Refractory thrombocytopenia, recurrent abortions and inferior vena cava thrombosis.

A 23-year-old woman was admitted in January 1983 with complaints of purpuric spots all over her body for 4 days prior to admission. She had been married for 3 years, had undergone three spontaneous abortions (one first trimester and two second trimester), and had one live birth. On examination, she had no splenomegaly. Investigations revealed a haemoglobin of 11.2 g/dl, total leukocyte count 4 × 10/l (polymorphs 64%, lymphocytes 36%), erythrocyte sedimentation rate 12 mm in first hour, platelets 40 × 10/l. Blood film showed isolated thrombocytopenia. Renal function, liver function tests, chest X-ray and ultrasound of the abdomen were normal. Bone marrow aspiration showed increased megakaryocytes while other cell lines were normal. Antinuclear antibody (ANA) and dsDNA were negative. A platelet antibody test was not available. The patient had isolated thrombocytopenia without any systemic illness. A diagnosis of idiopathic thrombocytopenic purpura (ITP) was considered. The patient was started on 60 mg prednisolone per day. The platelet count had risen to 70 × 10/l by the 10th day and she was discharged. In March 1983, she was re-admitted with falling platelet counts, mucosal and gingival bleeding, and epistaxis. The platelet count was 15 × 10/l. The dose of oral steroid was increased and platelet concentrate was transfused. The platelet count did not increase and the patient developed menorrhagia and haematuria while still in the hospital. She was referred to a surgeon and, in view of the refractory thrombocytopenia, a splenectomy was performed. Postoperatively, her platelet count increased transiently for 1–2 days and then stabilised at 15–20 ×10/l. She was put on danazol and cyclophosphamide after a period of 12 days postoperatively. Platelets were monitored and there was gradual but definite improvement. Six months after surgery, the platelet count was 150 ×10/l and the medicines were tapered gradually. Between January 1984 and September 1997, she did not return for follow-up. She had eight more spontaneous abortions, six of which were in the second trimester. The cause of this was investigated. Gynaecological examination and her husband’s semen analysis were normal. Abdominal ultrasound was also normal and TORCH test was negative. Her platelet count, bleeding time and clotting time were normal. In October 1997, the patient was re-admitted with complaints of gradually increasing abdominal distension and low-grade fever. She was febrile with no pallor or lymphadenopathy. Her abdomen was distended, the umbilicus was stretched transversely and there were no dilated veins over the abdomen or back. The liver was palpable and non-tender with evidence of free fluid in the abdomen. Investigations showed normal complete blood counts including platelet count, renal and liver function tests; urine examination revealed trace proteins and chest X-ray was normal. An ascitic tap showed 150 cells, 90% lymphocytes, and was transudative in nature. Contrast abdominal computed tomography (CT) scan (figure 1) showed gross ascites; the liver was enlarged and there was inferior vena cava (IVC) thrombosis. There were prominent retroperitoneal collateral vascular channels. On magnetic resonance angiography (MRA) (figure 2) the IVC was well visualised from its formation at the L5 level up to the L2 vertebral level. There was no