A 32-year-old Greek woman, para 1, at the 22nd week + day 3 of gestation, was referred to the A&Es with a 1-week history of abdominal discomfort and persistent vomiting. The woman had HbH disease (thalassemia intermedia) with a - a 3.7/Hb Icaria a genotype. She was splenectomized at the age of 6.5 years and she maintained a Hb around 8 g/dl without blood transfusions. Her medical history included incidents of superficial-vein thrombosis in 2005, 2008, and 2009 (with negative family history of thrombosis) for which she was receiving acenocoumarol. Since the confirmation of pregnancy she was receiving Salospir 100 mg p.o. and Enoxaparin 40 mg sc.
Laboratory exams revealed moderate hypochromic microcytic anemia (Ht 28.1%, Hb 8.46 g/dL, MCV: 67.2, and MCH: 20.2), mild thrombocytosis (PLTs: 519 x 109/L), and leukocytosis: WBC 27 x 109/L (neu: 87%, lymph: 9%, and mono: 4%). The C-reactive protein was 11.4 mg/L. INR and aPTT were within normal limits but fibrinogen was high, 519.9 mg/L, and D-dimers were >5000 mug/L. The rest of the biochemistry showed the following: TBil: 1.50 mg/dl; dBil: 0.8 mg/dl; SGOT 110 IU/L; SGPT 176 IU/L; ALP 165 IU/L; gamma-GT 46 IU/L; and ferritin: 63 ng/ml, indicative of hepatic impairment. An abdominal ultrasound revealed portal vein and spleen-vein thrombosis. The patient underwent a thrombophilia testing which was negative for hereditary thrombophilia (FV Leiden, FIIG20210A) as well as for anticardiolipins antibodies. Because of the site of the thrombosis (visceral), latent myeloproliferative neoplasms (MPN) were also excluded (she was negative for the JAK2V617F mutation).
The patient was treated with enoxaparin 60 mg bd, sc, and Salospir 100 mg p.o. o.d. Transaminases were normalized two weeks later and a new abdominal ultrasound, performed two months later, showed chronic portal vein and chronic spleen-vein thrombosis.
At the 35th week + day 3, the liver enzymes and the rest of the biochemistry were as follows: SGOT 466 IU/L, SGPT 931 IU/L, ALP 220 IU/L, gamma-GT 41 IU/L, TBil: 1.80 mg/dl, Ht 38%, Hb 10.2 g/dL, WBC 12.3 x 109/L, and PLTs: 487 x 109/L. The abdominal ultrasound showed no new findings. With the clinical suspicion of partial HELLP syndrome (the patient had only elevated liver enzymes) an urgent caesarian section (C-section) was performed. Anticoagulation bridging with heparin was used perioperatively. During the C-section, the patient developed hypertension episode [blood pressure: 190 (systolic)/ 110 (diastolic) mmHg] and she was treated with hydralazine. The premature newborn was 1810 gr but healthy and stayed in the neonatal ICU for 7 days.
Mother's liver enzymes were normalized within five days after the delivery and the patient was discharged. The patient is now on long-term anticoagulant therapy with acenocoumarol. The imaging of splenic veins is unchanged.