TN 7 (08-12)
DI 23022.815 Mastocytosis--Type IV
COMPASSIONATE ALLOWANCE INFORMATION
Leukemic Mastocytosis; Mast Cell Leukemia; MCL; Lymphadenopathic Mastocytosis; Type IV Leukemia
Mastocytosis is a rare neoplastic disorder that occurs when there is an abnormal accumulation (excess) of mast cells in the blood and bone marrow, skin, gastrointestinal tract (GI), liver and spleen. Mastocytosis can be cutaneous (skin) or systemic (involving the internal organs of the body). Depending on the number of mast cells in the different organ parts, it is classified as either indolent (slow growing) or aggressive. Mastocytosis type IV is a severe form of this disorder and is defined by malignant proliferation of mast cells present in the blood, with no skin involvement, multi-organ failure, and a short survival.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: Diagnostic testing for mastocytosis type IV includes skin exam, bone marrow biopsy, plasma tryptase, and urine mediators such as histamine. Imaging testing can include skeletal survey and gastrointestinal evaluation. Bone scans and bone marrow testing is conclusive.
ONSET AND PROGRESSION
The median age at diagnosis of the severe form of mastocytosis in adults is 55 years of age. The prognosis for people with type IV mastocytosis is poor with survival time of a few months once the diagnosis is established.
There is no current cure or standard treatment for mastocytosis type IV. Severe forms of mastocytosis have been treated with chemotherapy, immunotherapy, stem cell or bone marrow transplantation.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation: Clinical examinations including a thorough history of illness; oncology, hematology, dermatology, immunology consultation reports, pathology reports, and imaging study reports.
Suggested Listings for Evaluation:
Mastocytosis Type IV meets listings 13.06A or 113.06A upon confirmed diagnosis, regardless of effectiveness of treatment.
| || |
* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.