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Multisistemik Langerhans Hücreli Histiyositozis Hastalığında BT ve MR Bulguları

CT and MRI finding of Langerhans Cell Histiocytosis Disease

Journal Name:

  • Selçuk Tıp Dergisi

Publication Year:

  • 2011

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Keywords (Original Language):

Author NameUniversity of Author
Abstract (2. Language): 
Langerhans cell histiocytosis is a group of idiopathic disorders characterized by the abnormal proliferation of specialized bone marrow-derived Langerhans cells. In this report, we present a rare case of Langerhans cell histiocytosis with CT and MRI in a 2 yearold-boy who developed symptomatic diabetes insipidus and multiple bone ,cranial, lung and spleen metastases during the disease course. A 2-year-old male patient was hospitalized due to complaints of cough, fever, vomitting, diarrhea, achromasia, weakness and rash. Chest X-ray revealed reticulonodular infiltration in both lung fields. Thorax CT revealed diffuse micro and macro nodular type infiltration in both lung parenchyma. On CT and MRI revealed common lytic lesion of skull, vertebra and iliac bone. There are infundibular thickening and absence of posterior pituitary intensity on MRI of pituitary gland. The spleen involvoment is determined by abdominal CT. The diagnosis is confirmed with biopsy of iliac bone. Langerhans cell histiocytosis should be in the differential diagnosis in children having widespread lung, bone, pituitary gland, and spleen involvement on MRI and CT.
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Abstract (Original Language): 
Langerhans hücreli histiyositoz, kemik iliği kökenli Langerhans hücresinin anormal çoğalması ile karakterize ve nedeni bilinmeyen bir hastalık grubudur. Bu çalışmada nadir görülen akciğer, yaygın kemik, hipofiz, dalak tutulumu olan ve biyopsi sonucu Langerhans hücreli histiyositoz tanısı kesinleşen iki yaşındaki erkek olgunun BT ve MR bulguları sunulmuştur. Öksürük, ateş, kusma, ishal, solukluk, halsizlik, ve ciltte döküntü yakınmaları ile hastaneye başvuran 2 yaşındaki erkek çocuğun akciğer grafisinde her iki akciğerde retikülonodüler infiltrasyon gözlendi. Toraks BT’de akciğer parankim alanlarında yaygın mikro ve makronodüler karakterde infiltratif lezyonlar ile direkt grafi, BT ve MR tetkiklerinde kafatasında, vertebralarda, iliak kemiklerde yaygın litik lezyonlar görüldü. Hipofiz MRG’de ise T1A görüntülerde nörohipofize ait hiperintensite izlenmedi ve stalkta hafif kalınlaşma vardı. Batın BT’de ise dalakta hipodens nodül saptandı. İliak kemikten yapılan biyopsi ile Langerhans hücreli histiyositoz tanısı kesinleşti. BT ve MRG’de yaygın akciğer,kemik,hipofiz bezi,dalak tutulumu tespit edilen olgularda Langerhans hücreli histiyositoz ayırıcı tanıda mutlaka düşünülmelidir.
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  • 2
118-120
  • Turkish

REFERENCES

References: 

1. Gadner H, Heitger A, Grois N, Gatterer-Menz I, Ladisch
S. Treatment strategy for disseminated Langerhans cell
histiocytosis. Medical and Pediatric Oncology 1994;23:72-80.
2. Marioni G, De Filippis C. Langerhans’ cell histiocytosis:
Temporal bone involvement. The Journal of Laryngology &
Otology 2001;115:839–41.
3. Schmidt S, Georg Erich G. Extra-osseous involvement of
Langerhans’cell histiocytosis in children. Pediatr Radiol
2004;34:313–21.
4. Hadjigeorgi C, Parpounas C, Zarmakoupis P, Lafoyianni S.
Eosinophilic granuloma of the temporal bone: Radiological
approach in the pediatric patient. Pediatric Radiology
1990;20:546-9.
5. Lipton JM. Histiocytic disorders. In: Hoofman R, Benz EJ,
Shattil SJ, Furie B, Cohen HJ, Silberstein LE, McGlave P, eds.
Basic Principals and Practice. 3rd ed. New York: Churchill
Livingstone; 2000.p.783-93.
6. Gadner H, Grois N. Langerhans cell histiocytosis. In: PA
Voute, CA Kalifa, A Barrett, eds. Cancer in Children: Clinical
Management. Oxford University Press, Oxford, 1998, p.154-
169.
7. Helmbold P, Hegemann B, Holzhausen HJ, Klapperstuck
T, Marsch WC. Low-dose oral etoposide monotherapy in
adult Langerhans cell histiocytosis. Arch Dermatology
1998;134:1275-8.
8. Kilborn T.N, Teh J, Goodman T.R. Pediatric Manifestations
of Langerhans cell histiocytosis: a review of the clinical and
radiologic findings. Clinical Radiology 2003;58:269-278.
9. Meyer J.S, De Camargo B. The role of radiology in the diagnosis
and follow-up of Langerhans cell histiocytosis. Hematology/
Oncology Clinics of North America 1998;12:307-326.
10. Ödev K. Toraks Radyolojisi. Teknik, Klinik, Bulgular. İstanbul,
Nobel kitabevi;2005:253-56.
11. Grois N, Prayer D, Prosch H, Minkov M, PötschgerU, Gadner
H. Course and clinical impact of magnetic resonance imaging
findings in diabetes insipidus associated with Langerhans cell
histiocytosis. Pediatric Blood and Cancer 2004;43:59-65.

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