Brusellozisin Tetiklediği Herediter Sferositoz: Folik Asit Eksikliği, Hemolitik ve Megaloblastik Kriz

Yıl 2015, Cilt: 9 Sayı: 4, 280 - 282, 01.12.2015

Zafer Bıçakcı

Öz

Herediter sferositoz kalıtsal hemolitik anemiler içinde en sık görülen hemolitik anemi nedenidir. Herediter sferositozu olan hastalarda nonspesifik enfeksiyonların (çoğunlukla viral) tetiklemesi ile hemolizin artması sonucu var olan anemi, sarılık ve splenomegali artar. Brusellozu olan hastalarda da anemi, sarılık ve splenomegali gelişebilir. Aynı klinik belirti ve bulguları gösteren herediter sferositoz ve brusellozis birbiri ile karışabilir. Bu nedenle brusellozun tetiklediğini düşündüğümüz, herediter sferositoz komplikasyonu (folik asit eksikliği, hemolitik ve megaloblastik kriz) gelişen bir hasta sunulmuştur. On altı yaşında erkek hastada baş ağrısı, terleme, solukluk, sarılık ve splenomegali vardı. Lökosit 7600/μL, Hb 11 g/dL, Hct % 27.1, trombosit 215.000 μ/L, MCV 92.2 fL, RDW %18.3, MHC 37.3pg, MHCH 40.7 g/dL retikülosit %8 (düzeltilmiş retikülosit %5)’di. Periferik yaymada çok sayıda makrosit ve mikrosferositler gözlendi. Haptoglobin ve folik asit düzeylerinde düşüklük, total bilirubin ve LDH düzeylerinde yükseklik, osmotik frajilitede artış tespit edildi. Brusella tüp aglitünasyonu 1/320 olarak bulundu. Hastaya folik asit, rifampisin ve doksitetrasiklin başlandı. Tedavi sonrası şikayetleri kaybolan, dalak boyutları küçülen hastanın folik asit düzeyi normale döndü.Herediter sferositoz belirti ve bulguları daha çok nonspesifik (viral) enfeksiyonların tetiklemesi ile açık hale gelmektedir. Özellikle brusellozisin endemik olduğu bölgelerde (folik asiti düşüklüğü, anemisi ve splenomegalisi bulunan hastalarda), herediter sferositozun spesifik bir tetikleyicisi olarak brusellozis de akla getirilmelidir.

Anahtar Kelimeler

Anemi, Brusellozis, Folik asit, Herediter, Sarılık, Sferositoz, Splenomegali

Hereditary Spherocytosis Triggered by Brucellosis: Folic Acid Deficiency with Hemolytic and Megaloblastic Crisis

Öz

Hereditary spherocytosis is the most common hereditary hemolytic anemia. Anemia, jaundice and splenomegaly develop in hereditary spherocytosis patients as a result of the increased hemolysis following the triggering action of non-specific infections (mostly viral). Anemia, jaundice and splenomegaly may develop in patients with brucellosis as well. Hereditary spherocytosis can be confused with brucellosis as both have the same clinical signs and symptoms. We present a patient who developed hereditary hereditary spherocytosis (folic acid deficiency with hemolytic and megaloblastic crisis) that we believe was triggered by brucellosis. A 16-year-old patient presented with headache, sweating, pallor, jaundice and splenomegaly. Laboratory tests revealed a leukocyte count of 7600/µL, Hb 11 g/dL, Hct 27.1%, platelets 215,000 µ/L, MCV 92.2 fL, RDW 18.3%, MHC 37.3 pg, MHCH 40.7 g/dL, and reticulocytes 8% (corrected reticulocytes 5%). Peripheral blood smear showed a large number of macrocytes and micro-spherocytes. There was a decrease in haptoglobin and folic acid, increase in total bilirubin and LDH and increase in osmotic fragility. Brucella tube agglutination was 1/320. The patient was started folic acid, rifampicin and doxytetracycline. The symptoms disappeared, spleen dimensions regressed and the folic acid level returned to normal with treatment.In conclusion, the symptoms and signs of hereditary spherocytosis can become prominent with a triggering nonspecific infection (viral). Brucellosis should also be considered a specific trigger of hereditary spherocytosis (in patients with anemia, splenomegaly and low folic acid), especially in areas where brucellosis is endemic

Anahtar Kelimeler

Anemia, Brucellosis, Folic acid, Hereditary, Jaundice, Spherocytosis, Splenomegaly

Kaynakça

• Grace RF, Lux SE. Disorders of the red cell membrane. In: Orkin SH, Nathan DG, Ginsburg D, Look AT, Fisher DE, Lux SE (eds). Nathan and Oski’s Hematology of Infancy and Childhood, 7th ed. Philadelphia: Saunders Elsevier, 2009:659-839.
• Solera J. Update on brucellosis: Therapeutic challenges. Int J Antimicrob Agents 2010;36:18-20.
• Abbas AK, Lichtman AH, Pillai S. Cytokines. In: Abbas AK, Lichtman AH, Pillai S (eds). Cellular and Molecular Immunology. Philadelphia (PA): Saunders, 2007:267-301.
• Mori H, Takahashi N, Tada J, Higuchi T, Shimizu T, Harada H, et al. Megaloblastic anemia due to folate deficiency associated with hereditary spherocytosis. Rinsho Ketsueki 1994;35:397-402.
• Blecher TE. What happens to the microspherocytosis of hereditary spherocytosis in folate deficiency? Clin Lab Haematol 1988;10: 403-8.
• Tsuboyama A, Sasaki R, Takaku F, Mizoguchi H, Miura Y. Megaloblastic anemia due to folic acid deficiency observed in a pregnant women with hereditary spherocytosis (author’s transl). Rinsho Ketsueki 1976;17:1122-7.