Young Patient with BPDCN Benefits From ‘Great Support System’

After successfully donating stem cells to her brother, Drew, during his treatment for the blood disorder aplastic anemia, Sarah Montgomery was ready and willing to make the life-saving gesture again. When the call came to do so, however, she was forced to decline for a very understandable reason:

Her own stem cell transplant.

In the four years since donating to her brother, Montgomery, 26, had developed blastic plasmacytoid dendritic cell neoplasm (BPDCN) — a rare cancer that occurs in the bone marrow and blood. While her own transplant in April 2021 makes her ineligible for future donations, she still feels thankful: the Connecticut resident has healthy stem cells from an anonymous donor coursing through her bloodstream, and is receiving care at one of the first facilities in the United States to focus specifically on her cancer. The BPDCN Center at Dana-Farber/Brigham and Women’s Cancer Center (DF/BWCC) is staffed by a multidisciplinary team of medical oncologists, pathologists, dermatologists, and other specialists, who partner with basic and clinical researchers to develop individualized treatment plans and some of the first clinical trials for BPDCN.

 “When I was first diagnosed, I admit, I was shocked, angry, and scared,” says Montgomery. “The BPDCN Center was a great support system for me and my family, helping me to understand and fight my cancer.”

Sarah and Drew Montgomery a few months after Drew’s September 2017 stem cell transplant with Sarah’s donated cells.

A unique case

Although it has characteristics similar to both acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL), less than 1,000 people are diagnosed with BPDCN annually. Symptoms — including skin lesions, low blood counts, fever, night sweats, fatigue, and enlarged lymph nodes — vary widely from one patient to another.

Because many primary care physicians and even oncologists have not encountered cases of BPDCN, many patients with this cancer are initially misdiagnosed. Montgomery’s case was particularly unique. She was 25 when diagnosed, making her an excellent case study for BPDCN Center Director Andrew Lane, MD, PhD, and his Dana-Farber research colleagues as they try to zero in on the disease.

“While BPDCN is most frequent in older men, young people and women still do get the disease,” says Lane. “We have been studying the sex bias of BPDCN in the laboratory, and think at least half of the male bias is related to mutations in a gene on the X chromosome called ZRSR2; mutations only occur in males with BPDCN. Having more samples to study from women, young people, and young women like Sarah are very important to help us compare and contrast with the older, male BPDCNs.”

Montgomery is comforted by the expertise and clear treatment plan put in place by her care team, led by Lane and Joseph Antin, MD, who oversaw her stem cell transplant. And as she recovers from her transplant at home, she has another expert to turn to in 22-year-old Drew — now a healthy rising senior in college.

“Having someone who had experienced this before and was understanding all I was going through was so helpful,” says Montgomery. “You’d do anything for your sibling, and we were always there for each other.”

Drew visits Sarah just before her April 2021 stem cell transplant.

Brotherly love

Despite her brother’s medical challenges, Montgomery thought nothing of the flu-like symptoms that she couldn’t shake in late 2020. She had undergone genetic testing before donating her stem cells to Drew, and had no known mutations associated with major diseases.

Only when her mother grew concerned that her sickness might be COVID-19 did Montgomery visit a walk-in clinic for testing. Her blood counts concerned doctors enough to recommend a bone marrow biopsy, which confirmed she had BPDCN. As Montgomery learned more about the disease, she realized she had many of the aforementioned symptoms — including a longstanding bruise on her hip that had started turning purple.

“The hematologist had never heard of someone so young getting BPDCN,” says Montgomery. “She and my dad looked around for a specialist and found the BPDCN Center, and we went to Boston and met with Dr. Lane. He was wonderful, and the fact he had a game plan for fighting my specific case was very comforting.”

The plan included a drug called tagraxofusp, used specifically to target BPDCN. Montgomery spent a month as an inpatient at Brigham and Women’s Hospital while she was readied for and received tagraxofusp infusions in December 2020. After several more outpatient infusions, and another inpatient stay, she had her  April 2021 stem cell transplant.

“Younger BPDCN patients do better overall, and Sarah’s BPDCN was in a complete remission at the time of the transplant, which we know is another good prognostic sign that puts her in a better position for the long-term,” says Lane. “We will stay vigilant to watch out for any signs of the BPDCN coming back, but as of right now she’s doing great.”

All of which makes Montgomery’s favorite fellow transplantee breathe much easier.

“I cannot be prouder of my sister, and I feel honored that I was able to aid her in any way possible,” says Drew Montgomery.

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