Program Operations Manual System (POMS)
TN 33 (08-20)
COMPASSIONATE ALLOWANCE INFORMATION |
RICHTER
SYNDROME |
ALTERNATE NAMES
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Richter Disease; Richter Transformation; Richter’s syndrome; Richter’s Disease; Richter’s
Transformation
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Diagnostic testing for individuals with Richter syndrome includes:
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Computerized Tomography (CT/CAT) scan;
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Positron Emission Tomography (PET) scan;
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Magnetic Resonance Imaging (MRI);
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Bone marrow aspiration; and
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Physical findings: The signs and symptoms of Richter syndrome may include:
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Increased size of lymph nodes (lymphadenopathy);
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Painless swelling in the neck, axilla, abdomen, spleen (splenomegaly) or groin; and
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Unexplained weight loss, fevers and night sweats (commonly referred to as B-symptoms)
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Other signs and symptoms may include:
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Low Platelets with characteristic bruising or bleeding;
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Increase in serum lactate dehydrogenase (LDH); and
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Elevated serum calcium (hypercalcemia).
ICD-9: 200.7
ICD-10: C85.90
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for
Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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CT, MRI, or PET scan reports;
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Pathology/Biopsy reports of the cancer;
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Up-to-date progress reports.
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets |
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Equals |
13.05 D
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* 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.
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