Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria with marrow hypoplasia
Journal Title: Αρχεία Ελληνικής Ιατρικής - Year 2013, Vol 30, Issue 5
Abstract
A 34-year-old man was admitted to the hospital because of severe abdominal pain. The pain was continuous and started one day before admission, causing no changes in the stool and accompanied with nausea and vomiting. The patient had also complained about progressive weakness, fatigue, dyspnoea on slight exertion, dizziness, headache and arthralgias for the last 4 months. His past medical and family history was unremarkable. On admission the physical examination revealed jaundice and a generalized sensitivity in the examination of the abdomen with signs of peritoneal reaction in the upper abdomen. The patient’s temperature was 37.8 oC, the blood pressure was 145/95 mmHg and the pulse rate was 102/min. The liver was enlarged, 3 cm below the costal margin, and very sensitive. trephine biopsies were performed and were diagnostic. The osmotic fragility and the autohemolysis tests were within normal limits. The leukocyte alkaline phosphatase was almost absent. Caryotype was normal. The detection for CD55 and/or CD59 deficient red cell population in the peripheral blood, using the sephacryl gel microtyping system, revealed a 75% reduction of both antigens from the membrane surface (fig. 4). The diagnosis was established and the disease had a chronic course despite the appropriate treatment. The spleen was also palpable (2 cm below costal margin), but not painful while there was no peripheral lymphadenopathy. His hematological profile was as follows: WBC 1.9×109/L (differential count: Neutrophils 27%, lymphocytes 62%, monocytes 11%), Hb 8.2 g/dL, Ht 26.8%, and platelet count 94×109/L. The reticulocyte count was 0.2% and ESR was 62 mm/1 hour. The peripheral blood smears morphology is shown in figure 1. Serum biochemistry was as follows: Urea 72 mg/dL, creatinine 2.1 mg/dL, Na+ 138 mEq/L, K+ 5.4 mEq/L, Ca++ 4.9 mEq/L , SGOT 178 IU/L, SGPT 345 IU/L, γGT 181 IU/L, alkaline phosphatase 622 IU/L, bilirubin 4.2 mg/dL (conjugated 2.9 mg/dL) and LDH 340 IU/L. A mild prolongation in bleeding time (INR 2.1, aPTT 62 sec) was also observed while the fibrinogen degradation products were slightly increased. Chest X-rays and the ECG were normal. The ultrasonography of the abdomen revealed hepatomegaly, hepatic vein thrombosis with moderate ascites. The appropriate anti-coagulant medication was administered and the symptoms were vanished three weeks after the initiation of the treatment. Bone marrow aspiration findings are shown in figures 2 and 3;
Authors and Affiliations
J. ASIMAKOPOULOS, L. PAPAGEORGIOU, K. PETEVI, G. BOUTSIKAS, A. KANELLOPOULOS, P. FLEVARI, E. KOUTSI, V. TELONIS, T. VASSILAKOPOULOS, M. ANGELOPOULOU
Keywords
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