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FAMİLYAL POLİSİTEMİ TANISI İLE TAKİP EDİLEN İKİ KARDEŞ

FAMILIAL POLYCYTHEMIA IN TWO BROTHERS

Journal Name:

  • İstanbul Tıp Fakültesi Dergisi

Publication Year:

  • 2010

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Familial polycythemia (FP), namely primary familial congenital polycythemia is a rare automosal dominant disorder. FP is characterized by isolated erythrocytosis without evolution into leukemia or other myeloproliferative disorders, absence of splenomegaly, normal white blood cell and platelet counts, low plasma erythropoietin levels and hypersensitivity of erythroid progenitors to exogenous erythropoietin in vitro. We report here two brothers referred to our Hematology Department one of them was 22 years old the and other was 17. They had erytrocytosis and no other pathological findings. After excluding other diseases presenting with polycythemia we diagnosed familial polycythemia. We continue to treat with flebotomy when they are symptomatic.
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Abstract (Original Language): 
Familyal polisitemi (FP), tam ismiyle primer familyal konjenital polisitemi nadir görülen otozomal dominant bir hastalıktır. Ġzole eritrositoz ile seyreden bu hastalık; lösemi ile diğer myoleproliferatif hastalıklara dönüĢümün ve splenomegalinin olmaması, normal lökosit ve trombosit sayısı, düĢük eritropoetin düzeyi, in vitro koĢullarda eksojen eritropoetine eritroid progenitörlerin hipersensitivitesi ile karakterizedir. Bu yazıda Hematoloji Polikliniğimize yönlendirilen, izole eritrositoz dıĢında klinik ya da laboratuar bulgusu olmayan, biri 22 diğeri 17 yaĢında iki kardeĢ sunulmuĢtur. Polisitemiye neden olan diğer hastalıklar dıĢlandıktan sonra familyal polisitemi tanısı koyulmuĢtur. Hastalar semptomatik olduklarında flebotomi tedavisi yapılmaktadır.
FULL TEXT (PDF): 
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  • 1
31-32
  • Turkish

REFERENCES

References: 

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