a difficult leukemia. We would counsel the patient about expectations for remission, for disease-free survival, and for overall survival. And in a patient who’s 87, you can achieve important responses, but they’re palliative responses. The question is, how do you best do that with, especially in an 87-year-old with the right balance of toxicity and morbidity for the patient?
Transcript is AI-generated and edited for readability.