mycophenolic-acid and Thrombophilia

Thromboembolic complications are found in 2-3% of children with nephrotic syndrome (NS); this increased risk is caused by hypovolemia, hemoconcentration, increased number and activity of platelets, hyperfibrinogenemia and loss of coagulation inhibitors. Risk is even higher in case of additional factors e.g. congenital thrombophilia.. Girl with NS aged 17 11/12 years was admitted to hospital due to respiratory tract infection with cough and back pain. NS started 9 months earlier and she had two bouts of disease, and was treated only with prednisone (current dose - 60 mg/48h). On admission she was without any abnormalities on auscultation, with BP 111/65 mmHg, HR 80 bpm, satO2 99%. Lab results showed the increase of WBC 18.3×103/μL, D-dimers 23038 μg/L and proteinuria 900 mg/dL. Other values of examined parameters were in normal limits. Chest X-ray and ECG were also normal. Presumptive diagnosis of pulmonary embolism was made and the patient was given 1000IU of antithrombin III and nadroparine (2x90IU/kg/24h s.c.). In ECHO the occlusion of left pulmonary artery and preserved blood flow in right were revealed. In angioCT clot nearly filling lumen of left pulmonary artery, clot in intermediate part of right pulmonary artery, and focus of pulmonary infarction in 10th segment of left lung were found. Doppler USG of lower limb veins did not reveal thrombi or perforator vein incompetence. Treatment with nadroparine was continued, and rapid improvement of clinical condition and disappearance of pain and cough were observed. Mycophenolate mofetil was added, which resulted in subsidence of proteinuria. Rivaroxaban was used in prophylaxis of recurrences of thromboembolism. Tests for thrombophilia revealed factor V Leiden in patient.

Topics: Adolescent; Anticoagulants; Antithrombin III; Factor V; Female; Humans; Mycophenolic Acid; Nadroparin; Nephrotic Syndrome; Pulmonary Embolism; Thrombophilia

Heparin is commonly used as an anticoagulant but its many other pharmacologic properties are less well known. It has an important effect on complement regulation and has been shown in vitro to inhibit complement-mediated lysis of red cells. Although the beneficial effects of heparin for treatment of haemolytic anaemia were described many decades ago, its use in this scenario is not standard practice. Here we report a case where the use of heparin had a beneficial effect on a life-threatening episode of intravascular haemolysis. We also show unfractionated heparin to be more beneficial than low molecular weight heparin. We suggest that heparin has an important role to play in the management of complement-mediated haemolytic episodes.

Topics: 3' Untranslated Regions; Acute Disease; Adult; Anemia, Hemolytic, Autoimmune; Azathioprine; Combined Modality Therapy; Complement Activation; Enoxaparin; Female; Hemolysis; Heparin; Humans; Immunosuppressive Agents; Mycophenolic Acid; Plasma Exchange; Prednisolone; Prothrombin; Splenectomy; Thrombophilia; Venous Thrombosis