TN 33 (08-20)

DI 23022.887 Richter Syndrome

COMPASSIONATE ALLOWANCE INFORMATION
RICHTER SYNDROME

ALTERNATE NAMES

Richter Disease; Richter Transformation; Richter’s syndrome; Richter’s Disease; Richter’s Transformation

DESCRIPTION

Richter syndrome (RS) is a rare type of non-Hodgkin lymphoma. This condition occurs when chronic lymphocytic leukemia (CLL) transforms into a fast growing aggressive type of lymphoma, most commonly diffuse large B-cell lymphoma. (DLBCL). The risk of developing RS depends on the genetic mutations in the CLL cell; clinical characteristics; biologic and genetic features of the CLL B-cell clone; and therapy for progressive CLL.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnostic testing for individuals with Richter syndrome includes:

  • Blood chemistry studies;

  • Computerized Tomography (CT/CAT) scan;

  • Positron Emission Tomography (PET) scan;

  • Magnetic Resonance Imaging (MRI);

  • Bone marrow aspiration; and

  • Biopsy.

Physical findings: The signs and symptoms of Richter syndrome may include:

  • Increased size of lymph nodes (lymphadenopathy);

  • Painless swelling in the neck, axilla, abdomen, spleen (splenomegaly) or groin; and

  • Unexplained weight loss, fevers and night sweats (commonly referred to as B-symptoms) .

Other signs and symptoms may include:

  • Increased fatigue;

  • Shortness of breath;

  • Dizziness;

  • Palpitations;

  • Low Platelets with characteristic bruising or bleeding;

  • Increase in serum lactate dehydrogenase (LDH); and

  • Elevated serum calcium (hypercalcemia).

ICD-9: 200.7

ICD-10: C85.90

PROGRESSION

RS indicates the transformation of CLL into an aggressive lymphoma. The median age of occurrence is between 61 years and 70 years. People with CLL are at increased risk of developing a second malignant neoplasm such as cancers of the lung, brain, eye, and malignant melanoma. The median time to occurrence of CLL to RS is 1.8 to 5 years after diagnosis. Approximately 2% to 10% of people who have chronic lymphocytic leukemia develop Richter transformation. Prognosis is very poor with survival generally less than 1 year.

TREATMENT

Richter syndrome lymphoma is treated with the same chemotherapy drugs used for all other aggressive lymphomas, such as chemotherapy, radiation therapy, or antibody or biological therapy. RS is not very responsive to any of the available treatments.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:
  • Clinical history and examination that describes the diagnostic features of the impairment;

  • CT, MRI, or PET scan reports;

  • Pathology/Biopsy reports of the cancer;

  • Surgical procedures; and

  • Up-to-date progress reports.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

 

 

Equals

13.05 D

 

* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

To Link to this section - Use this URL:
http://policy.ssa.gov/poms.nsf/lnx/0423022887
DI 23022.887 - Richter Syndrome - 08/28/2020
Batch run: 08/28/2020
Rev:08/28/2020