Table 1 Summary of KLA patients

1^a

2 years/M

Mediastinum, abdominal cavity, pelvic cavity, bilateral inguinal regions and testes

Abdominal distention, pain and coagulation disorder

Corticosteroid, immune globulin and platelet transfusion

6

7.8–12.7 (10.4)

PD

2^a

4 months/F

Left mediastinum and left lung

Dyspnea pleural effusion and coagulation disorder

Thoracic drainage

18

6.1–15.7 (10.2)

PR

3^a

5 years/M

Lungs, vertebrae, ribs and mediastinum

Pleural effusion and coagulation disorder

Thoracic drainage, fibrinogen transfusion, exploratory thoracotomy

42

10.7–14.9 (13.3)

PR

4^a

6 months/M

Lungs, mediastinum, spleen, liver, pancreas and mesentery

Abdominal distention, vomiting and coagulation disorder

Vincristine plus corticosteroid, aspirin

41

5.1–13.7 (11.6)

SD

5^a

14 months/M

Lungs and spleen

Cough, respiratory distress, pleural effusion, pericardial effusion and coagulation disorder

Pericardiocentesis, corticosteroid, splenectomy and platelet transfusion

14

5.9–14.3 (12.1)

PR

6^a

4 years/M

Left lung and mediastinum

Cough and coagulation disorder

Sirolimus, sirolimus plus steroids, sirolimus and vincristine

21

9.4–15.8 (10.6)

SD

7^a

2 years/M

Mediastinum, lungs, neck and spleen

Dyspnea, cough and coagulation disorder

Propranolol and prednisone

21

10.0–14.7 (13.2)

SD

8^b

8 years/M

Bone, thoracic and mediastinal

Chylothorax and coagulation disorder

Interferon and propranolol

30 (cessation)

4.4–9.0 (7.5)

PR

9^b

8 years/M

Bone, thoracic and mediastinal

Scoliosis, chylothorax and coagulation disorder

Steroids and propranolol

24

8.1–12.4 (11.2)

SD

10^b

20 years/M

Bone, thoracic and right chest wall

Gastrointestinal hemorrhage and coagulation disorder

Steroids

12

4.7–6.0 (5.5)

PR

11^b

–/M

Thorax

NG

NG

NG

NG

PD

12^b

7 years/M

Left scapular, left arm and thoracic vertebral bodies

Dyspnea, shortness of breath, pleural effusion and coagulation disorder

Chinese traditional medicine, pleurocentesis and albumin

8

9.3–13.4 (12.3)

PR

13^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PR

14^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PR

15^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PR

16^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PR

17^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PR

18^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PR

19^b

NG

NG

NG

NG

12

10.0–15.0 (NG)

PD

20^b

7 years/M

Abdominal cavity, lower neck, mediastinum, bone and left upper arm

Cephalohematoma and coagulation disorder

Steroids, vincristine and zoledronate

12

9.4–14.8 (NG)

PR

21^b

13 years/F

Bone, mediastinum, spleen

Cough, fever, fatigue, intermittent wheezing, pleural effusion and coagulation disorder

Thoracostomy

3

NG

PR

22^b

20 months/M

Mediastinum and spleen

Cough, pleural effusion, pericardial effusion, epidural hematoma and coagulation disorder

Diaphragmatic fenestrations

7

NG

PD

23^b

11 years/F

Mediastinum, bone and spleen

Cough, chest pain, fatigue, listlessness, weight loss, coagulation disorder and pericardial effusion, coagulation disorder

Pericardiocentesis

31

NG

SD

24^b

4 years/M

Bone, spleen and mediastinum

Fatigue, decreased appetite, weight loss, cough, spontaneous bruising, back pain, coagulation disorder and pleural effusion

Cryoprecipitate, platelet infusions and prednisolone

NG

NG

SD