MASTOCYTOSIS TYPE IV
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ALTERNATE NAMES
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Leukemic Mastocytosis; Mast Cell Leukemia; MCL; Lymphadenopathic Mastocytosis; Mast
Cell Sarcoma
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DESCRIPTION
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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.
People with Mastocytosis Type IV have mast cell leukemia. Type IV is the most severe form of mastocytosis marked by
malignant proliferation of mast cells in the blood, no skin involvement, multi-organ
failure, and a short survival. Type IV is generally defined as having at least 10%
abnormal mast cells in the peripheral blood or at least 20% abnormal mast cells in
the bone marrow.
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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 Mastocytosis Type IV includes:
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Urine mediators such as histamine.
Imaging testing can include skeletal survey and gastrointestinal evaluation. Bone
scans and bone marrow testing are conclusive.
Physical findings: Signs and symptoms of this condition include:
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Anemia and bleeding disorders;
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Gastrointestinal symptoms such as abdominal pain, diarrhea, nausea, or vomiting;
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Itching, hives, or flushing of the skin;
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Enlarged liver (hepatomegaly);
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Enlarged spleen (splenomegaly); and
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Enlarged lymph nodes (lymphadenopathy).
ICD-9: 202.6
ICD-10: C96.2
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PROGRESSION
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The median age at diagnosis of the severe form of mastocytosis in adults is 55 years
of age. Type IV has also occurred in children as young as 4 years old. The prognosis
for people with type IV mastocytosis is poor with survival time of a few months once
the diagnosis is established.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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A thorough history of illness;
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Oncology, hematology, dermatology, and immunology consultation reports;
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.06 A
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Mastocytosis Type IV meets the criteria in listing 13.06 A upon confirmed diagnosis,
regardless of effectiveness of treatment.
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113.06 A
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Mastocytosis Type IV meets the criteria in listing 113.06 A upon confirmed diagnosis,
regardless of effectiveness of treatment.
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Equals
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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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