Paroxysmal nocturnal hemoglobinuria presenting with hemolysis and abdominal pain

INTRODUCTION: Abdominal pain, fatigue, erectile dysfunction, headache, back pain, dysphagia,renal insufficiency, vascular occlusion or hemolysis might be seen in PNH.
CASE: A 26-year-old male patient presented to the hospital with fatigue and abdominal pain. The patient complained of an abdominal pain that was not related with the meals. He had intermittent abdominal pain in the epigastric region for a few months. Labaratory values were as Hemoglobin: 10,04 mg / dL, WBC: 5100 /, creatinine: 0.62 mg / dL, sedimentation: 38 mm / h, CRP: 19.6, INR: 1.22, platelets: 143 thousand, direct coombs: negative, indirect coombs: negative, LDH: 964,8 U / L, indirect bilirubin: 1,3 mg / dL, reticulocyte count: 179 thousand, transferrin saturation: 15,6 %. Vitamin B12, folic acid and ferritin levels were with in normal limits. Peripheral blood smear showed an increase in lymphoplasmacytic cells, an increase in monocytes, no fragmented erythrocytes and no atypical cells or blasts. PNH test was done by flowcytometry to the patient with non-immun hemolytic anemia. Flowcytometric analysis showed the PNH clone in 95% of granulocytes, in 96% of monocytes and in 5.9% of erythrocytes (Type II + Type III). It has been interpreted that the clonal PNH rate difference between the white cells and erythrocytes in the flow cytometric analysis might be due to hemolysis and/or transfusion. The patient was started to enoxaparine 2x0,6 cc subcutaneously treatment due to the result of D-dimer value which was found as 1350. Eculizumab treatment was started. His symptoms have improved and his complaints have disappeared after eculizumab treatment.
DISCUSSION: If a patient with an abdominal pain had hemolysis as in our case, PNH should be kept in mind. Rapid eculizumab treatment would benefit these patients.

Hemoliz ve karýn aðrýsýyla ortaya çýkan paroksismal noktürnal hemoglobinüri

GÝRÝÞ: PNH da vücudun sýk tromboz görülmeyen bölgelerinde tromboz görülebilir. Karýn aðrýsý, halsizlik, erektil disfonksiyon, baþ aðrýsý, sýrt aðrýsý, yutma güçlüðü, damar týkanýklýðý veya hemoliz bulgularý görülebilir.
OLGU: 26 yaþýnda erkek hasta halsizlik ve karýn aðrýsý þikayeti ile hastaneye baþvurdu. Hastanýn epigastrik bölgede birkaç aydýr olan yemeklerden baðýmsýz ve kendi kendine gelip geçici tarzda aralýklý olan bir karýn aðrýsý þikayeti de mevcuttu. Hastanýn labaratuvar deðerlerinde: Hemoglobin: Hgb: 10,04 mg/ dL, WBC: 5100/µl, LDH: 964,8 U/L, indirekt bilirubin: 1,3 mg/ dL retikülosit sayýsý: 179 bin, transferrin saturasyonu: % 15,6, kreatin: 0,62 mg/dL, sedimantasyon: 38 mm/saat, CRP: 19,6 INR: 1,22 trombosit: 143 bin, direkt coombs: negatif, indirekt coombs: negatif. Vitamin B12, folik asit ve ferritin düzeyleri ise normal sýnýrlarda idi. Periferik yaymada lenfoplazmositer hücre artýþý mevcuttu, monositlerde artýþ mevcuttu, fragmente eritrosit izlenmedi, atipik hücre veya blast izlenmedi. Non-immün hemolitik anemisi mevcut olan hastaya PNH testi istenildi. PNH testi sonucu: Flow sitometrik analizde granülositlerde %95, monositlerde %96, eritrositlerde %5,9 ( TIP II+ TIP III ) PNH klonunun varlýðýný göstermektedir. Flow sitometrideki beyaz kürelerle eritrositlerin klon büyüklükleri arasýndaki farkýn hemolize ve/veya transfüzyona baglý olabileceði yorumunda bulunulmuþ. d-dimer: 1350 olan hastaya clexan 2x0,6 cc baþlanýldý.Hastaya eculizumab tedavisi baþlanýldý. Eculizumab tedavisi sonrasýnda hastanýn þikayetleri geriledi, semptomlarý duzeldi.
TARTIÞMA: Bizim olgumuzda da olduðu gibi karýn aðrýsý olan bir hastada eþlik eden hemoliz bulgusu varsa mutlaka PNH tanýsý da ayýrýcý tanýda düþünülmelidir. Hastalara hýzlý bir þekilde baþlanacak ekullizumab tedavisi ile tromboembolik riskin de azaltýlmasý saðlanýp saðkalým süresinin uzamasý saðlanabilecektir.