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Hematopoietic Cell Transplantation for Amyloid light-chain (AL) Amyloidosis (Primary Systemic Amyloidosis)

Section: Surgery
Effective Date: January 01, 2020
Revised Date: November 14, 2019

Description

Hematopoietic Cell Transplantation

Hematopoietic cell transplantation (HCT) refers to the infusion of hematopoietic stem cells to restore bone marrow function in cancer patients who receive bone-marrow-toxic doses of cytotoxic drugs with or without whole body radiation therapy. Hematopoietic stem cells may be obtained from the transplant recipient (autologous HCT) or from a donor (allogeneic HCT).

Primary Systemic or AL amyloidosis is the most common type of amyloidosis.  The clinical features are dependent on the organs involved, typically cardiac, renal, hepatic, peripheral, and autonomic neuropathy and soft tissue.

Criteria

Autologous HCT may be considered medically necessary to treat AL amyloidosis (primary systemic amyloidosis) when ALL of the following patient selection criteria are met:

  • Age greater than eighteen (18) years; and
  • Tissue diagnosis of amyloidosis by abdominal fat aspirate or biopsy of involved organ; and
  • Eastern Cooperative Oncology Group (ECOG) performance status score of zero-two (0- 2); and
  • New York Heart Association class I/II and no more than two involved major organs (liver, heart, kidney, autonomic nerve); and
  • Supine systolic blood pressure greater than 90 mm/Hg; and
  • Asymptomatic or compensated cardiac function (e.g. absence of congestive heart failure),echocardiographic left ejection fraction greater than 40%; and cardiac interventricular septal thickness is greater than 12 mm; and
  • Renal function with a creatinine clearance of at least 30 ml/min.

Note:  When available, a clinical trial should be utilized.

Procedure Codes

38206 38220 38221 38222 38232 38241 S2150

Allogeneic HCT is considered experimental/investigational and therefore non-covered to treat AL amyloidosis (primary systemic amyloidosis). Available scientific evidence does not permit conclusions concerning this intervention on health outcomes.

Procedure Codes

38205 38220 38221 38222 38230 38240 S2140
S2142 S2150

Diagnosis Codes

Covered Diagnosis Codes Applies to Autologous Hematopoietic Cell Transplants

E85.0 E85.1 E85.2 E85.81 E85.89 E85.9

Non-Covered Diagnosis Codes Applies to Allogeneic Hematopoietic Cell Transplants

E85.0 E85.1 E85.2 E85.81 E85.89 E85.9

Professional Statements and Societal Positions Guidelines

National Comprehensive Cancer Network (NCCN).  NCCN Guidelines Version 1. 2018, Systemic Light Chain Amyloidosis. 

The current NCCN Guidelines list the following as therapeutic considerations for management of patients with systemic light chain amyloidosis (all category 2A recommendation) along with best supportive care:  high-does melphalan followed by autologous SCT; oral melphalan and dexamethasone; dexamethasone in combination with alpha-interferon; thalidomide plus dexamethasone; lenalidomide and dexamethasone; Lenalidomide/cyclophosphamide/dexamethasone; pomalidomide and dexamethasone; bortezomib with or without dexamethasone; bortezomib with melphalan plus dexamethasone; cyclophosphamide, thalomide, and dexamethasone; bortezomib, and dexamethasone.

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