Choi et al reported a clinical score for disease activity in a patient with Langerhans Cell Histiocytosis (LCH). This can help to determine how intensive therapy should be. The authors are from Hanyang University Hospital, University of Pittsburgh, Sungjyunkwan University and University of Ulsan.
Patient selection: Langerhans Cell Histiocytosis (formerly Histiocytosis X)
Parameters:
(1) body temperature in °C
(2) skin rash
(3) gastrointestinal tract involvement
(4) endocrine involvement (endocrinopathy)
(5) lesions with risk for CNS involvement (orbital, temporal/mastoid, sphenoidal, zygomastical, ethmoidal, maxilla, sinuses, anterior or middle cranial fossa)
(6) lymphadenopathy
(7) number of sites of bone involvement
(8) hemoglobin in g/dL (with iron deficiency excluded)
(9) white blood cell count
(10) platelet count
(11) splenomegaly
(12) liver involvement
(13) lung involvement (high-resolution CT, by histopathology)
|
Parameter |
Finding |
Points |
|
body temperature |
< 38.5°C |
0 |
|
|
>= 38.5°C |
1 |
|
skin rash |
absent |
0 |
|
|
present |
1 |
|
GI tract involvement |
absent |
0 |
|
|
present |
1 |
|
endocrine involvement |
absent |
0 |
|
|
present |
1 |
|
CNS risk lesion |
absent |
0 |
|
|
present |
1 |
|
number of site of bone involvement |
0 |
0 |
|
|
1 |
1 |
|
|
>= 2 |
2 |
|
hemoglobin |
< 9 g/dL in infant |
1 |
|
|
< 10 g/dL noninfant |
1 |
|
|
other |
0 |
|
WBC count |
< 4,000 per µL |
1 |
|
|
>= 4,000 per µL |
0 |
|
platelet count |
< 100,000 per µL |
1 |
|
|
>= 100,000 per µL |
0 |
|
splenomegaly |
none or < 2 cm below costal margin |
0 |
|
|
>= 2 cm below costal margin |
3 |
|
liver involvement |
> 3 cm below constal margin |
3 |
|
|
liver dysfunction |
3 |
|
|
by histology |
3 |
|
|
other |
0 |
|
lung involvement |
absent |
0 |
|
|
present |
3 |
where:
• Bone marrow involvement was demonstrated by staining for CD1a.
• Risk organ involvement: liver, spleen, lung and bone marrow.
• Liver dysfunction: hyperbilirubinemia, hypoalbuminemia, elevated AST and ALT, ascites, edema.
total score =
= SUM(points for all 13 parameters)
Interpretation:
• minimum score: 0 (minimum score with LCH is 1)
• maximum score: 20
• The higher the score the worse the prognosis.
|
Score |
Reactivation-Free Survival at 10 Years |
Typical Patient |
|
1 to 2 |
88% |
single system disease |
|
3 to 5 |
75% |
multisystem disease without risk organ involvement |
|
>= 6 |
44% |
multisystem disease with risk organ involvement |
Specialty: Hematology Oncology
