Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis
Introduction. Erdheim-Chester disease (ECD) is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family with characteristic bilateral symmetrical long bone osteosclerosis, associated with xanthogranulomatous extras-keletal organ involvement. In ECD,...
| Main Authors: | , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Military Health Department, Ministry of Defence, Serbia
2016-01-01
|
| Series: | Vojnosanitetski Pregled |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0042-8450/2016/0042-84501500037P.pdf |
| _version_ | 1826574740735655936 |
|---|---|
| author | Perić Predrag Antić Branislav Knežević-Ušaj Slavica Radić-Tasić Olga Radovinović-Tasić Sanja Vasić-Vilić Jasenka Sekulović Leposava Tarabar Olivera Tukić Ljiljana Jovandić Stevo Magić Zvonko |
| author_facet | Perić Predrag Antić Branislav Knežević-Ušaj Slavica Radić-Tasić Olga Radovinović-Tasić Sanja Vasić-Vilić Jasenka Sekulović Leposava Tarabar Olivera Tukić Ljiljana Jovandić Stevo Magić Zvonko |
| author_sort | Perić Predrag |
| collection | DOAJ |
| description | Introduction. Erdheim-Chester disease (ECD) is a rare, systemic form of
non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family
with characteristic bilateral symmetrical long bone osteosclerosis,
associated with xanthogranulomatous extras-keletal organ involvement. In ECD,
central nervous system (CNS) and orbital lesions are frequent, and more than
half of ECD patients carry the V600E mutation of the proto-oncogene BRAF. The
synchronous or metachronous development of ECD and Langerhans cell
histiocytosis (LCH) in the same patients is rare, and the possible connection
between them is still obscure. Cladribine is a purine substrate analogue that
is toxic to lymphocytes and monocytes with good hematoencephalic penetration.
Case report. We presented a 23-year-old man successfully treated with
cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and
CNS involvement. ECD developed metachronously, 6 years after chemotherapy for
multisystem LCH with complete disease remission and remaining central
diabetes insipidus. During ECD treatment, the patient received 5 single-agent
chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4
weeks), with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to
severe neutropenia and thoracic dermatomal herpes zoster infection following
the fourth course. Radiologic signs of systemic and CNS disease started to
resolve 3 months after the end of chemotherapy, and CNS lesions completely
resolved within 2 years after the treatment. After 12-year follow-up, there
was no recurrence or appearance of new systemic or CNS xanthogranu-lomatous
lesions or second malignancies. Conclusion. In accordance with our findings
and recommendations provided by other authors, cladribine can be considered
an effective alternative treatment for ECD, especially with CNS involvement
and BRAF V600E-mutation-negative status, when interferon-α as the first-line
therapy fails. |
| first_indexed | 2024-12-13T03:59:12Z |
| format | Article |
| id | doaj.art-d285ad5009714dbd9ad76a3db03a739e |
| institution | Directory Open Access Journal |
| issn | 0042-8450 2406-0720 |
| language | English |
| last_indexed | 2025-03-14T13:03:37Z |
| publishDate | 2016-01-01 |
| publisher | Military Health Department, Ministry of Defence, Serbia |
| record_format | Article |
| series | Vojnosanitetski Pregled |
| spelling | doaj.art-d285ad5009714dbd9ad76a3db03a739e2025-03-02T02:30:35ZengMilitary Health Department, Ministry of Defence, SerbiaVojnosanitetski Pregled0042-84502406-07202016-01-01731838710.2298/VSP140915037P0042-84501500037PSuccessful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosisPerić Predrag0Antić Branislav1Knežević-Ušaj Slavica2Radić-Tasić Olga3Radovinović-Tasić Sanja4Vasić-Vilić Jasenka5Sekulović Leposava6Tarabar Olivera7Tukić Ljiljana8Jovandić Stevo9Magić Zvonko10Military Medical Academy, Clinic for Neurosurgery, Belgrade + University of Defence, Faculty of Medicine of the Military Medical Academy, BelgradeMilitary Medical Academy, Clinic for Neurosurgery, Belgrade + University of Defence, Faculty of Medicine of the Military Medical Academy, BelgradeFaculty of Medicine, Department of Pathology, Novi SadMilitary Medical Academy, Institute for Pathology and Forensic Medicine, BelgradeMilitary Medical Academy, Institute for Radiology, BelgradeMilitary Medical Academy, Institute for Radiology, BelgradeUniversity of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy, Institute for Radiology, BelgradeUniversity of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy, Clinic for Hematology, BelgradeUniversity of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy, Clinic for Hematology, BelgradeMilitary Medical Academy, Institute for Medical Research, BelgradeUniversity of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade + Military Medical Academy,Institute for Medical Research, BelgradeIntroduction. Erdheim-Chester disease (ECD) is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family with characteristic bilateral symmetrical long bone osteosclerosis, associated with xanthogranulomatous extras-keletal organ involvement. In ECD, central nervous system (CNS) and orbital lesions are frequent, and more than half of ECD patients carry the V600E mutation of the proto-oncogene BRAF. The synchronous or metachronous development of ECD and Langerhans cell histiocytosis (LCH) in the same patients is rare, and the possible connection between them is still obscure. Cladribine is a purine substrate analogue that is toxic to lymphocytes and monocytes with good hematoencephalic penetration. Case report. We presented a 23-year-old man successfully treated with cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and CNS involvement. ECD developed metachronously, 6 years after chemotherapy for multisystem LCH with complete disease remission and remaining central diabetes insipidus. During ECD treatment, the patient received 5 single-agent chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4 weeks), with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to severe neutropenia and thoracic dermatomal herpes zoster infection following the fourth course. Radiologic signs of systemic and CNS disease started to resolve 3 months after the end of chemotherapy, and CNS lesions completely resolved within 2 years after the treatment. After 12-year follow-up, there was no recurrence or appearance of new systemic or CNS xanthogranu-lomatous lesions or second malignancies. Conclusion. In accordance with our findings and recommendations provided by other authors, cladribine can be considered an effective alternative treatment for ECD, especially with CNS involvement and BRAF V600E-mutation-negative status, when interferon-α as the first-line therapy fails.http://www.doiserbia.nb.rs/img/doi/0042-8450/2016/0042-84501500037P.pdferdheim-chester diseasehistiocytosis, non-langerhans cellsorbital pseudotumorcentral nervous systembrain stemcerebellumproto-oncogene proteins b-rafcladribinemagnetic resonance imaging |
| spellingShingle | Perić Predrag Antić Branislav Knežević-Ušaj Slavica Radić-Tasić Olga Radovinović-Tasić Sanja Vasić-Vilić Jasenka Sekulović Leposava Tarabar Olivera Tukić Ljiljana Jovandić Stevo Magić Zvonko Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis Vojnosanitetski Pregled erdheim-chester disease histiocytosis, non-langerhans cells orbital pseudotumor central nervous system brain stem cerebellum proto-oncogene proteins b-raf cladribine magnetic resonance imaging |
| title | Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis |
| title_full | Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis |
| title_fullStr | Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis |
| title_full_unstemmed | Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis |
| title_short | Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis |
| title_sort | successful treatment with cladribine of erdheim chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis |
| topic | erdheim-chester disease histiocytosis, non-langerhans cells orbital pseudotumor central nervous system brain stem cerebellum proto-oncogene proteins b-raf cladribine magnetic resonance imaging |
| url | http://www.doiserbia.nb.rs/img/doi/0042-8450/2016/0042-84501500037P.pdf |
| work_keys_str_mv | AT pericpredrag successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT anticbranislav successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT knezevicusajslavica successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT radictasicolga successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT radovinovictasicsanja successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT vasicvilicjasenka successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT sekulovicleposava successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT tarabarolivera successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT tukicljiljana successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT jovandicstevo successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis AT magiczvonko successfultreatmentwithcladribineoferdheimchesterdiseasewithorbitalandcentralnervoussysteminvolvementdevelopingaftertreatmentoflangerhanscellhistiocytosis |