Hadi GEYLAN, Kamuran KARAMAN, Mecnun ÇETİN, Akkız Şahin YAŞAR

Kemoterapi İlişkili Akut Solunum Yetmezliği: Relaps Langerhans Hücreli Histiositozlu Bir Çocuk

Langerhans hücreli histiositoz LHH , CD1a, langerin ve S100 proteini eksprese eden ve ultrastriktürel incelemede Birbeck granülleri varlığı gösterilen, Langerhans tipi hücrelerin klonal neoplastik proliferasyonudur. Hastalık, klinik olarak çok değişken bir seyir gösterir. En fazla kemik, kemik iliği, cilt, lenf nodları, akciğer, karaciğer, dalak ve meninksler tutulur. Çoğu vaka çocukluk çağında tanı almaktadır, yıllık insidans yaklaşık olarak 5 milyonda birdir. Tutulum bölgesi ve lezyon sayısına göre en az yan etkisi olan ajan ile tedavi seçilmelidir. Hastaların çoğu başlangıç tedavisine yanıt verir. Son 3,5 yıldır Langerhans hücreli histiositoz tanısı ile takipli iken; idame kemoterapinin bitiminden yaklaşık iki yıl sonra hasta santral diabetes insipitus kliniği ile tarafımıza başvurdu. Yapılan görüntüleme tetkikleri neticesinde relaps Langerhans hücreli histiositoz tanısı konuldu. Hastaya kladribin ile sitarabin içeren kemoterapi protokolü başlandı. Bu tedavinin üçüncü küründen sonra, febril nötropenisi düzeldikten sonra ani gelişen ve hızlı ilerleyen solunum sıkıntısının eşlik ettiği pnömoni gelişti. Burada kladribin ve/veya sitarabin kemoterapilerden sonra ani gelişen solunum yetmezliği olan ve steroid tedavisine dramatik cevap alınan bir olgu sunulmuştur

Chemotherapy Associated Acute Respiratory Failure: a Child With Relapsed Langerhans Cell Histiocytosis

Langerhans cell histiocytosis LHH ; is a clonal neoplastic proliferation of Langerhans-type cells with expressing CD1a, langerin and S100 protein and presence of Birbeck granules in the ultrastructural examination. Clinical manifestations are very variable. The bone, bone marrow, skin, lymph nodes, lung, liver, spleen and meninges are most involved. Most cases are diagnosed during childhood and the annual incidence is approximately 5 million. The agent should be selected with the least side effect according to the number of lesions and the area of invasion. Most patients respond to initial treatment. In the last 3.5 years, with the diagnosis of LHH, approximately two years after the end of chemotherapy, the patient referred to our hospital with a central diabetes insipidus. Relapse Langerhans cell histiocytosis was diagnosed as a result of imaging studies. Chemotherapy protocol with cladribine and cytarabine was started for the patient. After the third session/round/cycle of the treatment, pneumonia accompanied by rapid onset of respiratory distress developed after the febrile neutropenia recovered. Here we present a case of sudden respiratory insufficiency after cladribine and/or cytarabine chemotherapy and a dramatic response to steroid treatment

Keywords:

Chemotherapy, Langerhans cell histiocytosis LHH, pneumonia,

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