Paroxysmal nocturnal hemoglobinuria presenting with hemolysis and abdominal pain

Abstract

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.