AHCI offers hematology care for malignant and benign blood diseases

February 03, 2023
Fiona He, MD
Nicholas Torgerson, DO

Almost 190,000 people in the U.S. are diagnosed with malignant blood diseases each year. These life-threatening conditions, such as lymphoma, leukemia and myeloma, account for nearly 10% of new cancer cases. In addition, nonmalignant yet serious blood disorders like pregnancy-related anemias and inherited bleeding conditions affect millions more people.

At Allina Health Cancer Institute (AHCI), we see adult patients with malignant and benign blood diseases. We concentrate our complex hematology care services at the metropolitan sites of Allina Health Cancer Institute — Minneapolis, Abbott Northwestern Hospital in Minneapolis and United Hospital in St. Paul. We also see patients in our clinics or during in-hospital consultations throughout the Allina Health system.

The AHCI team cares for patients with some of the most complex conditions. Our training and resources enable us to provide efficient, effective care to patients throughout the region with common and rare diseases.

Patient case study: a rare hematologic condition and AHCI’s rapid response

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A 64-year-old male without significant medical history presented to AHCI’s Abbott Northwestern Hospital with an initial complaint of chest pain and fatigue. Upon admission, the patient’s white blood cells were elevated at 95,000 cells/mm3, with 89% blasts. He had mild anemia with a hemoglobin of 13.1 g/dL and thrombocytopenia with platelets of 103,000 cells/mm3. He had palpable cervical and axillary lymph nodes on examination.

A CT scan of the chest, abdomen and pelvis revealed widespread adenopathy, with the largest lymph node diameter of 2.1 centimeters. The patient’s brain MRI was normal. His bone marrow biopsy was 70% cellular, with 83% immature primitive cells.

Extensive immunophenotyping by flow cytometry and immunohistochemistry showed an absence of markers to suggest acute myeloid/monocytic leukemia (including MPO, CD13, CD33, CD15, CD117, CD14, CD64, CD11b and lysozyme) or acute B or T lymphoblastic leukemia (TdT, cCD79a, cCD3, CD10, CD19, CD20, CD22 and CD1a). The combination of CD56, CD123, CD4, CD7 and TCL1 expression in conjunction with a lack of other lineage specific markers was most consistent with a blastic plasmacytoid dendritic cell neoplasm (BPDCN). Cytogenetics revealed two abnormal clones. Myeloid next-generation sequencing panel showed ASXL1, TET2 and NRAS mutations.

BPDCN is a rare hematologic malignancy that is derived from plasmacytoid dendritic cells (type 2 dendritic cells). The incidence of BPDCN is 0.04 cases in 100,000. It was initially described in 1995 as an acute agranular CD4-positive natural killer (NK) cell leukemia, or “Blastic NK cell lymphoma.” Its name was changed to BPDCN in 2016 to more closely reflect its cell of origin.

The exact incidence of BPDCN is unknown, and it most commonly occurs in older adults with a median age of diagnosis of 65. It can occur as an isolated disease, but 10% to 20% of diagnoses occur in association with antecedent hematologic malignancies. Clinically, it may present with cutaneous lesions, cytopenias, lymphadenopathy, splenomegaly and extranodal involvement including liver and central nervous system.  

We initiated a treatment plan with a CD123 antibody drug conjugate tagraxofusp, brand name Elzonris®. This drug has specific FDA approval as an orphan drug designation for BPDCN. In the clinical studies of tagraxofusp, complete remission was achieved in 54% of newly diagnosed BPDCN patients, which is higher than other historical reports utilizing multiagent chemotherapies similar to that of acute myeloid or acute lymphoblastic leukemia. Elzonris is currently the standard of care for first-line therapy in BPDCN based on its favorable results in trials leading to its FDA approval. However, the annual treatment cost is estimated at $285,000 per year. 

One of the unique side effects of Elzonris is its potential for cytokine release syndrome or vascular leak syndrome, which is mostly mild but can be severe in less than 10% of patients. When this happens, the blood vessels become leaky, and the patient can rapidly develop pulmonary edema, hypotension and multiorgan failure if severe.

AHCI’s team of physicians, advanced practice providers, pharmacists and nurses collaborated with the pharmaceutical company to obtain the treatment and train both inpatient and outpatient care teams on the administration and side effect management of this drug. The nurses were trained on the use of a special pump for tagraxofusp that was acquired from the anesthesia department.

Just five days after the diagnosis, and with our team trained, we initiated Elzonris at Abbott Northwestern Hospital. AHCI’s Abbott Northwestern team oversaw the patient’s care with Fiona He, MD, as the primary treating hematologist. The patient received bridging Hydrea® for cytoreduction and then received his five-day course of Elzonris without major complications. He had mild tumor lysis syndrome that required fluids and treatment of hyperkalemia. He was discharged home to New Ulm, approximately 90 minutes from Abbott Northwestern.

At the time of discharge, AHCI’s specialty oncology nurse navigators facilitated his transition home to continue some supportive care at AHCI New Ulm Cancer Center, including laboratory monitoring.

Learn more about AHCI’s hematologic treatment capabilities

We treat patients with a wide range of hematologic conditions, including rare diseases like BPDCN.

Malignant hematology diseases

We care for patients with critical conditions such as:

  • Acute and chronic leukemias
  • Lymphoma
  • Multiple myeloma

Benign hematology disorders

Many of our patients have benign but serious blood disorders such as:

  • Sickle cell anemia
  • Hemophilia
  • Iron deficiency anemia
  • Myeloproliferative disorders
  • Thalassemia

AHCI’s hematology care services

Abbott Northwestern Hospital is the central acute leukemia hub for the Allina Health system. We provide specialized care for patients needing acute inpatient and outpatient treatment for their hematologic malignancy and are a resource for Allina’s medical community on the care of hematology patients. We offer comprehensive hematology care that is integrated into the Allina system to provide high-quality, whole-person care. Our services include:

Diagnostics

Our hematology providers collaborate closely with pathology, transfusion medicine, molecular diagnostics and radiology for access to the most up-to-date tests and timely results. We order standard tests and provide specialized diagnostic tests such as:

  • CT, PET and MRI scans
  • Bone marrow and tissue biopsy
  • Platelet refractoriness workup
  • Hereditary cancer testing
  • Next-generation sequencing for oncologic mutations
Our hematopathology and molecular divisions have designed an in-house myeloid next-generation sequencing panel for patients with newly diagnosed acute myeloid leukemia (AML). We will be one of only three sites in the Twin Cities to have this capability.

Treatments

We offer nonsurgical standard-of-care treatments for most hematology disorders. These include:

  • Chemotherapy: We can provide multiple options for oral and infused medications on an outpatient or inpatient basis. We can also offer these treatments for patients who do not qualify for intensive surgery.
  • Radiation therapy: We provide treatments with high-wavelength radiation to destroy cancer cells and shrink tumors.
  • Blood transfusions: We provide standard and uncommon transfusions, such as highly specialized platelet transfusions for myelodysplastic syndrome.
  • Clinical trials: We provide access to early and late phase clinical trials as a member of the Metro Minnesota Community Oncology Research Consortium and have our own clinical trials research program through AHCI. In 2023, we plan to open two early phase trials for relapsed and refractory lymphoma.
  • Financial assistance: We evaluate the pharmaceutical and other out-of-pocket costs related to cancer care and identify sources to provide financial assistance, including grants and compassionate use.

How AHCI maintains leading-edge hematology expertise

Our expertise in hematology requires ongoing training of our team.

  • Hematology tumor board: We meet bi-weekly to review complex cases and exchange insights with colleagues. These meetings are attended by specialists in oncology, radiation, pathology and other clinical areas. We work together to evaluate diagnostic tests and discuss the best treatment options.
  • Hematology journal club: At this monthly meeting, one or two team members present findings from recent medical literature. Examples of topics discussed in 2022 include minimal residual disease in multiple myeloma, maintenance treatment in mantle cell lymphoma, infectious prophylaxis in acute myeloid leukemia and treatment of double-expressor diffuse large B-cell lymphoma.
  • Hematology working group: Starting in 2023, the hematology team will hold quarterly meetings with invited experts across disciplines. We will discuss topics such as inherited hematologic malignancy syndromes, updates on the use of irradiated blood products and clinical research initiatives, including a feasibility study of exercise interventions in newly diagnosed acute myeloid leukemia.
  • Hematology education: Our AHCI providers work closely with Abbott Northwestern Internal Medicine residents who rotate on the inpatient hematology consultation service.

Our timelines for hematology patient referrals

Our timeframes for patient visits are driven by each case’s severity. We see patients with an urgent need in one to two days in our clinics or in the hospital. We see non-urgent cases in our clinics within one to two weeks.

When you refer a patient to AHCI’s hematology services

Physicians often ask us to consult about ordering or interpreting diagnostic tests. Referring physicians can access a team of hematology subject matter experts who keep them informed and involved in their patients’ care.

We welcome the opportunity to work with referring physicians, both within and outside of Allina Health. Providers from within Allina Health can send referrals through Epic, our electronic medical records system.

Allina providers are encouraged to reach out directly to Dr. Fiona He or Dr. Nicholas Torgerson via Epic inbox or secure chat to expedite a hematology consultation due to medical urgency. Our trained hematology nurse navigator performs intakes for most referrals and ensures that patients have appropriate labs or imaging prior to their initial consultation.

Providers outside of Allina Health can refer patients by calling our nearest cancer center: