We report a severe hemorrhagic disorder in two pediatric patients with lupus anticoagulant (LA) associated to acquired factor II (prothrombin) deficiency. In both patients, hemorrhagic symptoms resolved after corticosteroid therapy. Serial coagulation studies showed that Staclot LA ா assay was more sensitive than DVVconfirm ா and Staclot PNP ா tests to confirm the presence of LA when associated with severe factor II deficiency. Both patients had non-neutralizing anti-prothrombin antibodies and their titers inversely correlated with factor II activity (r = −1.0, P < 0.0001). Associated findings in these patients included positive immunologic tests for systemic lupus erythematosus, a positive anticardiolipin antibody, and anti-␤ 2 GPI antibodies in one case. Our findings point out the difficulty in diagnosing LA associated with acquired factor II deficiency and suggest that, in confirmation of its phospholipid dependency, the inclusion of a source of normal human plasma in the test sequence to correct for any factor deficiency and a confirmatory step utilizing hexagonal (II) phase phospholipids may be crucial to the diagnosis of LA in some patients with LA-hypoprothrombinemia syndrome. Am.

SUMMARY Twenty women of child-bearing age with intracraniai venous thrombosis are reported. Thirteen developed thrombosis post-partum; 6 while on oral contraceptives. In one patient no cause was found. All diagnoses were confirmed by carotid angiography and in 10 out of 14 patients laboratory data indicated a hypercoagulable state. Coagulation studies were performed in 14 normal puerperal women, matched by age, who served as controls. No statistical differences was found between thrombotic and control groups. Radiologic signs of sino-venous occlusion are reviewed. Conservative treatment with anti-edematous agents, anticonvulsants and antiplatelet aggregants is advocated.

In order to analyze the usefulness of different types of treatment in relation to the interval since the onset of idiopathic thrombocytopenic purpura (ITP), a collaborative study of 934 adult patients was undertaken. Prednisone was administered to 818 patients, and 32% of them achieved prolonged complete remission (PCR). However, only 14% of patients who had ITP for more than six months achieved a prednisone- induced PCR (P less than .01). Splenectomy was done in 399 patients, and 65% of them achieved PCR; the remission rate did not vary with the interval since the onset of ITP. Of 120 patients with chronic ITP that was refractory to corticosteroids and splenectomy, 91 received either azathioprine or cyclophosphamide; 21% of them achieved PCR and 55% had a favorable response. None of 19 patients treated with vincristine and only one of ten patients treated with vinblastine-loaded platelets achieved PCR.

Accessory spleens were sought in 28 patients who had undergone splenectomy for chronic idiopathic thrombocytopenic purpura (ITP), using a variety of techniques. Abdominal scintigraphy with autologous erythrocytes labeled with Tc-99m and opsonized with anti-D IgG (radioimmune method) proved to be most useful, clearly demonstrating one or more accessory spleens in 12 cases (43%). Computed tomography (CT) was also helpful. Four out of five patients demonstrated an increased platelet count following surgery, the effectiveness of which was illustrated by the radioimmune scan. Patients who have had splenectomy for chronic ITP should be scanned using radioimmune techniques and CT to determine whether an accessory spleen is present.