langerhanse cell histiocytosis

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Efficacy and Safety of Cladribine as salvage therapy in patients with relapsed/refractory Langerhans Cell Histiocytosis : A single-center study of thirteen cases Authers: Dr. Ajay Yadav, Dept. of Medical Oncology, AIIMS, New Delhi

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Page 1: Langerhanse Cell Histiocytosis

Efficacy and Safety of Cladribine as salvage therapy in patients with relapsed/refractory Langerhans Cell Histiocytosis

: A single-center study of thirteen cases

Authers: Dr. Ajay Yadav,

Dept. of Medical Oncology, AIIMS, New Delhi

Page 2: Langerhanse Cell Histiocytosis

Introduction

• LCH is a heterogonous disease which can affect any organ or system of the body

• Clinical course varies from a self limiting disease to a rapidly progressive fatal disease

• Clinical manifestations depend on site & extent of organ involvement

• Response to chemotherapy is very good but some patients develop recurrence after first line treatment

Page 3: Langerhanse Cell Histiocytosis

Introduction

Localized Disease

Multi-system disease

Good Prognosis

Require minimal or no treatment

Poor Prognosis

Require Cytotoxic

agents

Relapsed DsRefractory Ds

Page 4: Langerhanse Cell Histiocytosis

Methods

• We reviewed the data of recurrent or refractory LCH treated at our center between March’2006 to March’2014

• Safety and efficacy (response rate & progression-free survival) were evaluated

• PFS was calculated from the date of progression after 1st line treatment to the date of progression after salvage therapy with cladrinbine

Page 5: Langerhanse Cell Histiocytosis

Results

• N=13

• Median age: 4 years (range: 1-28); Male: Female - 10:3

• All patients received prednisolone, 6-MP, vinblastine and Vp-16 (two patients without etposide) as their 1st line treatment

• Initial response- – PR-10; SD-1; PD-2

• Median time to progression after 1st line treatment: 16.6 months (range: 5-30.9)

Page 6: Langerhanse Cell Histiocytosis

Patient’s characteristics

Case Sex Age at diagnosis (Yrs) Site involved

1M 1

MULTISYSTEM “RISK” PATIENTS

Skin, Bone, Liver

2F 5

MULTISYSTEM “RISK” PATIENTS

Bone, Lung, Liver

3 M 6 Multisystem Low risk Skin, Bone

4M 5

MULTISYSTEM “RISK” PATIENTS

Skin, Bone, CNS, Lung

5 M3

MULTISYSTEM “RISK” PATIENTS

Bone, Lung

6M 2

MULTISYSTEM “RISK” PATIENTS

Skin, Bone, Liver

7M 4

MULTISYSTEM “RISK” PATIENTS

Skin, Bone, Bone marrow

8 M 1 Multisystem Low Risk Skin, Bone

9M 4

MULTISYSTEM “RISK” PATIENTS

Skin, Bone, Lung

10 F 2 Multisystem Low risk Skin, Bone

11 M 13 Single System Skin

12 F 1.5 Multisystem Low risk Skin, Bone

13 M 28 Single System Bone

Page 7: Langerhanse Cell Histiocytosis

Response

Response Number %

Complete response 5 38

Partial response 1 8

Stable disease 2 15

Progressive disease 3 24

Unknown response 2 15

Use: 2nd line-6; 3rd line-7

Overall response rate: 46% (n=6/13) Disease control rate: 62% (n=8/13)

Page 8: Langerhanse Cell Histiocytosis

Toxicity & outcome

• Grade ¾ febrile neutropenia: 3• Pneumonia: 1• No toxicity related death

• Median follow up: 72 months– Median PFS: 22 months – 5-year overall survival was: 92.3%

Page 9: Langerhanse Cell Histiocytosis

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Page 10: Langerhanse Cell Histiocytosis

Conclusion

• Cladribine is highly effective with manageable toxicity in patients with relapse/refractory LCH

• Many patients respond to subsequent line of therapy and had good overall survival

• Cladribine as salvage therapy can induce a long term disease control and even cure

Page 11: Langerhanse Cell Histiocytosis

THANK YOU