Description

Patients with Histiocytosis X can be separated into clinical groups with a scoring system using clinical and laboratory findings.


 

Parameters used for deriving the score:

(1) age of the patient in years

(2) number of affected organs (extent of disease)

(3) Lahey's criteria for hepatic, hematopoietic and/or pulmonary dysfunction

 

Organs which may become involved:

(1) bone

(2) skin

(3) teeth and/or gingival tissue

(4) ear

(5) endocrine organs

(6) lung

(7) liver

(8) spleen

(9) lymph nodes

(10) bone marrow

 

Parameter

Finding

Points

age

>= 2 years of age

0

 

< 2 years of age

1

extent of disease

< 4 organs involved

0

 

>= 4 organs involved

1

organ dysfunction

no evidence of organ dysfunction

0

 

one or more organs with dysfunction, due to disease and not attributable to superimposed infection

1

 

where:

• The presence of 1-3 organs (< 4) is considered localized disease involvement.

 

total score =

= (points for age) + (points for extent of disease) + (points for organ dysfunction)

 

Interpretation:

• minimum score: 0

• maximum score: 3

 

Score

Group

monostotic eosinophilic granuloma

0

0

I

1

II

2

III

3

IV

 

Note: In the implementation, if the patient is said to have monostotic eosinophilic granuloma, but there are points for organ involvement or dysfunction, then it is unlikely that it is truly monostotic, so it is grouped based on the total score.

 


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