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Innere Medizin 13. Oktober 2015

Clinical-Pathological Conference Series from the Medical University of Graz Case No 155: 26-year-old woman in third trimester of pregnancy with epigastric pain and thrombocytopenia

Presentation of case
Dr. F. Eisner

Due to epigastric pain, the unemployed pregnant woman had been examined at the outpatient clinic of the Styrian Public Health Service 2 months earlier. At that time, all laboratory tests were reportedly within normal limits and the epigastric pain was said not to be unusual during pregnancy. However, when the symptoms became more severe, she presented at the Obstetrics Outpatient Clinic at the University Medical Center in Graz, where thrombocytopenia with a platelet count of 9 G/l (normal: 140–440 G/l) was diagnosed. Other laboratory test results were: leukocytes 15.6 G/l (normal: 4.4–11.3 G/l), erythrocytes 2.4 T/l (normal: 4.1–5.1 T/l), hemoglobin (Hb) 7.8 g/dl (normal: 12.0–15.3 g/dl), hematocrit (Htc) 20.9 % (normal: 35–45 %), mean corpuscular volume (MCV) 86.7 fl (normal: 80–98 fl); differential blood count: 78 % neutrophils (normal: 50–75 %), 13 % lymphocytes (normal: 20–40 %); 100 ‰ reticulocytes (normal: 5–20 ‰), haptoglobin < 0.08 g/l (normal: 0.3–2.0 g/l), Hb in plasma 11.7 mg/dl (normal: < 5 mg/dl), total bilirubin 1.45 mg/dl (normal: 0.1–1.2 mg/dl), alkaline phosphatase (AP) 146 U/l (normal: 35–105 U/l), gamma-glutamyl-transpeptidase (GGT) 22 U/l (normal: < 38 U/l), aspartate-aminotransferase (AST) 86 U/l (normal: < 30 U/l), alanine-aminotransferase (ALT) 15 U/l (normal: < 35 U/l), creatinine 1.25 mg/dl (normal: < 1.0 mg/dl), urea 55 mg/dl (normal: 10–45 mg/dl), uric acid 7.8 mg/dl (normal: 2.4–5.7 mg/dl), glomerular filtration rate (GFR) 59 ml/min/l (normal: 80–140 ml/min/l), creatine kinase (CK) 249 U/l (normal: < 145 U/l), lactate dehydrogenase (LDH) 1719 U/l (normal: 120–240 U/l), glucose 92 mg/dl (normal: 70–115 mg/dl), prothrombin time (PT) 100 %, PT-INR 1.01, activated partial thromboplastin time (APTT) 28.5 s (normal: 26.0–36.0 s), fibrinogen 409 mg/dl (normal: 210–400 mg/dl), antithrombin (AT) 62 % (normal: > 75 %); C-reactive protein (CRP) 17.4 mg/l (normal: < 5 mg/l); urine test tape: Hb markedly increased, urinary protein 1377 mg/l (normal: < 130 mg/l), and 2025 mg/g creatinine (normal: < 110 mg/g creatinine). Since HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome was the suspected diagnosis for this primipara in her 36th week of pregnancy, after infusion of two platelet concentrates and one unit of packed red cells, a cesarian section was performed and a healthy boy delivered. There were no complications and afterwards the platelet count was 48 G/l, but there was no further improvement of her thrombocytopenia. Four days post partum laboratory tests showed: platelets 23 G/l, leukocytes 15.2 G/l, erythrocytes 2.6 T/l, Hb 8.1 g/dl, Htc 22.5 %, MCV 85.9 fl, reticulocytes 113 ‰, creatinine 1.15 mg/dl, urea 61 mg/dl, uric acid 8.9 mg/dl, GFR 65.4 ml/min/l, total bilirubin 1.8 mg/dl, AP 96 U/l, GGT 21 U/l, AST 83 U/l, ALT 21 U/l, CK 361 U/l, LDH 2331 U/L, and CRP 23.6 mg/l; fragmented red blood cells were seen on a blood smear. Plasmapheresis was planned and the patient was transferred to the ICU. When serum creatinine increased to 4 mg/dl, hemodialysis was ordered. Serum LDH increased to 3133 U/l; several immunopathological parameters (antinuclear antibodies (ANA), antineutrophil cytoplasmic auto-antibodies against myeloperoxidase (ANCA-MPO) and proteinase 3 (ANCA-Pr3), antibodies against double-strand DNA, mitochondria, smooth muscle cells, and basal membrane) and blood cultures were negative. Daily plasmapheresis (for 8 days) did not raise the platelet count. The patient was increasingly agitated and difficult to calm down. Computer tomography of the head was unremarkable. The patient developed progressive cardiorespiratory instability (troponin-T 2530 pg/ml, normal < 14 pg/ml) and suffered repeated apnea.

Elisabeth Fabian, Florian Eisner, Ingrid Pabinger, Christian Viertler, Sigrid Regauer, Andreas Lueger, Peter Neumeister, Eva-Christine Weiss, Wolfgang Schöll, Uwe Lang, Florian Prüller, Guenter J. Krejs, Wiener klinische Wochenschrift 17/18/2015

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