PROGRAM OPERATIONS MANUAL SYSTEMPart DI – Disability InsuranceChapter 230 – Special IssuesSubchapter 22 – Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS)Transmittal No. 27, 12/12/2019
This is a Quick Action Transmittal. These revisions do not change or introduce new policy or procedure.
Summary of Changes
DI 23022.483 Primary Peritoneal Cancer
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DI 23022.887 Richter Syndrome
COMPASSIONATE ALLOWANCE INFORMATION
Primary Peritoneal Carcinoma; Primary Peritoneal Neoplasm; Primary Peritoneal Tumor; Primary Peritoneal Sarcoma; Serous Surface Papillary Carcinoma; Serous Surface Papillary Cancer; Serous Surface Papillary Sarcoma; Serous Surface Papillary Tumor; Serous Surface Papillary Neoplasm; Extra Ovarian Serous Carcinoma; Extra Ovarian Serous Cancer; Extra Ovarian Serous Neoplasm; Extra Ovarian Serous Tumor; Extra Ovarian Serous Sarcoma; Primary Serous Papillary Carcinoma; Primary Serous Papillary Cancer; Primary Serous Papillary Sarcoma; Primary Serous Papillary Tumor; Primary Serous Papillary Neoplasm
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: The diagnosis of primary peritoneal cancer is made by:
History and physical exam;
CA 125 Assay;
Computerized Tomography (CT/CAT) scan;
Positron Emission Tomography (PET) scan;
Magnetic Resonance Imaging (MRI);
Chest X-ray; and
Physical findings: The signs and symptoms of primary peritoneal cancer may include:
Pain, swelling, or a feeling of pressure in the abdomen or pelvis;
Vaginal bleeding that is heavy or irregular, especially after menopause;
Vaginal discharge that is clear, white, or colored with blood;
Lump in the pelvic area; and
Gastrointestinal problems such as gas, bloating, or constipation
The prognosis for individuals with primary peritoneal cancer is often poor. Median life expectancy is 1-2 years, with a range of 4 months to >5 years, the 5-year survival rate is about 26%.
The presentation of PPC may be in the advanced stages at initial diagnosis, making curative treatment very difficult. Treatment may include surgery, radiation, and/or chemotherapy. The treatment of primary peritoneal cancer depends on the stage/grade of cancer; whether the patient has extra fluid in the abdomen that causes swelling; whether the cancer is unresectable, metastatic, or recurrent; changes in the BRACA1 or BRACA2 genes; and the patient’s age and general health.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Clinical history and examination that describes the diagnostic features of the impairment;
Pathology/biopsy report of the cancer; and
Imaging reports such as CT scan, MRI scan; PET scan, and Ultrasound
Primary peritoneal cancer, by definition equals 13.23E 1 a – extension to peritoneal surfaces.
Richter Disease; Richter Transformation; Richter’s syndrome; Richter’s Disease; Richter’s Transformation
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: Diagnostic testing for individuals with Richter syndrome includes:
Blood chemistry studies;
Bone marrow aspiration; and
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:
Shortness of breath;
Low Platelets with characteristic bruising or bleeding;
Increase in serum lactate dehydrogenase (LDH); and
Elevated serum calcium (hypercalcemia).
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.
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.
CT, MRI, or PET scan reports;
Pathology/Biopsy reports of the cancer;
Surgical procedures; and
Up-to-date progress reports.