Immune thrombocytopenic purpurae presenting with cortical vein thrombosis: is it rebalanced hemostasis?


Dear Editor, A 15-year-old previously healthy girl presented to our hospital with complaints of occipital headache, vomiting, and blurring of vision of 25 days duration. She also gave a history of recurrent epistaxis and menorrhagia. She denied trauma, and any significant history of bleeding diathesis in her family. Her vital parameters were stable. On examination, she had ecchymoses on the upper limbs. The rest of physical examination was unremarkable. The hematology revealed hemoglobin of 106 g/L, leukocytes 5.9×10/L, platelets 16×10/L, MCV 80 Fl, andMCHC was 30 g/dL. The peripheral blood smear showed normocytic normochromic anemia, thrombocytopenia with no evidence of schistocytes. The serum iron was 65 μg/dL, TIBC 420 μg/dL and s. LDH was 140 IU/L. The liver and renal function tests were within the normal limit and antiglobulin tests were negative. Coagulation profile including bleeding time, clotting time, prothrombin time, and activated partial thromboplastin time were within normal range. Fundoscopy showed bilateral mild papilledema. Cerebrospinal fluid examination was normal. Magnetic resonance imaging and a venogram of the brain showed an absence of flow in the superior sagittal sinus, right transverse sinus, and right sigmoid sinus suggestive of cortical vein thrombosis (CVT) (Fig. 1a– d). To evaluate the cause for hypercoagulable state, factor V Leiden mutation, protein C, protein S, homocysteine, and antiphospholipid antibody syndrome profile were tested which were all negative. The antinuclear antibody and anti ds-DNA antibodies also tested negative. The aspiration of the bone marrow showed normal erythroid and myeloid cells with increase inmegakaryocytes. There were no atypical cells. The clinical history and laboratory findings were consistent with the diagnosis of immune thrombocytopenic purpura (ITP); hence, high-dose intravenous methyl prednisone 1 g daily was given for 3 days followed by oral prednisolone 60 mg daily in two divided doses. The anticoagulation for CVT was done with un-fractionated heparin 5000 IU subcutaneous, twice a day for 5 days, after that she was switched over to oral warfarin tablets. On the third day of treatment, platelet count was 14×10/L and rose to 57×10/L on the 7th day, headache and vomiting gradually subsided. She was discharged after 15 days in better general condition on oral steroids and warfarin tablets. At 3 months follow-up, the magnetic resonance venogram showed partial re-canalization of the sinuses. The hemorrhagic events are common in the natural history of the ITP, whereas pro-thrombotic events are rarely found. The risk of thromboembolic events in adults with primary ITP has been little-investigated despite findings of increased susceptibility in other autoimmune thrombocytopenic conditions. Sarpatwari et al. [1] found that patients with ITP are at an increased risk for venous thromboembolic events compared with patients without primary immune thrombocytopenia. The postulated plausible mechanisms for primary immune thrombocytopenia induced venous thrombosis include increased platelet microparticle thrombogenicity following peripheral destruction and the activity of antiphospholipid antibodies. The thrombotic events in ITP can be explained by another hypothesis, Brebalanced hemostasis.^ This mechanism is based on the evolution of the pro-thrombotic state in patients of chronic liver disease with thrombocytopenia as a result of concomitant changes in both proand anti-hemostatic * Hans Raj Pahadiya


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