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Table 3 Characteristics of children that died of PLCH and comparison with surviving patients

From: Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Characteristics

Deceased patients, n = 6

Surviving patients, n = 11

P value

Age at PLCH diagnosis, median [min, max]

1.0 y [0.2–5.0]

1.4 y [0.1–13.7]

0.8

RO- LCH disease

3

7

0.6

RO+ LCH disease

3

4

 

Maximal DAS Score, median [min, max]

13.5 [7,27]

5 [4,10]

0.04

First-line LCH therapy

 VLB Steroid

5

11

0.4

 2CDA

1

0

Second-line LCH therapy

 2CDA

2

5

0.4

 VP16 ARA-C

2

1

 Targeted therapy

0

2

CT Score at PLCH diagnosis

(n = 4 evaluated cases)

(n = 9 evaluated cases)

 

 Cyst, median [min, max]

16 [8,24]

10 [1,18]

0.14

 Nodule, median [min, max]

0 [0,0]

3 [0,9]

0.08

BRAFV600E mutated LCH

0/2 investigated cases

4/9 investigated cases

0.5

Time to death after PLCH diagnosis, median [min, max]

6.9 months [4.6,13.7]

  

Cause of death

   

Repeated pneumothorax

3

_

 

Diffuse micronodular lung infiltration

2

Post lung transplant

1

  1. Values are the number of patients, unless indicated otherwise
  2. Abbreviations: DAS Disease Activity Score, CT computed tomography, LCH Langerhans cell histiocytosis, PLCH Pulmonary LCH, RO Risk organs involved (+) or not involved (−), VLB vinblastine, VP16 Ara-C Etoposide-Cytarabine, 2CDA Cladribine