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Langerhans cell histiocytosis with presentation as orbital disease.

Bhanage AB, Katkar AD, Ghate PS - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs.In rare instances, the affection of the orbit is the only and the first symptom.He was treated with salvage therapy for risk patients achieving complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India.

ABSTRACT
Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

No MeSH data available.


Related in: MedlinePlus

Computerized tomography scan after 4 cycles of chemotherapy (progression)
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Figure 4: Computerized tomography scan after 4 cycles of chemotherapy (progression)

Mentions: The patient was categorized as multisystem LCH with risk organ involvement and received 4 cycles of chemotherapy with vinblastine and prednisone. To determine response to therapy, CECT was repeated which showed a large destructive lesion involving central skull base, clivus, pituitary fossa, sphenoid sinus with associated well-defined soft tissue enhancing mass in the skull base. There was opacification of the right nasal cavity with the possibility of dural involvement [Figure 4]. Accordingly, a second course treatment with vinblastine and prednisolone and supportive treatment for Pneumocystis carinii and progressive neutropenia were initiated. On follow-up CECT done after 5 weeks, mandibular involvement was noted. The patient was staged as progressive active disease on treatment. Bone marrow study showed no evidence of infiltration. According to the guidelines, salvage therapy for risk patients with 2-chlorodeoxyadenosine was initiated. Response to therapy was assessed after 3 cycles that showed regression of the lesions on magnetic resonance imaging (MRI) brain with contrast. Three more cycles were given and MRI done showed disease remission [Figure 5]. The patient is in remission and doing well on follow-up at 4 years.


Langerhans cell histiocytosis with presentation as orbital disease.

Bhanage AB, Katkar AD, Ghate PS - J Pediatr Neurosci (2015 Apr-Jun)

Computerized tomography scan after 4 cycles of chemotherapy (progression)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489065&req=5

Figure 4: Computerized tomography scan after 4 cycles of chemotherapy (progression)
Mentions: The patient was categorized as multisystem LCH with risk organ involvement and received 4 cycles of chemotherapy with vinblastine and prednisone. To determine response to therapy, CECT was repeated which showed a large destructive lesion involving central skull base, clivus, pituitary fossa, sphenoid sinus with associated well-defined soft tissue enhancing mass in the skull base. There was opacification of the right nasal cavity with the possibility of dural involvement [Figure 4]. Accordingly, a second course treatment with vinblastine and prednisolone and supportive treatment for Pneumocystis carinii and progressive neutropenia were initiated. On follow-up CECT done after 5 weeks, mandibular involvement was noted. The patient was staged as progressive active disease on treatment. Bone marrow study showed no evidence of infiltration. According to the guidelines, salvage therapy for risk patients with 2-chlorodeoxyadenosine was initiated. Response to therapy was assessed after 3 cycles that showed regression of the lesions on magnetic resonance imaging (MRI) brain with contrast. Three more cycles were given and MRI done showed disease remission [Figure 5]. The patient is in remission and doing well on follow-up at 4 years.

Bottom Line: Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs.In rare instances, the affection of the orbit is the only and the first symptom.He was treated with salvage therapy for risk patients achieving complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India.

ABSTRACT
Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

No MeSH data available.


Related in: MedlinePlus