Overview

This document addresses the use of Elzonris (tagraxofusp-erzs), a CD123-directed cytotoxin for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in those 2 years of age or older. BPDCN is an aggressive and rare disease of the bone marrow and blood that can affect multiple organs, including the lymph nodes and the skin. It often presents as leukemia or evolves into acute leukemia. Previously there had been no FDA approval therapies for BPDCN.

Elzonris is a fusion protein comprised of a recombinant human interleukin-3 (IL-3) and truncated diphtheria toxin that inhibits protein synthesis and causes cell death in CD123- expressing cells. Tagraxofusp-erzs is constructed by recombinant DNA technology and produced in Escherichia coli cells.

Elzonris contains a black box warning for Capillary leak Syndrome (CLS). In clinical trials, the overall incidence of CLS was 53%, including Grade 1 or 2 in 43%, Grade 3 in 7%, Grade 4 in 1% and 4 fatal events. Common signs and symptoms (incidence ">= 20%) associated with CLS that were reported during treatment with Elzonris include hepatoxicity, hypoalbuminemia, edema, weight gain, and hypotension.

Before initiating therapy with Elzonris, ensure that the patient has adequate cardiac function and serum albumin is greater than or equal to 3.2 g/dL. During treatment with Elzonris, monitor serum albumin levels prior to the initiation of each dose of Elzonris and as indicated clinically thereafter, and assess patients for other signs or symptoms of CLS, including weight gain, new onset or worsening edema, including pulmonary edema, hypotension or hemodynamic instability.

The ECOG or Eastern Cooperative Oncology Group Performance Status

is a scale and criteria used by doctors and researchers to assess how an individual’s disease is progressing, assess how the disease affects the daily living abilities of the individual, and determine appropriate treatment and prognosis. This scale may also be referred to as the WHO (World Health Organization) or Zubrod score which is based on the following scale:

• Fully active, able to carry on all pre-disease performance without restriction
• Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature, for example, light house work, office work
• Ambulatory and capable of all self-care but unable to carry out any work activities. Up and about more than 50% of waking hours
• Capable of only limited self-care, confined to bed or chair more than 50% of waking hours
• Completely disabled. Cannot carry on any self-care. Totally confined to bed or chair
• Dead.

Clinical Criteria

When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to clinical review (including prior authorization), the following criteria will be used to determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose.

Elzonris (tagraxofusp-erzs)

Requests for Elzonris (tagraxofusp-erzs) may be approved if the following criteria are met:

1. Individual is 2 years of age or older; AND
2. Individual has a diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN); AND
3. Individual has a current Eastern Cooperative Oncology Group (ECOG) status of 0-1; AND
4. Individual is using as monotherapy; AND
5. At initial therapy, individual has a baseline serum albumin of 3.2 g/dL or higher (NCCN 2A).

Requests for Elzonris (tagraxofusp-erzs) may not be approved when the above criteria are not met and for all other indications.

Coding

The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

HCPCS

J9269

ICD-10 Diagnosis

• C86.4
• Z51.11

Injection, tagraxofusp-erzs, 10 micrograms [Elzonris]

Blastic NK-cell lymphoma

Encounter for antineoplastic chemotherapy

Document History

Reviewed: 02/25/2023