HISTIOCYTOSIS SYNDROMES
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ALTERNATE NAMES
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Hand-Schuller-Christian disease; Hemophagocytic Syndrome; Histiocytosis X; Langerhans
Cell Histiocytosis; Letterer-Siwe disease; LCH; Malignant Histiocytosis syndrome T-cell
Lymphoma; Non-Langerhans Cell Histiocytosis; Nonlipid Reticuloendotheliosis; Pulmonary
Histiocytosis X; Pulmonary Langerhans Cell Granulomatosis; Rosai-Dorfman Disease;
Sinus Histiocytosis with Massive Lymphadenopathy
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DESCRIPTION
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Histiocytosis is a general name for a group of syndromes where immune cells known as histiocytes
(monocytes/macrophages) proliferate and mistakenly attack the body instead of infections.
There are three major types of histiocytosis:
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Class I - Langerhans cell histiocytosis (LCH; previously known as Histiocytosis X);
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Class II - Hemophagocytic lymphohistiocytosis (HLH; non-Langerhans); and
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Class III - T-cell Lymphoma, also known as malignant histiocytosis syndrome.
The excessive increase in the number of histiocytes may form inflammatory tumors in
various body organs and bones, including the skull. For example, Langerhans cells
infiltrating the lungs leads to inflammation and stiffening of the lungs. Tumors in
weight-bearing bones, such as the legs or spine, may cause the bones to fracture without
cause.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Biopsy of skin or bone marrow documenting the presence of Langerhans or T-cell lymphoma.
Diagnosing HLH requires a set of defined criteria, including:
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Hemophagocytosis on tissue biopsy;
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Cytopenia (low blood cell levels);
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Splenomegaly (enlarged spleen);
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The following testing supports the diagnosis and provides information on clinical
severity:
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Complete blood counts (CBC);
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Bronchoscopy with biopsy;
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Pulmonary function tests.
Physical findings: Symptoms and signs depend on specific organ involvement. In children, these may include:
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Growth failure due to pituitary involvement;
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Frequent urination due to diabetes insipidus.
Children over age 5 usually only have bone involvement.
Adults may experience:
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Cough and shortness of breath;
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ICD-9: 202.5, 277.89
ICD-10: J84.82
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PROGRESSION
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LCH is relatively more common in children than in adults. The prognosis for children
is highly variable, with infants and young children more likely to have systemic disease
that leads to death. Adults with pulmonary histiocytosis X have a poor prognosis.
In familial HLH, only 21 to 25% survive 5 years after diagnosis.
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TREATMENT
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These disorders are treated with corticosteroids, anti-cancer immunosuppressive drugs,
radiation, and surgery, as well as supportive treatment.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and physical examination that describes the diagnostic features of
the impairment;
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Biopsy of affected organ system; and
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Functional assessment of organ system involved (for example, pulmonary function tests).
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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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3.02
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Listing level severity must be documented.
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8.09
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13.06 A
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103.02
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In children, solitary histiocytosis or eosinophilic granuloma treated with complete
surgical excision does not meet listing level severity.
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108.09
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113.03
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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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