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Miyeloproliferatif Neoplazilerde Nörolojik Bulgular

Miyeloroliferatif neoplaziler (MPN) pluripotent kök hücre düzeyinde genetik bozukluklardan kaynaklanan, her biri kendine özgü fenotipik, genetik ve klinik özellikleri olan klonal kök hücre hastalıklarıdır. MPN"li olgularda ortaya çıkan semptom ve komplikasyonlarda özellikle nörolojik olaylarda yaş önemli bir faktördür.Kronik miyelositik lösemi olgularında beyaz küre sayısının çok fazla olmasına bağlı (lökostaz) santral sinir sistemi (SSS) bulgularına yol açabilir. İlk tanıda veya hastalığın seyri sırasında kemik iliği dışında yani ekstra medüller alanlarda ekstramedüller miyeloid tümörler (EMMT) olarak isimlendirilen ve lösemik hücrelerin oluşturduğu kitleler diğer doku ve organların yanı sıra gerek SSS"inde gerekse spinal korda bası etkisine yol açan kitleler nedeniyle nörolojik semptom ve bulgulara yol açar. Santral sinir sistemi tutulumu ve trombositopenik kanamalar nörolojik semptom ve bulgulara yol açabilir.Polisitemia verada geçici iskemik atak ve trombotik inme en sık bulgulardır. Tromboz ve hemoraji yanısıra akut lösemiye transfromasyon nörolojik bulgu ve belirtilere yol açabilir. Esansiyel trombositozda geçici iskemik atak, trombotik inme ve zellikle hemoraji diğer MPN"lerdekine benzer nörolojik semptomlara yol açabilir. Miyelofibroziste ekstramedüller hematopoez vücutta yaklaşık her organ/dokuda ortaya çıkan kemik iliği dışındaki kan yapımı odaklarıdır. EMH odakları intrakranyal bası yapan kitle ve spinal kord kompresyonuna, nöbetlere, hidrosefaliye yol açabilir. Nadir olmakla birlikte kranyal/spinal meninkslerde, paraspinal dokuda ve intraserebral lokalizasyonda EMH saptanabilir. Santral ve periferik sinirlerde, koroid pleksusta, pituiter bezde, orbitada, orbital ve lakrimal fossada, sfenoid sinusta EMH rapor edilmiştir.

Anahtar Kelimeler:

Miyeloproliferatif neoplazi, nörolojik bulgular, onkoloji

Neurological Findings in Myeloproliferative Neoplasms

Myeloproliferative neoplasms (MPN) arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bone marrow named as extramedullary myeloid tumors (EMMT) could be detected at the initial diagnosis or during the prognosis of the disease, which may cause neurological symptoms due to pressure of leukemic cell mass on various tissues along with spinal cord. Central nervous system involvement and thrombocytopenic hemorrhage may lead to diverse neurological symptoms and findings.Transient ischemic attack and thrombotic stroke are the most common symptoms in polycythemia vera. Besides thrombosis and hemorrage, transformation to acute leukemia can cause neurological symptoms and findings. Transient ischemic attack, thrombotic stroke and specifically hemorrage can give rise to neurological symptoms similar to MPN in essential thrombocytosis.Extramedullary hematopoiesis refers to hematopoietic centers arise in organ/tissues other than bone marrow in myelofibrosis. Extramedullar hematopoietic centers may cause intracranial involvement, spinal cord compression, seizures and hydrocephalia. Though rare, extramedullary hematopoiesis can be detected in cranial/spinal meninges, paraspinal tissue and intracerebral regions. Extramedullary hematopoiesis has been reported in peripheral neurons, choroid plexus, pituitary, orbits, orbital and lacrimal fossa and in sphenoidal sinuses.

Keywords:

Myeloproliferative neoplasms, neurological findings, oncology,

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